UWorld Medicine

Basal Ganglia Signs

-Extrapyramidal signs including resting tremor, rigidity, bradykinesia, choreiform movements

Saw Palmetto

-Herbal remedy used for BPH ADRs: -Bleeding (esp perioperative, likely d/t platelet dysfxn), mild stomach discomfort, HA, dizziness, HTN

Ginseng

-Herbal remedy used for improved mental performance ADRs: -Increased bleeding risk -HA, insomnia, GI sx, vaginal bleeding, hypoglycemia

Black Cohosh

-Herbal remedy used for menopausal sx (e.g. hot flashes, vaginal dryness) ADRs: -Hepatotoxicity -Hypotension, adverse estrogenic effects

Echinacea

-Herbal remedy used for treatment & prevention of cold & flu ADRs: -Allergic rxn, dyspepsia

Management of Hypercalcemia

-If severe, associated w/ weakness, GI distress, neuropsych sx (confusion, stupor, coma), volume depletion -Typically volume-depleted from polyuria from hypercalcemia-induced nephrogenic diabetes insipidus, decreased oral intake -Hemodialysis reserved for pts w/ renal insufficiency or HF that cannot be aggressively hydrated

Aconite

-Ingredient in some Chinese herbal medicines used to treat pain or HF ADRs: -Can cause fatal arrhythmias (VTach, torsades)

AIDS-Defining Malignancies

-Kaposi sarcoma (HHV8) -Invasive cervical carcinoma (HPV) -NHL (EBV) -Primary CNS lymphoma (EBV)

Sudden Cardiac Death (SCD)

-Leading cause of mortality in young athletes <35 -Most commonly results from ventricular arrhythmias triggered by intense exertion in the setting of undiagnosed structural heart disease (e.g. hypertrophic CM, anomalous origin of coronary artery, arrhythmogenic RV CM) or congenital conduction abnormalities (e.g. long QT syndrome, Brugada syndrome) -Risk factors: family hx, use of performance-enhancing drugs (e.g. androgens)

Ginkgo Biloba

-Leaf extract, popular herbal supplement that pts may use as "memory booster" b/c of its suggested propensity for increasing cerebral blood flow -May be used for dementia, macular degen, peripheral vascular disease -Some studies suggest it may be somewhat effective in treatment of intermittent claudication & Alzheimer ADRs: -Risk of bleeding, potentiation of effects of anticoag therapy, seizures, HAs, irritability, restlessness, diarrhea, N/V -Has been associated w/ serious intracerebral bleeding

S3 Heart Sound

-Low-frequency diastolic sound produced by passive ventricular filling during early diastole, associated w/ turbulent blood flow to ventricles during passive diastolic filling due to increased volume -May be normal in young adults, children, athletes, pregnant women -Best heard over cardiac apex in left lateral decubitus position -Abnormal S3 is higher pitch & louder, "gallop"-like, may be heard in pts w/ CHF due to LV systolic dysfxn/HFrEF, correlates w/ elevated LA and/or ventricular filling pressures & serum BNP -Also abnormal in most adults >40, restrictive CM, high-output states, thyrotoxicosis, chronic severe mitral regurg, chronic aortic regurg

Tc-99m Perfusion Scan

-Normal tracer uptake w/ both rest & exercise indicates normal myocardial perfusion, low likelihood of ischemia, excellent prognosis w/ <1% annual risk of CAD -Decreased tracer uptake w/ both rest & exercise indicates a fixed defect, likely scar tissue w/ decreased perfusion & h/o CAD -Decreased tracer uptake w/ exercise but not w/ rest indicates reversible defect, inducible ischemia w/ likely CAD

Lead-Time Bias

-Occurs when test diagnoses disease earlier -> time from diagnosis until death appears prolonged even though there is actually no improvement in survival -Lead-time bias occurs when one test is in the lead

Indications for Long-Term Oxygen Therapy

-PaO2 55 or lower or O2 sat 88% or lower -If cor pulmonale, evidence of R HF, and/or hematocrit >55%, PaO2 49 or lower or SaO2 89% or lower -Should be titrated so that SaO2 is >90% during sleep, normal walking, at rest

Pemberton's Test

-Patient raises arms over head for up to 60 s; presence of facial plethora or engorgement of neck veins is strongly suggestive that thyroid is source of pt's obstructive sx

HIV Postexposure Prophylaxis

-Prophylaxis recommended for high-risk contact: exposure of mucous membrane, nonintact skin, percutaneous exposure; exposure to blood, semen, vaginal secretions, or any body fluid w/ visible blood (uncertain risk w/ CSF, pleural/pericardial/synovial/peritoneal/amniotic fluid) -Prophylaxis not recommended for low-risk contact: exposure to urine, feces, nasal secretions, saliva, sweat, tears, vomit w/o visible blood (counseling & reassurance) Timing: -Initiate urgently after exposure, preferably within the first few hrs, at latest <72 hrs after exposure -Continue for 28 days Regimen: -3+ drug regimen recommended w/ 2 nucleotide/nucleoside reverse transcriptase inhibitors (e.g. tenofovir, emtricitabine) + 1 of integrase strand transfer inhibitor (e.g. raltegravir), protease inhibitor, or non-nucleoside reverse transcriptase inhibitor Testing: -Should be tested immediately to establish serologic status, then retested at 6 wks, 3 mos, 6 mos

D-Xylose Testing

-Pt given oral dose of D-xylose (monosaccharide absorbed in prox small intestine w/o degradation by pancreatic or brush border enzymes), which is excreted into urine -Pt w/ small intestinal mucosal disease (e.g. celiac) poorly absorb D-xylose, demonstrate low urine D-xylose levels -Pt w/ malabsorption d/t enzyme deficiencies (e.g. chronic pancreatitis) show normal absorption of D-xylose b/c mucosa is intact & absorption does not rely on enzymatic processing -False-positive results may be seen in delayed gastric emptying, impaired glomerular filtration, SIBO (d/t bacterial fermentation of D-xylose before it can be absorbed)

Attrition Bias

-Refers to the systematic loss or withdrawal of participants from follow-up (e.g. cohort studies), often unequally from different study groups

Basophilic Stippling

-Represents ribosomal RNA precipitates that appear as blue granules dispersed throughout RBC cytoplasm -Seen in thalassemias, lead or heavy metal poisoning

Paradoxical S2 Splitting

-Reversed splitting in which A2 follows P2, w/ maximal splitting during inspiration -Seen in pts w/ fixed LVOF (e.g. aortic stenosis, bicuspid aortic valve, LBBB, RV-paced rhythm)

Extrapyramidal Symptom Treatment

-Rigidity, acute dystonia treated with benztropine/trihexylphenidyl, diphenhydramine -Akathisia treated with propanolol, benzo (lorazepam), benztropine -Parkinsonism treated w/ benztropine, amantadine -Tardive dyskinesia treated w/ valbenazine, deutetrabenazine

Screening Mammography

-Routine mammogram done even if the woman has no symptoms. Usually 2 views of each breast -Baseline at age 35, annually after age 40, not <30 b/c high density of breast tissue can impede visualization of masses and radiation exposure is risk for breast cancer -Every 2 yrs for women 50-74 yo, individualize b/t 40-50, uncertain benefit if 75+

Helmet Cells

-Schistocytes, fragmented RBCs seen in traumatic microangiopathic hemolytic conditions such as DIC, HUS, TTP

Length-Time Bias

-Screening test leads to increased detection of less aggressive cases w/ better prognosis -> false increase in survival, overestimation of benefits of screening tool

Comfrey

-Unsaturated pyrrolizidine alkaloid -Herbal supplement widely available in US -May be used for joint sx ADRs: -Hepatotoxicity

Rigid Knee Immobilizer

-Used for short-term management of patellar fx or rupture of quadriceps/patellar ligament complex

Multiple System Atrophy

Shy-Drager syndrome Presentation: -Parkinsonism -Autonomic dysfxn (postural hypotension, abnormal sweating, disturbance of bowel/bladder fxn, abnormal salivation/lacrimation, impotence, gastroparesis) -Widespread neuro signs (cerebellar, pyramidal, and/or LMN signs), ataxia -Bulbar dysfxn, laryngeal stridor can be fatal Treatment/Management: -Anti-parkinsonism drugs generally ineffective -Intravascular volume repletion w/ fludrocortisone, salt supplementation, alpha-adrenergic agonists, application of constrictive garments to lower body

Sexually Transmitted Infection Screening

Testing for all pts diagnosed w/ an STI or all interested in screening: -NAAT for Neisseria gonorrhea, Chlamydia trachomatis -RPR for syphilis -HIV testing (4th generation antigen/antibody) For certain populations: -For women only, wet mount for Trichomonas vaginalis (or NAAT if high suspicion & wet mount negative) -HSV screening (serology) only w/ hx of characteristic lesions

Neuropathic Tremor

Presentation: -Action tremor predominantly affecting distal extremities, may be seen in large-fiber demyelinating neuropathies (e.g. CIDP) -Associated w/ other signs of peripheral neuropathy (e.g. weakness, proprioceptive/sensory loss)

Binswanger Disease

Presentation: -Apathy, agitation, bilat corticospinal or bulbar signs Pathophysiology: -Type of vascular dementia involving white matter infarcts

Nummular Eczema

Presentation: -Chronic, relapsing/remitting condition w/ erythematous, pruritic, coin-shaped patches on extremities measuring 1-10 cm -May initially have scant exudate (intermittent seeping of clear/yellowish fluid) -Risk factors: middle-aged/older Diagnosis: -Usually clinical, occasionally bx Treatment: -Topical glucocorticoids (e.g. betamethasone diproprionate) -Lifestyle modifications to reduce xerosis (emollients, avoiding use of harsh soaps) Pathophysiology: -Inflammatory disorder thought to involve decreased production of skin lipids (occurs in setting of chronically dry skin)

Crystal-Induced Acute Kidney Injury

Presentation: -Commonly associated w/ acyclovir (esp IV), sulfonamides, methotrexate, ethylene glycol, protease inhibitors, uric acid (e.g. tumor lysis syndrome) -Usually asymptomatic, presents w/ AKI within 7 days of exposure, may present w/ nausea, flank/abd pain -Labs: UA w/ hematuria, pyuria, crystals -Risk factors: volume depletion, prior CKD Treatment/Management: -Discontinuation of drug, volume repletion, loop diuretics Pathophysiology: -Precipitation of low-solubility substance in renal tubules -> intratubular obstruction, direct renal tubular toxicity

Portal Vein Thrombosis

Presentation: -Complication of cirrhosis -Presents similarly to splenic vein thrombosis, but usually presents w/ both esophageal & gastric varices d/t HTN of entire portal system

Influenza Pneumonia

Presentation: -Complication of influenza infection, esp in elderly & pts w/ chronic medical illness (e.g. CAD, T2DM, COPD) -Acute worsening of influenza sx (dyspnea, cough), leukocytosis (usually <15), hypoxia -CXR w/ bilateral, diffuse, reticular/interstitial infiltrates Treatment/Management: -Inpatient treatment w/ supplemental O2 & antivirals (oseltamivir) Pathophysiology: -Secondary pneumonia; common complication of viral URIs

Systemic Lupus Erythematosus (SLE)

Presentation: -Constitutional: fever, weight loss, fatigue, lymphadenopathy -MSK: symmetric/migratory arthritis, butterfly rash, photosensitivity, painless aphthous ulcers, Raynaud phenomenon, livedo reticularis -Neuro: HA, seizures, cognitive dysfunction, transverse myelitis, psychosis, polyneuropathy, anxiety, mood changes -Serositis: pleuritis/pleural effusion, pericarditis, peritonitis -Renal: nephritic/nephrotic syndrome, lupus nephritis (GN) -Thromboembolic events (vasculitis, antiphospholipid antibodies) -Premature CAD w/ resultant MI & ventricular arrhythmia is common cause of death -Labs: hemolytic anemia, thrombocytopenia, leukopenia, hypocomplementemia (C3, C4), renal involvement (proteinuria, elevated Cr, hematuria, LE edema, low albumin), if neuro CSF may be normal or reflect mild inflammation (lymphocytosis) -Risk factors: female, African American Diagnosis: -Antibodies including ANA (sensitive), anti-dsDNA (sensitive + specific, titers correlated w/ disease activity, may be associated w/ lupus nephritis), & anti-Sm (specific), antiphospholipid antibody Treatment/Management: -Hydroxychloroquine for improving arthralgias, serositis, cutaneous sx, prevention of damage to kidneys & CNS -Low dose daily prednisone until hydroxychloroquine takes full effect -Cyclophosphamide reserved for pts w/ more severe manifestations (e.g. lupus nephritis, CNS involvement, vasculitis) -Methotrexate only indicated for pts w/ significant organ involvement w/ incomplete response to prednisone alone, rituximab has been used but limited d/t reports of PML -Can assess disease activity & treatment response w/ anti-dsDNA titers & complement levels Pathophysiology: -Lupus nephritis d/t deposition of anti-dsDNA immune complexes in glomerulus -> can cause either GN (neutrophil/macrophage recruitment w/ active urine sediment containing hematuria, cellular/granular casts, renal insuff) and/or nephrotic syndrome (deposition below basement membrane so no immune cell recruitment, bland sediment but high protein)

HIV Retinopathy

Presentation: -Cotton-wool retinal lesions that are rarely hemorrhagic, tend to resolve over wks to mos

Large Cell Lung Carcinoma

Presentation: -Cough, dyspnea, severe pain if parietal pleura or chest wall are infiltrated -CXR w/ large peripheral mass Pathophysiology: -Accounts for 10-15% of all lung cancers

Osmotic Diarrhea

Presentation: -Diarrhea occurring after ingestion of causative substance (e.g. lactose), does not persist w/ fasting Diagnosis: -High stool osmotic gap (>125)--high SOG = high stool solute concentration Pathophysiology: -Presence of nonabsorbed, osmotically active solute (e.g. polyethylene glycol, sorbital, lactose) inhibits water reabsorption

Thyroid Hormone Resistance Syndrome

Presentation: -Diffuse goiter -Labs: elevated TSH, elevated T3 & T4, normal Diagnosis: -Commercial assay for thyroid hormone receptor beta gene mutations Pathophysiology: -Mutations in thyroid hormone receptor beta gene, normal levels of alpha subunit & other pituitary hormones, normal sex hormone-binding globulin levels, TSH secretion suppressible w/ administration of thyroxine

Urticarial Vasculitis

Presentation: -Episodic wheals +/- purpuric center lasting 48-72 hrs (vs. urticaria <24 hrs) for 6+ wks -Pruritic & painful lesions (vs. urticaria only pruritic) -Resolution of lesions can leave residual purpura or hyperpigmentation -Recurrence common -May be associated w/ systemic sx (e.g. cough, dyspnea, abd pain), exhibit atypical features vs. urticaria, & be unresponsive to urticaria treatment Diagnosis: -Skin bx, inflammatory markers, UA, basic labs Pathophysiology: -Form of leukocytoclastic vasculitis -Resembles urticaria d/t complement-mediated mast cell degranulation -> wheals

Esophageal Carcinoma

Presentation: -Esophageal mass, oropharyngeal/esophageal dysphagia depending on location -Progressive dysphagia to solids & liquids Pathophysiology: -Most commonly d/t squamous cell carcinoma & adenocarcinoma -SCC: suspect w/ h/o EtOH & tobacco, other SCC lesions of head/neck, upper esopahgeal sx -Adenocarcinoma: suspect w/ chronic GERD, h/o Barrett's esopahgus, mid to distal-esophageal sx

Chronic Aortic Regurgitation

Presentation: -Exertional SOB, sensation of "pounding heart" (d/t hypertrophy & ventricular enlargement that brings ventricular apex closer to chest wall), esp in L lateral decubitus position -Decrescendo or high/pitched/sustained diastolic murmur, widened pulse pressure (increased SBP, decreased DBP), rapid rise-rapid fall ("water-hammer"/Corrigan) & bounding pulsation, abrupt carotid distention & collapse, "pistol-shot" femoral pulses, uvular pulsation (Muller sign) Pathophysiology: -Backflow from aorta into LV -> increased LV end-diastolic volume -> eventual compensation by LV w/ eccentric hypertrophy -> increased SV & CO -Eventual LV dysfxn from eccentric hypertrophy -> decreased SV & CO -> HF -Etiologies: congenital bicuspid aortic valve, postinflammatory (e.g. rheumatic heart disease, endocarditis), aortic root dilation (e.g. Marfan, syphilis)

Renal Cell Carcinoma

Presentation: -Gross or microscopic painless hematuria, painless abd/flank mass (nontender, firm, moves w/ respiration), flank pain, unintentional weight loss, anemia, intermittent fever -Left-sided varicocele that does not decompress -Paraneoplastic syndrome (e.g. hypercalcemia from PTHrP, EPO production -> polycythemia) -Risk factors: >50 yo, smoking, obesity, HTN, chemical/occupational exposure (e.g. asbestos), hereditary disorder (e.g. vHL) Diagnosis: -Abd CT (lesion within renal parenchyma that enhances w/ contrast, usually single solid or partially cystic lesion), staging imaging, partial/complete nephrectomy w/ histology Treatment/Management: -Nephrectomy (can be curative, 5 yr survival >70%)

Metabolic Syndrome

Presentation: -HTN, impaired fasting glucose, dyslipidemia, increased central/abd fat distribution -Associated w/ T2DM, NAFLD, PCOS Diagnosis: -3+ of 5 criteria: abd obesity (male waist circumference >40 in, female waist circumference >35 in), fasting glucose >100-110, BP >130/80, triglycerides >150, HDL cholesterol <40 in males & <50 in females Pathophysiology: -Insulin resistance

Nephritic Syndrome

Presentation: -HTN, oliguria, hematuria, proteinuria, casts -Pediatric causes include PSGN, HUS -Adolescent/adult causes include IgA nephropathy, membranoproliferative GN, crescentic GN, Goodpasture's, granulomatosis w/ polyangiitis (Wegener's)

Diffuse Axonal Injury (DAI)

Presentation: -Head trauma w/ sudden acceleration-deceleration -Severe neurologic impairment (GCS <8), typically results in immediate coma Diagnosis: -Minute white matter punctate hemorrhages w/ blurring of gray-white interface on brain imaging (MRI)

High-Output Heart Failure

Presentation: -Heart failure that occurs when cardiac output remains normal or above normal. It is usually caused by increased metabolic needs or hyperkinetic conditions such as septicemia (fever), anemia, and hyperthyroidism. This type of heart failure is different from left- and right-sided heart failure, which are typically low-output states, and is not as common as other types -Uncommon hypermetabolic state, may be a cause of HFpEF -Manifestations of HF (e.g. dyspnea, peripheral/pulm edema) & hyperdynamic circulation (bounding pulses, brisk carotid upstroke, systolic bruit/flow murmur, increased pulse pressure), laterally displaced PMI, S3, warm extremities Diagnosis: -Echo w/ dilated LV, RV, IVC, elevated resting cardiac index -Catheterization w/ increased PCWP, increased CI, decreased SVR & normal to decreased MvO2 Pathophysiology: -Etiologies: morbid obesity (most common), AVF (congenital, acquired by trauma/hemodialysis), pregnancy, thyrotoxicosis, thalassemia, severe anemia, beriberi, Paget's disease, VSD -Decreased SVR -> compensatory increase in CO -Decreased SVR may be d/t peripheral vasodilation d/t unmet metabolic demand (e.g. hyperthyroidism, severe anemia), increased quantity of peripheral vessels (e.g. Paget, morbid obesity), or bypass of systemic arteriolar pressure d/t large AVF

Variceal Bleeding

Presentation: -Hematemesis in the setting of hepatic disease (e.g. cirrhosis) -Associated complications: infection (SBP, UTI, aspiration PNA, primary bacteremia), hepatic encephalopathy, renal failure Treatment/Management: -IV octreotide, fluid resuscitation, upper endoscopy (diagnostic & therapeutic) -Infection prophylaxis w/ IV ceftriaxone for 7 days, then transition to TMP-SMX or oral FQ (norfloxacin, ciprofloxacin) prior to d/c -Prophylaxis w/ nonselective beta blockers (e.g. propanolol, nadolol),, oral nitrate to reduce risk of rebleeding

Acanthosis Nigricans

Presentation: -Hyperkeratotic, hyperpigmented, symmetric plaques w/ velvety texture on flexural areas (esp groin, axilla, posterior neck) -Benign form: seen in younger pts & associated w/ insulin resistance (obesity, T2DM, PCOS), may be seen in conjunction w/ acrochordons (skin tags) -Malignant form: associated w/ GI & GU malignancy, may present suddenly in older pts, usually not obese (instead have usually lost weight secondary to neoplasia), occur in more atypical regions (e.g. mucous membranes, palms, soles) Pathophysiology: -Benign form thought to be due to increased levels of insulin & insulin-like growth factors that stimulate epidermal & dermal proliferation

Cerebral Salt Wasting

Presentation: -Hypovolemic hyponatremia, high urine Na+ following neurologic insult (e.g. trauma, head surgery)

Aspiration Pneumonitis

Presentation: -In pts w/ periods of impaired consciousness (e.g. seizures, MVC), presents w/ low-grade fever, leukocytosis, sx of ARDS/respiratory failure mins to hrs after aspiration event -Diffuse pulm wheezes/crackles -Can range from asymptomatic to nonproductive cough, hypoxia, resp distress -CXR: fluffy/diffuse infiltrates of one or both lower lobes that resolves w/o abx Treatment: -Supportive, no abx (e.g. supplemental O2) -Oropharyngeal suction if event witnessed -Usually resolves in 24-48 hrs Pathophysiology: -Chemical irritation & inflammation of pulmonary parenchyma (bronchial/alveolar epithelial cells) from aspiration of gastric acid & direct tissue injury

Advanced Sleep-Wake Phase Disorder

Presentation: -Inability to stay awake in evening (usually after 7 pm), early morning insomnia

Chikungunya Fever

Presentation: -Incubation period of 3-7 days, sx usually resolve in 7-10 days -High fevers, severe POLYARTHRALGIAS (almost always present, joint swelling, starts in distal extremities then moves proximally, bilateral & symmetric, persist for mos-yrs), HA, myalgias, conjunctivitis, maculopapular rash -Labs: LYMPHOPENIA, thrombocytopenia, transaminitis Diagnosis: -Serologic testing or PCR Treatment/Management: -Supportive care (initial sx usually resolve in 7-10 days) -Methotrexate for chronic arthralgias/arthritis (occurs in >50%) Pathophysiology: -Mosquito-born viral illness by Aedes vector (also carries dengue, yellow fever, & Zika) endemic to tropical/subtropical areas of Central/South America, Africa, Asia, Caribbean -Alphavirus

Nonpurulent Cellulitis

Presentation: -Infection of skin w/ flat edges, poor demarcation, tender/warm erythema, indolent onset (days), localized involvement w/ fever later in disease course -No fluctuance or purulent drainage Treatment/Management: -Systemic abx (cephalexin) w/ MRSA coverage if febrile or risk factors present (e.g. prior MRSA infection, recent hospitalization) Pathophysiology: -Infection of dermis & subcutaneous fat, most commonly following break in skin (e.g. insect bite) -Most commonly due to GAS or MSSA infection

Amenorrhea in Hypothyroidism

Presentation: -Irregular, heavy menstrual bleeding or secondary amenorrhea -Galactorrhea Diagnosis: -TSH, T4 levels Treatment/Management: -Treatment of underlying hypothyroidism usually normalizes menses Pathophysiology: -Decreased circulating thyroid hormone -> feedback to hypothalamus increases release of TRH -> increased release of prolactin & TSH by anterior pituitary -> inhibition of hypothalamic GnRH release by increased prolactin -> decreased FSH & LH release -> anovulation, abnormal uterine bleeding

Pulmonary Cachexia Syndrome

Presentation: -Loss of lean muscle mass associated w/ chronic lung disease (esp COPD) -BMI 20 or less, weight loss >5% -Occurs in 20-40% of COPD pts, leads to impaired balance, increased susceptibility to lung infection, increased mortality Treatment/Management: -Optimization of lung fxn, exercise, nutritional supplementation Pathophysiology: -Increased work of breathing -> increased caloric use -> significant energy imbalance in setting of decreased appetite & low dietary intake -Systemic inflammation -> decreased appetite, increased skeletal muscle breakdown -Skeletal muscle hypoxia & glucocorticoid use may contribute -Diaphragmatic flattening in COPD may contribute to early satiety & decreased caloric intake

Hypertensive Encephalopathy

Presentation: -Marked BP elevation (usually 180+/120+) w/ progressive HA, N/V, nonlocalizing neurologic sx (confusion, restlessness, agitation, seizures, coma) due to cerebral edema Treatment: -Labetalol

Porcelain Gallbladder

Presentation: -May be asymptomatic (often incidentally found on imaging) or present w/ RUQ pain, firm/nontender RUQ mass on exam -Increased risk of gallstone adenocarcinoma (2-5%) Diagnosis: -Rimlike calcification on CT or X-ray, central bile-filled dark area on CT Treatment: -Prophylactic cholecystectomy, esp if symptomatic or have incomplete/punctate mural calcification (curvilinear calcifications minimally increase cancer risk, generally do not require intervention) Pathophysiology: -Calcium-laden gallbladder wall w/ bluish color & brittle consistency often associated w/ chronic cholecystitis -Pathogenesis thought to involve calcium salt deposition intramurally w/ natural progression of chronic inflammation or chronic irritation from gallstones

Chronic Myeloid Leukemia (CML)

Presentation: -May be asymptomatic, may present w/ fatigue, night sweats, weight loss, exercise intolerance, abdominal fullness due to splenomegaly, early satiety -Labs: thrombocytosis, anemia Diagnosis: -Peripheral blood smear w/ dramatic leukocytosis (often >100,000) w/ absolute basophilia & shift towards early neutrophil precursors (promyelocytes, myelocytes, metamyelocytes < myelocytes), low leukocyte alk phos score (indicating cytochemically/functionally abnormal neutrophils, vs. score is high in leukemoid rxn) Treatment: -Tyrosine kinase inhibitors including imatinib Pathophysiology: -Most commonly due to 9;22 translocation -> BCR-ABL transfusion gene -> constitutively active tyrosine kinase -> leukemogenesis

Hypothermia

Presentation: -Mild (32-35 C, 90-95 F): tachycardia, tachypnea, ataxia, dysarthria/slurred speech, increased shivering, confusion -Moderate (28-32 C, 82-90 F): bradycardia, lethargy, hypoventilation, hypotension, decreased shivering, atrial arrhythmias, QT prolongation -Severe (<28 C, <82 F): coma, cardiovascular collapse (marked hypotension), ventricular arrhythmias (VFib), areflexia, acidosis Treatment/Management: -Warmed crystalloid (42 C, 107 F) for hypotension -Endotracheal intubation in comatose pts -Rewarming techniques: -Mild - passive external warming by removing wet clothing, covering w/ blankets, goal of 1-2 C/hr -Moderate - active external warming w/ warm blankets, heating pads, warm baths -Severe - active internal warming w/ warmed pleural/peritoneal irrigation, warmed humidified oxygen Pathophysiology: -Initial tachycardia w/ peripheral vasoconstriction to attempt to increase delivery -Later bradycardia & hypotension due to decreased reactivity of pacemaker cells, salt/water loss from cold-induced diuresis

Factor V Leiden

Presentation: -Most common inherited thrombophilia, most common in Caucasian populations (4-5% prevalence) -DVT, PE, other VTE -Suspected in young pts <45 w/ first-time unprovoked DVT/PE, pts w/ recurrent DVT/PE, and/or unusual sites of thrombosis (e.g. cerebral, mesentery, portal veins) Diagnosis: -PT & PTT often normal Pathophysiology: -AD mutation in factor V gene resulting in activated protein C resistance -Slowed degradation of factor V leads to prolonged/continued thrombin formation, slowed degradation of factor VIII -Even higher risk if homozygous > heterozygous

Bronchial Carcinoid Tumor

Presentation: -Most common lung cancer in adolescents & young adults (often nonsmokers) -Proximal airway obstruction (cough, wheezing, dyspnea), recurrent PNA due to distal obstruction, hemoptysis -Carcinoid syndrome less common than w/ midgut carcinoid Diagnosis: -Chest CT w/ contrast enhancing (vascular) tumor w/ endobronchial component (extension of lesion into bronchial lumen) usually affecting central airways, may be homogenous in appearance or contain calcifications -Bronchoscopy w/ biopsy Pathophysiology: -Neuroendocrine tumor derived from bronchial Kulchitsky (enterochromaffin) cells in bronchial epithelium

Hepatic Veno-Occlusive Disease

Presentation: -Most commonly occurs in pts w/ BM transplant -Tender hepatomegaly, jaundice, ascites Pathophysiology: -D/t occlusion of terminal hepatic venules -> postsinusoidal portal HTN

Huntington Disease

Presentation: -Motor: chorea (abrupt, jerking, involuntary movements that seem to move from one muscle group to the next), delayed saccades, motor impersistence (e.g. inability to sustain grip), hyperreflexia -Psych: depression, irritability, psychosis, obsessive-compulsive sx -Cognitive: executive dysfxn -Survival 10-20 yrs Diagnosis: -Caudate nucleus & putamen atrophy on imaging Treatment: -Supportive Pathophysiology: -AD trinucleotide repeat disorder (CAG) -> abnormal huntingtin protein expression -Loss of GABA-ergic neurons in neostriatum

Dacryocystitis

Presentation: -Nasolacrimal duct infection, complication of dacryostenosis presenting w/ purulent eye drainage and erythema from medial canthus, warmth, tenderness, swelling over lacrimal sac -Epiphora (excessive tearing w/ drainage onto face) -Common in infants and adults >40 yo -May present w/ fever & leukocytosis Treatment: - Pathophysiology: -Infection of lacrimal sac, usually due to S. aureus or beta-hemolytic Strep

Peripheral Vertigo

Presentation: -Nystagmus never purely vertical, often inhibited by gaze fixation, is fatigable (<1 min), latency period of 2-40 s -Often associated w/ other inner ear sx like hearing loss, fullness, tinnitus -Walking usually preserved -No other CNS sx (e.g. HA, diplopia, weakness/numbness of face or limbs, dysarthria) Diagnosis: -Head imaging (CT first, if negative follow w/ MRI to exclude brainstem/cerebellar pathology) Pathophysiology: -Etiologies involve inner ear (BPPV, Meniere disease, vestibular neuritis, labyrinthitis)

Alopecia Areata

Presentation: -Painless, patchy, well-demarcated, often round nonscarring hair loss; skin left behind is smooth and hairless -No associated scaling, erythema, scarring -Narrowing of hair shafts close to skin surface, broken hairs tapered near the insertion into the scalp, particularly at peripheraly of alopecic plaque (exclamation point hairs) -Positive hair pull test (>5-6 hairs extracted) -Can occur independently or in association w/ other autoimmune disorders (e.g. vitiligo, lupus, autoimmune thyroid disease, pernicious anemia); may be associated w/ nail pitting -Recurring course is common, but most experience regrowth w/ treatment Treatment: -Mild/moderate hair loss treated w/ topical or intralesional corticosteroids -Extensive hair loss treated w/ topical immunotherapy (diphenylcylopropenone), oral corticosteroids -Second-line is minoxidil (more commonly used for androgenetic hair loss) Pathophysiology: -Does not typically cause permanent damage to hair follicle so most respond to therapy w/ regrowth, though recurrence is common -T-cell infiltration around hair follicles

Amaurosis Fugax

Presentation: -Painless, rapid, transient (<10 min) monocular vision loss, described as "curtain/shade descending" -Often associated w/ ipsilateral carotid bruit -Fundoscopy often normal, may show embolic plaques & retinal whitening (d/t ischemia) -Increased risk of stroke when d/t emboli -Risk factors: HTN, T2DM, CAD (& other vascular risk factors) Pathophysiology -Etiologies: retinal ischemia from atheroemboli (e.g. giant cell arteritis, carotid stenosis/atherosclerosis, fibromuscular dysplasia) -Vision returns after embolus is broken up or displaced & retinal circulation is restored

Catscratch Disease

Presentation: -Papule/nodule/vesicle at scratch/bite site, regional adenopathy, +/- fever of unknown origin (14+ days) -Affected lymph nodes are large, tender, erythematous; takes 1-2 mos to resolve Diagnosis: -Clinical +/- serology -Histo shows nonspecific lymphoid hyperplasia, areas of necrosis Treatment: -Generally self-limited, azithromycin Pathophysiology: -Bartonella henselae infection (fastidious gram- bacilli), transmitted by cat scratches/bites (esp kittens), rarely transmitted by fleas

Nonbullous Impetigo

Presentation: -Papules & pustules that coalesce & rupture to form honey-crusted lesions, may be painful or itchy, most commonly involving face and extremities in children -Fever uncommon -Often develops secondarily at a site of prior skin trauma, e.g. infection (HSV, VZV, scabies), inflammation (eczema, burns), open wounds (insect bites, cuts) -Complications: PSGN -Risk factors: warm/humid climate, poor hygiene, preexisting skin trauma/inflammation (e.g. eczema, scabies) Treatment: -Topical abx (e.g. mupirocin) if localized, oral abx if extensive (e.g. cephalexin, dicloxacillin) -Hand hygiene, coverage of actively draining lesions to reduce transmission Pathophysiology: -S. pyogenes (group A) or S. aureus (most common) infection of superficial epidermis

Malaria

Presentation: -Periodic/cyclic febrile paroxysms, malaise, headache, N/V, abd pain, diarrhea, myalgia, pallor, jaundice, petechiae, hepatosplenomegaly -Fever cycle usually consists of cold phase (chills, shivering), hot phase (high-grade fevers), sweating phase (diaphoresis, fever resolution) -Complications in children: cerebral malaria (seizures, coma, delirium), hypoglycemia, metabolic acidosis, cerebral -Complications in adults: jaundice, acute renal failure, pulmonary edema -Protective factors: sickle cell trait & other hemoglobinopathies (Hb S, Hb C, thalassemias), partial immunity from previous malarial illness -Labs: anemia, thrombocytopenia, hyperbili Diagnosis: -Thin & thick peripheral blood smears (Giemsa stain) Prevention: -Antimalarial drugs: atovaquone-proguanil, doxycycline, mefloquine, chloroquine, hydroxychloroquine -Insecticide-treated nets, household insecticide residual spraying, long sleeved clothing Pathophysiology: -Transmission of protozoans Plasmodium falciparum, vivax, ovale, or malariae parasites by Anopheles mosquito bite -Sickle cell trait protective b/c misshapen RBCs create suboptimal environment for parasitic proliferation

Ecthyma

Presentation: -Streptococcal skin infection related to impetigo that presents w/ round, erythematous plaques, acute onset of pustules & small ulcers

Lightning Injury

Presentation: -Uncommon but can involve multiple organ systems, can lead to severe multisystem dysfxn & death -Derm: thermal burns (superficial, partial, or full thickness) -CV: arrhythmias (e.g. asystole, VFib) -Neuro: LOC, temporary weakness/paralysis, autonomic dysfxn (fixed, dilated pupils), respiratory depression -Rhabdomyolysis w/ renal failure Treatment/Management: -Arrhythmias: excellent PCR, continuous chest compressions while administering pressors (e.g. epi in asystole)

Hemiballismus

Presentation: -Unilateral, violent arm flinging -Disruptive, tends to be self-limited Pathophysiology: -Damage to contralateral subthalamic nucleus

Vestibular Gait

Presentation: -Unsteady, staggering, falling to one side -Normal sensation, reflexes, motor strength -Nausea, vertigo, tinnitus, hearing loss Pathophysiology: -Benign positional vertigo, labyrinthitis, Meniere's

Abdominal Aortic Aneurysm

Presentation: -Usually asymptomatic, may present w/ abd/back/flank pain, LE ischemia and/or VTE -If ruptured, presents w/ abd distention & shock -Risk factors: smoking, male, age, Caucasian, fhx of AAA, atherosclerotic disease Diagnosis: -Abd US, often diagnosed during imaging study for unrelated cause Treatment/Management: -Smoking cessation, aspirin & statin therapy -Elective repair recommended for large aneurysms (5.5+ cm), rapidly enlarging aneurysms (0.5+ cm/6 mos), AAA associated w/ PAD or aneurysm., symptomatic aneurysm (back/abd pain) -Fup imaging: US every 6-12 mos if medium (4-5.4 cm), US every 2-3 yrs if <4 cm Pathophysiology: -Most commonly affects infrarenal aorta (3+ cm) -Smoking strongest risk factors -> increased inflammation & degen of connective tissue in aortic wall

Paraprotein Neuropathy

Presentation: -Usually begins in feet w/ alterations in proprioception/vibration sense, may progress to cause motor weakness -Associated w/ monoclonal plasma cell disorders

Dyshidrotic Dermatitis

Presentation: -Vesicular rash on palms, soles, sides of digits associated w/ pruritus, redness, scaling

Mycobacterium Marinum

Presentation: -Wound infection w/ papular/ulcerative lesions that develop over several days following exposure to salt/fresh water Pathophysiology: -Found in salt & fresh water

Prothrombin Mutation

Presentation: -2nd most common thrombophilia in Caucasian populations (2% prevalence) after factor V Leiden Pathophysiology: -AD mutation results in increased prothrombin levels

Gerstmann Syndrome

Damage to dominant parietal lobe Presentation: -Acalculia: difficulty performing simple arithmetic tasks -Finger agnosia: inability to name individual fingers -Agraphia: impaired writing -Right/left confusion: inability to identify or distinguish between R & L sides of body

Morphine

MOA: -Strong, short-acting opioid -May be used in persistent pain from ACS after nitroglycerin drip b/c of properties including decreasing cardiac preload, anxiolytic effects

Sulfasalazine

MOA: -TNF & IL-1 suppressor Indications: -IBD DMARD ADRs: -Hepatotoxicity, stomatitis, hemolytic anemia (G6PD def)

Membranoproliferative Glomerulonephritis (MPGN)

Presentation: -Nephrotic syndrome (>3.5 g/24 hrs, edema, hypoalbuminemia, hyperlipidemia, urine lipids) -Risk factors: hep B & C, lipodystrophy Diagnosis: -Dense deposits on IF that are + for C3

Minimal Change Disease

Presentation: -Nephrotic syndrome (>3.5 g/24 hrs, edema, hypoalbuminemia, hyperlipidemia, urine lipids), most commonly in children -Risk factors: NSAIDs, lymphoma (usually Hodgkin)

Slap Gait

Presentation: -Pts w/ sensory neuropathy that stomp feet against floor to help them know where lower limbs are relative to ground -May be associated w/ loss of proprioception, postural/gait instability, wide-based gait

Phase IV Clinical Trial

-Studies adverse effects cause over time by new treatment after it has been approved & is available on the market

Factorial Design Study

-Two more more experimental interventions, each with two or more variables studied independently -Utilizes 2+ interventions and all combinations of these interventions 3 drug tx each with 2 BP endpoint groups

Pica

Presentation: -Appetite for items other than food, e.g. paper products, clay, dirt, ice (pagophagia) -May be associated w/ iron deficiency anemia (esp pagophagia) or psychiatric disease Treatment: -Iron supplementation

Pearly Penile Papules

Presentation: -Arise in 2nd-3rd decade of life, normal skin variant -1+ rows of small, flesh-colored, dome-topped, or filiform papules on the penile corona/sulcus -Asymptomatic, harbor no malignant potential, not caused by a sexually transmitted disease Treatment/Management: -Reeassurance

Eustachian Tube Dysfunction

Presentation: -Asymmetric conductive hearing loss -Ear pain/fullness, popping/cracking sensation, middle ear effusion and/or tympanic membrane retraction Diagnosis: -Tympanometry Treatment: -If unresponsive to medical management (e.g. decongestants), tympanostomy tube placement

Mononeuritis Multiplex

Presentation: -Asymmetric peripheral nerve findings in multiple different body regions (e.g. wrist drop, foot drop) -Often associated w/ vasculitis (e.g. polyarteritis nodosa) Pathophysiology: -Nerve damage to 2+ separate nerves

Onychorrhexis

Presentation: -Brittle nails w/ vertical ridges Pathophysiology: -Zinc deficiency

Digoxin Toxicity

Presentation: -CV: life-threatening arrhythmias (atrial tachy w/ AV block most common, sinus brady, junctional rhythms, VTach) -GI: anorexia, N/V, abd pain -Neuro: fatigue, confusion, weakness, color vision alterations/scotoma/blindness, lethargy, disorientation -Acute toxicity more commonly presents w/ GI sx, chronic toxicity more commonly presents w/ neuro sx Pathophysiology: -May be precipitated by amiodarone/verapamil/quinidine/propafenone/spironolactone (interfere w/ digoxin metabolism -> increased serum digoxin levels) -Atrial tachycardia associated w/ increased automaticity somewhere in atrium resulting in typical P wave morphology, increased rate to ~200 bpm -AV block due to increased vagal tone -Hypokalemia can worsen cardiac toxicity b/c digoxin competes w/ K+ for binding to Na+/K+-ATPase

Bile Acid Diarrhea

Aka bile salt-induced diarrhea Presentation: -Secretory diarrhea (watery/brown, fasting diarrhea, nocturnal episodes), bloating, abd cramps, esp following a cholecystectomy (seen in 5-10% of pts) -May also be seen w/ ileal resection, short bowel syndrome -Unremarkable stool/labs Treatment: -Bile acid binding resins (cholestyramine, colestipol, colesevelam) Pathophysiology: -Primary bile acids produced in liver cells secreted into intestinal lumen -> converted to secondary bile acids by bacteria -> cause colonic stimulation in high quantities -Unresorbed bile acids spill into colon, resulting in mucosal irritation -Post-cholecystectomy, due to bile acid entering ileum too rapidly due to absence of gallbladder, overwhelming resorptive capacity -In Crohn disease or w/ abd radiation damage, due to ileal disease that impairs bile resorption

Medial Medullary Syndrome

Alternating hypoglossal hemiplegia -Mnemonic: Lary MD can't kick with his right leg, but can lick a tASAty ips cream cone from his left hand -Lary MD: medulLARY medial, can't kick with his right leg (contralateral paresis), can lick: tongue paralysis w/ deviation to affected side, tASAty: anterior spinal artery, ips: ipsilateral tongue paralysis Presentation: -Medullary pyramid: contralateral hemiparesis -Medial leminiscus: contralateral loss of tactile/vibratory/position sense -Hypoglossal nucleus/fibers: ipsilateral tongue paralysis w/ deviation to side of lesion

Grade III Astrocytoma

Anaplastic astrocytoma Diagnosis: -Increased mitoses relative to grade I/II astrocytoma

Pressure Ulcers

Decubitus ulcers Presentation: -Most common over bony prominences (e.g. sacrum, ischial tuberosities, malleoli, heels, 1st/5th metatarsal heads) -Stage I: nonblanchable erythema of intact skin -Stage II: shallow/open ulcer, red-pink wound w/o sloughing, possible intact or ruptured blister, superficial & partial thickness loss of epidermis/dermis -Stage III: full-thickness skin loss w/ possible visible subcutaneous fat w/o extension through underlying fascia, no exposed bone/tendon/muscle -Stage IV: full-thickness skin loss w/ exposed bone/tendon/muscle -Unstageable: full-thickness skin loss w/ ulcer base covered by slough and/or eschar requiring removal to stage -Risk factors: impaired mobility, malnutrition, abnormal mental status (e.g. dementia), decreased skin perfusion, reduced skin sensation (e.g. T2DM neuropathy) Treatment/Management: -Local wound care, repositioning of patient to reduce pressure, pain control, nutritional support -Shallow ulcer wound care (stages I & II) w/ occlusive/semipermeable/transparent dressings to maintain moist environment -Deep ulcers (stages III & IV) may require more complex dressings, surgical debridement of necrotic tissue or closure -Topical antiseptics may be considered if wounds not improve as expected within 2-4 wks (suggesting subclinical infection) Pathophysiology: -Constant, unrelieved pressure -> impaired blood flow to soft tissues -> necrosis of overlying skin and muscle Prevention: -Mobilization, proper pt positioning, careful skin care, moisture control, maintenance of nutrition -Beds w/ pressure redistribution measures, e.g. air-fluidized beds, pressure-relieving overlays (e.g. sheepskin), foam mattresses, active repositioning systems, alternating pressure supports

Major Neurocognitive Disorder

Definition of dementia Presentation: -Significant decline in 1+ cognitive domains -Irreversible global cognitive impairment -Marked functional impairment -Chronic & progressive over mos to yrs -Atypical if <60, rapidly progressive Diagnosis: -Cognitive testing w/ MMSE (score <24/30 suggestive of MCI/dementia), Montreal Cognitive Assessment (score <26/30), and/or Mini-Cog (abnormal 3-word recall +/- clock-drawing test) -Routine lab testing w/ CBC, B12, TSH, CMP to rule out reversible causes -Selective lab testing when relevant includes folate (e.g. taking methotrexate), syphilis, vit D level, CSF if very atypical/early onset -Routine imaging includes CT or MRI brain -If atypical/early onset, consider EEG

Dysphagia

Difficulty swallowing, may be characterized as oropharyngeal or esophageal -Oropharyngeal: difficulty initiating swallow, coughing/choking/drooling/aspiration after eating, workup using nasopharyngeal laryngoscopy, then standard barium esophogram prior to EGD (upper esophageal lesions not visualized well, perforation possible) -Esophageal: sensation of food stuck in esophagus; if both solids & liquids may indicate motility disorder that may be intermittent (e.g. esophageal spasm) or progressive (achalasia), if only solids may reflect mechanical obstruction (e.g. stricture, cancer, eosinophilic esophagitis) -If suspect lower esophageal sx, workup w/ EGD -Dysphagia initially involving both solids & liquids suggests neuromuscular disorder -Progressive dysphagia occurring initially for solids & later for liquids suggests mechanical obstruction

Sensory Aprosodia

Difficulty understanding meaning conveyed by tone of voice Pathophysiology: -Lesion affecting nondominant temporal lobe, which controls the ability to comprehend emotional gestures

Atopic Dermatitis

Eczema Presentation: -In infants, itchy, red, scaly, crusted lesions on extensor surfaces, trunk, cheeks, scalp -In children/adults, lichenified plaques in flexural creases/surfaces -May be triggered by excessive bathing, dry environment, stress, overheating, irritating detergents/soaps -Complications: eczema herpeticum, cellulitis/abscess, impetigo, molluscum contagiosum, tinea corporis, discomfort interfering w/ daily activities & sleep -Often associated w/ other atopic conditions (e.g. allergic rhinitis, asthma, food allergies) -Labs: elevated IgE, eosinophilia Treatment: -Topical thick emollients & steroid ointments -Trigger avoidance, hypoallergenic cleansers for bathing & laundry, cotton clothing > synthetic fabrics -Oral antihistamines -Topical glucocorticoids (low-potency agents like hydrocortisone for mild sx, moderate to high-potency agents like triamcinolone or betamethasone for more severe sx), not for use on face, eyelids, flexural areas -Possible use of calcineurin inhibitors (e.g. tacrolimus) for areas not covered by glucocorticoids (face, eyelids, etc.) -Severe refractory disease may require UV phototherapy or systemic immunosuppressants Pathophysiology: -Epidermal dysfxn due to improper synthesis of stratum corneum components -May be associated w/ mutation in epidermal barrier proteins (e.g. filaggrin) -> decreased water content, increased permeability, increased inflammation -Immune dysfxn w/ Th2-skewed response

Broca Aphasia

Expressive aphasia ("broken") Presentation: -Able to comprehend & follow commands, but unable to verbalize & write properly -Impaired repetition -May be associated w/ hemiparesis of face/upper limb (usually R-sided; 95% of R-handed & 70% of L-handed are L-hemisphere dependent) Pathophysiology: -Lesions of Broca's area (posterior inferior frontal gyrus), e.g. stroke of MCA territory -Lesion affecting surrounding motor cortex -> contralateral weakness, conjugate gaze deviation to side of lesion (contraversive frontal eye fields)

Parapneumonic Pleural Effusion

Exudative effusion secondary to PNA Presentation: -Sx of PNA (fever, cough), pleuritic chest pain -Focal rales, decreased/absent breath sounds, dullness to percussion over affected area -If small effusion, may have stable/normal work of breathing with stable vital signs -If moderate-large effusion, may show signs of respiratory distress (grunting, nasal flaring, subcostal retractions), tachypnea, hypoxia -Starts as uncomplicated parapneumonic effusion w/ fever, SOB, evidence of PNA, pleural effusion on CXR; effusion itself is sterile w/ pH 7.2+, low/normal glucose, LDH ratio >0.6, negative pleural fluid gram stain & culture -Can progress to complicated parapneumonic effusion (empyema) as bacteria & microorganisms invade pleural space and colonize effusion -> nonsterile effusion w/ pH <7.2, low glucose, LDH ratio >0.6, WBCs >50,000, but possibly negative pleural fluid gram stain & culture -Then progresses to empyema w/ grossly purulent fluid, pleuritic chest pain, signs of frank bacterial infection w/ pH <7.2, low glucose, LDH ratio >0.6/elevated LDH >1000, neutrophil predominance w/ leukocyte counts >50,000, positive gram stain & culture Diagnosis: -CXR confirming presence of pleural fluid (e.g. obscuring of costophrenic angle, layering fluid on lateral decubitus film), thoracentesis & pleural fluid analysis -If uncomplicated, small to moderate & free-flowing -If complicated, moderate to large, free-flowing or loculated Treatment: -If small/uncomplicated effusion w/ no respiratory distress or hypoxia, PO abx, close OP fup, serial CXR to ensure resolution -If moderate/large/complicated effusion OR respiratory distress OR hypoxia, IP management w/ IV abx, US, drainage (chest tube, video-assisted thorascopic surgery) Pathophysiology: -Pleural inflammation secondary to bacterial fluid -> leaking of exudative fluid into pleural space -If malignant effusion, 75% of cases associated w/ lung & breast carcinoma, lymphoma

Adynamic Bone Disease

Form of renal osteodystrophy associated w/ overtreatment Prevention: -Avoid overtreatment of renal osteodystrophy w/ phos binders (try to control by dietary phos restriction) Pathophysiology: -Excessive PTH suppression by overtreatment of renal osteodystrophy (phos restriction, phos binders to decrease serum phos) -> low bone turnover -> decreased cellularity & bone mineralization -> increased fx risk

Gait Apraxia

Frontal gait, Bruns ataxia Presentation: -Magnetic/freezing gait w/ start & turn hesitation -Normal strength, coordination, sensation -May be associated w/ dementia, incontinence, frontal lobe signs Pathophysiology: -Frontal lobe degeneration (damage to cortico-cortico white matter fibers in frontal lobe), normal pressure hydrocephalus

Glioblastoma

Grade IV astrocytoma, glioblastoma multiforme Presentation: -Most common primary brain malignancy -Nausea, vomiting, headache worse w/ changes in position/coughing/sneezing, papilledema, other signs of increased ICP -Location-dependent sx (e.g. personality changes, strange behavior w/ frontal lesions) Diagnosis: -Head CT/MRI w/ butterfly lesion w/ central necrosis and heterogeneous/serpiginous contrast enhancement, characteristically crosses midline -On histology, exhibits neovascularity, necrosis -Degree of anaplasia determines prognosis

Lynch Syndrome

Hereditary nonpolyposis colorectal cancer (HNPCC) Presentation: -Hereditary cancer syndrome associated w/ colorectal/endometrial/ovarian cancers Diagnosis: -Genetic testing, esp in pts w/ strong fhx of colon cancer Treatment/Management: -Regular colonoscopies, endometrial bxs starting at 30-35 yo -Prophylactic hysterectomy w/ bilat oopherectomy at 40 yo or once childbearing complete Pathophysiology: -ADH inheritance of germline mutation in DNA mismatch repair gene

Factor Precipitating DKA Mnemonic

Infection Ischemia/Infarction Iatrogenic (glucocorticoids) Intoxication Insulin missed Intra-abdominal process (e.g pancreatitis, cholecystitis) Intraoperative/perioperative stress Infant (pregnancy)

Internal Hordeolum

Infection of the meibomian gland Presentation: -Internal stye, tender nodule visible at palpebral conjunctiva Treatment: -Warm compresses

Methylnaltrexone

MOA: -Blocks mu opioid receptors in gut mucosa w/o reversing analgesic effect of opioids Indications: -Used to reverse opioid-induced constipation

Methotrexate

MOA: -Folate antimetabolite, inhibition of dihydrofolate reductase Indications: -RA DMARD, psoriasis, IBD, SLE, vasculitis, dermatomyositis -Abortifacient w/ misoprostol (e.g. ectopic, molar pregnancy) ADRs: -Hepatotoxicity (check LFTs), stomatitis/mucositis, cytopenias (megaloblastic anemia from folic acid def, myelosuppression), pulm fibrosis, baldness -May be administered w/ folic/folinic acid to reduce risk of ADRs (does not affect efficacy) Antidote: -Leucovorin/folinic acid (more potent than folic acid) -Folic acid supplementation used for prevention w/ chronic use

Medigap

Medicare supplement insurance: optional supplemental plans that cover copays, deductibles, & other services not covered by parts A & B; do not cover meds

Mild Neurocognitive Disorder

Mild cognitive impairment Presentation: -Mild decline in 1+ cognitive domains -Normal functioning in all ADLs w/ compensation

Motor Aprosodia

Monotone voice, inability to express emotion in speech Pathophysiology: -Lesion affecting nondominant frontal lobe, which controls the way a person conveys emotion through speech

Leprosy

Mycobacterium leprae (acid-fast bacillus) Presentation: -Macular, chronic, anesthetic skin lesions w/ raised/well-demarcated borders, often hypopigmented -Nodular, thickened, painful nearby nerves w/ loss of sensory/motor fxn due to segmental demyelination -Risk factors: immigrant/traveler (Asia, Africa, S. America) Diagnosis: -Full-thickness biopsy of skin lesion (active edge); not culturable Treatment: -Dapsone & rifampin if minimal lesions "paucibacillary" -Add clofazimine if severe/"multibacillary" -Lesions take mos/yrs to heal Pathophysiology: -Spread by respiratory droplets, nine-banded armadillo -Low infectivity

Memantine

Namenda MOA: -Blocks action of glutamate at NMDA receptors Indications: -Moderate to severe Alzheimers disease (can improve cognitive sx)

Keloid

Presentation: -Benign fibrous growths that develop in scar tissue, may be disfiguring & painful -Firm/hard to palpation, scar tissue extending beyond original wound, may have irregular borders -Thought to have genetic predisposition, tend to cluster in families & certain ethnicities (e.g. African American) Diagnosis: -Clinical; bx discouraged b/c can worsen keloid formation Treatment/Management: -Intralesional glucocorticoids, often requires serial injections for satisfactory response, treatment failure common -Surgical excision occasionally needed if steroids fail Pathophysiology: -Overproduction of extracellular matrix & dermal fibroblasts

Cardiac Myxoma

Presentation: -Benign neoplasm, 80% in LA -Position-dependent mitral valve obstruction (worse sitting up, better lying down) w/ apical mid-diastolic rumble (similar to mitral stenosis), dyspnea, lightheadedness, syncope, new-onset AFib -Embolization of tumor fragments -> stroke w/ multiple areas of infarction, other acute ischemia (e.g. limb, mesenteric) -Tumor "plop" sound at end of diastole on auscultation -Constitutional sx (low-grade fever, weight loss) -Complications: embolization, sudden cardiac death (due to impaired cardiac output) Diagnosis: -Echo w/ mass (usually in LA), TEE > TTE Treatment: -Prompt surgical resection Pathophysiology: -Obstructive sx b/c tumor is usually mobile, obstruction is intermittent -Some myxomas produce cytokines like IL-6 -> systemic/constitutional sx -Most common primary cardiac tumor, usually pedunculated w/ stalk coming from atrial septum & can be extremely friable (prone to release emboli)

Budd-Chiari Syndrome

Posthepatic venous thrombosis Presentation: -Acute: severe RUQ pain w/ jaundice, hepatic encephalopathy, variceal bleeding, rapidly developing ascites, hepatosplenomegaly -Chronic: vague/progressive abd pain, hepatosplenomegaly, ascites, signs of portal HTN (e.g. gastroesophageal varices) -Ascites usually straw yellow w/ normal amylase, high protein, high serum:ascites albumin gradient -Labs: prolonged INR/PT & elevated transaminases if acute, mild elevation in bili/transaminases/alk phos if subacute/chronic Diagnosis: -Doppler US w/ decreased hepatic vein flow -Testing for underlying disorders (e.g. JAK2 mutations for PV) Pathophysiology: -Hepatic venous outflow obstruction, thrombosis of hepatic veins or intra/suprahepatic IVC -May be d/t: myeloproliferative disorder (e.g. PV), malignancy (e.g. HCC), OCP use/pregnancy, other hypercoagulable state

Molluscum Contagiosum

Poxvirus skin infection Presentation: -Firm, round, domed, smooth, pink/flesh-colored papules with central umbilication, pruritus -May be widely scattered or in linear pattern due to adjacent spread of virus by scratching -Lower abdomen, genitals, upper thighs (more likely to affect face/trunk/extremities in children), spares palms/soles -Lesions are shiny, can be itchy, may become inflamed, can be surrounded by dermatitis -Self-limited & usually resolve w/o treatment in 6-12 mos Diagnosis: -Predominantly clinical, HIV testing if extensive lesions, STI testing if genital lesions Treatment: -Cryotherapy, curettage, topical therapy (cantharidin, podophyllotoxin) Pathophysiology: -Spread through fomites (e.g. towels), direct contact, or autoinoculation -Lesions usually due to sexual contact, may be spread by skin to skin contact or fomites in kids -Diffuse/severe infection (large lesions >10 mm, innumerable lesions, etc.) associated w/ cellular immunodeficiency (e.g. HIV)

Disc Herniation

Presentation: -Acute lumbosacral radiculopathy radiating in dermatomal distribution -Pain worse w/ flexion, usually begins after inciting event, worse w/ activity & better w/ rest -Positive straight leg test due to nerve root compression -Risk factors: age, degenerative disc disease

Initial Stabilization of Acute ST-Elevation MI

SNAP ABS -Pulmonary edema is a contraindication to beta-blocker -Hypotension, R-sided MI contraindications to nitrates

Painless Thyroiditis

Silent thyroiditis Presentation: -Variant of chronic autoimmune thyroiditis (Hashimoto) that presents w/ mild/brief hyperthyroid phase, sometimes followed by hypothyroid phase, usually resulting in spontaneous recovery -Small/nontender goiter Diagnosis: -Positive TPO antibody, low radioiodine uptake (indicating thyrotoxicosis due to release of preformed thyroid hormone rather than overproduction) Treatment: -Beta-blocker during thyrotoxic stage

Multiple Endocrine Neoplasia Type 2A

Sipple syndrome Presentation: 2 Ps, 1 M (parathyroid, pheo, medullary) mnemonic -Parathyroid hyperplasia (hypercalcemia) -Pheochromocytoma -Medullary thyroid carcinoma Diagnosis: -Genetic testing -Serum calcitonin, CEA, neck US, evaluation for coexisting tumors (e.g. metanephrines, catecholamines, abd imaging) Treatment/management: -Metanephrine screening prior to surgery -Thyroidectomy, adrenalectomy, parathyroidectomy Pathophysiology: -Mutation of RET gene

Lupus Anticoagulant

Type of antiphospholipid antibody Presentation: -Usually asymptomatic, increased risk for antiphospholipid antibody syndrome (associated w/ VTE, recurrent fetal loss) Diagnosis: -Prolonged PTT that fails to correct when mixed w/ plasma (mixing test, not a deficiency) d/t presence of antibody acting as inhibitor Pathophysiology: -LA binds to phospholipids used in most PTT assays, prevents them from inducing anticoag -> lab artifact prolonged PTT, resolves w/ addition of phospholipid

Dialysis Disequilibrium Syndrome

Presentation: -Changes in neuro status due to cerebral edema, N/V, HA Pathophysiology: -Osmotic shifts during hemodialysis

Global Cerebral Hypoperfusion

Presentation: -Confusion, vision loss, diffuse/nonfocal neuro sx in both UEs & LEs

Infective Endocarditis

Presentation: -Fever (>90%), weight loss, systemic embolization (e.g. sudden-onset weakness, stroke w/ multiple infarcts, limb/mesenteric ischemia) -Regurgitant murmur (85%) on auscultation (e.g. acute mitral regurg w/ holosystolic murmur at apex, tricuspid regurg w/ holosystolic LLSB murmur that worsens w/ inspiration) -Petechiae (50%), subungual splinter hemorrhages (<50%), Osler nodes (<50%, painful red nodules on pads of fingers/toes), Janeway lesions (<50%, nontender vascular lesions on palms/soles), embolic neurologic phenomena (40%), splenomegaly (30%), Roth spots (edematous retinal hemorrhages w/ pale centers, <5%) -If IV drug-related, most likely to involve tricuspid > aortic, often associated w/ septic pulmonary emboli (pleuritic chest pain, cough, nodular opacities on CXR) w/ fewer peripheral embolic phenomena like Osler/Janeway, if involves aortic more commonly associated w/ -Complications: chordae tendinae rupture (sudden onset dyspnea, pulmonary edema, murmur), valve insufficiency (common cause of death, presents w/ s/o HF), perivalvular abscess, pericarditis, intracardiac fistula, conduction abnormalities, mycotic aneurysm (-> subarachnoid hemorrhage), embolic stroke, cerebral hemorrhage, brain abscess, acute encephalopathy, meningoencephalitis, renal/other organ infarction, GN, drug-induced AIN from treatment, vertebral osteomyelitis, septic arthritis, MSK/metastatic abscess, other septic emboli -Labs: RBC casts w/ proteinuria in GN, RF+, normocytic anemia, elevated ESR -Risk factors: congenital heart disease (e.g. MVP, bicuspid aortic valve), prosthetic valve, prior endocarditis, intravascular catheters, IV drug use Diagnosis: -Major criteria: SERIAL BLOOD CULTURES (+ for typical organism incl S. aureus, Enterococcus, Strep viridans, recommended at 3 different venipuncture sites, prior to starting abx), Echo showing valvular vegetations (TEE preferred) -Minor criteria: predisposing cardiac lesion, IV drug use, temp >100.4, embolic phenomena, immunologic phenomena (e.g. glomerulonephritis), positive blood culture not meeting major criteria -Definite IE: 2 major or 1 major + 3 minor criteria -Possible IE: 1 major or 3 minor criteria Treatment: -Abx based on blood cultures -Vanc if suspect S. aureus & in empiric coverage -Amp-sulbactam if penicillin-resistant enterococcus/HACEK organisms -Penicillin G if suspect native-valve IE, penicillin-susceptible Viridans strep -Surgical consultation indicated if: acute HF, extension of infection (e.g. abscess, fistula, heart block), organism that is difficult to eradicate (e.g. fungus, MDR pathogen), persistent bacteremia on abx, large vegetation (>1 cm), persistent septic emboli Pathophysiology: -Most common valve involvement: mitral > aortic > tricuspid > pulmonic -Tricuspid IE: IV injected particulate matter tends to lodge on tricuspid valve & cause damage (allowing for bacterial adherence) before being filtered out by pulm capillaries (therefor never reaching mitral/aortic valve) -Caused by S. aureus in >50% of IE cases in IVD users

Viral Pleurisy

Presentation: -Fever, pleuritic chest pain, +/- pleural friction (distinguish from pericardial friction rub by disappearance during breath holding) Pathophysiology: -Inflammation of lung pleura

Exercise-Induced Hematuria

Presentation: -Gross hematuria, e.g. following participation in marathons (seen in ~24% of marathon participants) Diagnosis: -Hematuria, RBCs on microscopy (excluding myoglobinuria), no RBC casts -Repeat UA in 1 wk to ensure resolution -Cystoscopy not necessary unless persistent hematuria, but if performed may show bladder contusions Pathophysiology: -May be caused by repetitive up & down movements of bladder during running, direct trauma to kidneys or bladder in contact sports, shunting of blood flow towards muscles & away from kidneys

Functional/Psychogenic Tremor

Presentation: -High-amplitude, low-frequency tremors that occur inconsistently & associated w/ significant disability -Often improve w/ distraction

Whipple Disease

Presentation: -Hx of chronic malabsorptive diarrhea (steatorrhea, flatulence, abd distention), protein-losing enteropathy, weight loss, migratory non-deforming arthritis, generalized lymphadenopathy, low-grade fever, skin hyperpigmentation, chronic cough -May be associated w/ damage to eye, CNS, myocardium (e.g. aortic diastolic murmur w/ aortic regurgitation, CHF) -Later stages: dementia, supranuclear ophthalmoplegia, myoclonus -Risk factors: male, 40-60 yo Diagnosis: -Small intestinal bx w/ periodic acid schiff-positive macrophages in lamina propria containing non-acid-fast gram+ bacteria -PCR Pathophysiology: -Infection w/ gram+ bacillus Tropheryma whippelii

Cancer-Related Anorexia/Cachexia Syndrome

Presentation: -Hypercatabolic state associated w/ weight loss, anorexia, excessive reduction in skeletal muscle mass Treatment/Management: -Progesterone analogues (e.g. megestrol acetate) or corticosteroids to stimulate appetite; progesterone analogues preferred b/c decrease incidence of ADRs Pathophysiology: -Multifactorial weight loss thought to be due to systemic inflammation + decreased caloric intake

Neuroglycopenia

Presentation: -Hypoglycemia of the brain, associated w/ insulin use -Transient nausea, HA, focal neuro signs lasting mins to hrs

Alcoholic Ketoacidosis

Presentation: -Impaired mental fxn -Labs: ketonuria, ketonemia, mildly elevated BG (can also be low/normal), increased anion & osmolal gap Treatment/Management: -Dextros normal saline, thiamine supplementation -Insulin usually not required (dextrose increases insulin secretion -> metabolism of ketone bodies to bicarb)

Descending Aortic Aneurysm

Presentation: -May cause thoracic spinal cord ischemia; presents w/ urinary retention (bladder paresis), lower extremity paresis, crude touch/pain sensation loss, diminished reflexes Pathophysiology: -Thoracic spinal cord ischemia is rare complication (2-3%), attributed to interruption of intercostal and/or lumbar arteries, particularly at T10-T12 -> decreased anterior spinal artery supply -> anterior cord syndrome

Episcleritis

Presentation: -Mild-moderate eye discomfort, photophobia, watery discharge -Diffuse or localized bulbar conjunctival injection -Self-limited, does not affect vision or involve cornea -Risk factors: RA, other autoimmune disorders Pathophysiology: -Infection or inflammation of episcleral tissue between conjunctiva & sclera

Charcot-Marie-Tooth Disease

Presentation: -Most common hereditary polyneuropathy -Slowly progressive course w/ foot drop, muscle weakness, lower leg atrophy -Risk factors: fhx

Hemiplegic Migraine

Presentation: -Most common in adolescents -Headache, visual aura, self-resolving hemiplegia -Preceded by aura w/ motor weakness Pathophysiology: -Rare familial disorder

Mercury Toxicity

Presentation: -Motor neuropathy w/ weakness & areflexia Diagnosis: -24-hr urine testing

HIV Neuropathy

Presentation: -Occurs in pts w/ longstanding HIV -Bilateral distal extremity numbness, paresthesias (pinprick sensations, burning pain), loss of sensation (both light touch/vibration & pain/temp) & DTRs, usually motor strength preserved -Risk factors: advanced age, longer duration of illness, low CD4, nonadherence w/ antiretroviral meds, high viral loads, h/o exposure to neurotoxic antiretrovirals (e.g. didanosine, stavudine) Diagnosis: -Rule out other causes of distal symmetric polyneuropathy (e.g. diabetic, meds, vit def, uremia, etc.) Treatment: -Gabapentin first line to reduce sensory sx, SNRIs (duloxetine, venlafaxine), topical capsaicin Pathophysiology: -Thought to involve accumulation of HIV antigens (e.g. gp120) in distal sensory nerve fibers -> triggers macrophage/T-cell infiltration, cytokine release, axonal damage

Chylothorax

Presentation: -Pleural effusion w/ turbid or milky white appearance Diagnosis: -Exudative effusion rich in triglycerides (pleural fluid TG >110), lymphocyte predominance Pathophysiology: -Leakage of chyle into thoracic space, usually d/t obstruction of the thoracic duct

Left Ventricular Aneurysm

Presentation: -Post-MI complication (mos later), presents w/ HF (LE edema, pulm edema, JVD), angina, ventricular arrhythmia (e.g. VTach), systemic embolization (e.g. stroke) from mural thrombus Diagnosis: -ECG w/ persistent ST-elevation, deep Q waves -Echo w/ thin, dyskinetic myocardial wall w/ impaired EF Pathophysiology: -Scar tissue deposition following transmural MI in distribution of LAD -May also be rarely associated w/ HCM, Chagas disease -Increased risk w/ delay in coronary reperfusion -Systemic embolization from mural thrombus inside aneurysm

Syphilis

Presentation: -Primary: painless genital ulcer (single chancre/papule, regular borders & hard/clean base, indurated & well-circumscribed, nonexudative, shallow), usually resolves in 6-8 wks if untreated -Secondary: condyloma lata (flat/raised pink/gray velvety papules) on mucous membranes & moist skin of perineum/genitals/mouth, diffuse maculopapular rash involving palms & soles (begins in skin cleavage lines of trunk and spreads to extremities), widespread/epitrochlear lymphadenopathy, oral lesions, hepatitis, hair loss w/ lesions w/ moth-eaten appearance & scarring -Tertiary: CNS (dementia, tabes dorsalis: sensory ataxia, lancinating pains, reduced DTRs), CV (aortic aneurysm/insuff), cutaneous (gummas--soft, ulcerative masses w/ necrotic centers), Argyll Robertson pupils (normal pupillary constriction w/ accommodation but not w/ light) -Meningovascular form: stroke, confusion, HA -Latent: asymptomatic -Fetal complications (>80% if untreated): growth restriction, fetal demise, fetal infection, preterm labor, hepatomegaly, jaundice, hemolytic anemia, thrombocytopenia, long bone abnormalities, failure to thrive, intracranial/intrahepatic calcifications Testing: -VDRL/RPR screening, if pregnant universal at first prenatal visit (many false +, e.g. in APLS), repeat testing at 3rd trimester and delivery if high risk -FTA-ABS confirmatory testing (more specific for syphilis) -Microscopy w/ thin, delicate, corkscrew organisms on darkfield microscopy -If meningovascular, RPR & VDRL testing of CSF, CSF w/ lymphocytic predominance & elevated protein Treatment/Management: -IM benzathine penicillin G (1 dose for primary/secondary/early latent disease <12 mos, 1 dose weekly for 3 wks for late latent 12+ mos, unknown duration, or gummatous/CV syphilis), even during pregnancy, adequate treatment confirmed w/ 4x decrease in serologic titers @ 6-12 mos -If neurosyphilis, ocular syphilis, or congenital syphilis, IV aqueous penicillin G -If allergic to penicillin, skin testing and desensitization therapy -If unable to be desensitized (e.g. hx SJS/TEN, anaphylaxis), PO doxycycline for primary/secondary/latent/tertiary, IV/IM ceftriaxone for 2 wks for neurosyphilis, +f/u testing due to high failure rate w/ alternate treatments Pathophysiology: -Treponema pallidum readily crosses placenta

Cannon A Waves

Presentation: -Prominent jugular venous pulsation, may be associated w/ HA, jaw pain, sensation of neck pulsation Pathophysiology: -Intermittent, prominent A waves caused by surge in JVP due to RA contraction against closed tricuspid valve -Seen in AV dissociation (e.g. VTach), complete AV block, w/ frequent PVCs

Vibrio Vulnificus

Presentation: -Rapidly progressive disease (often <12 hrs) w/ septicemia (septic shock, bullous lesions) and/or cellulitis (hemorrhagic bullae, necrotizing fasciitis) -Increased risk of disease w/ liver disease (e.g. cirrhosis, hepatitis, hemochromatosis) or certain chronic medical conditions (e.g. DM, RA) -Labs: leukocytosis w/ left shift, renal insuff Diagnosis: -Blood & wound cultures Treatment/Management: -Empiric abx in those w/ likely illness as condition is highly fatal -IV ceftriaxone & doxycycline Pathophysiology: -Gram- rod, free living in marine environments, grows in brackish coastal water -May be seen w/ ingestion of oysters, wound infections following exposure to saltwater (e.g. recreational activities, raw seafood handling)

Transfusion Associated Circulatory Overload (TACO)

Presentation: -Respiratory distress/pulmonary edema within 6 hrs of transfusion, hypoxia, HTN due to fluid overload (vs. hypotension w/ TRALI) Treatment/Management: -Transfusion cessation, diuretic therapy, respiratory support Pathophysiology: -Involves rapid infusion of large volumes -> circulatory overload -Vs. TRALI, positive for JVD, possible S3, BNP elevated, EF decreased

Noonan Syndrome

Presentation: -Short stature, facial dysmorphism, spectrum of congenital heart defects in almost all pts (pulmonic stenosis, ASD, hypertrophic CM) Pathophysiology: -AD inheritance

Reactive Thrombocytosis

Presentation: -Slight thrombocytosis that usually resolves after inflammatory state has resolved -Risk factors: recent inflammatory state (e.g. infection, surgery, malignancy, autoimmune disease), splenectomy (decreased platelet removal) Pathophysiology: -Cytokine-driven platelet production

Filiform Cutaneous Warts

Presentation: -Solitary papule w/ finger-like keratotic projections Treatment/Management: -Snip/shave excision

Coronary Stent Thrombosis

Presentation: -Sx of ACS (crushing substernal CP) following stent placement -Can present as STEMI, nSTEMI, or unstable angina -Associated w/ high morbidity & mortality Pathophysiology: -Period of increased thrombosis risk (6-12 mos) until stent endothelization occurs b/c both bare metal & drug-eluting stents are thrombogenic -Most commonly due to nonadherence w/ meds required to reduce risk of thrombosis (dual antiplatelet therapy w/ aspirin & P2Y12 receptor inhibitor, e.g. clopidogrel, prasugrel, ticagrelor)

Neuroleptic Malignant Syndrome (NMS)

Presentation: -Tetrad of muscular rigidity ("lead pipe"), autonomic instability, AMS, & hyperthermia associated w/ reaction to antipsychotic agents Treatment/Management: -Supportive, dopamine agonists (e.g. bromocriptine)

Psychogenic Coma

Presentation: -Unresponsive to verbal/tactile stimuli, but DTRs, oculovestibular rxn, CN reflexes are all intact -Oculovestibular rxn: irrigation of external auditory canal w/ cold water leads to transient/conjugate/slow deviation of gaze to side of stimulus followed by saccadic correction to midline) -May have h/o prior episodes that resolved completely

Takayasu Arteritis

Presentation: -Usually Asian female pt 10-40 yo, gradual sx of arterial dilation/occlusion including lightheadedness, limb pain, UE ulcers & claudication -Constitutional sx (weight loss, fever, arthralgias, etc.) -BP discrepancy b/t arms (d/t subclavian stenosis), pulse deficits, arterial bruits -May develop coronary artery abnormalities -Labs: anemia Diagnosis: -Elevated inflammatory markers (ESR, CRP) -CXR showing aortic dilation, widened mediastinum -CT/MRI may show vessel wall thickening, narrowing of lumen Treatment: -Systemic glucocorticoids Pathophysiology: -Large vessel (e.g. subclavian) vasculitis primarily involving aorta & its branches -Characterized by mononuclear infiltrates, granulomatous inflammation of vascular media -> arterial wall thickening w/ aneurysmal dilation or narrowing/occlusion

Candida Endophthalmitis

Presentation: -Usually develops in context of neutropenia -Decreased visual acuity, eye pain, photosensitivity, fever -On fundoscopic exam, fungating, off-white, glistening, mound-like retinal lesions w/ vitreous extension, indistinct borders, vitreous haze -Risk factors: neutropenia (e.g. chemotherapy), CVC, TPN Treatment: -Systemic antifungals (e.g. amphotericin B) for 4-6 wks, intravitreal antifungal injection, vitrectomy (to reduce fungal microabscesses that may be recalcitrant to therapy) Pathophysiology: -May develop from hematologic seeding from TPN or other source

Erysipelas

Presentation: -Well-demarcated, raised, warm, tender area of erythema, may develop overlying bullae in severe cases -Often in areas of prior skin disruption (e.g. minor trauma, inflammation, concurrent infection, edema -Involvement of external ear common due to absence of lower dermis in skin -Constitutional sx (fever, chills), rapid spread & onset, regional lymphadenitis Diagnosis: -Usually clinical, blood cultures in pts who have extensive rash, systemic toxicity, underlying comorbidities (e.g. DM) Treatment: -IV abx (ceftriaxone, cefazolin), po abx if no systemic sx (amoxicillin, ampicillin) Pathophysiology: -Bacterial infection of dermis & lymphatics, most commonly by group A Strep -Other causes: beta-hemolytic Strep

Von Hippel-Lindau Syndrome

Presentation: HIPPEL mnemonic -Hemangioblastoma (retinal, spinal cord, cerebellar) -> decreased visual acuity from edema & distortion -> retinal detachment, glaucoma, vision loss -Increased clear cell carcinoma -Pheochromocytoma (HA, palpitations, severe HTN), Pancreatic neuroendocrine tumors, Papillary cystadenoma of epididymis, Port wine stains -Eye dysfunction (e.g. retinal hemangioma, angioblastoma), endolymphatic sac tumors of middle ear (hearing loss) -Liver/pancreatic (islet cell tumors)/kidney cysts Treatment/management: -Surveillance for associated malignancies (eye/retinal examination, plasma/urine metanephrines, MRI of brain/spine/abdomen) -Tumor resection (laser therapy for retinal hemagioblastoma) Pathophysiology: -AD mutation in VHL tumor suppressor gene on ch 3

Phencyclidine (PCP)

"Angel dust" Presentation: -Psychomotor agitation, combative/bizarre/violent behavior, diminished pain perception & delusions of strength, hallucinations, HTN, tachycardia, multidirectional nystagmus, ataxia -If mildly intoxicated, may exhibit dissociative sx (e.g. detachment, withdrawal) -Shorter duration (~8 hrs) of psychosis/delirium than bath salts Diagnosis: -Detectable on routine urine tox screens Treatment/Management: -Benzos for agitation, physical restraints if pt unable to be redirected verbally, antipsychotics used adjunctively if benzos fail to control agitation -If dissociative sx, provide low-stimulation environment Pathophysiology: -NMDA receptor antagonist

Transient Ischemic Attack (TIA)

"Mini-stroke" Presentation: -Focal neuro sx lasting <30 min w/ complete resolution -Neurologic emergency d/t acutely increased risk of stroke (up to 5% within first 48 hrs, 12% within first 30 days) Diagnosis: -Normal noncontrast CT -Within 24-48 hrs of event, should also obtain advanced brain imaging (MRI) even w/ normal CT, neurovascular imaging (e.g. CT/MR angiography, carotid duplex US), investigation for sources of embolism (e.g. cardiac rhythm monitoring, Echo) Treatment/Management: -Interventions to reduce risk of future TIA/stroke including aspirin, statin initiation, BP control, tobacco cessation

Spinal Stenosis

"Neuropathic claudication" Presentation: -Chronic low back & leg pain worse w/ extension (e.g. standing, walking downhill), better w/ flexion (pseudoclaudication, e.g. walking bent forward, "shopping cart sign," sitting), radiating into buttocks & thighs, +/- numbness & paresthesias -Usually presents w/ neurologic sx (sensory loss, leg weakness, numbness/tingling) -Normal pulses & ABI, often normal exam w/ negative straight leg testing -May complain of having "spaghetti legs" or walking "like a drunken sailor) -Risk factors: age (>60), degenerative disc disease Diagnosis: -Spinal MRI (shows narrowing of lumbar spinal canal, which can enclude encroaching of osteophytes at facet joints, hypertrophy of ligamentum flavum, protrusion of intervertebral discs) Treatment/Management: -Start w/ conservative management -Lumbar epidural block -Surgical decompression via laminectomy if refractory to above Pathophysiology: -Narrowing of central intraspinal canal, lateral recess, or neural foramen usually associated w/ degenerative disc disease (spondylosis, spinal OA) -Thickening of ligamentum flavum -> mechanical compression & narrowing of spinal canal

qSOFA Score

-1 pt each for RR >22, confusion, SBP 100 or lower -Pts w/ 2+ pts likely to be septic, should be treated w/ broad-spectrum abx within an hr, aggressive fluid resuscitation within 3 hrs -Fluids: rapid administration of relatively large volumes (30 mL/kg), 500 mL boluses w/ assessment for pulmonary edema between doses, aim to achieve MAP of 60-70, urine output of >0.5 mL/kg/hr -Abx: beta-lactam/beta-lactamase inhibitor (e.g. piperacillin-tazobactam), carbapenem (e.g. meropenem), or 4th gen cephalosporin (e.g. cefepime), vanco added to cover MRSA -Pressors indicated w/ recalcitrant hypotension after >3 L of fluid resuscitation over first hr (norepi preferred)

Radioactive Iodine Therapy

-131-I isotope taken up by thyroid follicular cells, beta emission results in slow necrosis of thyroid follicular cells -> clinical/biochemical resolution of hyperthyroidism over 6-18 wks ADRs: -Permanent hypothyroidism in >90% of pts w/ Graves (diffuse uptake vs. RAI in toxic nodular goiter & toxic adenoma usually focal in autonomous thyroid tissue)

Spur Cells

-Acanthocytes, irregularly sized RBCs w/ projections -Most commonly seen in liver disease

Management of Symptomatic Bradycardia

-Administer atropine -If inadequate response to atropine, transcutaneous pacing until can be transitioned to transvenous pacing/permanent pacemaker placement Pathophysiology: -Often d/t complete AV block

Ephedra

-Aka Ma-Huang -Herbal med/supplement used for respiratory sx (e.g. cold/flu remedy, weight loss, stimulant, improved athletic performance ADRs: -Hepatotoxicity -HTN, arrhythmias, MI, sudden death, stroke, seizures

Sentinel Event

-Any unanticipated even in a health care setting that results in death or serious physical/psychological injury, e.g. in-hospital suicide, abduction, discharge of infant to wrong family, surgery performed on wrong site/person

Model for End-Stage Liver Disease (MELD)

-Calculation used to predict mortality in pts w/ liver disease -Incorporates serum bilirubin, INR, Cr, and Na -Used to determine 90-day survival & calculate pt's priority on liver transplant list (pts w/ higher score take precedence)

Hypokalemia ECG Changes

-Can result in nonspecific ST changes (ST depression, decreased T wave amplitude), prominent U wave

Jin Bu Huan

-Chinese herbal med used as sedative & analgesic ADRs: -Hepatotoxicity

Pneumococcal Vaccination

-PPSV23 recommended for pts 65+ yo, pts <65 w/ certain comorbidities (chronic liver, lung, heart disease, T2DM, smoking)

Kappa Statistic

-Quantitative measure of inter-rater reliability (aka inter-rater concordance) -Reflects extent to which inter-rater agreement represents an improvement on chance agreement alone -Ranges from -1 (perfect disagreement) to +1 (perfect agreement) -Kappa = 0 suggests agreement due to chance, <0 suggests less than chance agreement, >0 suggests greater than chance agreement

Capnography

-Quantitative waveform or colorimetric analysis, noninvasive method for measuring CO2 concentration of exhaled breath -Most reliable method for verification of proper endotracheal tube placement -Normal capnogram has characteristic rectangular waveform w/ 4 phases -Colorimetric EtCO2 consists of litmus paper that changes color when exposed to CO2

Target Cells

-RBCs w/ central density surrounded by pallor (bulls eye appearance) -Seen in pts w/ hemoglobinopathies (thalassemia) or chronic liver disease (esp obstructive liver disease)

Guillain-Barre Syndrome (GBS)

Acute inflammatory demyelinating polyneuropathy Presentation: -Polyneuropathy presenting w/ symmetric, ascending weakness & areflexia (flaccid paralysis) that worsens over hrs to days, progresses to involvement of respiratory muscles, UEs, bulbar muscles of face (e.g. dysphagia) -Paresthesias & possible sensory ataxia (but otherwise few sensory sx), neuropathic/radicular pain (back pain w/o spinal tenderness), decreased/absent DTRs, autonomic dysfxn (e.g. arrhythmia, ileus, bradycardia, BP/HR instability/orthostatic hypotension, urinary retention, no sweating), respiratory compromise -Hx of preceding GI (e.g. Campylobacter) or respiratory (e.g. influenza, CMV) illness; also been associated w/ recent immunization, surgery, trauma -Typical course: 2 wks of progressive motor weakness, 2-4 wks of plateaued sx, slow/spontaneous recovery over mos (IVIg/plasmapheresis shorten time to recover by ~50%) -Prognosis: may have impaired recovery if precipitated by Campylobacter, if severe weakness on admission, or if require ventilatory support Diagnosis: -Clinical -Supportive findings include CSF w/ increased protein & normal leukocytes/glucose/RBCs (albuminocytologic dissociation), abnormal EMG & nerve conduction studies (peripheral motor/sensory NCV reduced), MRI normal or w/ enhancement of anterior nerve roots/cauda equina Treatment: -Monitoring of autonomic & respiratory fxn (e.g. spirometry; first priority is checking vital capacity) -IVIg (pooled human) or plasmapheresis; administered if nonambulatory and/or within 4 wks of sx onset -Elective intubation w/ deterioration of PFTs, respiratory distress, dysautonomia, and/or widened pulse pressure -Most recover fully within mos Pathophysiology: -Immune-mediated demyelinating polyneuropathy of peripheral nerves; molecular mimicry -> cross-reacting autoantibodies that damage peripheral nerve axons -Most commonly affects motor neurons, but can also affect autonomic & sensory neurons

Mononucleosis

EBV (sometimes CMV) infection Presentation: -Exudative tonsillitis/pharyngitis, (esp posterior cervical/diffuse) lymphadenopathy, hepatosplenomegaly (transient hepatitis), fever, malaise -Initial sx can last up to 1 mo, fatigue can persist for up to several mos -Maculopapular/morbilliform rash may occur on trunk w/ administration of beta-lactam abx like amoxicillin or ampicillin (typically spares extremities, no arthralgias, appears ~24 hrs after administration) -Labs: reactive lymphocytosis, mild transaminitis (transient hepatitis) -Complications: splenic rupture (most common in males in 3rd wk), acute airway obstruction, AIHA, thrombocytopenia Diagnosis: -Positive heterophile antibody (Monospot) test (25% false negative rate in 1st wk of illness, screens for IgM antibodies that agglutinate erythrocytes from unrelated species like horse & sheep), should repeat if negative or test anti-EBV IgG or IgM -Atypical lymphocytosis (large, vacuolated cytoplasm) -Abd US to confirm resolution of splenomegaly prior to resuming contact sports Treatment: -Supportive care (e.g. NSAIDs), usually resolves w/o sequelae -Airway obstruction treated w/ admission & IV corticosteroids to decrease airway edema -Avoid sports for 3+ wks (contact sports 4+ wks) due to risk of splenic rupture -Corticosteroids may also be used in cases of severe complications (e.g. overwhelming infection, aplastic anemia, thrombocytopenia) Pathophysiology: -Severe oropharyngeal inflammation & tonsillar enlargement can cause acute airway obstruction (presents w/ dysphagia, pooling oral secretions, tachypnea, mouth breathing, other signs of resp distress) -Reaction to beta-lactam abx thought to be due to delayed-type hypersensitivity rxn due to virus-induced immune modification (not a true drug allergy) -AIHA: IgM cold agglutinin antibodies cause complement-mediated destruction of RBCs, may occur ~2 wks after sx onset -> indirect hyperbili, reticulocytosis

Graves Disease

Hyperthyroidism Presentation: -Gen: heat intolerance, weight loss w/o decreased appetite, sweating, anxiety, insomnia -HEENT: lid lag (sclera seen above iris on downward gaze), ophthalmopathy/exophthalmos (proptosis, diplopia, impaired convergence, irritation w/ gritty/sandy sensation, redness, photophobia, pain w/ movement, tearing), periorbital edema -Skin: hair loss, infiltrative dermopathy (pretibial myxedema), onycholysis, clubbing (acropachy) -CV: HTN, tachycardia, AFib -Endocrine: goiter, hyperglycemia, hypercalcemia, bone loss, menstrual irregularities -GI: diarrhea -Neuro: tremors of fingers/hands, hyperreflexia, prox muscle weakness (e.g. difficulty combing hair, getting into/out of chair), muscle atrophy -Elderly more likely to present w/ "apathetic hyperthyroidism" w/ lethargy, apathy, decreased appetite, weight loss, muscle weakness (myopathy) -Risk factors: female, advanced age, smoking -Labs: hypercalcemia, hypercalciuria from hyperthyroid bone disease, hyperglycemia -Complications: arrhythmia, dilated cardiomyopathy, osteoporosis Diagnosis: -Low TSH, high T4, thyroid-stimulating abs Treatment/Management: -Antithyroid meds (PTU, methimazole) if mild hyperthyroidism, older age w/ limited life expectancy, preparing for radioactive iodine or thyroidectomy (+ beta-blocker for stabilization), or pregnant (PTU in 1st trimester), add propanolol for control of adrenergic sx -Radioactive iodine therapy if moderate to severe hyperthyroidism, mild/no ophthalmopathy (can worsen ophthalmopathy) -Thyroidectomy if very large goiter, suspicion of thyroid cancer, coexisting primary hyperparathyroidism, pregnant pt that cannot tolerate thionamides, severe ophthalmopathy, retrosternal goiter w/ obstructive sx -Start prednisone if moderate to severe ophthalmopathy prior to definitive treatment w/ surgery or RAI -Reassess thyroid fxn & response to treatment every 4-6 wks by total T3 & free T4 (TSH remains suppressed for mos & may not accurately reflect thyroid functional status) Pathophysiology: -Thyrotropin (TSH) receptor autoantibodies (TRAB) that bind and stimulate thyroid hormone production -> hyperthyroidism -> increased catecholamine sensitivity, sympathetic overactivation -Exophthalmos due to T cell activation and stimulation of orbital fibroblasts and adipocytes by TRAB -> orbital tissue expansion, lymphocytic infiltration

Apraxia

Inability to carry out learned purposeful movements (e.g. dressing apraxia--difficulty wearing clothes) Pathophysiology: -May occur w/ nondominant/contralateral parietal lobe lesions

Amyotrophic Lateral Sclerosis (ALS)

Lou Gehrig disease Presentation: -Usually initially presents as asymmetric distal limb weakness or w/ bulbar sx -Mixture of LMN (atrophy, fasciculations/twitching, weakness, cramping sensation in morning) & UMN (cramping, spasticity, hyperreflexia, pathologic reflexes e.g. +Babinski, jaw jerk) signs, incoordination -Oropharyngeal dysphagia (e.g. coughing after eating, choking, nasal regurgitation, aspiration, weak palatal reflex), bulbar sx, weight loss, dysarthria (changes in voice, slurred/hypernasal speech, difficulty understanding speech), pseudobulbar affect (inappropriate laughing, crying), tongue atrophy/fasciculations -Diaphragmatic weakness causes paradoxical breathing movement (expansion of abdomen w/ expiration instead of inspiration), elevation of diaphragm on CXR, hypoventilation, shallow breathing, orthopnea, dyspnea -Ocular motility, sensory, bowel, bladder, & cognitive functions preserved even in advanced disease -Progressive & universally fatal, usually over 3-5 yrs -Usually arises in pts of advanced age >70 -Labs: CK may be mildly elevated (<1000, d/t hypermetabolism of muscles or denervation) or normal, other labs normal (e.g. TSH, autoantibodies, B12) Diagnosis: -EMG, nerve conduction studies, MRI of head/spinal cord to exclude other causes, PFTs (restrictive pattern, normal DLCO) -Electrodiagnostic studies reflect LMN dysfxn, denervation in multiple muscle groups Treatment: -Riluzole (glutamate inhibitor, may help reduce glutamate-mediated excitotoxicity) + ederavone (antioxidant); improve survival and slow disease progression -Respiratory support (e.g. NIPPV), nutritional support (e.g. PEG tube), multidisciplinary symptom management Pathophysiology: -Loss of UMNs (e.g. corticospinal neurons in primary motor cortex) & LMNs (e.g. CNs, anterior horn cells) -Dysphagia due to CN involvement (hypoglossal, ambiguus, motor trigeminal): in 20%, CNs are affected first, associated w/ worse prognosis -Denervation of muscle fibers -> upregulation of ACh receptors (b/c of decreased input from NMJ d/t denervation) -> hypersensitivity to ACh -> spontaneous depolarization of muscle fibers -> widespread fibrillations, positive sharp waves (reflecting spontaneous depolarization) on EPS studies

Midodrine

MOA: -Alpha 1 agonist Indications: -Orthostatic hypotension

Vocal Cord Dysfunction

Paradoxical inspiratory adduction Presentation: -Dyspnea, noisy breathing (e.g. stridor), often in young women -Occurs in paroxysms Diagnosis: -Spirometric flow-volume loop abnormalities present

Agglutinin Disease

Pathophysiology: -Antibody-mediated hemolysis

Essential Tremor

Presentation: -Bilateral action/postural tremor of hands, usually w/o leg or trunk involvement, possible isolated head tremor w/o dystonia (hands > arms > head >> legs, can also affect voice/chin/trunk) -Often relieved by EtOH, more pronounced w/ hand extension, worse w/ movement & better but not obsolete w/ rest -Classically worsens at end of goal-directed activities (e.g. reaching for pen) -Usually bilateral & asymmetric -Slowly progressive, often hereditary (AD inheritance) -Usually no other neuro signs -Affects up to 5% of population -Vs. Parkinson disease, no resting tremor, rigidity, dyskinesia, or gait difficulty Treatment: -Propanolol (nonselective beta-blocker, may be contraindicated in airway disease & heart block), primidone (anticonvulsant), often used together -Indicated in pts w/ tremor-related disability (e.g. interference w/ work, impaired ADLs)

Hypertensive Retinopathy

Presentation: -Blurred vision, sometimes w/ limited visual field defects Diagnosis: -Fundoscopy w/ AV nicking, copper wiring, flame hemorrhages, cotton wool spots, optic disc edema

Cutaneous Larva Migrans

Presentation: -Creeping cutaneous eruption caused by dog (Ancylocystoma caninum) or cat (A. braziliense) hookworm larval infection -Primarily affects LEs, primarily cutaneous (deep infection rare), initially presents w/ erythematous/pruritic papule at site of entry -> intensely pruritic, serpiginous, reddish-brown tracks -Labs: eosinophils usually normal Treatment: -Antihelminths (ivermectin), otherwise usually clears spontaneously within wks Pathophysiology: -Acquired from walking barefoot on sand/soil

Scorpion Sting

Presentation: -Intense pain at bite site -Paralysis, hypertension, pancreatitis -Fasciculations, jerking movements of extremities -Usually no alterations in consciousness Pathophysiology: -Toxins cause neuronal membrane hyperexcitability leading to uncontrolled, repetitive firing of axons

Focal Segmental Glomerulosclerosis (FSGS)

Presentation: -Nephrotic syndrome (>3.5 g/24 hrs, edema, hypoalbuminemia, hyperlipidemia, urine lipids) -Risk factors: African American, Hispanic, obesity, HIV/heroin use

Membranous Nephropathy

Presentation: -Nephrotic syndrome (>3.5 g/24 hrs, edema, hypoalbuminemia, hyperlipidemia, urine lipids) -Risk factors: adenocarcinoma (e.g. breast, lung), NSAIDs, hep B, SLE

Clostridium Difficile Colitis

Presentation: -New-onset profuse watery diarrhea (3+ episodes/24 hrs) w/o frank blood/melena, low-grade fever, abd pain/cramping, nausea, leukocytosis, confusion Complications: fulminant colitis, toxic megacolon, GI perforation -Labs: leukocytosis, possible hypoalbuminemia (inflammatory protein-losing enteropathy), AKI d/t hypovolemia -Risk factors: recent abx (fluoroquinolones, cephalosporins, penicillins, clindamycin, carbapenems, monobactams), recent hospitalization, age >65, gastric acid suppression (PPIs, H2 blockers), comorbid GI disease (e.g. IBD), advanced age, chemotherapy, underlying IBD Diagnosis: -Stool studies: either stool PCR for C. diff toxin genes (stays positive for up to wks after treatment) or enzyme immunoassay for C. diff toxins & glutamate dehydrogenase antigen -NO COLONOSCOPY--risk of perf during insufflation Treatment/Management: -Oral vancomycin/fidaxomicin/metronidazole, recurrence in up to 25% of pts -If first episode: vanco PO or fidaxomicin -If first recurrence (common, 15-30% of pts): vanco PO in prolonged pulse/taper course or fidaxomicin if vanco was used in first episode -If multiple recurrences: vanco PO followed by rifaximin or above regimens, fecal microbiota transplant -If fulminant (e.g. hypotension/shock, ileus, megacolon): metronidazole IV + high-dose vanco PO (or PR if ileus present), surgical evaluation -If on abx w/ high risk of CDAD for other conditions, switch to abx w/ low CDAD risk if possible (e.g. TMP-SMX, macrolides, tetracyclines, aminoglycosides) -Infection control: hand hygiene w/ soap & water, contact isolation, sporicidal disinfectants (e.g. bleach) Pathophysiology: -Gram+ spore-forming bacterium transmitted fecal-oral route -After ingestion, spores germinate in colon to fully functional bacilli -Normal colonic flora usually able to suppress overgrowth, but abx can disrupt normal colonic flora, predisposing to C. diff proliferation -Release of C. diff exotoxins (A & B) cause inflammation & mucosal injury

Carpal Tunnel Syndrome

Presentation: -Numbness/paresthesias/pain over first 3 fingers and lateral 1/2 of 4th finger, if severe may result in weakness of thumb opposition & abduction, atrophy of thenar eminence & sensory deficits if advanced -Sx often intermittent, worse at night, may be provoked by repetitive/forceful hand/wrist use -Pain may be relieved by shaking ("flick sign"), holding arm in dependent position, running under warm water -Phalen (wrists in extreme flexion w/ dorsum of hands together)/Tinel (tapping of median nerve) signs (sensitivity/specificity limited), reverse Phalen (wrist hyperextension), hand elevation test (holding hand over head) -May be primary or secondary to other disorder (e.g. most common mononeuropathy in pts on hemodialysis) -Risk factors: obesity, occupation requiring frequent/strenuous hand/wrist involvement (e.g. keyboard/mouse work), see others below Diagnosis: -Clinical -If chronic/refractory, being considered for surgery: nerve conduction studies & EMG for confirmation & assessment of axonal loss (show slowed conduction velocities, high specificity & sensitivity) Treatment: -Wrist splinting (esp nighttime, to hold wrist in neutral position), glucocorticoid injections -Surgery (tunnel release) for severe/refractory sx Pathophysiology: -Entrapment mononeuropathy due to compression of median nerve as it traverses carpal tunnel (under tarnsverse carpal ligament) -Idiopathic/overuse: swelling & fibrosis of tendons & soft tissue -Hypothyroidism: soft tissue enlargement (mucopolysaccharides, infiltration of neural tissue & tendons by mucinous material), often bilateral -RA: extrinsic compression from joint deformity -Pregnancy: edema/fluid accumulation -ESRD: amyloid (from dialysis; inflammation stimulates formation of beta2 microglobulin) & calcium phosphate deposition, access related (e.g. bleeding, venous HTN during hemodialysis, vascular steal -> ischemic neuropathy, tracking of blood through fascial planes into carpal tunnel, usually in arm w/ vascular access) -Acromegaly: tendon enlargement, synovial edema -Gout: compression from tophi

Mitral Stenosis

Presentation: -Opening diastolic snap on auscultation followed by low-pitched diastolic rumble at apex (best heard w/ pt lying on L side at end-expiration), worse disease associated w/ earlier murmur, loud S1 (+/- loud P2 if pHTN) -Dyspnea on exertion, signs of increased PCWP (orthopnea, PND, cough, hemoptysis, pulm edema), +/- signs of RHF (e.g. hepatic congestion, peripheral edema, pleural effusion), AFib, increased risk of systemic emboli -Mitral facies: pinkish-purple patches on cheeks -Voice hoarseness from recurrent laryngeal nerve compression d/t LA enlargement (Ortner syndrome) -LA enlargement presents on CXR w/ straightening of L heart border, elevated L main bronchus, pulmonary vascular congestion w/ cephalization of blood flow to upper lobes -Lateral CXR may reveal posterior displacement of heart, impingement on esophagus -ECG w/ "P mitrale" (broad & notched P waves), atrial tachyarrhythmias, RVH (tall R waves in V1 & V2) -Most commonly associated w/ hx rheumatic heart disease (e.g. immigrants, latency period of 10-20 yrs, presentation in 40s-50s), may also be d/t age-related calcification, radiation-induced -Acute worsening may be associated w/ physiologic changes of pregnancy Diagnosis: -Echo w/ LA enlargement, increased transmitral flow velocity, mitral valve thickening/calcification/decreased mobility, possible coexisting mitral regurg Treatment/Management: -Percutaneous valvotomy or surgical repair/replacement -If female w/ exacerbation, check urine pregnancy test -Routine anticoag w/ warfarin (higher thrombogenic risk than nonvalvular AFib) Pathophysiology: -Blockage of LA outflow by stenosis -> increased LA pressure -> increased pulmonary pressures (both systolic & diastolic) -> pulmonary HTN -Increased pulmonary artery pressures also due to poorly understood reactive process, in which endothelin-mediated arteriolar vasoconstriction & remodeling occur -LV pressure distal to obstruction, pressure usually unaffected unless severe disease (low LV diastolic pressure) -Impaired diastolic filling of LV -> increased LA pressure -> LA wall stretching -> disruption of electrical conduction, arrhythmogenic foci

Piebaldism

Presentation: -Patchy absence of melanocytes from birth -> depigmentation usually confined to head & trunk -AD inheritance

Erythema Multiforme

Presentation: -Self-limited reaction to certain infections (e.g. HSV, Mycoplasma) & meds (e.g. sulfas, allopurinol) & malignancy & connective tissue diseases -Acute inflammatory disorder involving skin of extremities, face, trunk, neck, and rarely oral mucosa (erythema, erosions, bullae) if severe (begins on extensor surfaces of distal extremities & spreads centripetally) -Flu-like prodrome common (fever, malaise, sore throat) -Erythematous papules -> targetoid lesions that may have central bullae or dusky center, red inflammatory zone surrounded by pale ring & erythematous halo, negative Nikolsky Diagnosis: -Clinical, sometimes bx Treatment: -Antihistamines, topical glucocorticoids -Antiviral therapy does not shorten course -Usually self-limited & resolves w/o intervention within 1-2 wks Pathophysiology: -Cell-mediated response to certain infections, may involve immune response to antigens deposited in skin

Chloracne

Presentation: -Severe skin disorder caused by exposure to halogenated hydrocarbons (e.g. occupational exposure to pollutants like dioxin) -Inflammatory nodules & large comedones affecting head, neck, axilla

Parkinson Disease

Presentation: -Tremor--resting 4-6 Hz tremor w/ "pill-rolling" quality, frequently first manifests in 1 hand, then generalizes to involve other side & LEs, facial involvement less common (jaw, tongue, lips), decreases w/ voluntary movement, more pronounced w/ distractibility (e.g. mental tasks), exhibits re-emergence (goes away w/ movement, re-emerges when movement stops), often first sign of disease -Rigidity--baseline increased resistance to passive movement that may be uniform (lead pipe) or oscillating (cogwheel), often unilateral/asymmetric -Bradykinesia--difficulty initiating movements (e.g. starting to walk, rising from chair), narrow-based & shuffling gait w/ short strides & w/o arm swing (festinating/hypokinetic gait), micrographia, hypomimia (decreased facial expression, "mask-like" facies), hypophonia (soft speech) -Postural instability--flexed axial/stooped posture, loss of balance during turning/stopping, loss of balance when pushed slightly from stationary bipedal stance, frequent falls -Neurogenic orthostatic hypotension (lightheadedness, syncope, d/t autonomic dysfxn) -Parkinsonism signs precede dementia by 1+ yrs -REM sleep behavior disorder (e.g. dream enactment d/t loss of atonia during REM sleep, often precedes development of PD) Diagnosis: -Primarily clinical; LP & CT & EEG & peripheral nerve conduction studies often nonspecific/unremarkable/normal -Highly supported by response to dopaminergic therapy -MRI may be used to rule out other conditions Treatment: -Dopaminergic + anticholinergic drugs: -Mild & tremor-dominant disease: trihexyphenidyl + amantadine (counters anticholinergic side effects) -Mild: MAO-B inhibitors -Moderate: dopamine agonists like bromocriptine (poorly tolerated in elderly), levodopa -Severe: levodopa, carbidopa -COMT inhibitors (prolong levodopa's effects) -If psychotic sx d/t meds (d/t pro-dopaminergic activity): taper off meds to determine minimum effective dose, then consider addition of low-dosage antipsychotic (e.g. quetiapine, clozapine), pimavanserin Pathophysiology: -Neurodegenerative disorder associated w/ accumulation of alpha-synuclein in neurons of substantia nigra pars compacta -Involves progressive loss of dopaminergic neurons in basal ganglia leading to interruption of connection b/t basal ganglia & thalamus/motor cortex -> decreased dopaminergic activity, increased cholinergic activity -Baroreflex dysfxn: decreased postganglionic sypathetic release of norepi -> impaired reflex vasoconstriction, orthostatic hypotension

Orthostatic Tremor

Presentation: -Variant of essential tremor that presents in legs immediately on standing, relieved by sitting down, high frequency (14-18 Hz) and usually w/o other clinical signs/sx

Wernicke Aphasia

Receptive aphasia Presentation: -Difficulty comprehending & following commands, able to speak fluently (speech usually voluminous rambling w/o concrete meaning) -Repetition impaired -Lesions affecting surrounding areas may be associated w/ right superior visual field defects Pathophysiology: -Lesion of Wernicke's area in dominant parietal/temporal lobe (usually L side in both R-handed & L-handed people)

Synthetic Cathinones

"Bath salts," amphetamine analogs Presentation: -Similar to PCP intoxication, presents w/ severe agitation, combativeness, psychosis, delirium, myoclonus, seizures -Prolonged duration: vs. PCP intoxication, delirium & psychosis lasting up to a wk (vs. hrs-days) -HTN, tachycardia (increased sympathetic activity) -Often sold as white powder/crystal that is ingested/inhaled/ingested, may be labeled as "bath salts," "plant food," "cleaners" Diagnosis: -Not detected on routine urine tox screens Treatment: -Supportive care, benzos for seizures/agitation Pathophysiology: -Amphetamine analogs, increase release or inhibit reuptake of norepi, dopamine, serotonin

Hemochromatosis

"Bronze diabetes" Presentation: -Intrinsic liver disorder w/ elevated transaminases (hepatocellular injury), hepatomegaly, usually normal bili & alk phos (or mildly elevated w/o jaundice) -Also associated w/ DM, erectile dysfxn, skin hyperpigmentation (brown/bronze/gray skin more prominent on sun-exposed regions like face), arthropathy, increased risk of cirrhosis & hepatocellular carcinoma, restrictive/dilated cardiomyopathy, arrhythmias (conduction defects), hypopituitarism -> secondary hypogonadism, hypothyroidism -Degenerative arthritis most commonly involving 2nd & 3rd MCPs, radiographs w/ periarticular "hook-like" osteophytes, subchondral cysts, sclerosis, osteopenia, joint space narrowing, chondrocalcinosis (deposition of calcium pyrophosphate dihydrate in articular cartilage, pseudogout) -Increased susceptibility to Listeria, Vibrio vulnificus, Yersinia enterocolitica infections -Usually presents in 40s-60s in men, later in women d/t menstrual iron loss -Labs: elevated transaminases (hepatocellular pattern), transferrin saturation, ferritin, iron, low testosterone/FSH/LH Diagnosis: -HFE mutation testing -Liver bx may be used to stage liver involvement Treatment/Management: -Therapeutic/serial phlebotomy (urgent if ferritin >1000) -Echo to assess for HF -Liver transplantation for advanced liver disease w/ cirrhosis Pathophysiology: -Excessive iron absorption, deposition of iron in tissues -Secondary deposition in pituitary -> hypopituitarism, secondary hypogonadism -> testicular atrophy, low testosterone -Autosomal recessive inheritance

Euthyroid Sick Syndrome

"Low T3 syndrome" Presentation: -Abnormal thyroid function testing in pts w/ severe, acute illness (often ICU admission, taking high-dose glucocorticoid therapy) Diagnosis: -TFT testing; in early disease low T3 & normal TSH & T4, in late disease (if severe illness persists) low T3, TSH, & T4 -Reverse T3 (inactive metabolite of T4) elevated d/t reduced clearance Treatment: -Observe w/o treatment (thyroid hormone supplementation not indicated, does not improve pt outcomes) -F/u testing when pt has returned to baseline health Pathophysiology: -Severe illness causes decreased peripheral deiodination of T4 -> T3 due to caloric deprivation, increased glucocorticoid & inflammatory cytokine release (TNF, IFN-alpha), inhibitors of 5'-deiodinase (e.g. free fatty acids) -May be exacerbated by administration of exogenous glucocorticoids, other meds (e.g. amiodarone) -Thought to represent a mild transient central hypothyroid state that serves as adaptive response to severe illness (functions to minimize catabolism in acute illness)

Otitis Externa

"Swimmer's ear" Presentation: -Ear pain w/ discharge, edema, pruritus, inflammation of external auditory canal (w/ erythema, debris) -Pain w/ auricle manipulation -Tympanic membrane spared (clear, not inflamed, no middle ear fluid) -Risk factors: water exposure, trauma (e.g. cotton swabs, ear candling), foreign material (e.g. hearing aid, headphones), dermatologic conditions (e.g. eczema, contact dermatitis) Treatment/Management: -Gently clear cerumen & debris w/ wire curette or cotton swab, possible irrigation w/ dilute hydrogen peroxide -If mild, topical acidifiers (e.g. acetic acid, domeboro solution) -If moderate, ototopic antibiotic drops (e.g. fluoroquinolone like ciprofloxacin, antipseudomonal activity, neomycin/polymyxin B) +/- topical glucocorticoid -Consider wick placement to facilitate medication delivery if canal very swollen Pathophysiology: -Infection of the outer ear (ear canal), usually due to Pseudomonas aeruginosa or S. aureus -Water contains Pseudomonas and removes cerumen (has antimicrobial properties), alters ear canal pH, causes skin maceration

Lateral Epicondylitis

"Tennis elbow" Presentation: -Insidious elbow pain over wks-mos, usually ~1 cm distal to lateral epicondyle -Pain elicited by resisted wrist extension & passive wrist flexion, making fist (grip strength testing) -FROM Treatment/Management: -MSK US for doubtful cases -Initial treatment w/ activity modification, counterforce bracing/strap (tendinosis strap) to reduce load transmitted to tendon origin, compression sleeve -If refractory sx, short-term NSAIDs, corticosteroid injection, surgery consultation Pathophysiology: -Repetitive, forceful overuse of wrist & digit extensors (esp conjoined tendon of extensor carpi radialis brevis & extensor digitorum) -Angiofibroblastic tendinosis (disorganized tissue & neovessels, true inflammatory infiltrate normally scant) of extensor tendons at lateral epicondyle

Coccidiomycosis

"Valley fever" Presentation: -Fever, CP, cough, night sweats, can involve skin/meninges/bones -Cutaneous features: erythema nodosum + erythema multiforme, cutaneous soft tissue abscesses -Southwestern US (ARIZONA), CENTRAL + SOUTH AMERICA + CALIFORNIA = CLASSIC -Primary pulmonary infection has nonspecific features (fever, chest pain, productive cough, lobar infiltrate or normal CXR, hilar lymphadenopathy) Diagnosis: -Serologic testing, sometimes cultures -Bx w/ spherules w/ endospores Treatment/Management: -If mild/moderate & otherwise healthy, observe & f/u -If severe and/or risk factors, antifungal treatment (ketoconazole, fluconazole) Pathophysiology: -Mold easily aerosolized in dry mos after rainy season, via dust/soil -Inhalation of single arthroconidium sufficient to cause infection (7-14 days after inoculation)

Normal Pressure Hydrocephalus

"Wet, wacky, & wobbly" Presentation: -Progressive gait difficulties (slow & wide-based w/ small/shuffling steps, instability, frequent falls, "magnetic" gait that looks like pt's feet are stuck to ground), cognitive disturbances (psychomotor retardation, apathy, executive dysfxn), urinary incontinence/urgency -Depressed affect (frontal lobe compression), impaired executive fxn -UMN signs in LEs -Risk factors: age >60 yo Diagnosis: -Neuroimaging (MRI) w/ ventricular enlargement out of proportion to sulci enlargement or brain atrophy -Normal opening pressure on LP -Marked improvement in gait w/ spinal fluid removal (Miller Fisher/lumbar tap test) Treatment: -Ventriculoperitoneal or ventriculoatrial shunt placement Pathophysiology: -Decreased CSF resorption by arachnoid granulations -> CSF accumulation -Usually idiopathic and occurs in elderly, but may be secondary if associated w/ prior neurologic insults (e.g. subarachnoid hemorrhage, meningitis, trauma, chronic periventricular ischemia, elevated central pressures) that result in scarring/destruction of arachnoid granulations -Idiopathic form associated w/ chronic periventricular ischemia & increased venous resistance that alters arachnoid granulation fxn

Ecological Fallacy

-An error in reasoning in which incorrect conclusions about individual-level processes are drawn from group-level data (ecological studies) -Fallacy assumes that individuals of a study population have the avg characteristics of the population as a whole

Phase II Clinical Trial

-Assesses efficacy, optimal dosing, & adverse effects of new treatment in small number of pts w/ disease of interest

Phase I Clinical Trial

-Assesses toxicity, max tolerated dose, adverse effects, pharmacokinetics, pharmacodynamics, usually in small number of healthy subjects

Cerebellar Signs

-Ataxia (broad-based gait), intention tremor (low frequency of 3-4 Hz & high amplitude, increases steadily as hand meets target), dysmetria, impaired rapid alternating movements (dysdiadochokinesia), upward drift on pronator drift testing due to hypotonia, impaired heel-to-shin testing, nystagmus, dysarthria

Malaria Chemoprophylaxis

-Atovaquone-proguanil, doxycycline, mefloquine (beginning 2+ wks prior to travel, during trip, and discontinued 4 wks after return) -Not chloroquine (high levels of resistance) -Travelers also advised to wear protective clothing, use insect repellant, insecticide-treated bed netting

Inpatient Management of Diabetes

-Basal bolus regimen (intermediate or long-acting) used for all pts w/ T1DM (may be reduced to 25-50% of pre-hospital dose depending on nutritional status, T2DM pts treated before admission w/ basal bolus regimen, pts w/ T2DM inadequately controlled by sliding scale only regimen, & pts w/ newly diagnosed DM & high glucose levels -Insulin sliding scale only (short-acting) used for pts w/ T2DM that is well controlled w/ diet and/or oral meds before admission; addition of basal insulin if blood glucose suboptimally controlled on sliding scale alone -Insulin infusion (may require ICU admission) used for T1DM pts who are not eating & w/ glucose levels suboptimally controlled w/ SC insulin, T1DM pts in perioperative setting or in labor, & hyperglycemic emergencies (e.g. DKA, HHS) -American Diabetes Association suggests maintaining glucose at 140-180

Peripherally Inserted Central Catheter (PICC)

-Catheter inserted into a peripheral vein and designed and used for administration of sterile fluids, nutrition formulas, and medications into central veins -Advantages over centrally inserted central catheters: lower infection rate, fewer procedural complications (e.g. PTX, carotid artery puncture), increased pt comfort (e.g. less interference w/ activities like bathing) -Disadvantages compared to CICC: 2.5x higher risk of thrombotic events (UE venous thrombosis, can progress to PE, d/t longer catheter length that increases vascular endothelial contact/damage & higher catheter-to-vein diameter ratio) -Should be avoided in pts w/ clinically significant CKD (eGFR <60) that may need peripheral sites for future access; thrombosis can induce vessel stenosis & reduce available sites for future HD access

Bacterial Meningitis Coverage

-Cefepime: 4th gen cephalosporin, covers S. pneumo, N. meningitidis, GBS, H. flu, Pseudomonas -Vancomycin: covers cephalosporin-resistance pneumococci -Ampicillin: covers Listeria -Dexamethasone helps prevent neuro complications of S. pneumo meningitis (deafness, focal deficits), should be discontinued once S. pneumo meningitis is ruled out

HBV Post-Exposure Prophylaxis

-Considered in pts exposed to blood from suspected/known HBV pt -Usually none needed if vaccinated against HBV w/ known antibody response, but should receive HBV booster -If not vaccinated against HBV and/or no antibody response, should receive HB immune globulin within 24 hrs + HBV vaccine

Osteoporosis Screening

-DXA starting at 65 yo (osteoporosis defined as T-score of -2.5 or lower, osteopenia -1 to -2.5) -If multiple risk factors (e.g. weight <58 kg, parental h/o hip fx, early menopause), should estimate risk using FRAX calculator & possibly screen earlier -Bisphosphonates indicated if low bone mass w/ h/o fragility fx, bone density criteria for osteoporosis, or osteopenia w/ 10-yr probability for osteoporotic fx 20%+ or hip fx 3%+ w/ FRAX calculatorf

Virologic Failure

-Defined as failure to achieve viral load <200 copies/mL within 6 mos of antiretroviral therapy -May be due to drug resistance or noncompliance

Cardiac Resynchronization Therapy

-Device for simultaneous pacing of R & L ventricles (biventricular pacing, esp for pts w/ abnormal ventricular conduction that can impair ventricular synchrony) in select HF pts -Can improve exercise tolerance & NYHA functional class, reduce rates of hospitalization & overall mortality -Eligible pts must meet all of the following: LVEF <35%, NYHA class II/III/IV HF sx, LBBB w/ QRS duration >150 ms

Protein Gap

-Difference b/t total protein & albumin >4 -Indicates non-albumin protein in serum -May be seen in polyclonal gammopathies (e.g. infection, connective tissue disease) or monoclonal pathologies (e.g. Waldenstrom macroglobulinemia, multiple myeloma)

Standard Medical Therapy in CAD

-Dual antiplatelet therapy (aspirin + P2Y12 inhibitors including clopidogrel, prasugrel, ticagrelor) -Beta-blockers -ACEi/ARBs -Statins (HMG-CoA reductase inhibitors) -Aldosterone antagonists (eplerenone, spironolactone) in pts w/ LVEF of 40% or lower w/ HF sx and/or T2DM

Phase III Clinical Trial

-Evaluates efficacy, risks, & benefits of new treatment compared to current standard treatments and/or placebo in large sample of affected patients w/ condition of interest -Usually randomized, blind, & compares 2+ treatments -Determines whether treatment is approved by FDA for commercial market

Female Genital Cutting

-Female circumcision, genital mutilation -Cultural practice predominantly performed in some parts of Africa involving manipulation or removal of external genital organs -Considered an important rite of passage into womanhood by some cultures -Usually performed by nonmedical practitioners w/o anesthesia or abx -Complications: genital pain, scarring, infection, infertility, difficulty w/ coitus and/or vaginal delivery

Metaiodobenzylguanidine (MIBG) Scan

-Functional scintigraphy w/ I123-labeled MIBG, which resembles norepi, is taken up by adrenergic tissue, can detect tumors not detected by CT or MRI (e.g. high index of suspicion for pheo but negative imaging) -May also be considered in pt w/ large tumors >5 cm d/t higher risk of malignancy & possible extra-adrenal disease -Also considered in younger pts, pts w/ confirmed familial disorder d/t higher risk for multiple & extra-adrenal tumors

Verification Bias

-Gold standard testing selectively in order to confirm result, esp in situations where gold standard testing is invasive & has risk of complications and is not feasible to perform in all patients (e.g. liver bx), esp w/ pts w/ negative results -Can over or under estimate sens/spec -Overcome by randomly performing gold standard testing on random sample of participants w/ negative results

Funnel Plot

-Graphical tool used to assess publication bias -Plot of study's treatment effect on x-axis against study's size or precision on y-axis (usually represented by standard error) -If no bias, any scatter b/t study results should be d/t sampling variation, 95% should lie within triangle centered on summary estimate & extending 1.96 SD on either side -Should be symmetric in absence of study heterogeneity & publication bias -Asymmetry suggests publication bias, heterogeneity, methodological anomalies, artifact, or chance

Hepatitis B Serology

-HBsAg: first serological marker, precedes onset of clinical sx & aminotransferase elevation, detectable throughout infection -Anti-HBs: appears in serum after successful vaccination or clearance of infection, usually remains detectable for life, indicates noninfectivity/immunity, window period = time b/t HBsAg disappearance & appearances of anti-HBs -HBcAg: core antigen, not usually detectable in serum (normally sequestered in HBsAg coat) -Anti-HBc: IgM appearance shortly follows HBsAg, may be only marker present during window period, IgG remains detectable during recovery or progression to chronic infection -HBeAg: detectable shortly after appearance of HBsAg, indicates active viral replication, associated w/ presence of HBV DNA, tends to disappear after aminotransferase levels peak & before HBsAg is eliminated -Anti-HBe: represents cessation of active viral replication, low infectivity

Licorice

-Herbal remedy used for stomach ulcers & bronchitis/viral infections -Often found in herbal teas & food products ADRs: -HTN, hypokalemia Pathophysiology: -Contains glycyrrhetinic acid, which inhibits 11-beta-hydroxysteroid dehydrogenase, preventing conversion of cortisol -> cortisol -Excess cortisol binds mineralocorticoid receptors -> HTN, hypokalemia, metabolic alkalosis, suppression of aldosterone & renin production

Colon Cancer Screening

-If no increased risk, colonoscopy every 10 yrs starting at 50 -If found to have small adenomatous polyps, every 5 yrs -If fhx of adenomatous polyps or CRC (1 FDR relative <60 yo or 2+ FDRs at any age), start at age 40 or 10 yrs prior to age of diagnosis of affected relative, repeat every 5 yrs -If IBD (UC or CD w/ colonic involvement), begin 8 yrs after disease onset, then every 1-3 yrs after -Classic FAP: begin @ 10-12 yo, repeat annually -HNPCC/Lynch: begin @ 20-25 yo, repeat every 1-2 yrs -If h/o abdominopelvic radiation: begin @ 30-40

Proprioception Signs

-Indicate dorsal column dysfxn -Positive Romberg test

Platelet Transfusion

-Indicated if platelets are <10,000 or <50,000 w/ active bleeding

Renal Transplant

-Living-donor transplant long-term survival rate is ~91% @ 5 yrs (increases risk of donor gestational complications, e.g. fetal loss, preE, gestation DM, gestational HTN) -Deceased-donor transplant long-term survival rate is ~84% @ 5 yrs (lower due to longer cold ischemic time -> higher risks of hypoxic injury, graft failure) -Significant survival benefit over any type of dialysis, better QOL, improvement in HTN & endocrine abnormalities associated w/ ESRD (e.g. anemia, impaired bone metabolism), improvement in microvascular disease in recipients w/ DM -Post-transplant risk of new-onset DM (med adverse effects to glucocorticoids & calcineurin inhibitors w/ reversible toxicity to pancreatic islet cells, increased insulin excretion by transplanted kidney -> increased gluconeogenesis)

Thyroid Nodule Ultrasound Findings

-Nodules w/ microcalcifications, irregular margins, internal vascularity, hypoechoic texture carry higher risk of malignancy; should undergo FNA if >1 cm -Nodules w/ cystic/spongiform lesions have lower risk of malignancy; should undergo FNA if >2 cm

Kava

-Piper methysticum -Herbal remedy used for anxiety, insomnia, menopause ADRs: -Severe hepatotoxicity, liver failure

Sheehan Syndrome

-Postpartum hypopituitarism/infarction and necrosis of pituitary (which enlarges after 20 wks gestation), usually secondary to obstetric blood loss (postpartum hemorrhage) Presentation: -Inability to breastfeed, fatigue, oligomenorrhea/amenorrhea, hot flashes, vaginal atrophy, hypotension, bradycardia, anorexia, weight loss, orthostatic hypotension -Does not occur w/ abortions <20 wks -Labs: iron deficiency anemia from PPH, hyponatremia (normal K b/c aldosterone preserved), hypocortisolemia Diagnosis: -Decreased GnRH, FSH, prolactin, estrogen Treatment: -Pituitary hormone replacement Pathophysiology: -Decreased prolactin -> lactation failure -Decreased FSH/LH -> amenorrhea, hot flashes, vaginal atrophy, decreased body hair -Decreased TSH -> fatigue, bradycardia, dry skin (central hypothyroidism) -Decreased ACTH -> anorexia, weight loss, hypotension, lethargy (secondary adrenal insufficiency), hyponatremia (d/t increased ADH secretion, normally inhibited by cortisol), no skin hyperpigmentation vs. primary AI -Decreased GH -> decreased lean body mass

Sertoli-Leydig Cell Tumor

-Sex cord-stromal (often ovarian) tumor composed of Sertoli cells that form tubules and Leydig cells (between tubules) with characteristic Reinke crystals, presents w/ increased testosterone Presentation: -Rapid-onset virilization (voice deepening, male-pattern bitemporal baldness, increased muscle mass, clitoromegaly), signs of hyperandrogenism (hirsutism, nodulocystic acnes), oligomenorrhea, unilateral/solid adnexal mass (arises from ovaries or adrenal glands) -Signs of estrogen deficiency: breast atrophy, vulvovaginal atrophy, dyspareunia, oligomenorrhea/amenorrhea, AUB -More common in postmenopausal women -If during pregnancy, high risk of both maternal & fetal virilization Diagnosis: -Testosterone levels markedly elevated (>150 ng/dL) but DHEAS normal in ovarian tumors -Elevated DHEAS but testosterone normal in adrenal tumors -Decreased FSH/LH due to suppression by testosterone Treatment: -Surgical removal (if during pregnancy, 2nd trimester or postpartum Pathogenesis: -Testosterone inhibits GnRH and FSH/LH release -> estrogen deficiency

Howell-Jolly Bodies

-Single, round, blue RBC inclusions representing retained RBC nuclear remnants usually removed by spleen -Usually seen in asplenia or functional asplenia/hyposplenism (e.g. SCD, sarcoidosis, thrombosis)

Clinical Pathways

-Standardized decision tools often integrated into the EMR -Typically specify an algorithm, or a sequential, evidence-based series of actions to take based on pt data -Increase quality of care by reducing unnecessary variation in health care services for & by reducing errors caused by physician cognitive bias -Intended to guide physicians rather than substitute their clinical judgment or dictate how they should provide care to every patient -Allow for exceptions at the physicians' discretion (e.g. patient preferences, severity of illness, external factors, etc.)

S4 Heart Sound

-Stiff/hypertrophic ventricle (aortic stenosis, restrictive cardiomyopathy, HCM, LVH w/ longstanding HTN) -Corresponds w/ atrial contraction (atrial gallop), believed to result from sound of blood hitting stiffened LV -Ten-ne-ssee -Normal in healthy older adults (>70), abnormal in younger adults & children, w/ hypertrophy, or w/ MI (acute phase)

End of Life Medication Management

-Stop: as-needed insulin (& fingerstick BG checks), antihypertensives, statins, aspirin -Continue: pain meds, antiemetics, laxatives, anxiolytics, antidepressants, secondary seizure ppx -Individualize: HF therapy, antianginal therapy, scheduled DM meds, primary seizure ppx

Cross-Over Study

-Subjects are exposed to different treatments or exposures sequentially -They cross over from one study arm to another and serve as their own controls

Familial Medullary Thyroid Cancer

-Subset of MEN2A only associated with medullary thyroid cancer (w/o pheochromocytoma or hyperparathyroidism)

Recall Bias

-Systematic error due to differences in accuracy or completeness of recall to memory of past events or experiences -Is a problem in case-control studies, esp when questionnaires are used to ask participants about distant past exposures

Observer Bias

-Systematic errors in observation or misclassification of data that occur because of an observer's expectations or differences in interpretation -A concern in single-blind studies (where individuals observing outcomes are aware of treatment assignments) and studies that rely on subjective outcomes

Tattoo Removal

-Techniques include dermabrasion, cryosurgery, thermal cautery, surgical resection, laser removal -Dermabrasion: removal of superficial layer of epidermis to allow pigment to leach out of skin -Laser removal: use of lasers of different wavelengths to break up pigments into smaller molecules that are taken up & cleared by macrophages in skin; can leave scar marks & cause skin hypo/hyperpigmentation

Ad Hoc Interpretation

-Temporary/informal interpretation by nonqualified individuals (e.g. bilingual or partially fluent family members or medical staff) not professionally trained in interpreter skills, ethics, & medical terminology -Generally not recommended d/t high error risk, but may be necessary in limited situations meeting conditions including: 1. Pt w/ urgent medical need that is ill-appearing, 2. Qualified interpreter services are not readily accessible (e.g. system is inoperable), 3. Delaying care to access interpreter services may result in preventable pt harm -If necessary given the situation, should select the most fluent staff/family member -Do not use minors <18 as interpreters unless true emergency

Hawthorne Effect

-Tendency of a study population to affect outcome b/c participants are aware that they are being studied -> behavioral changes -Commonly seen in studies studying behavioral outcomes -Alterations in behavior d/t participation awareness -> dramatized reporting by subjects on outcomes under study -> overestimation of effect of intervention, threat to internal validity of study

Causes of Hematuria

-Terminal hematuria may present w/ clots, no proteinuria or casts on UA -Total hematuria may present w/ other signs of glomerular disease (proteinuria, casts, etc.)

Transjugular Intrahepatic Portosystemic Shunt (TIPS)

-Therapy for portal hypertension that involves the placement of a stent between the portal veins and hepatic veins to reduce portal systemic pressure -Salvage therapy for pts w/ refractory ascites or varices that have failed endoscopic/medical management -Associated w/ portosystemic encephalopathy in up to 35% of patients

Terrible Ts of Anterior Mediastinal Masses

-Thymoma -Teratoma & other germ cell tumors -Terrible lymphoma -Thyroid neoplasm (e.g. ectopic thyroid, substernal goiter) Presentation: -Mass effect compression of other structures -> cough, SOB, postural wheezing (esp while lying down)

Causes of PSA Elevation

-Transient, mild elevation: urinary retention, mild acute prostate infection/inflammation, urologic procedure (e.g. cystoscopy), digital rectal exam, recent ejaculation; repeat PSA after 6-8 wks to document improvement -Persistent, significant elevation: BPH, prostate cancer, severe/chronic prostatitis

Healthy Worker Effect

-Type of selection bias in which working populations are generally healthier than the general population & exhibit lower mortality rates (results from severely ill or disabled individuals being excluded from or not entering the workforce) -May be seen in occupational cohort studies where general population is used as reference group & compared to a working population (e.g. analyzing the influence of vinyl chloride occupational exposure on cancer risk)

Pyramidal/Corticospinal Signs

-UMN signs including pronator drift (greater weakness in supinator > pronator muscles in UEs), focal weakness, spasticity, hyperreflexia, Babinski sign

Arterial Catheters

-Used for continuous BP monitoring & frequent arterial blood sampling -Radial artery most commonly used due to superficial location & collateral vasculature (ulnar artery) -Brachial artery in antecubital fossa may be used b/c higher reliability (lower occlusion rates), accuracy (closer correlation to central aortic pressure), if difficulty w/ radial artery cannulation Complications: -Median nerve injury (runs along brachial artery, may be lacerated during cannulation or compressed by hematoma or local swelling)

Inferior Vena Cava Filter

-Used for proximal DVT when there are contraindications to or complications from anticoag (e.g. significant active bleed, recent surgery, acute hemorrhagic stroke, bleeding diathesis) -Also placed if anticoag fails (e.g. pt develops new or recurrent/extending proximal DVT while on therapeutic anticoag) -Placed by transvenous approach to inhibit large thrombi in the LEs from embolizing into lungs -Preferable to place retrievable filter that is removed once pt has stabilized Complications: -Short term: bleeding/hematoma, infection, thrombus, arteriovenous fistula at puncture site, guidewire entrapment within filter during placement -Long term: recurrent DVTs (prevents PEs but not DVTs), filter migration/erosion into surrounding structures, recurrent thrombus (extending into IVC & around filter)

Transcutaneous Pacing

-Used in pts w/ symptomatic bradyarrhythmias and/or complete heart block

Criteria for Extubation

-pH >7.25 -Adequate oxygenation (e.g. PaO2 of 60+) on minimal support (FiO2 of 40% or less, PEEP of 5 or less) -Intact inspiratory effort & sufficient mental alertness to protect airway -If meets above, may undergo spontaneous breathing trial (SBP) in which pt remains intubated but ventilatory support is turned off -Candidacy for extubation may be assessed during SBP w/ rapid shallow breathing index (RSBI), calculated by dividing RR by TV (pts w/ high RSBI breath fast/shallow, likely to need continued ventilatory support)

Turner Syndrome

45,X Presentation: -US w/ nuchal thickening, cystic hygroma (fluid-filled lymphatic sac in neck), hydrops fetalis -Gonadal dysgenesis (small, nonfunctioning streak ovaries) -> short stature, minimal breast development, primary amenorrhea, shield chest -Horseshoe kidney (can cause oligohydramnios), aortic coarctation (systolic murmur at L infraclavicular area, upper extremity HTN), bicuspid aortic valve, mitral valve prolapse, hypoplastic heart -Lymphedema of hands and feet, broad chest w/ widely spaced nipples, webbed neck, cubitus valgus (angling of forearms away from body), nail dysplasia -Normal pubic/axillary hair b/c does not affect adrenals -Low estrogen -> increased risk of osteoporosis & fxs -Usually normal cognitive abilities, some increased risk of impaired nonverbal skills (e.g. mathematics), ADHD, problems w/ executive functioning -Congenital lymphedema at birth is usually nonpitting due to high protein content (vs. pitting in liver disease, nephrotic syndrome, congestive HF) -Astigmatism, far-sightedness, other vision/hearing abnormalities -Increased risk of autoimmune endocrinopathy (esp primary hypothyroidism) Diagnosis: -Karyotype, FSH & LH (both elevated), estrogen (low, as are inhibin and progesterone), FISH to detect mosaicism if karyotype normal and high suspicion of TS -If diagnosed, echocardiogram, visual/hearing assessment, renal US, TSH levels Treatment/Management: -Estrogen replacement therapy to induce sexual development, improve growth/height, prevent osteoporosis after pt is 14 yo (earlier may lead to premature fusion of epiphysis, which can decrease final height) -Growth hormone to improve height potential Pathophysiology: -Lymphatic network dysgenesis -> accumulation of protein-rich interstitial fluid in hands, feet, & neck -> congenital lymphedema

Parallel Study

A study where two or more groups receive different treatments and the outcomes are compared.

Cluster Analysis

A technique used to divide an information set into mutually exclusive groups such that the members of each group are as close together as possible to one another and the different groups are as far apart as possible

Neurofibromatosis Type 1

AD NF1 mutation, von Recklinghausen disease Presentation: CAFE SPOT mnemonic -Cafe au lait macules (flat, uniformly hyperpigmented, numerous and regularly contoured vs. McCune-Albright) -Axillary freckling (clustered, particularly axillary/inguinal regions), astrocytomas -Fibromas (neurofibromas--type of nerve sheath tumor that are nontender/pedunculated and can undergo malignant transformation) -Eye Lisch nodules (raised, tan hamartomas of iris) -Skeletal bowing, seizures, scoliosis -Pseudoarthrosis of tibia -Optic Tumor (optic glioma compression of hypothalamus can cause precocious puberty, usually presents during toddlerhood w/ headache, monocular eye proptosis, compression of optic nerve -> decreased visual acuity, alterations in color vision, optic nerve atrophy) -Other: brainstem gliomas Treatment/Management: -Annual skin exam -Brain/orbit MRI Pathophysiology: -Mutation in gene for neurofibromin (NF1 tumor suppressor gene) on ch17

Neurofibromatosis Type 2

AD NF2 mutation, central neurofibromatosis Presentation: -Bilateral vestibular schwannomas aka acoustic neuromas (sensorineural hearing loss, balance deficits), cataracts, cutaneous plaques and tumors, intracranial meningiomas, retinal hamartomas, spinal tumors (schwannomas, ependymomas), peripheral neuropathy -Hypopigmented cafe-au-lait spots -Variable expressivity, usually presents in 20s-30s Treatment/Management: -Surveillance w/ audiograms, ophthalmologic evals, MRI of spine/brain Pathophysiology: -Mutation in gene encoding merlin protein (NF2 tumor suppressor gene) on ch22 -Vertigo uncommon b/c slow growth of vestibular schwannoma allows for central compensation of gradual loss of input

Indications for Dialysis Mnemonic

AEIOU Acid base disorders Electrolytes Intoxications with drugs Overload of volume Uremia

HIV-Associated Dementia

AIDS dementia complex Presentation: -Almost exclusive to chronic untreated HIV pts w/ CD4 <200, often 50+ yo -Apathy, attention/concentration/memory impairment, subcortical motor sx (e.g. impaired psychomotor speed), subacute/chronic cognitive deficits, depressive sx, personality/behavior changes -Associated myelopathy can present w/ spastic paraparesis, urinary incontinence Diagnosis: -Brain MRI w/ diffuse brain atrophy, ventricular enlargement, reduced attenuation/increased intensity of white matter structures Pathophysiology: -HIV replication in CNS

Hepatojugular Reflux

Abdominojugular reflux -Application of firm & sustained pressure over upper abd for 10-15 s -> sustained elevation of JVP >3 cm during compression -Reflects a failing RV that cannot accommodate increase in venous return w/ abd compression -May be caused by constrictive pericarditis, RV infarction, restrictive CM

Hidradenitis Suppurativa

Acne inversa Presentation: -Typically starts w/ single solitary/painful inflamed nodule -Multiple, recurrent, painful inflammatory nodules/abscesses w/ purulent or serosanguineous drainage & chronic scarring in the intertriginous regions: axilla, groin (inguinal/perineal regions), scalp -Mild: painful nodules, draining abscesses -Moderate: sinus tracts, scarring -Severe: extensive sinus tracts, widespread disease -Risk factors: smoking, metabolic syndrome (obesity, T2DM), fhx of hidradenitis, mechanical stress on skin -Complications: depression, SCC Treatment: -SMOKING CESSATION, weight loss, daily skin cleansing of affected area -Mild: topical clindamycin, intralesional steroids or oral abx for flare-ups -Moderate: oral tetracyclines, oral clinda + rifampin in refractory cases -Severe: TNF-alpha inhibitors (e.g. adalilumab, infliximab), oral retinoids (e.g. acitretin), surgical excision Pathophysiology: -Disordered folliculopilosebaceous units (ductal keratinocyte proliferation -> follicular occlusion -> follicular rupture from mechanical friction in intertriginous areas -> inflammation, infection) -Nicotine, tobacco thought to stimulate follicular occlusion, have proinflammatory effects on neutrophils & T lymphocytes & keratinocytes (promote TNF-alpha production)

Cervicofacial Actinomyces

Actinomyces israelii infection Presentation: -Slowly progressive, nontender, indurated submandibular mass that can form abscess and draining sinus tracts w/ thick serous discharge w/ classic, purulent yellow sulfur granules -Most commonly affects mandible (>50%), but can also affect nearby skin of chin & cheek -Fever & lymphadenopathy uncommon -Can cause indolent pulmonary disease -Risk factors: dental procedures/infections/trauma (e.g. tooth extraction), immunosuppressed, T2DM, malnutrition Diagnosis: -FNA, culture >14 days Treatment: -High-dose PO penicillin for 2-6 mos for mild cases (no fistulas), IV penicillin & surgical resection for severe disease Pathophysiology: -Direct extension from oral cavity, oblivious to tissue planes

Pseudogout

Acute calcium pyrophosphate dihydrate (CPPD) crystal arthritis Presentation: -Acute mono- or oligoarticular arthritis, most commonly of peripheral joints (knees, ankles most common) -Most commonly presents >65 yo -Episodes may be precipitated by trauma/overuse, surgery, illness -Can also be associated w/ hyperparathyroidism, hemochromatosis, hypothyroidism, hypercalcemia Diagnosis: -Inflammatory effusion (15-30,000 cells/mm^3), CPPD crystals on aspiration (rhomboid shape, positive birefringence), CHONDROCALCINOSIS on imaging (signs of chronic calcification of articular cartilage, distinguishes from gout) Treatment/Management: -Intraarticular glucocorticoids, NSAIDs, colchicine

Primary Adrenal Insufficiency

Addison disease Presentation: -Chronic fatigue, weakness, weight loss, diffuse abd pain, anorexia, GI disturbance (nausea, vomiting, diarrhea), generalized/patchy skin hyperpigmentation (prominent in areas exposed to friction/light, e.g. elbows, knees, face, dorsum of hands, may also be associated w/ vitiligo), lymphoid hyperplasia (e.g. tonsil enlargement) -Hypoaldosteronism: hyponatremia, salt craving, postural hypotension -Hypocortisolemia: hypoglycemia, peripheral eosinophilia (normally inhibited by glucocorticoids) -If undiagnosed prior, can present w/ adrenal crisis during times of stress (e.g. surgery, injury, infection), w/ severe/unresponsive hypotension & shock (does not respond to volume resuscitation or pressors) & confusion -In females, can lead to hypoandrogenism/hypogonadism (low libido, loss of pubic hair), but not seen in men due b/c most androgens produced by testes -Labs: low cortisol & aldosterone, high ACTH, hyponatremia, hyperkalemia, hypoglycemia, eosinophilia, hyperchloremic metabolic acidosis -Risk factors: other autoimmune conditions (e.g. Hashimoto, vitiligo) Diagnosis: -8 am cortisol, plasma ACTH -ACTH analog (cosyntropin) stimulation testing; shows low cortisol & high ACTH in Addison disease (vs. low cortisol, low ACTH in secondary/tertiary adrenal insuff) Treatment/Management: -Glucocorticoid replacement (e.g. hydrocortisone, prednisone) -Mineralocorticoid replacement (fludrocortisone) Pathophysiology: -Autoimmune adrenalitis: destruction of all 3 layers of adrenal cortex bilaterally -> hypocortisolemia, hypoaldosteronism, hypoandrogenism -Other etiologies: infection (e.g. disseminated TB, histoplasmosis), malignancy, sarcoidosis -Elevated proopiomelanocortin (converted to ACTH + melanocyte-stimulating hormone) -> hyperpigmentation -Volume depletion due to aldosterone deficiency + cortisol def -> loss of vascular tone -> hypotension, dizziness, syncope, hyperkalemia, increased ADH secretion -> hyponatremia

Age-Related Sicca Syndrome

Age-related dry eye syndrome Presentation: -Dry eyes (sandy/gritty sensation), dry mouth, blurry vision, photophobia, can progress to corneal epithelial erosions (impaired vision) -Sx often worse at end of day -Risk factors: women, DM, thyroid disease, age Diagnosis: -Rule out Sjogren (anti-Ro, anti-La, ANA, RF), ophtho exam, Schirmer test Treatment/Management: -Artificial tears, ocular lubricants (e.g. methylcellulose drops), use of humidifier, avoidance of triggers (e.g. windy environments) -Cyclosporine (may increase tear secretion) 2nd-line -Salivary flow stimulation (mechanical/topical), artificial saliva for xerostomia Pathophysiology: -Decline in exocrine output from lacrimal/salivary glands w/ age, associated w/ atrophy, fibrosis, ductal dilation of glands -Other contributing factors: decreased blink rates, oxidative damage over time, excessive evaporation of tears, use of anticholinergic meds

Vasospastic Angina

Aka Prinzmetal/variant angina Presentation: -Young pt <50 yo w/ recurrent chest discomfort/pain, often occurs during sleep or during rest w/ spontaneous resolution within 15 min -Episodes may be associated w/ diaphoresis, N/V, palpitations, dyspnea -Risk factors: smoking, usually few risk factors for CAD Diagnosis: -Periodic ST elevation on ambulatory ECG -Coronary angiography shows no CAD Treatment/Management: -Smoking cessation -Calcium channel blockers (preventive, e.g. diltiazem), sublingual nitroglycerin (abortive) Pathophysiology: -Hyperreactivity of coronary intimal smooth muscle -> intermittent coronary artery vasospasm -> transient ischemia & angina -Imbalance in autonomic stimulation -Calcium channel blockers facilitates coronary artery dilation

Inferior Myocardial Infarction

Aka R-sided MI Presentation: -Hypotension (e.g. sudden-onset dizziness), bradycardia/bradyarrhythmias, other signs of MI but no pulm edema (usually no SOB, however may see pulm edema w/ severe bradycardia) -Epigastric pain & nausea vs. classic substernal chest pain -R-sided HF -> JVD, increased RV preload, decreased LV preload, increased SVR Diagnosis: -ECG w/ ST elevation in II, III, AVF -If involves posterior wall, may also see depression in V1-V3 -If involving RCA, may present w/ AV block -Right-sided precordial ECG w/ ST elevation in V4R Treatment: -Avoid preload reduction, aka NO NITROS, caution w/ beta blockers, CCBs, diuretics, opioids (all decreased RV preload/HR) -Reperfusion therapy, fluid resuscitation (IV NS bolus to increase RV preload, do not use if pulmonary edema secondary to sinus brady) -Other standard STEMI therapies: aspirin & antiplatelet, anticoag, statin -If sinus brady, IV atropine (if refractory, pacing) to increased CO; if AV block (e.g. anterolateral MI) unresponsive to atropine, transcutaneous/transvenous cardiac pacing -If persistent hypotension despite aggressive fluid resuscitation, inotropes (dopamine) Pathophysiology: -Presentation differs from L-sided MI b/c R side is highly sensitive to inadequate preload (thus no nitros, which can profoundly worsen hypotension) -Most commonly due to occlusion of the RCA > LCX (4:1) -RCA supplies the AV node, so occlusion can cause AV block -Increased vagal tone & decreased blood supply to SA node by RCA can also result in bradycardia

Ischemic Colitis

Aka acute colonic ischemia Presentation: -Moderate abd pain & tenderness (sudden, crampy, localized, lateral, mild compared to periumbilical/severe pain of acute mesenteric ischemia of small bowel), sudden bloody stools, diarrhea, tenesmus/fecal urgency -Rectal bleeding or bloody diarrhea within 24 hrs of sudden onset abd pain -Labs: leukocytosis, lactic acidosis -Risk factors: atherosclerosis, loss of collateral circulation, vessel manipulation during surgery Diagnosis: -Colonoscopy w/ pale mucosa w/ petechial bleeding, bluish hemorrhagic nodules, edematous/friable/cyanotic mucosa w/ hemorrhagic ulcers, sharp transitions b/t affected & unaffected regions -CT w/ colonic wall thickening (edema), fat stranding, possible pneumatosis (air in wall) -X-rays often normal (may show thumbprinting/submucosal edema, colonic distention) Treatment/Management: -IV fluids, bowel rest, abx w/ enteric coverage, colonic resection if necrosis/gangrene/perforation develop Pathophysiology: -Usually due to nonocclusive ischemia of watershed regions of colon (e.g. rectosigmoid junction, L transverse colon aka splenic flexure) -Often complicated by underlying atherosclerotic disease, states of hypotension/low blood volume (e.g. vascular surgery) -Splenic flexure = watershed b/t superior & inferior mesenteric arteries -Rectosigmoid junction = watershed b/t sigmoid & superior rectal arteries

Mixed Cryoglobulinemia Syndrome

Aka cryoglobulinemia types II or III Presentation: -Fatigue, arthralgias/arthritis, peripheral neuropathy (both sensory & motor deficits), weakness -Skin: nonblanching, palpable purpura, leukocytoclastic vasculitis -Renal: membranoproliferative GN (dysmorphic RBCs, RBC casts, protein), HTN, hematuria, proteinuria -Pulm: dyspnea, pleurisy -Labs: low C3/C4/C50, transaminases commonly elevated, positive RF Diagnosis: -Serological tests (assays for cryoglobulins/cryocrit, low C4 complement levels), skin/renal bx -Viral/ANA/RF autoantibodies Treatment: -Treat underlying disease (e.g. antiviral therapy) -Plasmapheresis, immunosuppression to stabilize end-organ damage (glucocorticoids, rituximab) if rapidly progressive, life-threatening Pathophysiology: -Chronic HCV, HBV, HIV, SLE -Lymphoproliferative disorders, other autoimmune/rheumatologic diseases (e.g. SLE, Sjogren) -Immune complex deposition (polyclonal IgG & IgM RF) in small to medium-sized vessels -> endothelial injury, end-organ damage -HCV complexes formed by HCV, anti-HCV IgG, IgM anti-IgG abs (rheumatoid factor), & complement

Anterior Uveitis

Aka iritis Presentation: -Moderate eye pain, conjunctival injection, limbus erythema, blurred vision/decreased acuity, hazy cornea, constricted/irregular pupil w/ poor light response (miosis), photophobia -Anterior chamber w/ flare, cells on slit lamp exam, hazy flare in aqueous humor, hypopyon, ciliary flush (scleral hyperemia at corneal margin) Diagnosis: -Slit lamp exam w/ leukocytes in anterior segment -Depending on suspected etiologies, HLA-B27 testing, pelvic/spinal X-ray, CXR, HIV, syphilis serology Treatment: -Dilating eyedrops (e.g. cyclopentolate), antimicrobials depending on etiology -Topical glucocorticoids Pathophysiology: -Inflammation of the anterior uveal tract, esp the iris -Etiologies: infection (e.g. herpesviruses, toxoplasmosis, syphilis), sarcoidosis, spondyloarthritis (e.g. ankylosing spondylitis, reactive arthritis, IBD-associated arthritis), traumatic iritis (blunt trauma)

Vestibular Neuritis

Aka labyrinthitis (when associated w/ unilat hearing loss) Presentation: -Acute, single vertigo episode that may last up to days -Often follows viral syndrome -Sensation of imbalance & unsteady gait -Peripheral vertigo rather than central (able to be suppressed by fixating on stationary target) -Abnormal head thrust test (pt asked to look at fixed object while head is rapidly rotated, pt unable to maintain normal fixation and eyes move and return to object w/ horizontal corrective saccade) -N/V, impaired gait, falling towards affected side -VN + unilateral hearing loss = labyrinthitis Treatment/Management: -Self-limited, so managed expectantly w/ vestibular suppressants (e.g. meclizine), corticosteroids, vestibular rehab Pathophysiology: -Self-limited disorder of vestibulocochlear nerve (viral or post-viral inflammation)

Upper Airway Cough Syndrome

Aka postnasal drip Presentation: -Chronic, nonproductive cough, often more frequently/severe at night -Sensation of dripping in back of throat, rhinorrhea -On exam, clear lungs & oropharyngeal cobblestoning Treatment/Management: -Oral first-gen antihistamine, combined antihistamine-decongestants (e.g. brompheniramine, pseudoephedrine)

Complex Regional Pain Syndrome

Aka reflex sympathetic dystrophy, algodystrophy, causalgia, Sudeck atrophy, transient osteoporosis, acute atrophy of bone Presentation: -Usually follows injury -Severe regional (but not dermatomal), burning/stinging pain, worsened by movement or light touch (allodynia, hyperesthesia) -Edema, abnormal sweating, vasomotor changes, altered skin temp/color, trophic skin/hair/nail changes, motor weakness, contractures -Triggers include surgery, trauma (e.g. fx, sprain), usually 4-6 wks after insult -Stage 1: burning pain, edema, vasomotor changes -Stage 2: progression of edema, skin thickening, muscle wasting -Stage 3: limited ROM & bone demineralization on X-ray Diagnosis: -Primarily clinical -X-ray w/ patchy demineralization -Bone scintigraphy w/ increased uptake in affected limb -autonomic testing to measure increased resting sweat output Treatment/Management: -PT/OT, exercise -Meds: NSAIDs, antineuropathic meds (e.g. pregabalin, TCAs), sometimes bisphosphonates, topical lidocaine/capsaicin -Treat comorbid conditions (e.g. MDD) -Regional sympathetic nerve block, IV regional anesthesia Pathophysiology: -May be due to inflammatory cytokines (exact etiology not known) -Injury causing increased sensitivity to sympathetic nerves

Trichorrhexis Nodosa

Aka split ends Presentation: -Fragility of hair w/ easy breaking of strands, fractured strands w/ splitting of hair -May be congenital or acquired (e.g. excessive heat, hair dyes, salt water

External Hordeolum

Aka stye/sty Presentation: -Abscess over upper or lower eyelid; localized/red/tender swelling at lid margin -After days, may develop into pustule that ruptures w/ purulent discharge & pain relief -May leave persistent granulomatous chalazion after rupture that regresses over mos Treatment: -Warm compresses Pathophysiology: -Most commonly due to S. aureus, may often be sterile -Acute inflammatory disorder of eyelash follicle or tear gland

Peptostreptococcus

Anaerobic gram positive cocci Presentation: -Rare cause of pulmonary infection w/ putrid sputum & abscess/empyema

Aromatase Inhibitors

Anastrozole, letrozole, exemestane Indications: -Treatment of postmenopausal estrogen-receptor positive breast cancer

Condyloma Acuminatum

Anogenital warts caused by low-risk HPV subtypes (usually 6 & 11) Presentation: -May be asymptomatic or pruritic (mild itching/burning), vary from papules to exophytic, cauliflower-like/verrucous/filiform/papilliform lesions that are white/gray/pink/skin-colored, may be friable -Lesions in internal/external vaginal, vulvar, anal regions, penile shaft -Lesions persist for long periods of time w/o other consequences/sx -Risk factors: immunosuppression, chronic tobacco use Treatment: -Pharm: trichloroacetic acid, topical imiquimod, podophyllin resin -Surgical: cryotherapy, laser therapy, excision -Prevention w/ vaccination & barrier protection

Ascariasis

Ascaris lumbricoides roundworm Presentation: -Fecal-oral transmission, usually from contaminated water, endemic to Asia, Africa, South America -Usually asymptomatic until advanced infection with large worm burden -Sx of SBO w/ abd pain and distention, HIGH-PITCHED BOWEL SOUNDS, obstipation, N/V -Obstruction of bowel & biliary tree can also cause biliary colic, cholangitis, acute pancreatitis -Pulm sx incuding cough, eosinophilic pneumonitis (rare, occur within first wks of infection) -Eosinophilia (one of first signs), malnutrition (anemia, vitamin def) Diagnosis: -Visualization of eggs/worms in stool or respiratory secretions Treatment: -SBO treatment supportive w/ nasogastric suction, fluid/electrolyte repletion -Albendazole, mebendazole Pathophysiology: -Ingestion contaminated water containing eggs -> hatch in colon -> penetrate colonic wall -> spread hematogenously to lungs -> mature in lungs then climb up respiratory tree -> coughed and swallowed -> mature into egg-laying adult worms in small intestine

Cholesterol Embolization

Atheroembolic disease, cholesterol crystal embolization Presentation: -Plaque embolization into small arteries following coronary angiography or other vascular procedure (or trauma) -> skin findings (livedo reticularis/mottled erythema that blanches, gangrene, cyanosis, ulcers), usually on lower extremities (e.g. blue toe syndrome w/ cyanotic toes w/ intact pulses), AKI (may take wks to resolve), pancreatitis, mesenteric ischemia, GI bleeding, stroke, amaurosis fugax -Fundoscopy w/ Hollenhorst plaques (bright yellow, refractile plaques in retinal artery, origin usually from carotid) -Can be delayed in presentation (usually within 30 days) -Risk factors: recent vascular procedure, advanced age, obesity, HLD, HTN, T2DM -Labs: AKI, eosinophilia, hypocomplementemia, UA typically benign but may show eosinophiluria Diagnosis: -Labs + renal bx (biconvex/needle-shaped clefts within occluded vessels, perivascular inflammation w/ eosinophils) Treatment/Management: -Supportive care (pain control, IV fluids, bowel rest), NPO except essential meds, continued statin therapy -Pancreatitis usually improves in 4-7 days w/ conservative management Pathophysiology: -During vascular procedure, atherosclerotic plaque is disrupted -> cholesterol crystals & debris showered into circulation -> partial or total occlusion of arterioles

Antiphospholipid Antibody Syndrome (APLS)

Autoimmune disorder caused by antiphospholipid antibodies that cause a hypercoagulable state leading to venous thrombosis (e.g. placental thrombosis during pregnancy) Presentation: -Arterial or venous thromboses (DVT, PE, stroke/TIA) -Pregnancy morbidity-3+ unexplained fetal losses <10 wks, 1+ unexplained fetal losses >10 wks, 1+ premature births <34 wks due to preE, eclampsia, or placental insufficiency -Mild, immune-mediated thrombocytopenia -Occurs in up to 40% of SLE pts, may occasionally occur w/o SLE in minority of healthy individuals for transient period (retest after 12 wks to confirm dx) Diagnosis: -Testing for lupus anticoagulant, anticardiolipin antibody, and anti-beta-2 glycoprotein antibody -ANA if no prior SLE diagnosis -Positive VDRL (false+ b/c test contains cardiolipin) -Lupus anticoagulant effect: prolonged activated PTT not reversed on plasma mixing studies (despite increased thrombotic risk, d/t presence of bound inhibiting ab) Treatment: -Chronic anticoagulation with warfarin in nonpregnant, low molecular weight heparin if pregnant -Nonhormonal contraceptive options (e.g. copper IUD, though not recommended if heavy menstrual bleeding or on anticoag), progestin-releasing IUD or implant, estrogen-containing contraceptives contraindicated -If concomitant SLE confirmed, add hydroxychloroquine

Cobalamin Deficiency

B12 Presentation: -Macrocytic/megaloblastic anemia, glossitis (pain/swelling of tongue, shiny/smooth appearance), neuropsychiatric sx (e.g. peripheral neuropathy w/ tingling, SCD, irritability, crying spells) -Platelets and WBCs normal or mildly decreased -Occurs in patients w/ restricted diets (e.g. limited meat intake) or GI pathology affecting B12 absorption (pernicious anemia, intestinal bacterial overgrowth, gastric surgery e.g. gastrectomy, autoimmune gastritis), chronic pancreatitis, may be precipitated by supplementing folic acid but not cobalamin, metformin (30% of pts within 5+ yrs of starting therapy) -Labs: low reticulocytes, high LDH, indirect hyperbili, low haptoglobin, low Hgb, high MCV, may also cause thrombocytopenia/leukopenia w/ hypersegmented PMNs Diagnosis: -Elevated methylmalonic acid (vs. folate def) Pathophysiology: -Stored in liver, takes yrs to develop -Deficiency results in defective myelin synthesis -Required cofactor for formation of thymidylate & purine molcs for DNA synthesis; deficiency causes ineffective erythropoiesis d/t delayed nuclear maturation -> decreased transition to mature RBC forms, increased immature megaloblasts in bone marrow -> increased intramedullary hemolysis of megaloblasts -> indirect hyperbilirubinemia, increased LDH c/w hemolysis -May be d/t loss of intrinsic factor needed for B12 absorption in causes including autoimmune gastritis, pernicious anemia, post-gastrectomy

Folate Deficiency

B9 Presentation: -Dyspnea, fatigue, pallor, weakness -May be associated w/ chronic hemolysis (e.g. SCD), poor dietary intake, malabsorption (e.g. gastric bypass), medications (e.g. methotrexate, phenytoin) -Vs. B12 def, no neuro manifestations -Labs: pancytopenia if severe Diagnosis: -Macrocytic anemia, low/norm reticulocytes, hypersegmented neutrophils on smear, low serum folate, elevated homocysteine but normal methylmalonic acid (vs. B12 def) Treatment: -Supplementation Pathophysiology: -Acquired from meats, leafy vegetables -Not stored like B12, easily becomes depleted within 5-6 wks, esp in alcoholics or w/ poor nutrition -Causes impaired DNA synthesis in cells w/ rapid turnover

Bacillary Angiomatosis

Bartonella henselae Presentation: -Vascular cutaneous lesions that begin as small reddish/purple papules -> friable pedunculated, nodular lesions -Systemic sx (fever, night sweats, fatigue, malaise) -Rarely organ involvement (hypodense lesions in liver, bone, CNS) -Risk factors: severe immunodeficiency (e.g. advanced HIV w/ CD4 <100), cat exposure, homelessness (e.g. lice exposure) Diagnosis: -Lesional bx w/ microscopy/histopathology Treatment: -Doxycycline or erythromycin -Initiation of antiretroviral therapy (usually 2-4 wks later) Pathophysiology: -Bartonella henselae/quintana: gram-, intracellular bacteria that usually establishes infection following cutaneous penetration after cat scratch or body/head lice bite -Causes local dermal infection -> spread to vascular endothelium -> attacks RBCs, causes endovascular/lymphatic/cutaneous manifestations

Blastomycosis

Blastomyces dermatiditis Presentation: -Great lakes & Canadian provinces, Mississippi & Ohio river basins (esp Wisconsin), Southern & upper Midwest states -Low-grade fever, night sweats, productive cough, weight loss, lytic bone lesions, ulcerated skin lesions (verrucous, heaped-up, crusted, well-circumscribed, wart-like, violaceous nodules that can evolve into microabscesses), osteomyelitis, prostatitis, epididymo-orchitis, meningitis, epidural/brain abscesses -CXR resembling TB or histoplasmosis, acute or chronic PNA -Incubation period of 3-6 mos -Disseminated disease may occur even in immunocompetent pts Diagnosis: -Culture (blood, sputum, tissue specimens) -Microscopy (broad based, single budding yeast) -Antigen testing (urine, blood) Treatment: -Mild disease in immunocompetent: may consider not treating -Mild to moderate pulm disease, mild disseminated disease: oral itraconazole -Severe pulm disease, moderately severe to severe disseminated disease, immunocompromised: IV amphotericin B Pathophysiology: -Primary infection via inhalation, associated w/ contact w/ soil or rotting wood

Aplastic Anemia

Bone marrow failure Presentation: -May present as autoimmune phenomenon, after infection (parvovirus B19, EBV, hepatitis), after exposure to drugs (carbamazepine, chloramphenicol, sulfonamides), after exposure to radiation/toxins (benzene, solvents, etc.) -Pancytopenia: anemia (fatigue, weakness, pallor, normocytic or macrocytic), thrombocytopenia (mucosal bleeding, easy bruising, petechiae), leukopenia (recurrent infections, fever) Diagnosis: -Biopsy w/ hypocellular bone marrow w/ fat and stromal cells -Peripheral smear shows decreased counts but normal morphology Treatment: -Identification and removal of offending agent, if relevant -Supportive care -If severe/refractory, may require immunosuppression w/ hematopoietic stem cell transplant Pathophysiology: -Bone marrow failure (usually idiopathic in kids) due to hematopoietic stem cell deficiency (CD34+)

Lyme Disease

Borrelia burgdorferi (spirochete transmitted by deer tick Ixodes scapularis) Presentation: -Early localized (days to 1 mo): erythema migrans ("bulls-eye" rash, may be pruritic, but not scaly, flat, usually on trunk, may start as confluent macule before developing central clearing), fatigue, fever, headache, myalgias, arthralgias, neck stiffness, HA -Early disseminated (wks to mos): multiple erythema migrans, unilateral/bilateral CN 7 Bells palsy, lymphocytic meningitis, radiculopathy, carditis (AV block), migratory arthralgias -Late (mos to yrs): arthritis (e.g. joint effusion, most commonly involving knee), encephalitis, peripheral neuropathy Diagnosis: -Synovial fluid analysis w/ leukocytes ~25,000, negative gram stain/culture -Detection of Lyme antibodies in serum (ELISA followed by Western blot) to confirm analysis; usually negative d/t developing humoral response in early disease (should empirically treat, do not test unless disseminated infection) -CSF w/ lymphocytic predominance (only perform LP if c/f meningitis) Treatment: -PO doxycycline (or amoxicillin or cefuroxime if pregnant or doxy contraindicated) for early localized/primary disease -Ceftriaxone for severe complications Pathophysiology: -Transmission of spirochete from Ixodes tick gut to salivary glands occurs after 36-48 hrs of attachment; tick attached <36 hrs unlikely to transmit disease -Low risk of transmission if tick found not engorged

Thromboangiitis Obliterans

Buerger disease Presentation: -Young male patient (usually <45) that smokes -Distal extremity ischemia, digital ulcers, digital gangrene, superficial thrombophlebitis -Usually normal distal pulses Pathophysiology: -Small & medium vessel vasculitis precipitated by smoking -Involves formation of inflammatory microthrombi

Renal Osteodystrophy

CKD-mineral bone disorder Presentation: -Bone pain, increased risk of pathologic fx -Radiographic evidence of widespread osteopenia, subperiosteal bone reabsorption, eventual progression to osteitis fibrosa cystica -Labs: hyperphosphatemia, hypocalcemia, elevated PTH, elevated alk phos (elevated PTH -> increased bone turnover) Treatment: -Low-phosphate diet (first-line), phosphate binders (e.g. calcium carbonate, calcium acetate, sevelamer, avoided b/c can cause vascular calcifications) -Vit D & calcium supplementation Pathophysiology: -Bone lesions, increased bone turnover due to secondary hyperparathyroidism due to chronic renal disease (vit D def from ESRD -> low phos/Ca -> increased PTH production, parathyroid hyperplasia -> secondary hyperparathyroidism -> increased bone resorption) -Can eventually lead to tertiary hyperparathyroidism (chronic parathyroid stimulation -> development of autonomously functioning adenomas -> very high PTH, hypercalcemia)

Calciphylaxis

Calcific uremic arteriolopathy Presentation: -Painful subcutaneous nodules & ulcers (violaceous/purpuric), can progress to ischemic/necrotic ulcers & become infected, cause sepsis -Most common on areas of adiposity (trunk, thighs), usually presents w/ intact peripheral pulses vs. atherosclerotic ischemia -Soft tissue calcification on imaging -Risk factors: ESRD (esp dialysis, kidney transplant), hypercalcemia, hyperphosphatemia, hyperparathyroidism, hypomagnesemia, obesity, DM, oral anticoag (e.g. warfarin), vit K def -Labs: high PTH, high phosphate, high/normal calcium Diagnosis: -Skin bx w/ arterial calcification/occlusion, subintimal fibrosis Pathophysiology: -Arteriolar & soft tissue calcification, local tissue ischemia & necrosis

SGLT2 Inhibitors

Canagliflozin, dapagliflozin, empagliflozin MOA: -Increased urinary glucose excretion by blocking sodium-glucose cotransporter in proximal tubule -> low insulin:glucagon ratio -Benefits: reduced progression of nephropathy/albuminuria, reduced CV morbidity/mortality (reduced cardiac remodeling, unknown mechanism), reduced hospitalizations for HF, weight loss Indications: -Add-on for treatment of T2DM in addition to lifestyle modification & metformin (esp if pt also has CV disease, e.g. CAD, HF, MI) -May be added for GDMT of HFrEF (esp if pt has comorbid T2DM) ADRs: -Euglycemic diabetic ketoacidosis (can be triggered by prolonged fasting, major illness, intense exercise, abrupt reduction in insulin dose, EtOH), increased risk of GU infections (UTI, candidiasis, Fournier gangrene), hypotension (from diuresis -> AKI), worsening of renal fxn, orthopedic complications (low-trauma fx, foot ulcers that may increase amputationi risk) Contraindications: -T1DM, h/o DKA, impaired renal fxn w/ eGFR <30

Cardioversion vs. Defibrillation

Cardioversion: in sync with QRS, used in tachyarrhythmias w/ clinical sx (hypotension, cardiogenic shock, signs of ischemia, acute HF), e.g. hemodynamically unstable AFib w/ RVR (w/ pulse), atrial flutter, monomorphic VT w/ a pulse, SVT Defibrillation: not in sync with QRS (high energy shock at random point in cardiac cycle), used in VFib and VT without a pulse

Clostridium Perfringens

Causes gas gangrene w/ myonecrosis, clostridial myonecrosis Presentation: -Penetrating wound -> signs of cellulitis, crepitus (gas gangrene) of soft tissues -Severe pain (vs. ecthyma gangrenosum painless), fever, purple bullae, signs of systemic toxicity (shock, multiorgan failure) Treatment/Management: -Usually requires extensive surgical debridement

DIAPPERS

Causes of urinary incontinence in elderly Delirium Infection (e.g. UTI) Atrophic urethritis/vaginitis, Alcohol Pharmaceuticals (e.g. alpha blockers, diuretics, anticholinergics, opiates, CCBs) Psychological (e.g. depression) Excessive urine output (e.g. T2DM, CHF) Restricted mobility (e.g. postsurgery) Stool impaction

Cavernous Hemangioma

Cavernous malformations Presentation: -Soft blue, compressible vascular masses that can grow up to a few cms in skin, mucosa, deep tissues, viscera -Lesions of brain and viscera associated w/ VHL Pathophysiology: -Dilated vascular spaces w/ thin-walled endothelial cells

Psittacosis

Chlamydia psittaci Presentation: -Fever, dry cough, HA, hx of exposure to birds

Eosinophilic Granulomatosis with Polyangiitis

Churg-Strauss disease Presentation: -Difficult to control asthma, allergic rhinitis w/ nasal polyps, chronic sinusitis, mononeuropathy multiplex -Skin findings: granulomas, palpable purpura Pathophysiology: -Uncommon autoimmune vasculitis

Amiodarone

Class III antiarrhythmic Indications: -Acute management of ventricular arrhythmias (wide complex), esp in pts w/ CAD, ischemic CM -Maintenance of sinus rhythm in pts w/ AFib ADRs: -Cardiac: sinus bradycardia, heart block, risk of proarrhythmias (QT prolongation, torsades) -Pulm: chronic interstitial pneumonitis (nonproductive cough, fever, dyspnea, reticular/ground glass pulm infiltrates, restrictive PFTs, decreased DLCO, pleuritic CP, weight loss), pulm fibrosis, organizing PNA, ARDS, diffuse alveolar hemorrhage, pulm nodules, masses -Endocrine: hypothyroidism, hyperthyroidism -GI/hepatic: elevated transaminases, hepatitis -Ocular: corneal microdeposits, optic neuropathy -Derm: blue-gray skin discoloration, photosensitivity -Neuro: peripheral neuropathy -Other: BM suppression

3rd Degree AV Block

Complete Heart Block Presentation: -Fatigue, lightheadedness, syncope Diagnosis: -ECG w/ complete dissociation of P waves & QRS complexes Treatment/Management: -Initially managed w/ atropine if hemodynamically unstable -> transcutaneous pacing if still unstable -> dopamine if still hypotensive -> transvenous pacing -Permanent pacemaker placement

Focal Seizure with Impaired Awareness

Complex partial seizure Presentation: -Focal seizure (originating in a single hemisphere) in which pt appears awake but does not interact appropriately w/ environment (e.g. blank staring) -Often engage in repetitive, stereotyped actions (aka automatisms), e.g. repeating words, gesturing, fumbling hand movements

Central Pontine Myelinolysis

Complication of overly rapid correction of hyponatremia (>0.5 mEq/L/hr) Presentation: -Locked-in syndrome: flaccid quadriplegia w/ intact consciousness, sparing of upper CNs (intact vertical EOM, blinking, pupil reflexes), paralysis of lower CNs (loss of horizontal EOMs, oral movements) -Preserved wakefulness (e.g. spontaneous eye opening) & awareness (blinking on command) -LMN & intrinsic muscle fxn remain intact (peripheral nerve conduction & EMG normal) -Loss of voluntary respirations -If delayed onset, paralysis may present several days after rapid sodium rise -Typically irreversible Diagnosis: -Brain MRI (may demonstrate demyelinating lesions) Treatment/Management: -Supportive care, eye-computer interface Pathophysiology: -Rapid correction of Na+ in setting of hyponatremia -> excess water movement out of neurons & glia into extracellular compartment -> disruption of cellular activity, osmotic shock -> cell damage from rapid cell shrinkage, apoptosis, demyelination, oligodendrocyte death -Central pontine gray matter most susceptible to damage d/t highest density of oligodendrocytes -Highest risk w/ severe hyponatremia (120 or lower) of significant duration (>2 days)

Primary Hyperaldosteronism

Conn syndrome Presentation: -Secondary HTN, muscle weakness, cramping, paresthesias d/t hypokalemia (esp w/ concurrent use of loop/thiazide diuretics), polyuria (may be d/t hypokalemia) -Labs: hypokalemia, hypernatremia, metabolic alkalosis Diagnosis: -Elevated plasma aldosterone, low plasma renin activity, plasma aldosterone/renin ratio of >20, aldosterone remains elevated following oral saline load (unable to be suppressed) -CT of adrenals, adrenal vein sampling if CT does not reveal discrete mass to differentiate b/t hyperplasia & adenoma Treatment: -If adrenal adenoma, adrenalectomy may be curative -If bilat adrenal hyperplasia, med management w/ aldosterone antagonists (spironolactone, eplerenone) Pathophysiology: -Bilat adrenal hyperplasia (~60%) or unilateral adrenal adenoma producing aldosterone (~40%) -Aldosterone escape: increased plasma volume -> increased secretion of natriuretic factors -> increased sodium excretion (counters initial hypernatremia)

Erythrasma

Corynebacterium minutissimum Presentation: -Superficial bacterial skin infection w/ red/brown patches in intertriginous areas -Flat, +/- fine scaling, mild pruritus Diagnosis: -Wood lamp revealing coral-red fluorescence caused by Corynebacterium porphyrins

Medicare Part A

Covers IP hospital services, skilled nursing facilities (limited), hospice & home health care

Medicare Part B

Covers OP physician services, preventive care, OP diagnostics (lab tests, X-ray), OP surgery, hospital observation services

Subacute Thyroiditis

De Quervain thyroiditis Presentation: -Painful/tender goiter following viral infection (e.g. URI), neck pain -Prominent fever & hyperthyroid sx for few wks followed by hypothyroid stage lasting mos -Most eventually recover to euthyroid state -Labs: elevated ESR & CRP, milk leukocytosis, mild anemia, mild transaminitis Diagnosis: -Suppressed TSH, elevated T4 during hyperthyroid stage -Low radioiodine uptake on scintigraphy Treatment: -Beta-blockers to treat thyrotoxicosis sx, NSAIDs for pain, glucocorticoids for severe pain refractory to NSAIDs -Antithyroid drugs like methimazole ineffective b/c thyrotoxicosis is d/t release of preformed thyroid hormone Pathophysiology: -Likely d/t postviral inflammatory process -> follicular injury -> release of thyroid hormone

Low-Grade Astrocytoma

Defined as grade I or II Presentation: -Vs. glioblastoma, longer duration of sx, usually presents w/ seizures -Contrast enhancement on imaging less likely than w/ glioblastoma Diagnosis: -On histology, have increased cellular proliferation & atypia, but lack necrosis/mitoses/neovascularity

Pseudodementia

Dementia syndrome of depression, depression-related cognitive impairment Presentation: -Cognitive changes in elderly pt associated w/ mood disturbance (e.g. depression), often reversible cognitive impairment -Anhedonia, hypersomnia, low energy, weight gain/loss, cognitive impairment/poor concentration -Episodic over wks to mos -Pts often more aware (more insight) of their cognitive deficits than pts w/ dementia -During cognitive assessment, have deficits in attention, concentration, memory, & executive fxn, may put forth poor effort during testing -Risk factor for developing major neurocognitive disorder (dementia, Alzheimers/vascular) Treatment: -Psychotherapy, pharmacotherapy -Often leads to complete resolution of sx

Anosognosia

Denial of disability Pathophysiology: -May occur w/ nondominant parietal lobe lesions

Inflammatory Myopathies

Dermatomyositis, polymyositis, inclusion body myositis Presentation: -Affects proximal muscles > distal muscles (e.g. difficulty climbing stairs, combing hair, getting into/out of high chair or car), mild muscle pain & tenderness to palpation, weakness, muscle atrophy in late disease -No sensory deficits or LMN signs b/c pathology involves musculature, not innervation -May also present w/ rash, inflammatory polyarthritis, ILD, Raynaud, dysphagia/regurgitation/aspiration, myocarditis -Dermatomyositis: above + rash w/ purple/dusky hue involving eyelids (heliotrope), forehead, neck, chest, hands; Gottron papules (violaceous rash on dorsum of fingers) -Risk factors: female, 40-50 yo -Labs: elevated ESR, CRP, muscle enzymes (CK, aldolase, AST, LDH), transaminitis Diagnosis: -Autoantibodies (ANA, anti-Jo-1, anti-Mi2) -Bx w/ endomysial infiltrate (macrophages, CD8+ lymphocytes), patchy necrosis, regeneration/fibrosis of muscle fibers -EPS studies including EMG may show fibrillation potentials in affected muscle groups only (vs. diffuse in ALS) Treatment/Management: -Initial remission induced w/ po glucocorticoids (e.g. prednisone) + glucocorticoid-sparing agent (methotrexate, azathioprine) to minimize long-term adverse effects of steroid treatment -Should also receive cancer screening b/c can be paraneoplastic -Pulm screening for ILD (CXR) Pathophysiology: -Inflammatory infiltrates cause myofiber necrosis -Dermatomyositis may be associated w/ certain solid tumors (e.g. cervical, ovarian, lung, gastric, pancreatic, breast adenocarcinoma)

Traumatic Lumbar Puncture

Diagnosis: -CSF findings w/ RBC count >6000 w/o xanthochromia (vs. SAH), WBC elevation in ratio of 1 WBC:750-1000 RBCs, elevated protein & glucose Pathophysiology: -Accidental damage of blood vessel during LP

Giant Cell Myocarditis

Diagnosis: -Echo w/ LV dilation w/ segmental and/or global LV systolic dysfxn Pathophysiology: -Rare form of idiopathic myocarditis thought to be mediated by autoimmune process

Prerenal Acute Kidney Injury

Diagnosis: -Increase in serum Cr (50% from baseline), decreased urine output, BUN:Cr of >20:1, FENa <1%, unremarkable or "bland" urine sediment -Improvement in UOP & Cr following IVF resuscitation (usually back to baseline within 72 hrs) Treatment/Management: -Restoration of renal perfusion Pathophysiology: -Due to decreased RBF (eg, hypotension) -> decreased GFR. Na+/H2O and urea retained by kidney in an attempt to conserve volume -> increased BUN/creatinine ratio (urea is reabsorbed, creatinine is not) and decreased FENa -May be due to decreased CO (e.g. shock), intravascular/true volume depletion, renal artery stenosis, decreased EABV (e.g. HF, cirrhosis, even if volume overloaded), displacement of intravascular volume (e.g. sepsis, pancreatitis), afferent arteriole constriction (e.g. NSAIDs)

Alpha Thalassemia Minor

Diagnosis: -Microcytic anemia (low MCV), normal RDW, normal RBC count, target & teardrop cells on smear, slightly increased reticulocyte count, mild/moderately reduced Hct -Normal iron studies (normal iron, ferritin, TIBC) -No response to iron supplementation -Normal Hgb electrophoresis

Beta Thalassemia Minor

Diagnosis: -Microcytic anemia (low MCV), normal RDW, normal/elevated RBC count, target & teardrop cells on smear, slightly increased reticulocyte count, mild/moderately reduced Hct -Normal iron studies (normal iron, ferritin, TIBC) -No response to iron supplementation -Abnormal Hgb electrophoresis w/ increased Hgb A2 Treatment: -No specific treatment, no improvement w/ supplementation

Asthma

Diagnosis: -Positive bronchodilator response (>12% increase in FEV1) indicating reversibility of obstruction, reduced FEV1/FVC ratio w/ normal/slightly elevated TLC & DLCO -Distinguished from COPD by reversibility of obstruction, increased/normal DLCO -Cough-variant asthma: cough & chest tightness triggered by exercise & forced expiration, primarily at night -Exacerbation: acute worsening of cough, wheezing, SOB, chest tightness -Severe findings: AMS, pulsus paradoxus, absent wheezing due to poor aeration, marked accessory muscle use -Risk factors: fhx of atopy (eczema, asthma, allergies), hx of other atopic conditions -Triggers: allergens, URI, exercise, aspirin, meds (e.g. beta-blockers -> bronchoconstriction) Treatment (Acute): -Supplemental O2 if SpO2 <90% -SABA (short acting beta-2 agonist): inhaled albuterol (bronchodilator), may be used continuously if severe or w/ repeated doses -Anticholinergics: inhaled ipratropium bromide, bronchodilator used w/ repeated dosing only in acute setting & in conjunction w/ albuterol -Glucocorticoids: prednisone, methylprednisolone, dexamethasone (oral or IV), have delayed anti-inflammatory effects (6 hrs), used for control of late-phase inflammation (discharge from ED w/ 1 wk course) -One-time infusion of Mg sulfate if severe & w/o improvement w/ above treatments in 1 hr -Possible addition of SC/IV terbutaline or epinephrine -If severe (e.g. impending resp failure indicated by elevated/normal PaCO2, absent wheezing, decreased breath sounds, decreased mental status, hypoxia w/ cyanosis, PEF <25% of baseline, marked tachycardia, diaphoresis, accessory muscle use w/ suprasternal retractions, pulsus paradoxus, etc.), intubation Treatment (Chronic): -Step 1: SABA prn -Step 2: add on low-dose inhaled corticosteroid -Step 3: add on daily LABA or switch to medium-dose ICS -Step 4: daily LABA + medium dose ICS -Step 5: daily LABA + high dose ICS, consider adding omalizumab for pts w/ allergies -Step 6: daily LABA + high dose ICS + oral steroid, consider adding omalizumab for pts w/ allergies Pathophysiology: -In acute exacerbation, trigger (e.g. allergen, URI, exercise) causes mast cell degranulation -> histamine & leukotriene release -> immediate bronchoconstriction, increased vascular permeability (bronchial edema) & mucus production -Late phase reaction also occurs hrs later due to continued mobilization of inflammatory cells including eosinophils -> recurrent bronchoconstriction & inflammation -Most common causative allergens: house dust mite, cat, dog, cockroach allergens

Cholinesterase Inhibitors

Donepezil, rivastigmine, galantamine ADRs: -N/V, diarrhea, bradycardia

Granuloma Inguinale

Donovanosis, Klebsiella granulomatis Presentation: -Extensive & progressive, painless, ulcerative, beefy red lesions w/o lymphadenopathy -Base may have granulation-like tissue -Seen primarily in India, Guyana, New Guinea Diagnosis: -Microscopy w/ deeply staining gram-negative intracytoplasmic cysts (Donovan bodies) Treatment: -Azithromycin

Dawn Phenomenon

Early morning glucose elevation produced by the release of growth hormone & cortisol, which decreases peripheral uptake of glucose resulting in elevated morning glucose levels. Results in elevated fasting glucose levels. Admin of insulin at a later time in day will coordinate insulin peak with the hormone release.

IBD-Associated Arthritis

Enteropathic arthritis Presentation: -Occurs in up to 45% of IBD pts, can involve axial (e.g. spine, SI) and/or peripheral (e.g. knee, MCP) joints -Spondyloarthritis/sacroiliitis presents w/ prolonged stiffness + low back/buttock pain that improves w/ activity, limited spine flexion on exam w/ tenderness to palpation over lower back Treatment/Management: -NSAIDs may be used but can worsen underlying bowel disease -Treatment of underlying IBD can improve sx (e.g. sulfasalazine)

Plateau Pressure

Equal to elastic pressure + PEEP Peak airway pressure = resistive pressure + plateau pressure Elastic pressure = product of lung elastance & volume of gas delivered (dependent on lung compliance/elastance)

Essential Thrombocytosis

Essential thrombocythemia, autologous/primary thrombocytosis Presentation: -Stroke, transient eye sx, other sx of thrombosis, hemorrhage, erythromelalgia (burning pain w/ erythema) -Labs: persistent elevation in plts >600,000 Diagnosis: -BMBx Pathophysiology: -Clonal megakaryocyte proliferation

Erythroderma

Exfoliative dermatitis Presentation: -Widespread, scaly skin eruption Pathophysiology: -May be drug-induced, idiopathic, or secondary to underlying dermatologic or systemic disease

Zollinger-Ellison Syndrome (ZES)

Gastrinoma Presentation: -20-50 yo, 80% cases sporadic, 20% associated w/ MEN1 -Multiple & refractory peptic ulcers (refractory to antacids), ulcers distal to duodenum (jejunal), chronic diarrhea & steatorrhea, burning epigastric pain, dyspepsia, reflux sx, abd pain, weight loss, frank GI bleeding Diagnosis: -Markedly elevated serum gastrin (>1000 pg/mL) in presence of normal gastric acid (pH <4) -If intermediate (100-1000 pg/mL), workup w/ secretin stimulation test -Endoscopy (shows thickened gastric folds, multiple ulcers), CT/MRI & somatostatin receptor scintigraphy for tumor localization -If tumor confirmed, pt should be screened for MEN1 w/ assays for PTH, serum calcium, prolactin Treatment/Management: -High dose PPIs, surgery (exlap & resection) considered in pts w/ sporadic gastrinoma & no evidence of metastatic disease Pathophysiology: -Gastrin-producing tumor in pancreas or duodenum -> uncontrolled gastrin production -> parietal cell hyperplasia -> excess gastric acid production -Excess gastric acid in small bowel can cause diarrhea/steatorrhea by inactivation of pancreatic enzymes & injury to mucosal brush border

Globus Sensation

Globus hysterica Presentation: -Functional disorder of esophagus characterized by sensation of foreign body in throat -Worse when swallowing saliva, frequently associated w/ anxiety

Streptococcus Bovis

Group D Strep, aka strep gallolyticus Presentation: -Bacteremia, strongly associated w/ colon cancer (~60% of cases) & IBD -May cause endocarditis Diagnosis: -Colonoscopy to screen for occult malignancy -LFTs, hepatitis serologies b/c damage to hepatic sinusoids can predispose to infection Pathophysiology: -Bacteria has adhesion molcs that bind to overexpressed surface ligands on cancer cells -> permits aggregation, entry into bloodstream

Chancroid

Haemophilus ducreyi infection Presentation: -Erythematous papules & pustules -> erosion into painful genital ulcers (large, deep, w/ gray/yellow/purulent exudate, well demarcated borders & soft, friable base) w/ painful, suppurative inguinal lymphadenitis (buboes) Diagnosis: -Microscopy w/ gram negative rods that clump in long parallel strands ("school of fish") Treatment: -Azithromycin, ceftriaxone Pathophysiology: -Gram- rod, leading cause of genital ulcers in sub-Saharan Africa, Southeast Asia, Latin America

Trichotillomania

Hair-pulling disorder Presentation: -Recurrent hair pulling resulting in hair loss -Repeated attempts to stop/decrease hair pulling -Not due to medical/dermatologic condition (e.g. alopecia areata, presents w/ total hair loss or patches that are circular, smooth, & completely devoid of hair) -Not due to another mental disorder (e.g. body dysmorphic disorder) -On exam, presents w/ irregular patches of hair loss, hair shafts of varying lengths, noninflammatory/nonscarring, no erythema (vs. tinea capitis), often affects side of dominant hand -Commonly affects front/sides of scalp, eyebrows, eyelids -May attempt to hide hair loss (e.g. wigs, hats) -Prevalence of 1-2%, more common in children & girls -May be associated w/ trichophagia (eating hair) -> trichobezoars that can lead to abd pain, bowel obstruction Treatment: -CBT

Allergic Rhinitis

Hay fever Presentation: -Rhinorrhea, nasal congestion, sneezing, nasal itching, cough secondary to postnasal drip, ocular itching & tearing -Early age of onset common (<20), commonly associated w/ other allergic disorders (e.g. eczema, asthma, eustachian tube dysfxn) -Identifiable allergen or seasonal pattern -Pale/bluish/enlarged turbinates, pharyngeal cobblestoning, conjunctival edema, thick/green nasal discharge, "allergic shiners" (infraorbital edema & darkening), "allergic salute" (transverse nasal crease), "allergic facies" (high-arched palate, open-mouth breathing) Diagnosis: -If not responsive to treatment, allergy skin testing or serum radioallergosorbent testing to identify allergen triggers Treatment/Management: -Allergen avoidance, intranasal corticosteroids (e.g. fluticasone, mometasone) or antihistamines (e.g. azelastine) -Less effective: oral antihistamines, cromolyn nasal sprays, leukotriene modifiers (e.g. montelukast)

Shingles

Herpes zoster Presentation: -Burning, itching, tingling, localized pain & hyperesthesia in dermatomal pattern followed by development of vesicular rash (up to days after, grouped papules/vesicles on erythematous base, progresses to ulceration/crusting 7-10 days later) -Risk factors: immunosuppression, age >50 yo, severe physical stress -Complications: post-herpetic neuralgia (>4 mos after) Diagnosis: -Usually clinical -Direct fluorescent antibody testing Treatment/Management: -Antiviral therapy (acyclovir, valacyclovir, famciclovir) to shorten duration of sx & reduce risk of post-herpetic neuralgia, ideally within 72 hrs, for at least 7 days (oral preferred unless immunocompromised) -If localized, standard precautions & lesion covering -If disseminated, admission w/ IV acyclovir & contact/airborne precautions Pathophysiology: -Reactivation of herpes zoster virus in sensory dorsal root ganglion following prior chickenpox infection -> spreads to form rash in dermatomal distribution of ganglion -Infectious by direct contact w/ shedding lesions (not yet crusted), very rarely infectious by aerosolization; prevent spread by covering rash to prevent direct contact

Histoplasmosis

Histoplasma capsulatum Presentation: -Usually asymptomatic, disseminated form usually seen in immunocompromised (CD4 <100), presents 2-4 wks after exposure -Systemic: subacute febrile wasting disorder w/ chills, malaise, weight loss, cachexia, HA, myalgias -Pulm: dry cough, dyspnea, CXR w/ reticulonodular opacities or interstitial/miliary infiltrates -Hepatosplenomegaly, lymphadenopathy, mucocutaneous & skin lesions (crusted papules, nodules, erythema nodosum) -Labs: pancytopenia (from bone marrow infiltration), transaminitis, increased LDH & ferritin Diagnosis: -Urine/serum Histoplasma antigen, serology, fungal blood cultures (takes 4-6 wks) -Tissue bx w/ caseating or noncaseating granulomas w/ narrow-based budding yeast Treatment: -Amphotericin B for moderate/severe disease and/or HIV positive, itraconazole for mild disease & maintenance -Usually resolves completely over wks w/o intervention Pathophysiology: -Endemic to midwest & central US (Ohio, Mississippi River Valleys), temperate regions worldwide, most common endemic mycosis in US -AIDS-defining illness in regions where endemic (risk of disseminated infection d/t inability of macrophages to destroy H. capsulatum in phagolysosomes) -Spread via soil contaminated by bird/bat droppings (chicken coops, farm buildings, bird roosts, caves) -Seen in pts w/ high levels of exposure (<5% of infected immunocompetent pts develop sx) or immunodeficiency -Organism targets histiocytes and reticuloendothelial system -> lymphadenopathy, pancytopenia, hepatosplenomegaly

Cushing Syndrome

Hypercortisolism Presentation: -Truncal obesity, "buffalo hump" fat pad, increased libido, virilization/hirsutism, irregular menses, skin atrophy & purple striae, skin hyperpigmentation if due to ACTH excess, HTN, glucose intolerance, depression, anxiety/mood swings, facial plethora, muscle weakness/wasting, ecchymoses, osteopenia/osteoporosis -Labs: hyperglycemia, hypernatremia, hypokalemia, metabolic alkalosis Diagnosis: -1. Confirm hypercortisolemia w/ at least 2/3 of: low-dose dexamethasone suppression test, 24-hr urinary cortisol excretion, late-night salivary cortisol assay -2. After above, test ACTH to determine if cause if ACTH-dependent or independent -If ACTH-dependent, should undergo high-dose dexamethasone suppression test to determine if ACTH production is pituitary or ectopic -Inferior petrosal sinus sampling may be used to differentiate source of ACTH production (pituitary vs. ectopic) Treatment: -Metyrapone used to inhibit cortisol production until definitive surgical treatment Pathophysiology: -Most commonly due to exogenous administration of glucocorticoids, can also be caused by ACTH-producing pituitary tumor (Cushing disease), ectopic ACTH production (e.g. small cell lung cancer, bronchial carcinoid, medullary thyroid carcinoma), primary adrenal disease (adrenal adenoma) -ACTH-dependent causes: Cushing disease, ectopic ACTH production, involve bilateral hyperplasia involving zona fasciculata & reticularis in adrenal cortex, present w/ skin hyperpigmentation d/t excess MSH (ACTH & MSH both derived from POMC, both bind MSH receptors), androgenic sx d/t ACTH stimulation of zona reticularis -ACTH-independent causes: adrenal adenoma/carcinoma, if benign usually well-circumscribed w/ similarity to zona fasciculata, if malignant usually irregular/poorly demarcated, associated w/ atrophy of zona fasciculata & reticularis in uninvolved cortical regions

St. John's Wort

Hypericum perforatum -OTC herbal supplement used for antidepressant, anti-inflammatory, wound-healing properties -In studies, may have efficacy in treatment of mild to moderate depression equivalent to TCAs & SSRIs ADRs: -Poorly regulated in US, high potential for drug interactions d/t induction of CYP450 isozymes (1A2, 2C19, 2C9, 3A4) & P-glycoprotein systems -Drug interactions can lead to treatment failure w/ hormonal contraceptives, anti-retrovirals, immunosuppressives, narcotics, anticoagulants, antifungals -Risk for serotonin syndrome when combined w/ other serotonergic antidepressants -Hypertensive crisis

Thyrotoxicosis

Hyperthyroidism Presentation: -Weight loss, tachycardia, lid retraction, tremor, weight loss, insomnia, palpitations, heat intolerance -CV: sinus tach, PACs & PVCs, AFib/AFlutter, high-output HF or decompensation of existing HF, angina (due to coronary vasopasm), systolic HTN, widened pulse pressure Treatment: -Beta blockers (propanolol, atenolol) to control HR & hyperandrenergic sx, continued until treated adequately w/ thionamides, radiotherapy, surgery, etc. Pathophysiology: -Thyroid hormone increases sensitivity to circulating catecholamines, has inotropic & chronotropic effects leading to hyperdynamic CV state -Also causes increased myocardial oxygen demand due to increased cardiac output & increased systemic O2 consumption; can manifest as angina esp if pt already has predisposing factors like atherosclerosis

Secondary Hyperparathyroidism

Hypocalcemia of chronic kidney disease Presentation: -Labs: hypocalcemia, hyperphosphatemia, high PTH Pathophysiology: -Chronic kidney disease -> decreased production of active vit D -> hypocalcemia -> increased PTH -Chronic kidney disease -> impaired excretion of phosphate -> hyperphosphatemia, + high PTH -> further hyperphosphatemia -> phosphate binds calcium to form complexes in bloodstream -> worsening of hypocalcemia -Can also be mediated by low vit D (associated w/ low-normal phosphate, normal serum calcium)

Tetanus Prophylaxis

If clean/minor wound: -Booster tetanus toxoid-containing vaccine only if last dose 10+ yrs ago and 3+ doses in past/childhood, no tetanus immunoglobulin -Tetanus toxoid-containing vaccine & no tetanus immunoglobulin if unimmunized, uncertain, or <3 toxoid vaccine doses in past/childhood If dirty/severe wound: -Booster tetanus toxoid-containing vaccine if last dose 5+ yrs ago & 3+ doses in past/childhood, no tetanus immunoglobulin -Tetanus toxoid-containing vaccine & tetanus immunoglobulin if unimmunized, uncertain, or <3 tetanus toxoid doses in past/childhood

Treatment of Agitation

In elderly: -1st line is low-dose haloperidol -2nd lines include atypical antipsychotics (quetiapine, risperidone) -Only to be used in acute setting (e.g. delirium/agitation that compromises medical safety), prolonged use can be associated w/ increased mortality -Benzos not used in elderly d/t risk of worsening agitation (paradoxic effect), increased risk for adverse events (e.g. withdrawal, dependence, motor impairment) -Typical antipsychotics should not be used in Lewy body dementia (can cause neuroleptic hypersensitivity -> severe parkinsonism & impaired consciousness w/ administration)

Observational Biases

Inaccurate measurement or classification of disease, exposure, or other variable -Recall bias: common in retrospective studies, subjects w/ negative outcomes are more likely to report certain exposures than control subjects -Observer bias: observers misclassify data due to individual differences in interpretation or preconceived expectations regarding study -Reporting bias: subjects over or under-report exposure history due to perceived social stigmatization -Surveillance/detection bias: risk factors itself causes increased monitoring in exposed group relative to unexposed group, which increases probability of identifying diseases

Selection Biases

Inappropriate selection or poor retention of study subjects -Ascertainment/sampling bias: study population differs from target population due to nonrandom selection methods (results from atypical population are extrapolated to general population) -Nonresponse bias: high nonresponse rate to questionnaires/surveys can cause errors if nonresponders differ in some way from responders -Berkson bias: disease studied using only hospital-based patients may lead to results not applicable to target population -Prevalence/Neyman bias: exposures that happen long before disease assessment can cause study to miss diseased patients that die early or recover, occurs when an exposure of interest is a prognostic factor that impacts overall survival (usually a rapidly fatal disease, e.g. study of MI in pts snow shoveling will miss pts that die at home) -Attrition bias: significant loss of study participants may cause bias if those lost to follow up differ significantly from remaining subjects

TNF inhibitors

Include adalilumab, certolizumab, infliximab, golilumab, etanercept ADRs: -Infection (e.g. reactivation of TB, test w/ IFN-gamma assay), demyelination, CHF (pre-treatment Echo), malignancy

Androgen Abuse

Include anabolic steroids such as testosterone, trenbolone, boldenone, stanozolol, nandrolone Presentation: -Men: decreased testicular volume & sperm production, gynecomastia, acne, aggressive behavior, increased muscle mass, normal libido & erectile fxn during use but decreased libido & impotence during withdrawal -Women: acne, hirsutism, irreversible voice deepening, menstrual irregularities, clitoromegaly, bitemporal hair loss, ovarian dysfxn -Msk: increased muscle mass -CV: LVH, decreased HDL & increased LDL -Psych: mood disturbance, increased aggression -Heme: polycythemia, hypercoagulability -Include use of exogenous steroids (e.g. testosterone replacement therapy), synthetic steroids (e.g. stanozolol, nandrolone), androgen precursors (e.g. DHEA), estrogen blockers, gonadotropins -Labs: erythrocytosis (high Hct), hepatotoxicity (e.g. stanozolol), dyslipidemia (decreased HDL), low testosterone/FSH/LH Pathophysiology: -Testosterone levels usually recover in wks to mos after discontinuation, but chronic abuse can lead to prolonged & even permanent suppression of endogenous testosterone production

Progesterone Analogs

Include megestrol acetate, medroxyprogesterone acetate Indications: -Used in palliation of anorexia, to promote weight gain in pts w/ cancer-related anorexia/cachexia syndrome

Alcohol Toxicity

Includes: EtOH, isopropanol (solvents, mouthwashes, rubbing alcohol), methanol (windshield wiper fluid), ethylene glycol (antifreeze) Presentation: -Intoxication, CNS depression, may be comatose, EtOH considered sedative-hypnotic and may cause bradycardia, hypotension, respiratory depression, hyporeflexia, renal failure, anion gap metabolic acidosis (lactic/ketotic acidosis) -> Kussmaul respirations, epigastric pain -Methanol may present w/ papilledema/vision loss/blurring/scotomata/afferent pupillary defect (damage by formic acid) -Ethylene glycol may present w/ hematuria, oliguria, flank pain, CN palsies, tetany, calcium oxalate crystals in urine, nystagmus, slurred speech, ataxia, pulmonary edema -Isopropanol may present w/ CNS depression, dysconjugate gaze, absent ciliary reflex, no increased anion gap or metabolic acidosis Treatment: -Isopropanol treated w/ supportive care and rarely with hemodialysis -Methanol and ethylene glycol treated with displacement via EtOH or fomepizole (first choice, simultaneous use w/ EtOH not recommended), hemodialysis to remove toxin, sometimes bicarbonate and glucose; EtOH treated with thiamine Pathophysiology: -Glycolic acid from ethylene glycol injures renal tubules, oxalic acid binds calcium -> hypocalcemia, calcium oxalate crystal deposition

Cholinergic Toxidrome

Includes: organophosphates (suicide attempt, farm worker, pediatric ingestion), insecticides (e.g. carbamate), bioterrorism weapons (nerve agent, suspect w/ mult pts presenting w/ similar sx) Presentation: -Muscarinic—SLUDGE; salivation, lacrimation, urination, diarrhea/defecation, GI upset, emesis; others include miosis (pinpoint pupils), diaphoresis, rhinorrhea, bradycardia -Nicotinic—muscle weakness, paralysis, HTN, fasciculations -CNS—seen with organophosphates, includes anxiety, confusion, psychosis, seizures -Also DUMBBELLS—diarrhea/diaphoresis, urination/incontinence, miosis/muscle weakness, bronchorrhea/bronchospasm, bradycardia, emesis, lacrimation, salivation/sweating -Agent may be associated w/ garlic-like odor (petroleum base) Treatment/Management: -Remove clothing & irrigate skin -Early intubation to prevent airway compromise, atropine (reverse muscarinic sx) pralidoxime (displaces organophosphates from cholinesterase, reverses muscarinic & nicotininc sx including neurotoxic sx, administer after atropine), benzos for seizures, rarely decontamination by whole bowel irrigation Pathophysiology: -Acetylcholinesterase inhibition -> excessive action of acetylcholine at synapse

Endocarditis Prophylaxis

Indicated for high-risk cardiac conditions incl: -Prosthetic heart valve, previous infective endocarditis, structural valve abnormality in transplant heart, certain congenital heart disease subtypes (unrepaired cyanotic CHD, repaired CHD w/ residual defect, repaired CHD w/ use of prosthetic material in preceding 6 mos) Indicated for certain procedures: -Gingival manipulation or respiratory tract incision (Strep viridans group coverage w/ amoxicillin) -GU or GI tract procedure in setting of active infection (Enterococcus coverage w/ ampicillin) -Surgery on infected skin/muscle (Staph coverage w/ vancomycin) -Surgical placement of prosthetic cardiac material (Staph coverage w/ vancomycin)

Coronary Revascularization

Indicated for: -Pts w/ refractory angina despite max medical therapy -Pts in whom revascularization will improve long-term survival, including those w/ L main coronary stenosis & multivessel CAD (esp involving proximal LAD) w/ LV systolic dysfxn Options: -CABG superior to PCI w/ drug-eluting stents d/t lower rate of all-cause mortality & MI, lower rates of repeat revascularization -PCI w/ bare metal or drug-eluting stents may be options for pts w/ refractory angina d/t severe single or 2-vessel CAD not involving proimal LAD -CABG most indicated w/ multivessel CAD (esp involving prox LAD) & LV dysfxn

Ranolazine

Indications: -Antianginal agent effective in reducing frequency/severity of anginal sx in pts w/ chronic exertional angina refractory to conventional medical therapy

Pylephlebitis

Infective, suppurative portal vein thrombosis Presentation: -May present as complication of intraabd or pelvic infection (e.g. appendicitis, diverticulitis) -Acute/chronic fever, RUQ pain, jaundice, hepatomegaly -Labs: elevated alk phos & GGT -Complications: bowel ischemia, portal HTN, hepatic abscess Diagnosis: -CT or US revealing thrombus Treatment/Management: -Prolonged broad-spectrum abx Pathophysiology: -Portal venous system drains majority of GI tract; GI infection can lead to localized thrombophlebitis that can extend to portal vein Polymicrobial bacteremia common

Von Willebrand Disease

Inherited deficiency of vWF Presentation: -Easy bruising, mucocutaneous bleeding (e.g. epistaxis, heavy menstrual bleeding), anemia -Age of sx onset depends on disease severity -Normal CBC (platelet count) and coag studies (normal PT, possible prolonged PTT & bleeding time but may be normal in mild pts or pts w/ increased vWF production due to pregnancy, OCP use, acute stress, thyroid hormone supplementation) Diagnosis: -CBC, coag studies, vWF-specific testing (vWF antigen, ristocetin cofactor activity, factor VIII levels) -PTT sometimes prolonged, corrects w/ mixing study Treatment: -Rarely needed outside of surgery/trauma, includes desmopressin (ADH analog), vWF replacement therapy, antifibrinolytics (e.g. tranexamic acid) Pathophysiology: -Impaired platelet adhesion (vWF acts as bridging glycoprotein b/t platelets and endothelial factors at sites of vascular injury, also acts as carrier for factor VIII to prevent degradation by protein C)

Induction Therapy

Initial dose of treatment to rapidly kill tumor cells & send patient into remission (<5% tumor burden)

Common Peroneal Neuropathy

Innervation: -Motor: foot eversion, dorsiflexion, toe extension -Sensory: posterolateral leg, dorsolateral foot Presentation: -Unilateral foot drop, numbness/tingling over dorsal foot & lateral shin, impaired ankle dorsiflexion (walking on heels), preserved plantar flexion & reflexes (walking on toes) -Typically caused by fx or compression at proximal fibula -Sx may be transient following compression (e.g. hrs in duration), may be precipitated by leg immobility, crossing legs, protracted squatting Diagnosis: -EMG, nerve conduction studies Treatment: -Reducing pressure on nerve (e.g. avoid crossing legs), ankle-foot orthosis splint, PT

Chronic Mesenteric Ischemia

Intestinal angina Presentation: -Recurrent episodes of cramping postprandial pain, occult blood in stool, weight loss due to food aversion -Risk factors: smoking, hyperlipidemia/atherosclerosis, hypotension Diagnosis: -Contrast angiography (CT or MR), duplex US Treatment: -Mesenteric angioplasty and/or stenting Pathophysiology: -Usually affect watershed areas (splenic flexure, rectosigmoid colonic junction) -Due to large number of collateral vessels supplying bowel, multiple high grade stenoses typically needed in mesenteric vasculature before pts become symptomatic

Differentiating Microcytic Anemias

Iron Deficiency Anemia vs. Thalassemia vs. Anemia of Chronic Disease -IDA associated w/ decreased erythrocyte count, thalassemias have normal erythrocyte count -IDA associated w/ increased RDW, thalassemias have normal RDW -IDA often associated w/ reactive thrombocytosis (elevated platelets in response to low blood count b/c megakaryocytes & erythrocytes share common progenitor cell), thalassemia platelet counts normal -Ferritin decreased in IDA, normal/slightly elevated in thal due to high RBC turnover, elevated in anemia of chronic disease (due to chronic inflammation, acts as acute phase reactant) -If favor IDA, order iron studies; if favor thalassemia, order Hgb electrophoresis

Ventricular Fibrillation

Irregular contractions of the ventricles; may be fatal unless reversed Presentation: -Most frequent underlying arrhythmia responsible for sudden cardiac arrest in setting of acute MI -Syncope, lightheadedness, CP, palpitations Treatment: -If w/ pulse, CPR & cardioversion -If w/o pulse, CPR & defibrillation Pathophysiology: -In setting of MI, if occurring within 10 min of occlusion, often d/t heterogeneity of conduction w/ areas of marked conduction slowing & delayed activation that predisposes to ventricular reentrant arrhythmias like VFib & VTach (phase 1a/immediate periinfarction ventricular arrhythmias) -In setting of MI, if occurs 10-60 min following occlusion, usually d/t abnormal automaticity (phase 1b/delayed periinfarction ventricular arrhythmias)

Progressive Multifocal Leukoencephalopathy (PML)

JC virus infection of oligodendrocytes (white matter) Presentation: -Focal neuro deficits, often in untreated HIV or immunosuppressed (e.g. transplant) pt w/ CD4 <200 -Subacute onset of altered mentation (e.g. confusion), multifocal neuro sx (e.g. visual sx like diplopia, hemiparesis/monoparesis, ataxia, seizures) -Often fatal, even w/ treatment, often have irreversible neurologic sequelae Diagnosis: -Brain CT w/ multiple asymmetric/nonenhancing/hypodense white matter lesions -Brain MRI w/ asymmetric, multifocal white matter demyelination w/ no mass effect (no edema/enhancement) -LP w/ CSF PCR + for JC virus -Brain bx (rarely needed) Treatment: -If HIV+, initiate antiretroviral therapy Pathophysiology: -Reactivation of JC virus that is usually initially acquired in childhood, lies dormant in kidneys & lymphoid tissue -Reactivated virus spreads to CNS & lyses oligodendrocytes, causing white matter demyelination

Lymphogranuloma Venereum

L1-L3 subtypes of Chlamydia trachomatis Presentation: -Painless, small, shallow genital ulcers (often missed) w/ painful, fluctuant inguinal lymphadenitis -Initially presents as small, painless papules/pustules that ulcerate -> painful, swollen lymph nodes that coalesce to form buboes, which can rupture -Damage can eventually cause scarring & strictures in inguinal region -Not a recurring condition like hidradenitis suppurativa or HSV Diagnosis: -Microscopy w/ intracytoplasmic chlamydial inclusion bodies in epithelial cells & leukocytes Treatment: -Doxycycline

Legionella Pneumonia

Legionnaire's disease Presentation: -Atypical lobar PNA, rust-colored sputum, GI sx (esp diarrhea, abd pain, hepatitis), high fever (usually >39C/102F), neurologic sx (e.g. confusion), bradycardia relative to high fever -Risk factors: smoking, exposure to possibly contaminated water (recent travel, esp cruise or hotel stay within previous 2 wks, contaminated potable water in hospitals/nursing homes) -Labs: hyponatremia, hepatic dysfxn (elevated transaminases), hematuria, proteinuria, sputum gram stain w/ neutrophils but few/no organisms Diagnosis: -Urine antigen testing -Can be made from sputum culture (usually from bronchoscopy), usually difficult to recover even when selective media used (e.g. buffered charcoal yeast extract) Treatment: -Macrolides (azithromycin), FQs second-line, not responsive to beta-lactam & AG abx Pathophysiology: -Intracellular gram- rod -In water sources, lives inside protozoa & is transmitted to humans via droplets or aerosols (e.g. air conditioning, showers)

Transudative Pleural Effusion

Light Criteria: -Does not meet criteria for exudative effusion Presentation: -SOB, focally decreased breath sounds, dullness to percussion, CXR w/ pleural effusion -Labs: pleural fluid w/ normal glucose (60+), pH 7.4-7.55 Pathophysiology: -Increased hydrostatic (e.g. CHF) or decreased plasma oncotic pressure (e.g. hypoalbuminemia), decreased intrapleural pressure (e.g. atelectasis) -Etiologies: cirrhosis (hepatic hydrothorax), nephrotic syndrome, HF, constrictive pericarditis, dilated cardiomyopathy

Extranodal Marginal Zone Lymphoma

Low-grade B cell lymphoma of mucosa-associated lymphoid tissue (MALT) of the stomach Presentation: -Stomach mass, dyspepsia, epigastric pain improved w/ PPIs Diagnosis: -Upper endoscopy, bx shows lymphocyte proliferation in gastric lamina -H. pylori testing Treatment: -If H. pylori identified (~90% of cases), quadruple therapy (eradication therapy) w/ bismuth/PPI/metronidazole/tetracycline (alternatively triple therapy w/ PPI/clarithromycin/amoxicillin); usually sufficient for complete remission -If H. pylori negative, radiation, immunotherapy (e.g. rituximab), or single-agent chemotherapy Pathophysiology: -H. pylori infection -> chronic inflammation -> stimulates proliferation of antigen-dependent B & T cells in gastric lamina -> chronic activation leads to monoclonal B cell population that no longer depends on normal stimulatory pathways for growth

Phenytoin

MOA: -Inhibits voltage-gated sodium channels Indications: -Narrow-spectrum antiepileptic ADRs: -First manifests w/ signs of cerebellar dysfxn (horizontal nystagmus, ataxia/wide-based gait, dysmetria, slurred speech, hyperreflexia), N/V, blurred vision, diplopia, dizziness, drowsiness, lethargy -Severe toxicity: AMS (lethargy, confusion), coma, paradoxical seizures -Rapid IV infusion can result in hypotension, bradyarrhythmias -Treatment: gastric decontamination, possible hemodialysis Pharmacokinetics/dynamics: -Highly protein-bound, hepatically metabolized by CYP450 (inhibitors increase risk of toxicity)

Theophylline

MOA: -Phosphodiesterase inhibitor Indications: -Moderate to severe asthma ADRs: -Narrow therapeutic index, CYP450 interactions -CNS stimulation (HA, insomnia, tremor, seizures), GI disturbance (N/V), cardiac arrhythmias

Hydroxychloroquine

MOA: -TNF & IL-1 suppressor Indications: -RA DMARD ADRs: -Retinopathy (regular ophtho evals)

Leflunomide

MOA: -Pyrimidine synthesis inhibitor Indications: -RA DMARD ADRs: -Hepatotoxicity, cytopenias

Nitrofurantoin

Macrobid, Macrodantin Indications: -First-line for acute bacterial cystitis (w/ fosfomycin, SMX-TMP) ADRs: -Exacerbation if underlying G6PD deficiency -Drug-induced lung injury, acute lung hypersensitivity in short term & ILD in long term, pleural effusion, eosinophilia & leukocytosis -Maculopapular rash

Construction Apraxia

Marked difficulty in copying single line drawings Pathophysiology: -May occur w/ nondominant parietal lobe lesions

Attributable Risk Percent

Measure of the impact of a risk factor ARP = (risk in exposed - risk in unexposed)/risk in exposed = (RR-1)/RR

Medicare Part C

Medicare Advantage: allow private health insurance companies to provide Medicare benefits, plans that provide IP & OP services as an alternative to parts A & B

Pill Esophagitis

Medication-induced esophagitis Presentation: -Dysphagia, retrosternal chest pain, severe odynophagia -May be unable to swallow saliva due to pain -Often associated w/ po NSAIDs, KCl, Fe, tetracyclines, bisphosphonates, esp w/ drug ingestion w/ minimal water/food at bedtime Diagnosis: -Clinical, EGD if severe sx Treatment/Management: -Discontinue offending drug -If continuation of drug necessary, ingestion of pill w/ full glass of water while upright for at least 30 min after ingestion Pathophysiology: -Due to direct effects of med on esophageal mucosa -May occur when pills become obstructed in esophagus, most commonly at level of aortic arch d/t esophageal compression by aorta -> localized chemical burn to esophageal mucosa -May be injury d/t pH (e.g. tetracyclines highly acidic w/ pH <3) or hyperosmolarity (KCL) or disruption of prostaglandin protective barrier (NSAIDs)

Chalazion

Meibomian cyst Presentation: -Chronic, granulomatous inflammation of the meibomian gland presenting as hard, painless lid nodule -Residual nodule following internal/external hordeolum Treatment: -If large/persistent, incision & curettage

Cancer Pain Management

Mild pain: -Acetaminophen, NSAIDs Moderate pain: -Weak opioids (codeine, hydrocodone, tramadol) +/- nonopioids Severe pain: -Strong short-acting opioids (morphine, hydromorphone) -If still refractory, calculate total daily dose and convert to long-acting formulation (fentanyl patch, oxycodone) + short-acting opioids for breakthrough pain

CYP450 Inhibitors

Mnemonic: SICKFACES.COM Sodium valproate Isoniazid (INH) Cimetidine Ketoconazole Fluconazole Alcohol & grapefruit juice, amiodarone Chloramphenicol Erythromycin (macrolides except azith) Sulfonamides Ciprofloxacin Omeprazole Metronidazole

Toxic Adenoma and Multinodular Goiter

Most common causes of hyperthyroidism after Graves disease Presentation: -Sx of hyperthyroidism + goiter/focal nodule Diagnosis: -Radioactive iodine scan w/ focally increased uptake (in 1 region in toxic adenoma, multiple regions in multinodular goiter) Treatment: -Initially treated w/ beta-blocker to relieve adrenergic sx, thionamide (methimazole, PTU) to decrease thyroid secretion -Definitive management: surgical excision, radioiodine ablation Pathophysiology: -Most often caused by activating mutations in TSH receptor -> TSH-independent thyroid hormone secretion, focal (TA) or multifocal (MNG) follicular hyperplasia

Mycobacterium Avium Complex (MAC)

Mycobacterium avium, intracellulare Presentation: -Common cause of watery diarrhea & abd pain in AIDS pts CD4 <50 -Presents w/ weight loss, HIGH FEVER >102.2, night sweats, cough, lymphadenopathy, hepatosplenomegaly -Cutaneous lesions uncommon, usually nodular & ulcerating -Labs: elevated alk phos (d/t hepatic involvement) Diagnosis: -Small intestinal bx w/ PAS-positive macrophages & acid-fast bacilli -Blood/lymph node culture w/ nontuberculous Mycobacteria Treatment: -Macrolide (clarithromycin, azithromycin) + ethambutol -If HIV+, initiate antiretroviral therapy a few days after starting above

Cutaneous T-cell Lymphoma

Mycosis fungoides, Sezary syndrome Presentation: -Scaly, pruritic papules/patches/tumors, slowly progressive skin lesions -Generalized erythroderma Treatment: -Interferon alfa, skin-directed corticosteroids, chemotherapy, phototherapy, and/or radiation

Disseminated Gonorrhea

Neisseria gonorrheae infection Presentation: -Purulent monoarthritis w/ painless vesiculopustular rash (look like furuncles/pimples, rarely involves face, usually affects distal extremities and only lasts few days) and w/o systemic sx like fever OR triad of tenosynovitis (pain over flexor tendons), dermatitis (painless pustules on distal extremities or thorax), migratory polyarthralgia (asymmetric, in multiple proximal/distal joints) -Rash, chills, fever, polyarthralgia in sexually active patient -May have no signs of venereal disease (e.g. purulent genital discharge) Diagnosis: -NAAT detection of N gonorrheae in urine, cervical, or urethral sample -Culture of blood, synovial fluid (less sensitive, negative in >50% of pts) -Synovial WBC count usually lower (<50,000) than other causes of septic arthritis Treatment: -3rd gen IV cephalosporin (ceftriaxone) + azithromycin (only if chlamydial status unknown or +) Pathophysiology: -Hematogenous dissemination of infection from other source (e.g. GU infection--cervicitis, urethritis)

Lichen Simplex Chronicus

Neurodermatitis Presentation: -Thickened, excoriated plaques caused by persistent scratching/rubbing -Often associated w/ anxiety disorders -Usually occurs in areas that are easy to reach (e.g. arms, legs, neg)

Tabes Dorsalis

Neurosyphilis Presentation: -Sensory ataxia (wide-based gait, +Romberg), lancinating/shooting/burning pains in face/back/extremities, neurogenic urinary incontinence, diminished pain/temp/proprioception/vibration/reflexes -Argyll Robertson pupils (poor constriction w/ light but normal constriction w/ accommodation, miotic & irregular in size) -Presents yrs to decades after initial exposure, increased incidence & more rapid progression in HIV pts Treatment: -10-14 days of IV penicillin G Pathophysiology: -Treponema pallidum infection of & damage to dorsal sensory roots -Secondary degeneration of dorsal columns

Vitamin B3

Niacin Sources: meat, whole grains, legumes Deficiency: pellagra (dermatitis, dementia/delusions/depression/distraction, diarrhea, death) -Photosensitive dermatitis (erythematous, well-demarcated, thickened, hyperpigmented, in sun-exposed areas, w/ vesicles & blisters), atrophic glossitis -Watery diarrhea w/ GI sx like nausea/vomiting, abd pain, epigastric discomfort -Neurologic manifestations include insomnia, anxiety, disorientation, delusions, dementia, encephalopathy -May be associated w/ alcoholism, anorexia nervosa, malabsorptive disease (e.g. IBD), dietary niacin deficiency (e.g. primarily corn diet), carcinoid syndrome, Hartnup syndrome (congenital disorder of tryptophan absorption)

Thyrotoxicosis Etiologies

Normal or increased radioactive iodine uptake (overproduction of thyroid hormone): -Graves disease, toxic multinodular goiter, toxic nodule -Methimazole & radioactive iodine target production, used for normal/increased RAIU but not decreased Decreased radioactive iodine uptake (release of preformed hormone): -Painless/silent thyroiditis, subacute (de Quervain) thyroiditis, amiodarone-induced thyroiditis, excessive dose (surreptitious intake) of levothyroxine, struma ovarii, iodine-induced, extensive thyroid cancer metastasis -Treated w/ beta blocker (e.g. propanolol) for hyperadrenergic sx -Glucocorticoids to reduce peripheral conversion of thyroid hormone

Odds Ratio

Odds of exposure in cases / odds of exposure in controls = (a/c)/(b/d) Value >1 indicates a risk factor

Acute Colonic Pseudoobstrution

Ogilvie syndrome Presentation: -Abd distention, pain, obstipation, vomiting -Abd tympany on percussion, decreased bowel sounds, mild/diffuse tenderness to palp -If perforation, may present w/ guarding, rigidity, rebound tenderness -Risk factors: major surgery, traumatic injury, severe infection, electrolyte derangements (low K+/Mg2+/Ca2+, e.g. w/ severe diarrhea, diuretic use), medications (opiates, anticholinergics), neuro disorders (dementia, stroke) Diagnosis: -X-ray w/ diffuse colonic dilation (esp of R colon), normal haustra, nondilated small bowel -CT w/ diffuse colonic dilation w/o evidence of anatomic obstruction Treatment/Management: -NPO, nasogastric/rectal tube decompression -Neostigmine if no improvement in 48 hrs or if cecal diameter >12 cm (impending perforation) Pathophysiology: -Autonomic disruption of colon

Hereditary Hemorrhagic Telangiectasia

Osler-Weber-Rendu disease, AD inheritance Presentation: -Mucocutaneous telangiectasias (oral & cutaneous, recurrent epistaxis from nasal telangiectasias often beginning in childhood), multiorgan AVMs (lungs, liver, brain), iron def anemia -CNS: hemorrhagic stroke from AVM, brain abscesses due to paradoxical bacterial embolization across pulmonary AVM -Pulm: recurrent hemoptysis due to pulm AVM (usually symptomatic in early adulthood), pulmonary arterial HTN -> R sided HF, multifocal & nodular/smooth/well-circumscribed lung lesions on imaging representing AVMs, continuous pulm systolic bruit (from turbulent flow through AVM) -GI: chronic GI bleed from angiodysplasias (esp stomach, small bowel), portal HTN, high output HF -Chronic hypoxemia from shunting -> digital clubbing, reactive polycythemia -Labs: elevated Hct Treatment/Management: -Correction of iron def anemia w/ iron supp, blood transfusions -Localization & embolization of symptomatic pulm AVMs by pulm angiography Pathophysiology: -AD mutations of genes involved in angiogenesis (e.g. ENG, ACVRL 1) -Pulm AVM = anastamoses b/t branches of pulm artery & companion pulm veins

Hip Osteonecrosis

Osteonecrosis of femoral head, osteochondritis dissecans Presentation: -Progressive hip pain on weight-bearing, esp in patients with sickle cell (in absence of fever/warmth/erythema), pain worse w/ activity & better w/ rest, often localized to hip or referred to groin/thigh/knee/buttock -Limp, limited ROM (esp internal rotation, abduction, but ROM may be normal early in disease), atrophy of proximal thigh muscles, no erythema/point tenderness/swelling -X-rays can be normal or show flattened/fragmented femoral head (lucency = necrotic bone replaced by new bone), subchondral sclerosis, loss of sphericity Diagnosis: -MRI most sensitive, crescent sign if advanced, serpiginous low-intensity lines on T1, bone marrow edema -Normal WBC, ESR, CRP Treatment: -No weight bearing, conservative treatment w/ bracing splinting -Surgery in cases where femoral head is not well contained within acetabulum e.g. joint replacement (stage 4), core decompression (stages 1 or 2), osteotomy Pathophysiology: -Chronic loss of cartilage and periarticular bony tissue at femoral heads due to avascular necrosis, occlusion of end arteries supplying femoral head (macro or microvascular occlusion) -> trabecular thinning & collapse -In sickle cell disease, due to disruption of microcirculation in bone by sickling, increased intraosseous pressure due to bone marrow hyperplasia -Foveal artery and ascending arteries supply femoral head, foveal artery may become obliterated w/ aging (lies in ligamentum teres) -Other etiologies of avascular necrosis: steroid use, EtOH abuse, SLE, antiphospholipid syndrome, other hemoglobinopathies, infections (osteomyelitis, HIV), renal transplantation/chronic renal insuff, hemodialysis, decompression sickness, arterial disruption from prior fx, trauma, Gaucher, Caisson's disease

Anti-NMDA Receptor Encephalitis

Paraneoplastic syndrome w/ ovarian teratoma Presentation: -Psych sx (anxiety, insomnia, psychosis), autonomic instability (hyperthermia, HTN, tachycardia), cognitive impairment, rigidity, hyperreflexia, dystonia, focal seizures -Median age of onset 21, 4x more common in women Diagnosis: -CSF w/ antibodies to GluN1 subunit of NMDA receptor -Imaging for tumor localization Treatment: -Prompt tumor removal -Immunosuppressive treatment -Slow recovery, most recover w/o sequelae in 2 yrs

Postrenal Acute Kidney Injury

Pathophysiology: -Due to outflow obstruction (stones, BPH, neoplasia, congenital anomalies, malignancy). Develops only with bilateral obstruction or in a solitary kidney.

Intrinsic Acute Kidney Injury

Pathophysiology: -Most commonly due to acute tubular necrosis (from ischemia or toxins); less commonly due to acute glomerulonephritis (eg, RPGN, hemolytic uremic syndrome) or acute interstitial nephritis. In ATN, patchy necrosis -> debris obstructing tubule and fluid backflow across necrotic tubule -> decreased GFR. Urine has epithelial/granular casts. Urea reabsorption is impaired -> decreased BUN/creatinine ratio and increased FENa

Bartter Syndrome

Pathophysiology: -Reabsorbing defect in the thick loop of Henle affecting the Na/K/2CL co-transporter (like a loop diuretic) leads to hypokalemia, metabolic alkalosis and hypocalcemia (hypercalciuria) -High urine [Cl]

Gitelman Syndrome

Pathophysiology: -Reabsorptive defect of NaCL in DCT (like a thiazide diuretic). -Leads to hypokalemia, hypomagnesemia, metabolic alkalosis, and hypercalcemia.

Anserine Bursitis

Pes anserinus pain syndrome (once cause) Presentation: -Subacute medial knee pain, well-defined & highly localized area of tenderness & swelling over medial tibial plateau below joint line, where the conjoined tendon of semitendinosus/gracilis/sartorius inserts on the tibia -Risk factors: OA, T2DM, obesity, mechanical derangements of knee (e.g. medial meniscus protrusion, MCL displacement) Treatment/Management: -Rest, NSAIDs, exercises to increase strength/flexibility of quadriceps & hamstring muscles to stabilize knee Pathophysiology: -Inflammation of pes anserine bursa over medial tibial condyle, deep to pes anserinus tendon complex

Frontotemporal Dementia

Pick disease = behavioral variant FTD Presentation: -Early personality changes (e.g. euphoria, apathy/detachment, disinhibition--e.g. flirting/public urination, loss of empathy), compulsive behaviors/rituals (e.g. peculiar eating habits, hoarding), hyperorality, executive dysfxn -Some pts (~20%) develop motor neuron disease w/ UMN & LMN (e.g. hyperreflexia, fasciculations) similar to those seen in ALS -Memory deficits later in disease (vs. Alzheimers) -Earlier age of onset than other forms of dementia (usually 50s-60s) -Frontotemporal atrophy on neuroimaging -40% have fhx of FTD -Avg survival length of 8-10 yrs after onset Diagnosis: -Primarily clinical, neuroimaging (CT, MRI) to rule out other disorders & evaluate for frontal/anterior temporal lobe atrophy Treatment/Management: -Nonpharmacologic: behavior modification (e.g. exercise, trigger avoidance, distraction, redirection) -Pharmacologic (symptomatic): SSRI, atypical antipsychotics Pathophysiology: -Focal degeneration of frontal and/or temporal lobes -Involves abnormal inclusions of either hyperphosphorylated tau protein (aka Pick bodies) or 43 kD transactive response DNA-binding protein (TDP-43) -Pick disease: silver-staining cytoplasmic inclusions in neurons of hippocampus

Male Secondary Hypogonadism

Pituitary/hypothalamic disease, central hypogonadism Presentation: -Fatigue, decreased libido, testicular atrophy, decreased body hair, gynecomastia (less common than primary) -Labs: low testosterone, low sperm count, low/normal LH Diagnosis: -LH & FSH low -Prolactin, transferrin, +/- cranial MRI Pathophysiology: -Etiologies: pituitary tumors, hyperprolactinemia, infiltrative diseases (e.g. hemochromatosis), chronic/severe illness, eating disorders, severe weight loss, meds (opioids, glucocorticoids, exogenous androgens-withdrawal phase), congenital (Kallman syndrome) -Pituitary tumors & direct pituitary trauma associated w/ low LH, elevated prolactin from disruption of dopaminergic neurons in pituitary stalk

Tinea Versicolor

Pityriasis versicolor, malassezia globosa Presentation: -Initial lesion = "herald patch," larger than subsequent lesions -Hypopigmented, hyperpigmented, or mildly erythematous ("salmon-colored") lesions/macules (face in children, trunk & upper extremities in adolescents & adults), often coalesce -May have faint scaling, pruritus -May be noticed after sun exposure b/c surrounding skin darkens, most common in summer -Typically resolves within 6 wks of presentation Diagnosis: -KOH preparation shows large/blunt hyphae & thick-walled budding yeast cells in spaghetti & meatballs pattern Treatment: -Topical ketoconazole, clotrimazole, terbinafine, or selenium sulfide -If extensive disease or recalcitrant infection, oral antifungals (ketoconazole, itraconazole, fluconazole) Pathophysiology: -Superficial fungal skin infection caused by Malassezia species (furfur, globosa, sympodialis)--nondermatophytic, lipid-dependent yeasts -Malassezia globosa skin flora, grows in exposure to hot/humid weather -Organism inhibits pigment transfer to keratinocytes & makes affected skin paler than unaffected tanned skin

Pneumocystis Pneumonia

Pneumocystis jiroveci Presentation: -Subacute/indolent dyspnea/respiratory failure (hypoxia, tachypnea), fever, dry nonproductive cough, weight loss -CXR w/ diffuse pulm infiltrates, bilateral reticulonodular interstitial infiltrates -Labs: elevated LDH, CD4 usually <200, respiratory alkalosis -Risk factors: AIDS/HIV (esp untreated), immunosuppressed (e.g. transplant) Diagnosis: -Pneumocystis identification in induced sputum (w/ hypertonic saline) -If above negative/inconclusive, fiberoptic bronchoscopy w/ BAL sample (requires specific stains to identify, e.g. methanamine silver, sputum has low diagnostic sensitivity of 50-90% so negative results still necessitate further testing w/ BAL) Treatment: -TMP/SMX (also for prophylaxis), concomitant corticosteroids if severe (ABG PaO2 of 70 or lower, A-a gradient of 35+, SpO2 <92% on RA, used to prevent inflammation from organism lysis w/ abx) -Alternate oral regimens for mild/moderate disease: dapsone-TMP, primaquine + clindamycin, atovaquone suspension -Alternate IV regimens for moderate/severe disease: IV pentamidine (only if cannot tolerate TMP/SMX, severe ADR profile), primaquine + IV clindamycin -Initiation of antiretroviral therapy in HIV pts after 1-2 wks (immediate initiation associated w/ increased risk of worsening resp status from immune reconstitution syndrome) Pathophysiology: -Fungus proliferates within alveoli & cause strong inflammatory response -> hypoxia, large A-a gradient -Abx initiation can worsen hypoxia by release of pro-inflammatory intracellular macromolecules w/ fungal lysis, thus administered w/ steroids -Seen in pts w/ defects in cell-mediated immunity

Dressler Syndrome

Post-cardiac injury syndrome, postpericardiotomy syndrome Presentation: -Fever, leukocytosis, tachycardia, pleuritic chest pain (worse w/ deep breathing) wks to mos following procedure involving pericardial incision or ACS/MI -Usually self-limited disease course, but can cause chronic/recurrent disease leading to constrictive pericarditis -Elevated ESR, CRP, widespread ST segment elevation on ECG (like other forms of acute pericarditis) -Risk factors: MI, cardiac surgery/trauma, percutaneous coronary intervention Diagnosis: -CXR w/ pleural effusion +/- enlarged cardiac silhouette, Echo w/ pericardial effusion Treatment: -NSAIDs, colchicine, steroids for inflammation in refractory disease or if NSAIDs contraindicated -Pericardial puncture indicated if tamponade develops -Colchicine may be used for prevention following cardiac surgery Pathophysiology: -Autoimmune mechanism, immune complex deposition in pericardium and pleura following an event or intervention that leads to immune exposure to cardiac antigens from damaged mesothelial pericardial cells in pericardial space

Concussion

Postconcussive syndrome Presentation: -Transient neurologic disturbance (e.g. dizziness/vertigo, disorientation, amnesia, headache, cognitive dysfxn, confusion, sleepiness, difficulty concentrating, irritability, slurred/delayed speech, transient abnormalities in coordination, e.g. stumbling/falling) after mild TBI w/o structural intracranial abnormality -Headache most common, may be triggered by physical/cognitive exertion, visual tasks (e.g. moving eyes b/t 2 points), +/- light/sound sensitivity -Sx usually resolve within wks but can persist for up to 6+ mos -No structural intracranial injury on imaging (e.g. vs. hematoma, contusion) -High risk features like vomiting, loss of consciousness, focal neuro signs, amnesia for events >30 min prior to injury favor other etiologies; physical signs like battle sign (bruising behind ears), raccoon eyes, temporal hematoma favor bleed -Complications: second impact syndrome, chronic traumatic encephalopathy, posttraumatic epilepsy (w/ severe/multiple concussions) Diagnosis: -Clinical, but may use imaging to rule out if high suspicion for other structural etiology Treatment/management: -Remove from same-day physical activity, physical/cognitive rest for 24-48 hrs -Neurologic evaluation -Sx management (NSAIDs, ondansetron) -Gradual return to normal activity if sx do not worsen (physical: light aerobic exercise -> noncontact sports -> contact sports, gradual return to play over minimum of 5 days; neurocognitive: limited screen time, school accommodations w/ frequent breaks, shortened days) -Usually return to normal activity within a mo Pathogenesis: -Axonal shearing from rotational acceleration of brain after fall/strike to head -> neurologic disturbance w/ no structural intracranial injury -Head trauma -> widespread neuron depolarization, decreased cerebral blood flow, localized lactic acidosis

Medicare Part D

Prescription drug coverage, provided by private insurance companies w/ government contracts

Ichthyosis Vulgaris

Presentaiton: -"Fish scales"--diffuse dermal scaling w/ skin that is dry & rough, w/ horny plates resembling skin/reptile scales, mild pruritus, worse on extensor extremities, spares intertriginous areas & face -Lifelong condition, sx early in life (infancy, early childhood) often mild & may be attributed to dryness, worsens later in life & in the winter due to decreased ambient humidity -Associated conditions: keratosis pilaris, palmar hyperlinearity, atopic disease Diagnosis: -Clinical, bx if uncertain (shows reduced/absent granular layer in epidermis) Treatment/Management: -If emollients ineffective, treated w/ keratolytics (e.g. urea, alpha-hydroxy acid, coal tar, salicylic acid, lactic acid) -Long baths to remove scales Pathophysiology: -Inherited condition d/t mutations in filaggrin gene -> impaired epidermal barrier w/ epidermal hyperplasia & defective keratinocyte desquamation, reduced skin moisturization -Worse in homozygous individuals

Behcet Disease

Presentation -Recurrent, painful aphthous ulcers, genital ulcers (painful, round, white-yellow necrotic base, usually scrotal in men), eye lesions (uveitis) -Skin lesions: erythema nodosum, acneiform lesions, thrombosis -Exaggerated skin ulceration with minor trauma (needlestick) -Risk factors: young adults of Turkish, Middle Eastern, Asian descent Diagnosis: -Biopsy: Nonspecific vasculitis of different sized vessels Pathophysiology: -Autoimmune

Marijuana Intoxication

Presentation -Tachycardia, tachypnea, dry mouth, conjunctival injection, euphoria with inappropriate laughter, anxiety, paranoid delusions, perception of slowed time, delayed reflexes, dizziness, impaired judgment, social withdrawal, increased appetite, hallucinations -Sx improved w/ hot showers Pathophysiology: -Pharmaceutical form is dronabinol (tetrahydrocannabinol isomer): used as antiemetic (chemotherapy) and appetite stimulant (in AIDS).

Reactive Arthritis

Presentation: -"Can't see, can't pee, can't climb a tree"--conjunctivitis/uveitis, (aseptic) urethritis/cervicitis/prostatitis, asymmetric oligoarthritis w/ enthesitis (esp Achilles)/dactylitis/tendinitis -Arthritis presents w/ warm/inflamed joints & elevated synovial leukocytes -Also presents w/ malaise, cutaneous findings (e.g. keratoderma blennorhagica, balanitis circinata), ORAL ULCERS, afebrile -Classically follows infection (Chlamydia, Shigella, Salmonella, Yersinia, Campylobacter, C. diff), 2-3 wks after onset of diarrhea -Balanitis circinata: painless, shallow ulcers of glans penis -risk factors: HLA-B27 positivity Diagnosis: -Labs: elevated synovial leukocytes w/o organisms, sterile pyuria Treatment: -NSAIDs -If NSAID failure/contraindication, try 1. intraarticular glucocorticoids, then 2. systemic glucocorticoids, then 3. DMARDs (e.g. sulfsalazine, methotrexate) -Topical steroids for balanitis circinata -Abx & repeat testing if persistent infection (e.g. chlamydia, non-self-limiting GI infection) Pathophysiology: -HLA-B27-associated (thought to be d/t defective antigen presentation through class I HLA), postinfectious, seronegative reactive arthropathy following GU/GI infection -Thought to involve activated lymphoid cells from infection migrating to joints/extraarticular sites & causing inflammation

Papular Urticaria

Presentation: -0.5 to 1 cm papules localized to insect bites, lesions often surrounded by pale halos d/t dermal edema -New lesions may recur sporadically, leading to chronic sx -One affected person in household (vs. bedbugs, scabies), more commonly in children -Usually spontaneous resolution within mos Treatment/Management: -2nd gen H1 blocker for severe pruritus, topical corticosteroids -Removal from insect exposure (e.g. treat pets, wear insect repellent) Pathophysiology: -Delayed hypersensitivity to insect bites (e.g. ticks, bedbugs) -Not true urticaria

Somatic Symptom Disorder

Presentation: -1+ somatic symptoms causing distress/functional impairment for 6+ mos -Excessive thoughts, anxiety, or behaviors (time & energy) related to sx -Associated w/ high healthcare utilization/multiple providers, repeated testing rarely reassuring -Risk factors: female, lower educational level, chronic childhood illness or fhx of chronic illness, childhood abuse/neglect, sexual trauma Treatment/Management: -Antidepressants (SSRIs, SNRIs, TCAs), CBT if treatment resistant -Schedule regular visits w/ same provider, limit unnecessary workup & specialist referrals -Legitimize sx but focus on stress reduction/coping strategies (e.g. sleep hygiene, relaxation techniques, exercise, diet, return to productive activities)

Fat Embolism Syndrome

Presentation: -1-3 days following traumatic injury, presents w/ triad of petechial rash (typically on trunk), diffuse pulm infiltrates w/ hypoxia & tachypnea/respiratory distress (appears similar to pulmonary edema in ARDS), altered mentation/confusion (visual field deficits, focal neuro findings, obtundation, seizures, coma) -Thrombocytopenia, mild respiratory alkalosis from tachypnea, no arterial filling defects on CT angiography b/c emboli are too small to be soon -Risk factors: fx (esp of long bones, pelvis), delayed immobilization & operative fixation of fxs Diagnosis: -Clinical, brain MRI may reveal multiple small, hyperintense foci Treatment: -Supportive care (e.g. mechanical ventilation) -Prevention by early immobilization of fxs Pathophysiology: -Blocking of small blood vessels by fat globules subsequent to a fracture or surgical manipulation, especially one of long bones or pelvis, may also occur w/ pancreatitis -Marrow containing fat and hematopoietic cells mobilizes into bloodstream -> microvascular occlusion in lungs, brain, dermal capillaries (-> erythrocyte extravasation -> petechiae) -Fat globules also induce systemic inflammatory response that further contribute to microvascular dysfxn

Skin Squamous Cell Carcinoma

Presentation: -2nd most common type of skin cancer -Firm/scaly plaque, papule, or nodule on sun-exposed areas +/- hyperkeratosis or ulceration -If in situ (Bowen disease), likely to be slow-growing, red, scaly plaque/patch -Neurologic signs possible if perineural invasion (numbness, paresthesias) -May be seen overlying/within burn wounds (Marjolin ulcer), chronic wounds/scars (avg latency of 30 yrs, excessive granulation tissue that bleeds on contact, nonhealing ulcers/nodules, eversion of wound margions), over focus of osteomyelitis, radiotherapy scars, venous ulcers -Risk factors: sun/UV light exposure, ionizing radiation exposure, immunosuppression (esp organ transplant), chronic scars/wounds/burn injuries, fair skin Diagnosis: -Skin biopsy (punch/shave/excisional) including deep reticular dermis shows dysplastic/anaplastic keratinocytes Treatment: -Low risk (in situ, trunk/extremities) & small lesions treated w/ surgical excision or local destruction (cryotherapy, electrodessication, radiation therapy, topical 5-flurouracil, imiquimod) -High risk lesions (large size 1+ cm, poorly differentiated histology, perineural invasion, face/ears/neck/hands/feet/genitalia) or lesions in cosmetically sensitive areas treated w/ Mohs micrographic surgery or excision w/ 4-6 mm margins -If untreated, can cause extensive local destruction and lymphatic/distant metastases (esp if arising in wound/burn aka Marojlin ulcer) Pathophysiology: -SCC in chronic wounds often aggressive w/ high risk for recurrence, local, invasion, & metastasis; likely develop from chronic inflammation & vascular compromise from scarring

Polycystic Ovarian Syndrome (PCOS)

Presentation: -3 main criteria-1. evidence of hyperandrogenism, 2. hx of irregular menses indicating anovulation, 3. polycystic ovaries on US ("pearls on string" cysts) -Hyperandrogenism: severe nodulocystic acne, male pattern baldness, hirsutism -Menstrual irregularities: oligoovulation/anovulation -Obesity, comorbidities include metabolic syndrome (DM, HTN), OSA, nonalcoholic steatohepatitis, endometrial hyperplasia/cancer -Mild elevation of testosterone, esp in obese patients, but usually not enough elevation to cause virilization (voice deepening, clitoromegaly) Diagnosis: -Must exclude other causes of hyperandrogenism -Increased GnRH and estrogen, normal FSH, high LH, LH/FSH imbalance, (mildly) increased testosterone (may be normal), low progesterone (causes unbalanced endometrial prolif by estrogen) Treatment: -Weight loss (first line), OCPs (cyclic progesterone, combined OCPs, progesterone-containing IUD), letrozole or clomiphene for ovulation induction if desiring pregnancy Pathophysiology: -Hyperandrogenism, decreased sex hormone-binding globulin -> elevated estrone (from peripheral androgen conversion) -> high-frequency, short interval GnRH bursts -> preferential production/release of LH -> LH/FSH imbalance, lack of LH surge -> failure of follicle maturation & oocyte release, multiple follicles develop at same time w/o ovulation -> anovulation, AUB -Very high estrogen levels due to peripheral conversion/aromatization of androgens to estrone in adipose tissue -Combined OCPs contain progesterone to stimulate endometrial differentiation & limit continued prolif, estrogen to stabilize uterine lining, reduce androgenism by blocking androgen secretion & increasing production of sex hormone-binding globulin -> binds & decreases free testosterone

Rocky Mountain Spotted Fever (RMSF)

Presentation: -3-4 days of fever, headache, myalgias, arthralgias, N/V (resembles viral prodrome) -Rash several days into illness, begins as maculopapular rash involving palms/soles and wrists/ankles, spreads centrifugally before becoming petechial -Severe abd pain in children -Complications: shock, encephalitis, pulmonary edema, bleeding, arrhythmias -Labs: CSF w/ normal glucose, leukocytes <100, thrombocytopenia, hyponatremia, elevated AST & ALT Diagnosis: -Rickettsia serology, skin biopsy -Serology often negative early in disease; therefore often diagnosed clinically Treatment: -Doxycycline, supportive care (IVF) Pathophysiology: -Dermacentor tick spread of Rickettsia rickettsii, esp in grassy/wooded areas during summer mos in US -Thrombocytopenia due to intravascular platelet destruction, hepatic injury due to hepatic endothelial vascular injury -Bacteria attacks vascular endothelial cells -> progressive capillary permeability, fluid extravasation, hypovolemia, poor tissue perfusion

Interventricular Septal Rupture

Presentation: -3-5 days following MI, presents w/ chest pain, hypotension, biventricular HF & shock -Pansystolic murmur best heard at L sternal border, often has accompanying thrill Diagnosis: -Echo w/ L-to-R ventricular shunt, -Pulm artery catheterization showing increased oxygen levels in R side of heart (increased gradient b/t RA & RV), increased R-sided pressures, low CO Pathophysiology: -May involve LAD (apical septum) or RCA (basal septum) occlusion

Acute Limb Ischemia

Presentation: -6Ps: pain, pallor, paresthesias/numbness, pulselessness, poikilothermia/cyanosis (cool extremity), paralysis (late) -Risk factors: PAD, AFib, heart vegetations Treatment: -Immediate anticoag (IV heparin infusion) prior to imaging or surgical intervention -If refractory after anticoag, may require percutaneous thrombolysis (e.g. alteplase) or surgical thrombectomy to restore perfusion Pathophysiology: -Usually due to either embolism (e.g. AFib, infective endocarditis, LV thrombus from LV aneurysm after recent MI, prosthetic valve thrombosis) or thrombosis following atherosclerotic plaque rupture (e.g. PAD), rarely due to iatrogenic/blunt or blunt trauma/traumatic vessel disruption or dissection -If no evidence of prior PAD, more likely to be embolic source (usually cardiac) -If due to PAD/plaque disruption may be delayed in presentation due to presence of collateral circulation (cap refill may still be present)

Lichen Planus

Presentation: -7 Ps: pruritic, purple/pink/violaceous, polygonal, planar plaques & papules; nail pterygium (longitudinal ridges, fissuring, thinning); phenomenon of Koebner (formation at sites of trauma) -Wickham striae (overlying white, lacy pattern) -May involve skin (usually ankles, volar wrists, extremities, trunk, flexural surfaces), oral mucosa (white papules/plaques +/- erythema on tongue & buccal mucosa, Wickham striae, mucosal atrophy, ulcers, longer course than cutaneous lichen planus often lasting yrs), and/or external genitalia (penis, vulva) -Chronic sx that usually spontaneously resolve within 2 yrs -If drug-induced, may be more diffuse than idiopathic form & may persist despite discontinuation of drug, w/ residual hyperpigmentation -Risk factors: hep C or other hepatic disease, ACEi, thiazide diuretics, beta blockers, hydroxychloroquine, recent trigger (e.g. infection, med, contact allergen) -Labs: LFT abnormalities if d/t HCV Diagnosis: -Usually clinical, lesion punch biopsy if needed for confirmation (e.g. in pts w/ mucosal atrophy/ulceration) Treatment/Management: -Topical high-potency glucocorticoids (e.g. betamethasone, flucinonide), antihistamines for pruritus -If widespread lesions, systemic glucocorticoids, phototherapy Pathophysiology: -Autoimmune disorder driven by CD8+ cells, may occur after exposure to a trigger -Activated T-cells directed against basal keratinocytes of epidermis

Acute Hepatitis B

Presentation: -70% of pts asymptomatic, the rest present w/ sx including anorexia, nausea, jaundice, RUQ discomfort, hepatomegaly Diagnosis: -Hep B serology & DNA PCR Treatment/Management: -Most asymptomatic; schedule regular fup to follow for disease resolution or progression to chronic hep B (>6 mos, 5% of adult pts) -Hospitalization for pts w/ severe illness including hemodynamic instability, impaired hepatic synthetic fxn, signs of acute liver failure (e.g. encephalopathy, INR 1.5+), pts >50, poor PO intake, etc. -Antiviral therapy in pts w/ immunosuppression, concurrent hep C or D, severe hepatitis, fulminant hepatic failure, preexisting liver disease (antivirals not demonstrated to significantly course of infection) -If progression to chronic: viral genotyping, possible interferon therapy, liver bx to evaluate for fibrosis

Fibromuscular Dysplasia

Presentation: -90% in women (in adults) -Severe, refractory HTN in young female -Internal carotid artery stenosis: recurrent severe headaches, recurrent epistaxis, pulsatile tinnitus, neck pain, subauricular systolic bruit, TIA (e.g. focal weakness, vision loss, amaurosis fugax, Horner syndrome), stroke -Renal artery stenosis: secondary HTN, flank pain, systolic-diastolic epigastric/abd bruit, renal size discrepancy, recurrent flash pulm edema -Less commonly affects vertebral, mesenteric, iliac, subclavian, visceral arteries -Increased risk of arterial stenosis, aneurysms, dissections -Labs: increased Cr after starting ACEi or ARB w/o significant effect on BP Diagnosis: -Noninvasive imaging preferred (e.g. duplex US, CTA, MRA) -Catheter-based arteriography (digital subtraction arteriography for pts w/ inconclusive noninvasive imaging) -Aldosterone:renin ratio of ~10 (<20) -Consider screening if: severe/resistant HTN, onset <35 yo, increase in BP from baseline, bruit Treatment/Management: -Antihypertensives (ACEi or ARBs 1st line), BP & Cr f/u every 3-4 mos w/ renal US every 6-12 mos -Percutaneous transluminal angioplasty -Surgery if PTA unsuccessful Pathophysiology: -HTN due to secondary hyperaldosteronism from renal artery stenosis -Noninflammatory, nonatherosclerotic condition caused by abnormal cell development in arterial wall that can lead to vessel stenosis, aneurysm, dissection

Entamoeba Histolytica

Presentation: -90% of pts asymptomatic -Dysentery/colitis (wks) w/ diarrhea, bloody stool w/ mucus, abd pain (dull, may be referred to R chest wall or shoulder), anorexia, weight loss -Liver abscess (fever, RUQ pain, hepatomegaly, single/smooth/cystic/subcapsular/low-density mass, usually in R lobe), rupture complications into pleura/peritoneum possible -Labs: elevated leukocytes, alk phos, transaminases, negative blood cultures -Risk factors: travel/residence of developing countries (regions w/ poor sanitation, esp India, Africa, Mexico, Central/South America), men 20-50 yo, MSM, highest risk of liver abscess if underlying cirrhosis/hepatitis Diagnosis: -Stool microscopy w/ O&P, stool antigen/PCR testing for colitis (preferred), E histolytica serology for isolated liver abscess -Imaging w/ round, well-defined hypoechoic mass usally in R hepatic lobe Treatment: -Metronidazole/tinidazole + intraluminal agent (paromomycin, iodoquinol) -Abscesses rarely require drainage (vs. Echinococcus), only if refractory to therapy or risk of imminent rupture; aspirate resembles "anchovy paste" & is often aseptic w/ negative gram stain Pathophysiology: -Abscess develops when parasite spreads from colonic mucosa to liver through portal vein -Usually acquired by ingestion of water/food contaminated w/ cysts in developing countries, may also rarely be acquired by sexual intercourse (MSM)

Chronic Pulmonary Aspergillosis

Presentation: ->3 mos of weight loss (>90%), cough, hemoptysis, fatigue -Risk factors: lung disease/damage (e.g. cavitary TB) Diagnosis: -Imaging w/ cavitary lesion +/- fungus ball -Positive Aspergillus IgG serology Treatment/Management: -Resect aspergilloma if possible -Azole (e.g. voriconazole) -Embolization if severe hemoptysis

Chronic Radiation Proctitis

Presentation: ->3 mos to yrs after radiation exposure (usually within a yr), presents w/ severe hematochezia from rectal bleeding, +/- strictures w/ constipation and rectal pain, fecal incontinence, fecal impaction, fistulas Diagnosis: -Multiple telangiectasias, focal hemorrhage, mucosal pallor & friability, strictures on colonoscopy Treatment: -Endoscopic thermal coagulation, sucralfate or glucocorticoid enemas Pathophysiology: -Obliterative endarteritis, chronic mucosal ischemia, submucosal fibrosis -Chronic tissue hypoxia -> neovascularization -> hemorrhage, telangiectasia formation, secondary anemia (e.g. conjunctival pallor)

Hiatal Hernia

Presentation: ->90% sliding, may be asymptomatic or present w/ signs of heartburn -Nausea/vomiting, dysphagia, epigastric/chest pain, postprandial fullness -Large hernias can result in herniation of stomach and intraabd organs -> compression of stomach & other organs -> respiratory compromise, gastric volvulus -If paraesophageal, may see retrocardiac air-fluid level on imaging Diagnosis: -Barium swallow, upper endoscopy Treatment: -Sliding usually treated w/ medical management of reflux -Hiatal usually require surgical repair -Observation if asymptomatic Pathophysiology: -Protrusion of intraabd organs into thoracic cavity, may be sliding (GE junction & prox stomach slide at esophageal hiatus) or paraesophageal (fundus migrates through membrane defect in diaphragm) -Laxity of phrenoesophageal membrane that attaches distal esophagus to diaphragm @ esophageal hiatus (may be age-related degeneration or repetitive stress-related)

Pulseless Electrical Activity (PEA)

Presentation: -A condition in which the heart's electrical rhythm remains relatively normal, yet the mechanical pumping activity fails to follow the electrical activity, causing cardiac arrest -Organized rhythm (including AFib, sinus brady) on ECG w/o palpable pulse -Often degenerates into asystole Treatment/Management: -CANNOT BE CARDIOVERTED, CPR + IV epinephrine every 3-5 min as attempts are made to identify & treat reversible causes of PEA -Airway management Pathophysiology: -Etiologies (5 H&Ts): hypovolemia, hypoxia, hydrogen ions (acidosis), hypo/hyperkalemia, hypothermia, tension PTX, tamponade (cardiac), toxins (opioids, benzos, etc.), thrombosis (pulm or coronary), trauma -Absence of adequate cardiac mechanical contraction to generate palpable pulse, even in presence of electrocardiographic rhythm

Melanoma

Presentation: -ABCDE (asymmetry, border irregularity, color variegation, diameter 6+ mm, evolution) -May exhibit inflammatory changes (itching, crusting, bleeding, sensory changes like paresthesias) -Ugly duckling sign: lesion significantly different from other lesions on same pt -May develop thickening or nodularity indicating vertical growth Diagnosis: -Full thickness excisional (elliptical or punch) biopsy w/ 1-3 mm margins of normal tissue -Partial excision (deep shave biopsy, punch biopsy) usually only used if lesion very large or in region where complete excision may be problematic (e.g. ear, face) Treatment: -Excision w/ wide margins -If BRAF mutation+, BRAF kinase inhibitors (e.g. vemurafenib) Pathophysiology: -Most common subtype is superficial spreading (horizontal growth) -Nodular subtype grows vertically, often is nodular/pedunculated, firm to palpation, exhibits continuous growth over ~1 mo

Hypopituitarism

Presentation: -ACTH deficiency (secondary adrenal insuff): postural hypotension, tachycardia, fatigue, anorexia/weight loss, hypoglycemia, eosinophilia, pale skin (decreased production of melanocyte-stimulating hormone) -Central hypothyroidism: fatigue, cold intolerance, constipation, dry skin, bradycardia, slowed DTRs -Central hypogonadism: amenorrhea/infertility in women, infertility/loss of libido/testicular atrophy/erectile dysfxn in men -Labs: low cortisol, low ACTH, low FSH & LH, low testosterone, low free T4, low or inappropriately normal TSH, mild hyponatremia (d/t ADH release), normal aldosterone Pathophysiology: -Pituitary causes: primary (adenoma), metastatic mass, infiltration (e.g. hemochromatosis, lymphocytic hypophysitis), hemorrhage (pituitary apoplexy), infarction (Sheehan syndrome) -Hypothalamic causes: mass lesions, radiation therapy, infiltration (e.g. sarcoidosis), trauma to skull base, infections (e.g. tuberculous meningitis) -Vs. adrenal insufficiency, aldosterone is normal (no hyperkalemia or severe hypotension)

Marfan Syndrome

Presentation: -AD disease w/ marfanoid habitus (scoliosis, kyphosis, joint hypermobility, ectopia lentis, arachnodactyly, pectus excavatum/carinatum, tall stature), skin striae, arched palate, crowding of teeth, high myopia (from elongation of globe, severe and requiring 6+ diopters of correction), iridodonesis (rapid contraction and dilation of iris) -Aortic root dilation -> aortic regurgitation (diastolic murmur), aortic aneurysm, aortic dissection, MVP -Upward lens dislocation (predisposed to lens/retinal detachment), normal intellect, spontaneous pneumothorax Treatment/management: -Regular echo to evaluate for CV sx -Beta blockers to decrease myocardial contractility & reduce stress on aortic wall (prophylaxis for dissection) -Surgical replacement of aortic root if diameter is significantly or rapidly enlarging Pathophysiology: -AD mutation in fibrillin-1 (FBN1) gene -> systemic weakening of connective tissues, degeneration & weakening of medial layer of aortic wall -Fibrillin-1 found in MSK, CV systems (aorta, heart valves), zonular fibers suspending ocular lens

Primary CNS Lymphoma (PCNSL)

Presentation: -AIDS-defining illness -Confusion/lethargy/AMS, seizures, focal neuro deficits Diagnosis: -Brain imaging w/ single, irregular, well-defined, nonhomogenous, ring-enhancing lesion w/ surrounding edema -EBV DNA on PCR of CSF -Brain bx not usually needed Treatment/Management: -HAART, corticosteroids, radiation therapy -Improvement in immune status (CD4 count, viral loads) associated w/ improved prognosis Pathophysiology: -Impaired immunosurveillance, unchecked expression of latent viral oncogenes

Contrast-Induced Nephropathy (CIN)

Presentation: -AKI following administration of radiologic contrast agent -Labs: acute rise in Cr 24-48 hrs following arterial contrast administration, w/ gradual return to baseline after ~1 wk, muddy brown granular & epithelial cell casts -Risk factors: >75, CKD (esp diabetic nephropathy), reduced renal perfusion (e.g. hypotension/hypovolemia), high contrast load, NSAID use Diagnosis: -Clinical (bx usually not needed), FeNa <1% w/ muddy bown granular casts -Patchy necrosis of renal tubular cells on bx Prevention: -Periprocedural saline hydration, use lowest volume of contrast agent possible, hold NSAIDs & nephrotoxins Pathophysiology: -Can involve direct cytotoxicity causing ATN w/ muddy brown casts and/or renal vasoconstriction causing prerenal injury (FENa <1%, even in absence of clinical volume depletion)

Acute Diverticulitis

Presentation: -Abd (LLQ) pain, fever, n/v, ileus from peritoneal irritation, leukocytosis -Some present w/ urinary frequency, urgency, dysuria (due to bladder irritation from inflamed sigmoid colon) -Diverticula present on colonoscopy, CT scan shows inflammatory changes + soft tissue stranding + colonic wall thickening -Rarely develop peritoneal signs, LLQ palpable mass -Labs: sterile pyuria (+leukocyte esterase, -nitrites, due to bladder irritation from adjacent sigmoid colon inflammation) -Complications: abscess, obstruction, fistula, perforation -Risk factors: chronic constipation, low fiber/high fat & red meat diet, obesity, tobacco Diagnosis: -Abd CT (PO/IV contrast) w/ increased inflammation from pericolic fat, presence of diverticula, bowel wall thickening, soft tissue masses (e.g. phlegmons), possible pericolic fluid collection if abscess -No colonoscopy d/t risk of perforation Treatment: -Bowel rest, abx (ciprofloxacin + metronidazole), f/u colonoscopy 4-8 wks later to screen for colon cancer -If no improvement after 2-3 days of treatment, repeat CT to evaluate for complications -If abscess, may require perc drainage & IV abx followed by interval elective partial colectomy -If elderly, immunosuppressed, high fever/leukocytosis, or significant comorbidities, admit for IV abx & observation -Prevent recurrence w/ dietary changes (increased fiber), bowel regimen Pathophysiology: -Inflammation due to microperforation of a diverticulum -Diverticulosis: arises from herniation through circular & longitudinal muscle layers (through vasa recta) due to increased intraluminal pressure (e.g. constipation, obesity) -Diverticulitis: elevated luminal pressure and/or inspissated food particles cause erosion of diverticular wall -> micro/macroperforation, colonic wall inflammation, most commonly in sigmoid colon

Crohn Disease

Presentation: -Abd pain (often RLQ), watery diarrhea (bloody if colitis), anemia, elevated inflammatory markers (ESR, CRP) -Can affect anywhere in GI from mouth to anus (mostly ileum/colon), classically presents w/ skip lesions, perianal disease w/ rectal sparing -Extraintestinal involvement: erythema nodosum, PSC, aphthous ulcers (cankersores), arthritis, lung disease, uveitis/scleritis/episcleritis -Risk factors for severe disease: smoking, young age at diagnosis (<30), extensive anatomic involvement, perianal disease, deep ulcerations, strictures, fistulization, prior intestinal surgery -Intestinal complications: fistulae, abscesses, strictures (bowel obstruction w/ bilious vomiting, abd distention, tympanic bowel) Diagnosis: -Gross findings on colonoscopy: transmural inflammation, linear mucosal (deep serpiginous) ulcerations, cobblestoning, creeping fat -Microscopy findings on biopsy: noncaseating granulomas, transmural inflammation, lymphoid aggregate formation, skip regions -GI imaging: strictures, bowel wall thickening, fistulas -> abscess, phlegmon Treatment/Management: -Fecal calprotectin may be used in pts w/ established disease to confirm flares -Surgical resection for strictures, fistulae -5-aminosalicylic drugs for mild disease (e.g. sulfasalazine, mesalamine) -Corticosteroids, biologics for severe disease (e.g. infliximab, other TNF-alpha inhibitors), azathioprine

Ulcerative Colitis

Presentation: -Abd pain in varying locations, bloody diarrhea, anemia, elevated inflammatory markers (ESR, CRP) -Urgency, tenesmus, incontinence -Always involves rectum, can involve colon, continuous lesions (vs. skip lesions in CD) -Intestinal complications: toxic megacolon Diagnosis: -Gross findings on colonoscopy: mucosal & submucosal inflammation/edema, pseudopolyps, continuous & shallow ulcerations, loss of vascular markings, exudates, spontaneous hemorrhage -Microscopic findings on biopsy: no granulomas, acute + chronic inflammation usually confined to mucosa but can extend to submucosa (vs. transmural in CD), crypt abscess formation Treatment: -Total proctocolectomy w/ ileoanal anastomosis if severe -TNF-alpha inhibitors used as first-line induction & maintenance (infliximab, adalimumab, golilumab) for moderate to severe, 5-aminosalicylic agents used for mild disease (sulfasalazine, mesalamine) -Other maintenance agents: azathioprine -Steroids for flare

Small Bowel Lymphoma

Presentation: -Abd pain, malabsorption, SBO, unintentional weight loss, N/V, bowel wall thickening on imaging, occult blood in stool -Very rare form of SBO Diagnosis: -Bowel bx w/ diffuse infiltrate of atypical lymphocytes

Small Intestinal Bacterial Overgrowth (SIBO)

Presentation: -Abd pain, watery diarrhea, bloating, flatulence -In severe cases, malabsorption (steatorrhea), weight loss, anemia (may be macrocytic from vitamin def), fat-soluble vitamin def -Labs: elevated fecal fat, macrocytic anemia (B12 deficiency due to consumption by bacteria, folate excess from bacterial production) -Risk factors: anatomic (strictures, surgery, e.g. gastric bypass), motility disorder (T2DM, scleroderma), alteration in gastric/pancreatic secretions (atrophic gastritis, chronic pancreatitis, small intestinal diverticula), chronic opioid use, gastric hypochlorhydria (e.g. chronic PPI use) Diagnosis: -Jejunal aspirate & culture showing >10^5 organisms/mL -Carbohydrate breath testing (e.g. lactulose, glucose) Treatment: -Abx (rifaximin, amoxicillin-clavulanate, neomycin) -Avoid antimotility agents (e.g. opioids) -Dietary changes -Promotility agents (e.g. metoclopramide) Pathophysiology: -Bacteria originating from colon grow in excess in small bowel -May involve Streptococci, Bacteroides, E. coli, and/or Lactobacillus sp -Proximal small intestine contains minimal bacterial colonization due to gastric acidity & peristalsis; surgery or other conditions may predispose to overgrowth (e.g. gastric bypass creates blind loop of intestine) -> excessive fermentation, inflammation, malabsorption

Ascites

Presentation: -Abdominal distention, shifting dullness Diagnosis: -Paracentesis w/ protein, SAAG, cell count & diff -If neutrophils of 250+, associated w/ peritonitis -If high protein (2.5+), may be associated w/ CHF, constrictive pericarditis, peritoneal carcinomatosis, TB, Budd-Chiari syndrome, fungal -If low protein (<2.5), may be associated w/ nephrotic syndrome, cirrhosis -If high serum:ascites albumin gradient (1.1+), indicates portal HTN, may be due to cardiac ascites, cirrhosis, Budd-Chiari -If low serum:ascites albumin gradient (<1.1), indicates absence of portal HTN, may be due to TB, peritoneal carcinomatosis, pancreatic ascites, nephrotic syndrome

Uremic Coagulopathy

Presentation: -Abnormal bleeding & bruising in pt w/ chronic renal failure -Ecchymoses, epistaxis while on dialysis -May be associated w/ serious bleeding including GI bleed, hemopericardium, subdural hematoma, bleeding from surgical/invasive sites -Labs: plt count, PT/PTT/TT/BT all usually normal Treatment: -DDAVP, cryoprecipitate, conjugated estrogens -Plt transfusion not indicated; transfused plts quickly become inactive Pathophysiology: -Uremic toxins (e.g. guanidosuccinic acid) implicated in platelet dysfxn -DDAVP helps by increasing release of factor VIII:vWF multimer release from endothelial storage sites

Cardiogenic Syncope

Presentation: -Abrupt, transient loss of consciousness w/ loss of postural tone followed by spontaneous, complete recovery -If aortic stenosis/HCM: exertional syncopal episodes, systolic murmur on exam -If VTach: no preceding sx, may be in context of cardiomyopathy or previous MI -If sick sinus syndrome: preceding fatigue or dizziness, sinus pauses on ECG -If advanced AV block: bifascicular block or prolonged PR interval, prolonged QRS interval or dropped QRS complexes on ECG, prior bradyarrhythmic episodes (intermittent dizziness) -If torsades: no preceding sx, may occur in context of hypokalemia, hypomagnesemia, meds that prolong QT

Heat Stroke

Presentation: -Absence of sweating with hot, flushed skin, sudden collapse, altered consciousness (change in mental status distinguishes from heat exhaustion) -Core temp >40C/104F w/ CNS dysfxn (e.g. AMS, seizure, confusion, weakness, dizziness, lethargy, ataxia), tachycardia, tachypnea, diarrhea -Organ or tissue damage: renal/hepatic failure, DIC, ARDS, pulm edema, rhabdomyolysis -Labs: AKI, rhabdo, acute liver injury, severe metabolic acidosis, hypokalemia, hypophosphatemia -Risk factors: strenuous activity during hot & humid weather, dehydration, poor acclimatization, lack of physical fitness, obesity, meds (e.g. anticholinergics, antihistamines, phenothiazines, tricyclics, antipsychotics, alcohol, amphetamines) Treatment/Management: -Pt stabilization: ABCs -Rapid cooling: ice water immersion preferred, may consider high-flow cold water dousing, ice-wet towel rotation, evaporative cooling (spraying w/ tepid water mist or covering w/ wet sheet while large fans circulate air to maximize evaporation), misting systems, ice packs, ice water lavage -Fluid resuscitation, electrolyte correction -Management of end-organ complications -No role for antipyretic therapy Pathophysiology: -Exertional causes: strenuous activity in hot & humid weather -Nonexertional causes: advanced age, impaired mobility, obesity, neuropsych disease, recreational drugs (e.g. cocaine, amphetamines), certain meds (e.g. anticholinergics, antipsychotics, beta blockers, diuretics)

Massive Pulmonary Embolism

Presentation: -Accentuated P2, elevated CVP (JVD) -Echo demonstrating bowing of interventricular septum into LV (due to RV pressure exceeding LV diastolic pressure), RV free wall hypokinesis w/ sparing of apex -Labs: elevated BNP, troponin, Pathophysiology: -RV ischemia/infarction -> RV failure & shock -> syncope, hemodynamic collapse

Acne Mechanica

Presentation: -Acneiform rash (nonpruritic papules & pustules) in distribution where friction/pressure is applied (e.g. axillae, crutches, bra straps, turtlenecks, sports padding, helmets, heavy backpacks, etc.) Treatment/Management: -Eliminate source of pressure Pathophysiology: -Recurrent mechanical pressure -> damage to pilosebaceous follicles -> obstruction, comedone formation, acneiform lesions

Hypersensitivity Pneumonitis

Presentation: -Acute HP: short-lived episodes following exposure (usually within ~6 hrs of exposure) that are self-resolving, w/ abrupt-onset fever, chills, cough, dyspnea, fatigue, leukocytosis, CXR w/ scattered micronodular interstitial opacities (patchy interstitial inflammation, alveolitis) -Chronic HP: continued exposure causing fibrotic evolution (e.g. bagpipe players due to mold overgrowth) w/ progressive nonproductive cough, dyspnea, fatigue, weight loss, hypoxemia that worsens w/ exertion, CXR w/ diffuse reticular interstitial opacities (extensive network of interstitial inflammation & fibrosis) -Risk factors: hx of atopy (eczema, asthma), smoking Diagnosis: -PFT w/ restrictive or mixed pattern, decreased DLCO (chronic only) -BAL w/ high relative lymphocyte count -Lung bx w/ lymphocytic infiltrate, poorly formed noncaseating granulomas, interstitial inflammation or fibrosis (chronic only) Treatment/Management: -Remove antigen exposure (resolves acute disease) -Glucocorticoids and/or lung transplant (chronic only) Pathophysiology: -Exposure & exaggerated immunologic reaction to organic antigens (e.g. molds, animal proteins, certain bacteria incl Mycobacterium avium, Aspergillus)

Acute Decompensated Heart Failure

Presentation: -Acute dyspnea, orthopnea, PND, chronic cough -HTN common (suggests severe disease) -Accessory muscle use, tachycardia, tachypnea, diffuse crackles w/ possible wheezes (cardiac asthma, pulm edema), possible S3 (highest specificity), JVD, peripheral edema -L-sided: pulm edema (bilat crackles, vascular congestion, tachypnea, hypoxia) -CXR may show diffuse pulm congestion, cardiomegaly -ECG may show e/o LV hypertrophy (e.g. increased voltage in precordial leads) Diagnosis: -Serial cardiac markers (elevated BNP), Echo, ECG Treatment/Management: -If normal or elevated BP w/ adequate end-organ perfusion: supplemental O2, IV loop diuretic (e.g. furosemide), consider IV vasodilator (e.g. nitroglycerin, nitroprusside, esp w/ pulmonary edema) -If hypotension or signs of shock: supplemental O2, IV loop diuretic as appropriate, IV vasopressors (e.g. norepinephrine) -Noninvasive ventilation if hypoxemia & respiratory distress -Hold beta blockers; contraindicated d/t decrease in cardiac contractility & HR, may worsen pulmonary edema Pathophysiology: -Most commonly due to LV systolic or diastolic dysfxn w/ or w/o additional cardiac disease (e.g. MI, arrhythmias, acute severe mitral/aortic regurg)

Guttate Psoriasis

Presentation: -Acute eruption of droplet like lesions on trunk, w/ erythematous papules & small plaques w/ fine scales -May be triggered by strep infection Treatment/Management: -Observation, phototherapy

Orbital Compartment Syndrome

Presentation: -Acute eye pain, diplopia, vision loss -Periorbital swelling, ecchymosis, tightness/induration (hard eyelid) -Proptosis, diffuse subconjunctival hemorrhage, limited EOM, relative afferent pupillary defect, decreased visual acuity, resistance to retropulsion (pushing on eye) -May follow eye trauma (most commonly) or be associated w/ coagulopathy, surgery, infection -Rare ophthalmologic emergency that can lead to permanent blindness Diagnosis: -CT/MRI should be delayed until after surgery Treatment/Management: -Emergent surgical decompression (lateral canthotomy w/ cantholysis), emergent ophtho consultation -Temporizing measures to limit increases in intraorbital pressure prior to surgery (elevation of head of bed, pain control, topical timolol) -Orbital imaging (CT) Pathophysiology: -Hemorrhage/swelling within confined orbital space -> rapidly increased intraorbital pressure -> compression of ophthalmic artery -> ischemia of optic nerve & globe

Septic Arthritis

Presentation: -Acute monoarthritis (hot, swollen, decreased ROM) -Fever, chills, cloudy fluid on joint aspiration -Labs: elevated ESR, CRP, peripheral leukocytosis -Risk factors: abnormal joint (RA, prosthetic joint, gout, OA), age >80 yo, T2DM, IV drug abuse, alcoholism, intraarticular glucocorticoid injections Diagnosis: -Blood cultures (+ in 50%), synovial fluid analysis w/ leukocytosis >50,000, gram stain, culture Treatment: -Joint drainage by serial needle aspiration (e.g. knee, ankle, wrist), arthroscopy w/ irrigation (e.g. hip, shoulder), or open arthrotomy if severe -IV abx: vancomycin for gram+ cocci, 3rd gen cephalosporin for gram- rods, vancomycin if negative microscopy (+ 3rd gen cephalosporin if immunocompromised) Pathophysiology: -Commonly S. aureus, may involve indolent organisms like Propionibacterium w/ prosthetic joints -GNRs including E. coli & Pseudomonas seen in IV drug use, severe immunosuppression, advanced age -Neisseria gonorrhea in sexually active, young adults

Drug-Induced Lupus

Presentation: -Acute onset of constitutional sx (e.g. fever, malaise, night sweats), arthralgias/arthritis, serositis (e.g. pleuritis, pericarditis), rash (less common compared w/ SLE), hepatomegaly/splenomegaly, can affect women & men of any age -Implicated drugs: procainamide, hydralazine, penicillamine, minocycline, TNF-alpha inhibitors (e.g. etanercept, infliximab), isoniazid, phenytoin, sulfasalazine -Risk factors: high drug dose, prolonged use (>3 mos), slow acetylator status Diagnosis: -Antihistone abs in >90%, ANA+ in >95% Treatment: -Stop causative agent, treat sx w/ NSAIDs -Sx resolve within wks to mos of discontinuation

Acute Bacterial Prostatitis

Presentation: -Acute onset of systemic sx (fever, chills, malaise), irritative urinary sx (dysuria, frequency), pelvic/perineal pain, cloudy urine -Difficulty urinating, acute urinary retention w/ suprapubic fullness/discomfort if prostate impinges on urethra (esp w/ h/o BPH) -Tender prostate on digital rectal exam w/ warmth/edema (distinguishes from cystitis) -Labs: leukocytosis, +urine culture & UA -Complications: prostatic abscess, bacteremia, chronic prostatitis Diagnosis: -Clinical, UA -No prostatic massage w/ prostatic fluid analysis vs. chronic bacterial prostatitis d/t risk of bacteremia & no additional diagnostic benefit Treatment/Management: -Bladder scan, then suprapubic catheter bladder decompression (urethral catheter passage can cause prostatic rupture, sepsis by translocating bacteria from prostate) -Prolonged abx for ~6 wks (fluoroquinolone like levofloxacin/ciprofloxacin or SMX-TMP) Pathophysiology: -Usually caused by colonic pathogens (e.g. E. coli, Proteus) that have contaminated urethra, enter via intraprostatic urine reflux

Erythema Nodosum

Presentation: -Acute panniculitis presenting w/ tender, nonpruritic, violaceous, erythematous nodules/plaques (usually 2-3 cm) usually on LE anterior shins, can also develop on upper extremities, buttocks, trunk, face -Does not ulcerate, usually spontaneously resolves w/o scarring in 2-8 wks, sometimes leaving behind residual hyperpigmentation -Associated w/ systemic/autoimmune disorders (esp. IBD, CD > UC), worsens during flares and resolves as flares improve -May also be associated w/ sarcoidosis, malignancy (HL), certain infections (e.g. streptococcal, endemic fungal, viral mononucleosis), certain meds (e.g. penicillins, sulfonamides, OCPs) Pathophysiology: -Delayed-type hypersensitivity rxn to antigen exposure -Biopsy w/ septal panniculitis w/o vasculitis

Hepatitis A

Presentation: -Acute-onset fever, nausea, vomiting, anorexia, RUQ pain -Few days later, dark urine (bilirubinemia) and/or pale stools (due to decreased bili excretion), pruritus -Jaundice (less common in kids), tender hepatomegaly -Risk factors: poor sanitation/hygiene, travel to endemic areas, contaminated food/water Diagnosis: -Elevated transaminases >1000 (followed by alk phos & bili elevation), anti-hepatitis A serology (anti-HAV IgM) Treatment: -Supportive/expectant, spontaneous recovery in most pts within 3-6 wks (few have prolonged course w/ cholestatic/relapsing hepatitis) -Post-exposure ppx for close contacts of infected, within 2 wks of exposure (vaccine if 40 or younger, hep A Ig if 41+) Pathophysiology: -Fecal-oral transmission, outbreaks often linked to contaminated water & food (e.g. shellfish), overcrowded homeless shelters, day cares, international travel, men who have sex w/ men, illicit drug use

Peptic Ulcer Perforation

Presentation: -Acute-onset, severe generalized abd pain followed by peritoneal signs (guarding, rebound), fever/tachycardia/tachypnea, abd distention -Often have hx of mos of intermittent, postprandial epigastric/abd pain -Can progress to sepsis/shock if untreated -Risk factors: hx PUD -Labs: +stool guaiac Diagnosis: -Abd X-ray w/ free air under diaphragm (pneumoperitoneum) -Endoscopy contraindicated in acute perforation (can worsen injury) Treatment: -Immediate surgical consultation (exlap) -IV PPIs, broad-spectrum abx (gram- coverage), fluid resuscitation, nasogastric suctioning Pathophysiology: -Full-thickness erosion of peptic ulcer through stomach/duodenal wall -> release of air & caustic gastric secretions & contents into peritoneum -> chemical peritonitis

Mucormycosis

Presentation: -Acute/severe facial pain, headache, periorbital swelling, proptosis, fever, black necrotic eschar in the nasal cavity, purulent nasal discharge, nasal congestion, sinus pain -Complications: cerebral venous thrombosis, CN infarction (e.g. facial numbness), blindness, death (mortality ~60%) -Risk factors: thrombotic disorders, advanced malignancy (e.g. hematologic), severe infections, DKA/DM, solid organ or stem cell transplant Diagnosis: -Sinus endoscopy showing necrotic invasion of palate/orbit/brain w/ bx & culture -Mucosal biopsy w/ broad noseptate hyphae with right-angle branching Treatment/Management: -Surgical debridement, liposomal amphotericin B -Elimination of risk factors (e.g. hyperglycemia, acidosis) -After clinical improvement, transition fro amphotericin to oral antifungal med (several wks of step-down therapy) Pathophysiology: -Most commonly d/t infection by Rhizopus oryzae (ubiquitous fungus), which possesses ketone reductase, which catalyzes growth in high glucose & acidic environments

Subdural Hematoma

Presentation: -Acute: gradual onset 1-2 days following injury, presents w/ impaired consciousness (e.g. AMS, coma), confusion, HA, N/V (increased ICP) -Chronic: insidious onset wks after injury, presents w/ HA, somnolence, confusion, lightheadedness, focal neuro deficits (e.g. contralateral hemiparesis), personality changes, seizures -Risk factors: cerebral atrophy (e.g. alcoholics, elderly), increased fall risk (e.g. elderly, CNS disease), infants (thin-walled vessels), anticoag Diagnosis: -Head CT w/ crescent-shaped hyperdensity (acute) or hypodensity (chronic) crossing suture lines Treatment: -Reverse/discontinue anticoag, surgical evacuation of large/symptomatic bleeds -If small may be managed conservatively Pathophysiology: -Rupture of bridging veins due to traumatic shearing forces (head injury)

Atrial Septal Defect (ASD)

Presentation: -Acyanotic lesion, wide & fixed splitting of S2 & mid-systolic ejection murmur over LUSB due to increased blood flow across pulmonic valve, mid-diastolic rumble from increased flow across tricuspid -May develop dyspnea/fatigue w/ exertion, atrial arrhythmias (e.g. AFib, AFlutter), stroke from paradoxical embolism, cerebral abscess, etc. in mid-late adolescence, early adulthood -If untreated, can lead to severe pulm HTN & R-sided HF Diagnosis: -TTE may show abrupt discontinuity of interatrial septum w/ RA & RV dilation (from volume overload d/t L -> R shunt) Pathophysiology: -Wide/fixed splitting d/t delayed closure of pulmonic valve from prolonged emptying of enlarged RV -Shunt across ASD itself is low-velocity & usually does not produce a murmur

Acute Opioid Withdrawal

Presentation: -After acute opioid cessation/dose reduction following prolonged use (after 6-12 hrs since last dose, peaking at 24-48 hrs) -GI: N/V, diarrhea, cramping, increased bowel sounds -CV: tachycardia, diaphoresis, HTN -Psych: insomnia, yawning, dysphoric mood -Myalgias, arthralgias, lacrimation, rhinorrhea, piloerection, mydriasis Treatment: -Opioid agonist like low-dose methadone (preferred) or buprenorphine--emergency management w/ methadone in IP setting allowed for up to 3 days if primary disease is medical, per federal law -Nonopioids including clonidine, adjunct meds (antiemetics, antidiarrheals e.g. loperamide, benzos, NSAIDs, baclofen for cramping)

Warfarin Skin Necrosis

Presentation: -After initiating oral warfarin, presents as dark/violaceous lesions on extremities, breast, trunk, penis that marginate over period of hrs -If untreated, lesions become edematous, purpuric, & necrotic -Risk factors: large loading dose, underlying hereditary protein C def Treatment: -Immediate warfarin cessation, administration of protein C concentrate Pathophysiology: -Acquired protein C deficiency (natural anticoagulant) upon starting warfarin -Warfarin inhibits production of anticoagulant proteins C & S -> reduction of protein C to 50% activity within first day while other procoagulant factors (II, IX, X) decline more slowly -> transient hypercoagulable state -> increased risk of VTE & skin necrosis

Anaphylaxis

Presentation: -Allergic sx affecting 2+ organ systems (skin, respiratory, GI, CV) or hypotension after exposure to allergen -CV: vasodilation -> hypotension, tissue edema, tachycardia -Respiratory: upper airway edema -> stridor, hoarseness; bronchospasm -> wheezing, sensation of throat closing, nasal discharge -Cutaneous: urticaria, pruritus, flushing, swelling of lips/tongue -GI: N/V, abd pain -Neuro: confusion, seizure -Ocular: conjunctival edema, periorbital edema -Risk factors for fatal anaphylaxis: older age, asthma, CV comorbidities, delayed epi administration -Complications: biphasic anaphylaxis (recurrence of sx after initial period of resolution, can be fatal) Diagnosis: -If dx unclear, serum tryptase or plasma histamine Treatment/Management: -IM epi (IV epi drip if necessary) -Airway management, fluid resuscitation -Adjunctive therapies: glucocorticoids, antihistamines, albuterol -Even if full recovery after epi, should be admitted to hospital for observation if severe sx at presentation (e.g. hypotension, upper airway edema, resp distress) and/or if multiple doses of epi were required d/t high risk for biphasic anaphylaxis Pathophysiology: -NSAIDs can worsen sx by causing nonimmunologic mast cell activation -Beta-blockers can worsen sx by leading to unopposed alpha stimulation

Alpha Thalassemia

Presentation: -Alpha thalassemia minima = aa/a- (one gene loss) = asymptomatic, carrier -Alpha thalassemia minor = aa/-- or a-/a- (2 gene loss) = mild microcytic anemia -Hemoglobin H disease aka alpha thalassemia major = a-/-- (3 gene loss) = chronic hemolytic anemia -Hemoglobin Barts (hydrops fetalis) = --/-- (4 gene loss) = high-output cardiac failure, anasarca, death in utero, severe hypoxemia Diagnosis: -Hemoglobin H: microcytic anemia (erythrocytosis, but cells are small), reticulocytosis, extra newborn screen band representing gamma tetramers on Hgb electrophoresis, target cells on peripheral smear -Low MCV but high/normal ferritin & iron, high bilirubin, normal RDW & RBC count Pathophysiology: -In Hg Barts, fetuses form homotetramers of gamma globin due to absence of alpha gene, which has extreme affinity for O2 -> severe fetal hypoxemia -> high-output cardiac failure -> hydrops fetalis, death -In Hemoglobin H disease, fetuses/infants form homotetramers of gamma globulin at birth, transitions to homotetramers of beta chains as children/adults (hemoglobin H)

HIV-Associated Lipodystrophy

Presentation: -Alterations in fat deposition w/ no change in lean tissue mass, including: -Lipoatrophy: loss of SC fat from face, arms, legs, abdomen, and/or buttocks, which can give pts a skeleton-like appearance; associated w/ NRTIs in particular (e.g. stavudine, zidovudine) -Fat aaccumulation: dorsocervical fat accumulation leading to buffalo hump & visceral abdominal fat accumulation -> increased abd girth despite minimal SC abd fat, hepatomegaly; not clearly associated w/ any particular antiretroviral drug and can occur w/ any regimen Treatment/Management: -Lifestyle modifications, possible additional statin treatment if 10 yr CV risk >7.5-10% Pathophysiology: -Abnormal lipid/glucose metabolism -> insulin resistance, dyslipidemia, increased risk of CV disease -Antiretroviral therapy can cause dyslipidemia via increased cholesterol, LDL, TGs, & reduced HDL

Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD)

Presentation: -Anemia w/ episodic hemolysis exacerbated by oxidant stress (triggers include sulfonamides, fava beans, infection) -Jaundice, splenomegaly, scleral icterus, dark urine, back/abd pain, pallor, fatigue -Familial hx (X-linked recessive), esp in African descent (also Mediterranean, Middle Eastern, Asian) -Labs: normocytic anemia, reticulocytosis, negative Coombs, low MCHC (vs. hereditary spherocytosis), indirect hyperbili, Heinz bodies & bite cells on smear, high LDH, decreased haptoglobin, blood+ on UA but no RBCs on microscopy Diagnosis: -Decreased G6PD activity (may be normal during attack, should repeat 3 mos after) Treatment: -Avoidance of hemolytic triggers (infections, fava beans, medications), supportive care -Meds to definitely avoid: dapsone, isobutyl nitrite, nitrofurantoin, primaquine, rasburicase -Meds to use w/ caution: acetaminophen, aspirin, benadryl, SMX-TMP Pathophysiology: -X-linked erythrocyte glycolytic RBC enzyme deficiency -Inadequate NADPH production -> inadequate glutathione -> accumulation of oxidative metabolites (superoxide, hydrogen peroxide) in erythrocytes -> denaturing/precipitation of Hgb into Heinz bodies -> predisposes to RBC damage, lysis in RES -> bite cells -Predominantly presents in males, but can present in females w/ homozygous mutations or skewed lyonization of X chromosomes

Diffuse Alveolar Hemorrhage

Presentation: -Anemia, hemoptysis, hypoxia & respiratory distress, diffuse alveolar infiltrates Pathophysiology: -Alveolar filling process -Etiologies: Ebola and hantavirus, rheumatologic conditions (e.g. Goodpasture, GPA, Churg-Strauss, Behcet, antiphospholipid antibody), crack cocaine use

Fabry Disease

Presentation: -Angiokeratomas, telangiectasias, peripheral neuropathy, asymptomatic corneal dystrophy, renal/heart failure (glomerular disease), elevated risk of thromboembolic events Pathophysiology: -Alpha-galactosidase deficiency

Uremia

Presentation: -Anorexia, N/V, confusion, lethargy, pruritus -Labs: CrCl usually <10 w/ Cr >7, electrolyte derangements (e.g. hyperkalemia), acidosis Diagnosis: -BUN >50 Treatment: -Dialysis Pathophysiology: -Renal failure -> reduced renal clearance of uremic toxins -> fluid, electrolyte, metabolic derangements

Acetaminophen Toxicity

Presentation: -Anorexia, malaise, pallor, diaphoresis, nausea/vomiting at 0-24 hrs; RUQ pain, LFT abnormalities at 24-48 hrs; encephalopathy, coagulopathy (bleeding), hypoglycemia, extreme LFT elevation at 48-96 hrs; declining hepatic enzymes, necrosis at >96 hrs -Labs: lactic acidosis, hyperbili, AKI Diagnosis: -Check serum acetaminophen levels Treatment/Management: -N-acetylcysteine (most effective when given prior to onset of hepatotoxicity, within 8 hrs on Rumack-Matthew line) -Charcoal no longer routinely used (before was used for 7.5+ g ingestion 4 hrs ago or earlier)

Thymoma

Presentation: -Anterior mediastinal mass, usually in middle-aged adult -Mass effect compression of other structures -> cough, SOB, postural wheezing (esp while lying down) -Paraneoplastic syndromes common (e.g. myasthenia gravis w/ abnormal levels of anti-ACh receptor abs)

Tick-Borne Paralysis

Presentation: -Ascending flaccid paralysis over hrs to days (rapid compared to GBS), may be localized or more pronounced in 1 leg or arm, weakness & loss of DTRs but normal cranial nerves, gait ataxia -Usually no fever, normal sensation, no autonomic dysfxn (vs. GBS) -Labs: normal CSF, normal WBCs Diagnosis: -Meticulous skin examination Treatment: -Tick removal (improvement of sx usually seen within an hr, complete recovery in days) Pathophysiology: -Neurotoxin release by tick (requires 4-7 days of feeding prior to release of neurotoxin) -In US, most commonly d/t Dermacentor andersoni (Rocky Mountain wood tick) & D variabilis (American dog tick)

Acute Hyperkalemia

Presentation: -Ascending muscle weakness w/ flaccid paralysis -ECG changes: T wave peaking, short QT interval, PR prolongation -> disappearance/flattening of P wave -> widened QRS -> sine wave w/ VFib (conduction delay), conduction blocks (-> bradycardia), ectopy/arrhythmias (e.g. VTach) -> asystole Treatment: -If ECG abnormalities: calcium gluconate/chloride first (stabilize cardiac membranes) -Next, insulin w/ glucose, beta-2 adrenergic agonists (e.g. albuterol), sodium bicarb--shift K+ intracellularly -K+ removal by: diuretics (e.g. furosemide), GI cation exchangers (e.g. patiromer), hemodialysis -Review medications Pathophysiology: -Most common etiologies are acute or chronic kidney disease, meds that impair RAAS, missed dialysis session -Meds: ACEi, ARBs, cyclosporine, digitalis, heparin, nonselective beta-blockers, NSAIDs, K+-sparing diuretics, succinylcholine, trimethoprim -Increased K+ movement out of cells: uncontrolled hyperglycemia, metabolic acidosis -Increased tissue catabolism: trauma, tumor lysis syndrome

Portal Hypertension

Presentation: -Ascites, peripheral edema, hepatomegaly, splenomegaly, spider angiomata, scleral icterus, esophageal/rectal varices (hematemesis, hematochezia), caput medusae -Complications: SBP Pathophysiology: -May be due to intrinsic liver disease (e.g. viral hepatitis, cirrhosis), right HF, extrahepatic portal vein thrombosis -In intrinsic liver disease, increased venous pressure below diseased liver due to high-resistance portal circulation -> portal HTN -Portal HTN associated w/ increased NO and other vasodilatory factors (may be from bacterial products that accumulate due to reduced host defenses, increased portosystemic shunting resulting in decreased clearance) -> splanchnic vasodilation -> decreased SVR -> compensatory increase in HR & CO (hyperdynamic circulation) -Third-spacing of fluid from ascites + decreased renal perfusion from splanchnic vasodilation -> activation of RAAS -> Na+ & water retention -Hypoalbuminemia from intrinsic liver disease + hyperdynamic blood flow cause movement of fluid into peritoneal cavity

Echinococcus Granulosus

Presentation: -Associated w/ contact w/ dog feces -Initially asymptomatic (often for yrs until rupture or size 10+ cm), then may present w/ liver cyst (most common) or lung cyst -Liver cyst causes sx of mass effect (RUQ pain, n/v, hepatomegaly) and w/ rupture (fever, eosinophilia, anaphylaxis) -Lung cyst causes cough, chest pain, hemoptysis -Risk factors: immigrant, exposure to sheep/dogs (e.g. SW US) Diagnosis: -Imaging (US) w/ large (often >10 cm), smooth, unilocular hydatid cyst, often w/ internal septations/daughter cysts, "eggshell" calcifications on CT -Cysts may also be found in other organs (e.g. lung, muscle, bone) -IgG E granulosus serology Treatment: -Albendazole if <5 cm, percutaneous therapy if large or complex cyst (>5 cm or w/ septations), surgery if ruptured -Cysts must be injected w/ ethanol or hypertonic saline solution prior to removal b/c can precipitate anaphylactic rxn Pathophysiology: -Dog tapeworm endemic to rural/developing countries (South America, Middle East), esp high in areas of sheep farming b/c sheep are intermediate hosts, dogs (definitive host) acquire by eating sheep viscera -Humans acquire infection via water contaminated w/ dog feces -> eggs hatch in small intestine -> penetrate through intestinal wall -> travel to liver/lungs to form 1+ hydatid cyst -Vs. entamoeba, no fever unless rupture, may be asymptomatic for yrs

Wernicke Encephalopathy

Presentation: -Associated w/ deficiency from alcoholism (most common), malnutrition (e.g. anorexia, bowel resection), hyperemesis gravidarum -Encephalopathy (AMS, lethargy, disorientation), oculomotor dysfxn (horizontal nystagmus, bilateral abducens palsy/ophthalmoplegia), postural/gait ataxia (wide-based), loss of reflexes -If hyperemesis gravidarum, may be associated w/ hypochloremic metabolic alkalosis, hypokalemia, hyponatremia, and elevated transaminases, all due to protracted vomiting -Risk factors: alcoholism, malnutrition -Complications: Korsakoff syndrome (late-stage, esp w/ alcoholism, presents w/ retrograde/anterograde amnesia, confabulation) Diagnosis: -MRI demonstrating mammillary body atrophy, periaqueductal gray matter hyperintensity Treatment: -Antiemetics, fluid resuscitation, thiamine supplementation (high dose infusion), glucose infusions (after thiamine supplementation) Pathophysiology: -Thiamine deficiency -Can be precipitated in malnourished pts w/ administration of dextrose (resulting in depletion of remaining stores of thiamine)

Milk-Alkali Syndrome

Presentation: -Associated w/ excessive intake of calcium & absorbable alkali (e.g. calcium carbonate in antacids, for osteoporosis) -N/V, constipation, polyuria, polydipsia, neuropsych sx -Labs: hypercalcemia, metabolic alkalosis (due to increased bicarb from increased intake & decreased excretion), AKI, suppressed PTH, hypophosphatemia (due to intestinal binding by calcium bicarb), hypomagnesemia (decreased renal absorption) -Risk factors: CKD, use of thiazides (promotes calcium retention, volume depletion), ACEi, NSAIDs Treatment/Management: -Discontinuation of causative agent, isotonic saline followed by furosemide Pathophysiology: -Excessive intake -> renal vasoconstriction, decreased GFR -Inhibition of Na-K-Cl cotransporter due to activation of calcium-sensing receptors in thick ascending loop + impaired activity of ADH -> renal loss of Na+ & water, reabsorption of bicarb

Candidal Skin Lesions

Presentation: -Associated w/ hematogenous dissemination of Candida (e.g. associated w/ endophthalmitis, sepsis) -Lesions manifest as grouped pustules on erythematous base w/ or w/o necrotic centers

Analgesic Nephropathy

Presentation: -Associated w/ long-term use of 1+ analgesics (e.g. aspirin, ibuprofen) for chronic HAs or other somatic complaints -Usually asymptomatic, but can have chronic tubulointerstitial nephritis or painless hematuria d/t papillary necrosis, polyuria, HTN -If significant papillary necrosis, sloughing can cause renal colic -Most common in females 50-55 yo -Associated w/ increased risk of premature aging, atherosclerotic CV disease, urinary tract cancer Diagnosis: -Elevated Cr w/ UA showing hematuria or sterile pyuria, WBC casts (similar to AIN), sterile urine culture -May have mild proteinuria <1.5 g/day, can also present w/ nephrotic range proteinuria -CT w/ small kidneys, bilat renal papillary calcifications if papillary necrosis Pathophysiology: -Analgesic-induced vasoconstriction of medullary blood vessels (vasa recta) -> papillary necrosis, sloughing

Occupational Asthma

Presentation: -Asthmatic sx (coughing, wheezing, dyspnea) closely linked to workplace -Once sx become chronic, they persistent throughout work wk, abating after sustained absence from workplace Diagnosis: -Workplace-specific change in airway physiology (e.g. measurement of peak expiratory flows at workplace vs. home, serial sputum eosinophil counts, in-workplace spirometry, serial methacholine bronchoprovocation testing) -Often normal spirometry, exam, CXR Treatment: -Antigen avoidance (removal from workplace preferred) or reduction (e.g. respirator use, rotation scheduling) -Bronchodilators & inhaled corticosteroids, desensitization therapy, immunotherapy Pathophysiology: -Antigens -> inflammation (IgE dependent or independent) -> reversible bronchoconstriction -Animal proteins (e.g. veterinary/laboratory animal workers, seafood processors), grain antigens (e.g. bakers, food processors), isocyanates (e.g. painters, epoxies)

Atrial Fibrillation

Presentation: -Asymptomatic or episodes of palpitations +/- dyspnea, fatigue, CP, lightheadedness, syncope -Irregularly irregular pulse -Increased risk of thromboembolic events from LA thrombus (e.g. stroke) -Risk factors: age (65+), HTN, mitral valve dysfxn, LV failure, CAD & CAD risk factors (e.g. smoking, T2DM), obesity, OSA, chronic hypoxic lung disease (e.g. COPD), ASD, hyperthyroidism, excess EtOH use, increased sympathetic tone (e.g. acute illness like PE/MI/sepsis, cardiac surgery), sympathomimetic drug use (e.g. cocaine) Diagnosis: -ECG w/ variable R-R intervals & no P waves -Continuous ECG (e.g. zio patch) often needed for paroxysmal disease (intermittent arrhythmia) -Echo may show LVH, LA enlargement Treatment/Management: -If CHADS-VASc of 1, aspirin or oral anticoag or no treatment -If CHADS-VASc of 2+, oral anticoag (NOACs incl apixaban, dabigatran, rivaroxaban, edoxaban) -Rate control w/ beta blockers (metoprolol) & CCBs (can use digoxin if unable to tolerate beta blockers & CCBs) -Rhythm control indicated if unable to achieve adequate control of rate with above agents, if recurrent symptomatic episodes (e.g. palpitations, lightheadedness, dyspnea, angina) despite rate control therapy, or if HF sx -Antiarrhythmic therapies: flecainade or propafenone if no CAD or structural heart disease, dronedarone or amiodarone if LVH, sotalol or dronedarone if CAD w/o HF, amiodarone or dofetilide if HF -If hemodynamically unstable, cardioversion Pathophysiology: -Atrial remodeling due to age-related myocardial changes, atrial enlargement from heart disease (e.g. HTN, mitral stenosis, MI, CAD) w/ atrial fibrosis, atrial conduction remodeling -Foci of rapidly electrical atrial activity that commonly originate in pulmonary veins, may worsen w/ EtOH intake & increased sympathetic drive

Vitamin D Deficiency

Presentation: -Asymptomatic or nonspecific MSK sx -Low bone density, bone fxs, fatigue -Osteomalacia: bone pain, muscle weakness, abnormal gait, deformity of weigh-bearing bones -Risk factors: decreased sun exposure (e.g. nocturnal occupation, nursing home residence, northern latitudes), heavy skin pigmentation, obesity, malabsorption, advanced age, CKD, change in dietary intake (found in fortified dairy products, oily fish) Diagnosis: -Serum 25-hydroxyvitamin D levels (insufficiency defined as 20-30 ng/mL, deficiency defined as <20) Treatment: -Supplementation

Hepatitis C

Presentation: -Asymptomatic or nonspecific sx (e.g. fatigue, anorexia, arthralgias/myalgias), hepatomegaly (nodular, shrunken liver on US) -Hematologic: mixed cryoglobulinemia (palpable purpura, GN, low complement), ITP, AIHA -Renal: membranoproliferative GN -Derm: porphyria cutanea tarda, lichen planus, leukocytoclastic vasculitis -Complications: cirrhosis, hepatocellular carcinoma -Labs: elevated transaminases (often normal in chronic infection), thrombocytopenia (in cirrhosis, ITP), false+ ANA & RF, hypoalbuminemia, coagulopathy (elevated PT/INR) Diagnosis: -Positive hepatitis C IgG antibody (indicates exposure, current or past infection but takes 2-6 mos to develop), followed by confirmation testing for HCV RNA (indicates chronic disease, active infection) -Evaluation to determine HCV genotype, extent of liver fibrosis -Liver bx shows portal triad lymphocytic infiltration, lymphoid follicles, fibrosis & regenerative nodules (cirrhosis) Treatment/Management: -Direct-acting antiviral agents (e.g. ledipasvir-sofosbuvir) -Surveillance abd US for HCC -Vaccination against HAV & HBV if no preexisting immunity (d/t risk of superimposed infection) -Screening for complications/comorbidities (liver fibrosis, renal disease, cryoglobulinemia) -Substance abuse & EtOH cessation counseling

Type I Cryoglobulinemia

Presentation: -Asymptomatic usually -Sx of hyperviscosity (e.g. blurry vision, vertigo, ataxia), thrombosis (e.g. Raynaud, digital ischemia) -Skin: livedo reticularis, retiform purpura (net-like), digital ischemia -Labs: normal complement, negative RF Diagnosis: -Positive cryocrit, normal complement, elevated ESR, +monoclonal spike on SPEP Pathophysiology: -Associated w/ lymphoproliferative & hematologic disorders (e.g. multiple myeloma, Waldenstrom macroglobulinemia, other plasma cell dyscrasias/B cell lineage malignancies) -Monoclonal IgM forms cryoprecipitates -> vascular occlusion

Cerebellar Gait

Presentation: -Ataxic, staggering/swaying side to side, wide-based gait -May be associated w/ dysdiadochokinesia, dysmetria, nystagmus, +Romberg sign, impaired tandem gait, titubation (truncal tremor) Pathophysiology: -May be caused by cerebellar degeneration, stroke, drug/EtOH intoxication, B12 def -Truncal ataxia if involving vermis, limb ataxia if involving hemispheres

Mixed Connective Tissue Disease (MCTD)

Presentation: -Autoimmune disorder w/ variable features of SLE, polymyositis, & systemic sclerosis (e.g. sclerodactyly, esophageal dysmotility) -Diagnostic criteria include Raynaud phenomenon, hand/finger swelling, inflammatory arthritis/synovitis (similar to RA), inflammatory myopathy -Other sx: pulm HTN (most common cause of death), malar/discoid rash, mild CNS (e.g. CN neuropathies) and/or renal disease -Labs: elevated CK, anemia/cytopenias, may be + for ANA, RF, anti-CCP Diagnosis: -Positive for anti-U1 ribonucleoprotein Treatment/Management: -Systemic glucocorticoids, corticosteroid-sparing agents e.g. methotrexate

Brugada Syndrome

Presentation: -Autosomal dominant disorder most common in Asian males -ECG pattern of pseudo-right bundle branch block and ST elevations in V1-V3. -Increased risk of ventricular tachyarrhythmias and SCD (esp may be triggered by fever/infection), most commonly at night (may report nocturnal palpitations) Treatment/Management: -Prevent SCD with implantable cardioverter-defibrillator (ICD)

Dubin-Johnson Syndrome

Presentation: -Autosomal recessive condition, benign hereditary hyperbilirubinemia -Conjugated hyperbilirubinemia, usually asymptomatic apart from scleral icterus & dark urine (bilirubinuria) starting in adolescence/early adulthood, no long-term sequelae -Scleral icterus may be triggered by illness, pregnancy, OCPs Diagnosis: -Liver bx not required for dx, but shows grossly black liver & dark, granular pigment accumulation within hepatocytes (vs. normal bx in Rotor syndrome) Treatment: -Reassurance

Non-Hodgkin Lymphoma (NHL)

Presentation: -B symptoms (intermittent fever, night sweats, weight loss), >70% present w/ painless peripheral lymphadenopathy, hepatosplenomegaly -Risk factors: EBV infection, HIV Pathophysiology: -In HIV pts, associated w/ reactivation of oncogenic viruses like EBV associated w/ immunosuppression

Anorexia Nervosa

Presentation: -BMI <18.5, fear of weight gain, distorted body image, distress at having to limit physical activity -Decreased BMD/osteoporosis, stress fxs, amenorrhea, lanugo, hair loss, dry skin, constipation, gastroparesis (postprandial nausea, early satiety), GERD, enlarged parotid glands (if binge/purge type), cold intolerance, hypotension/hypothermia/bradycardia, cardiac atrophy, arrhythmias -Labs: hypokalemia, hyponatremia, hypomagnesemia, metabolic alkalosis, elevated LFTs Diagnosis: -Comprehensive dietary hx Treatment: -Nutritional rehab, psychotherapy Pathophysiology: -Decreased food intake, electrolyte abnormalities -> slowed colonic transit time -> constipation

Varicocele

Presentation: -Bag of worms/soft scrotal mass finding on exam/US, NO TRANSILLUMINATION, usually present in adolescents/young adults, more common on L side -Often asymptomatic or mild/dull pain worse w/ standing/valsalva, smaller/improved when supine -Associated w/ subfertility, testicular atrophy Diagnosis: -US w/ retrograde venous flow, tortuous/anechoic tubules adjacent to testis, dilation of pampiniform plexus veins -If R-sided, may be a sign of malignant compression (e.g. RCC), warrants CT scan Treatment/Management: -If testicular atrophy/infertility, gonadal vein ligation in boys/young men w/ testicular atrophy -Scrotal support & NSAIDs in older men w/ no concerns about fertility or if asymptomatic Pathophysiology: -Dilation of pampiniform plexus within spermatic cord -Subfertility may be due to mildly increased scrotal temperature -More common on L side b/c L spermatic vein drains into L renal vein (susceptible to compression b/t superior mesenteric artery & abd aorta) -Unilateral R sided varicoceles rare, should raise suspicion for thrombosis or malignant compression

Basilar Migraines

Presentation: -Basilar/brainstem aura sx (vertigo, dysarthria, ataxia, tinnitus, diplopia & other vision disturbances) w/o motor weakness, followed by severe migraine-type HA (throbbing, usually occipital) -May be associated w/ loss of consciousness

Cardiac Tamponade

Presentation: -Beck triad: hypotension, JVD, muffled heart sounds, nonpalpable pt of max impulse -Evidence of shock w/ hypoperfusion (reduced urine output, cool extremities, AMS) -Pulsus paradoxus (SBP decrease of 10+ mm Hg w/ inspiration, e.g. loss of palpable radial pulse w/ inspiration), +hepatojugular reflex -May be due to blood in pericardial space (e.g. LV rupture, cardiac surgery) or pericardial effusion (e.g. malignancy, infection, uremia) -Can precipitate obstructive shock (CVP/PCWP/SVR increased, CI/output decreased), syncopal episodes -If acute tamponade, develops over mins-hrs, effusion of ~100-200 mL, normal cardiac silhouette -If subacute tamponade, develops over days-wks, effusion of 1-2 L, enlarged & globular cardiac silhouette, associated w/ slowly progressive causes (e.g. malignancy, renal failure) Diagnosis: -ECG w/ low-voltage QRS complex, electrical alternans -CXR w/ enlarged cardiac silhouette (IF ACUTE MAY HAVE NORMAL CARDIAC SILHOUETTE), clear lungs -Echo w/ early diastolic RA & RV collapse, IVC plethora, bowing of intraventricular septum into LV during inspiration -Elevation & equalization of intracardiac diastolic pressures (PCWP, RA, RV) on pulmonary artery catheterization Treatment: -IV fluids to increase right-sided preload -Drainage via pericardiocentesis or pericardial window Pathophysiology: -Affects R side of heart more than L side (b/c R sided chambers are lower pressure) -> absence of pulmonary edema -As tamponade progresses, diastolic pressures across all chambers increase & equalize w/ pericardial pressure -> impaired blood flow, decreased ventricular preload -> decreased SV, CO -Inspiration -> decreased intrathoracic pressure -> increased venous return to R heart -> expansion of R ventricle & bowing of interventricular septum into LV -> decreased LV filling -> decreased SBP -> pulsus paradoxus

Pyoderma Gangrenosum

Presentation: -Begins w/ small papule/pustule -> rapidly progressive, painful ulcer w/ purulent base & irregular, violaceous border -Single or multiple lesions, usually on trunk or LE-30% of time triggered by local trauma (pathergy) -Peak onset 40-60 yo, F>M, associated w/ IBD, RA, other autoimmune arthropathies, hematologic malignancy, vasculitis (e.g. GPA) Diagnosis: -Exclusion of other causes of ulceration (e.g. infection, venous ulcer, cutaneous cancer), skin biopsy of ulcer margin w/ neutrophilic infiltrate, mixed cellular infiltrate w/ dermal & epidermal necrosis Treatment: -Local/systemic glucocorticoids -Surgical debridement typically avoided due to potential of inducing pathergy

Subconjunctival Hemorrhage

Presentation: -Benign condition w/ collection of thin layer of blood on surface of eye lateral to the iris -May be caused by violent coughing, sneezing spells, minor trauma (e.g. rubbing eyes, contact lens use), or may appear w/o cause -Asymptomatic w/o disturbance of vision, otherwise normal eye examination, no pain/discharge/itching/photophobia -Over days, affected area may increase in size d/t redistribution of extravasated blood within tight subconjunctival space; subsequently spreads, becomes yellowish in color & fades, disappears completely within 1-3 wks -No short or long-term complications Treatment/Management: -Reassurance Pathophysiology: -Rupture of small conjunctival blood vessels -> blood collection b/t sclera & conjunctiva

Simple Renal Cyst

Presentation: -Benign, often incidental finding on imaging, most common in pts >50 -Usually not associated w/ sx (e.g. infection, HTN, flank pain, hematuria, proteinuria -CT/MRI features: sharply demarcated, round, thin/smooth/regular wall, unilocular, not septated, homogenous internal contents, absence of contrast enhancement Treatment/Management: -None, reassurance

Hypertensive Nephrosclerosis

Presentation: -Bilat small/atrophic kidneys on US, other findings of chronic HTN (e.g. S4, strong apical impulse, LVH) -Over time can lead to anemia from decreased EPO, other manifestations of CKD -Azotemia usually slowly progressive & irreversible -Labs: elevated Cr & BUN, bland UA (no WBCs, RBCs, casts), trace proteinuria (<1 g) -Risk factors: comorbid T2DM, severe HTN, African American Diagnosis: -Clinical, bx not usually necessary Pathophysiology: -Chronic HTN -> medial hypertrophy & intimal fibrosis of renal arterioles -> endothelial damage -> plasma protein & basement membrane material deposition into arterial walls (hyaline arteriolosclerosis) -> luminal narrowing -> decreased renal blood flow -> glomerula ischemia, glomerular & tubular fibrosis & atrophy (glomerulosclerosis)

Central Herniation

Presentation: -Bilateral midposition & fixed pupils (loss of both parasympathetic & sympathetic tone) -Decorticate posturing (flexor) -> progresses to decerebrate (extensor) posturing -Unconsciousness (dysfxn of ascending reticular activating system responsible for arousal) Treatment: -Treat underlying cause, reduce ICP to prevent tonsillar herniation (can cause cardiac/respiratory arrest) Pathophysiology: -Caudal displacement of diencephalon & brainstem through tentorium cerebelli -Rupture of paramedian basilar artery branches -Abnormal limb positioning d/t disruption of descending tracts (rubrospinal, vestibulospinal) typically involved in postural movements

Acute Bacterial Rhinosinusitis

Presentation: -Bilateral, purulent nasal discharge, inflamed nasal mucosae, fever, cough, ill-appearing, nasal congestion/obstruction, maxillary tooth discomfort -Facial pain/headache/pressure worse w/ bending forward, sinus tenderness to palp (common in adults but may not be seen in children) -Diagnostic criteria (need 1 of 3): persistent sx for 10+ days w/o improvement, severe onset (fever of 39/102.2+ and drainage) for 3+ days, worsening sx 5+ days following initial improvement after viral URI -Risk factors: viral URI (most common), allergic rhinitis, environmental irritant exposure (e.g. cigarette smoke, pool chemicals, air pollution), nasal septal/palatal structural abnormalities Treatment: -1st line: amoxicillin + clavulanate -2nd line: doxycycline, fluoroquinolones -Supportive: analgesics, decongestants, saline irrigation, topical glucocorticoids -Not recommended d/t resistance: amox, macrolides, TMP-SMX, 2nd/3rd gen cephalosporins Pathophysiology: -Infection of paranasal sinuses, most commonly by nontypeable Haemophilus influenzae, Strep pneumo, or Moraxella catarrhalis -Strep pneumo less common now due to vaccine -Thickened secretions and mucosal inflammation (often from initial viral URI or allergic rhinitis) prevent sinus drainage and impair mucociliary clearance of contaminating bacteria -> secondary infection

Type 1 Diabetes Mellitus

Presentation: -Bimodal age distribution: peaks at 4-6 yo and 10-14 yo -Polyuria, polydipsia, nocturia, enuresis/urinary incontinence, dehydration (dry mucous membranes), weight loss, fatigue, blurred vision Diagnosis: -Serum and/or urine glucose Treatment: -Insulin replacement: -Start w/ basal-bolus insulin (long acting like glargine or intermediate acting like NPH insulin once or twice daily to provide baseline coverage) -Premeal bolus insulin (short acting like regular or rapid-acting insulin prior to meals) -Once well-established glycemic control w/ above, can either remain on multiple daily injection therapy or convert to continuous insulin infusion pump Pathophysiology: -Autoimmune destruction of pancreatic beta islet cells

Cauda Equina Syndrome

Presentation: -Bladder/bowel incontinence (e.g. urinary hesitancy, dribbling), sexual dysfxn, saddle anesthesia (sensation loss around perineum/buttocks/perianal region), decreased rectal sphincter tone, bilateral LE motor/sensory/reflex abnormalities (LMN signs including weakness, loss of reflexes, decreased muscle tone) -Severe back pain radiating into one or both legs -Positive straight leg test in >90% Diagnosis: -Urgent lumbosacral spinal MRI Treatment: -Surgical decompression within 24-48 hrs to prevent permanent neurologic sequelae -IV glucocorticoids Pathophysiology: -Centrally herniated lumbar disc (L4-S1 into lumbar cistern) -> multiple compressed nerve roots (cauda equina includes L2-L5 + S1-S5 + coccygeal nerve) -Bladder & rectal sphincter paralysis from damage to S3-S5 nerve roots -Loss of ankle reflexes from damage to S1-S2 -Etiologies: most commonly d/t large/central lumbar disc herniation, may also be d/t epidural tumors (e.g. metastasis), epidural abscess, inflammatory/infiltrative disease (e.g. sarcoidosis)

Disseminated Intravascular Coagulation (DIC)

Presentation: -Bleeding from mucosal surfaces, surgical sites, and IV lines, purpuric rash -May be associated w/ obstetric complications (maternal sepsis, preE, amniotic fluid embolism, postpartum hemorrhage, placental abruption), malignancy (esp gastric, breast, lung), severe traumatic injury, sepsis -Sx similar to PE, w/ dyspnea, chest pain, tachypnea -Organ damage common (e.g. renal insuff w/ decreased urine output) Diagnosis: -Thrombocytopenia, prolonged PT/INR & PTT, decreased fibrinogen, increased D-dimer, microangiopathic hemolytic anemia (schistocytes, formed due to shearing of RBCs by thrombi) -> elevated total bili, elevated LDH, reticulocytosis Treatment: -Treatment of underlying etiology (e.g. uterine atony in PPH) -Blood product resuscitation (pRBCs, FPP) Pathophysiology: -Procoagulant excessively triggers coagulation cascade -> formation of fibrin/platelet-rich thrombi (paradoxical thrombosis) & fibrinolysis -> bleeding & organ damage (e.g. kidneys, lungs) -Consumptive coagulopathy

Traumatic Hyphema

Presentation: -Blood within anterior chamber, usually due to blunt force to eye (e.g. baseball, paintball, airbag deployment, etc.), less commonly d/t penetrating trauma -Vision loss, eye pain, photophobia, layering of blood in anterior chamber, anisocoria (unequal pupils) -Complications: rebleeding, intraocular HTN -> optic nerve atrophy (glaucoma), permanent vision loss Treatment/Management: -Ophtho consultation (serial measurement of intraocular pressure, cyclopegic/glucocorticoid eye drops) -Rigid eye shield, sometimes soft patching to block ambient light -Bed rest (elevate head to keep blood away from visual axis & aid in clearance) & admission for large hyphema (e.g. >50% of pupil obscured) or if risk factors for rebleeding (e.g. anticoag use, sickle cell trait/disease) -Surgical intervention (e.g. clot evacuation) if intraocular HTN refractory to topical/systemic therapies Pathophysiology: -Acute increase in intraocular pressure -> blood vessel rupture in ciliary body & iris -If spontaneous, may be associated w/ underlying bleeding disorder like vWD or microvascular disorder like DM

Choroidal Rupture

Presentation: -Blurred vision following blunt ocular trauma Diagnosis: -Fundoscopy w/ central scotoma, retinal edema, hemorrhagic detachment of macula, subretinal hemorrhage, crescent-shaped streak concentric to optic nerve

Hypertrophic Osteoarthropathy

Presentation: -Bone & soft tissue enlargement in distal extremities -Clubbing, arthropathy w/ skin thickening, periostosis of long bones (excessive bone formation), joint effusions Pathophysiology: -Paraneoplastic syndrome of pulmonary malignancy, primarily adenocarcinoma, intrathoracic malignancies, CF, other pulmonary disease (e.g. bronchiectasis, chronic pulm infection) -Clubbing: megakaryocytes skip normal route of fragmentation within pulm circulation, become entrapped in distal fingers & release platelet-derived growth factor (PDGF) & vascular endothelial growth factor (VEGF) -> growth promoting properties increase tissue hypertrophy, capillary permeability & vascularity

Tertiary Hyperparathyroidism

Presentation: -Bone cysts, brown tumors, bone pain, pruritus -Calciphylaxis: vascular calcification w/ skin necrosis -Only seen in pts w/ ESRD (very elevated Cr, often on dialysis) -Labs: hypercalcemia, extremely high PTH (>800), elevated alk phos (high bone turnover) Treatment/Management: -Parathyroidectomy indicated w/: persistently elevated Ca (>10.5), phos, or PTH (>800) levels, soft tissue calcification or calciphylaxis, intractable bone pain/pruritus Pathophysiology: -Results when parathyroid activity becomes autonomous and excessive in patients with secondary hyperparathyroidism, leading to autonomously functioning adenomas that produce PTH; results in hypercalcemia & metabolic bone disease

Multiple Myeloma

Presentation: -Bone pain worsened by movement, absent at night, weight loss, fatigue -X-rays w/ osteolytic lesions -Rapidly progressive generalized bone loss, may present w/ fragility fx -Labs: normal alk phos, normocytic anemia, hypercalcemia, renal failure (intrinsic AKI, elevated Cr, UA may have glassy light chain/granular casts, proteinuria may be absent as UA only detects albumin, no hematuria), low PTH, elevated protein gap indicating monoclonal gammopathy (serum total protein - serum albumin >4), high total protein, blood smear w/ rouleaux formation (stacking of RBCs), elevated ESR (d/t increased plasma viscosity from paraproteinemia) -Complications: acute/chronic kidney injury, pathologic fxs, infection (esp bacterial URIs & UTIs d/t hypogammaglobulinemia), hyperviscosity syndrome (nasal/oral bleeding, blurry vision, neurologic sx, HF), arterial/venous thrombosis -Mnemonic: CRAB--calcium, renal insuff, anemia, lytic bone lesions Diagnosis: -Skeletal survey w/ osteolytic lesions, osteopenia -Serum protein electrophoresis/SPEP (w/ monoclonal M-spike of IgG, IgA, or light chains), serum free light chain analysis or urine protein electrophoresis (UPEP) -Diagnosis confirmed w/ bone marrow bx w/ >10% clonal plasma cells Treatment/Management: -Whole-body low dose CT, MRI or PET to locate/quantify bone lesions -Hypercalcemia management w/ hydration & dexamethasone if mild, bisphosphonates if moderate/severe -Renal insuff may eventually require plasmapheresis or dialysis -Plasmapheresis for hyperviscosity syndrome -PPx for infections (highest risk first 3-4 mos of therapy) w/ vaccine, ppx abx during therapy Pathophysiology: -Plasma cell dyscrasia -> increased monoclonal paraprotein production (of Ig) -Increased levels of osteoclast-activating factor secreted by neoplastic cells -> generalized bone loss -IgG/IgA/light chain monoclonal antibodies -Monoclonal light chains form intratubular casts within renal tubules -> myeloma cast nephropathy, deposition disease -> can eventually cause normocytic anemia d/t chronic kidney damage -Other causes of renal insuff include myeloma amyloidosis, monoclonal Ig deposition disease (MIDD), both cause glomerular injury & nephrotic syndrome -Hyperviscosity syndrome: IgM clogs microcirculation of brain -> HA, dizziness, vertigo, nystagmus, hearing loss, vision impairment, impaired platelet fxn -> mucosal hemorrhage

Tourette Syndrome

Presentation: -Both multiple motor & 1+ vocal tics (not necessarily concurrent, >1 yr); tics preceded by irresistible urges, followed by feelings of relief, exacerbated by stress/fatigue/anxiety, tend to subside during sleep -Motor tics: facial grimacing, blinking, head/neck jerking, shoulder shrugging, tongue protrusion, sniffing, nose twitching -Vocal tics: grunting, snorting, throat clearing, barking, squeaking, coughing, yelling, coprolalia (obscenities), echolalia -Unlike chorea, tics can be suppressed -Normal tone/strength -Onset age <18 yo, severity peaks 10-12 yo, majority improve/resolve in adulthood (tics persist in 1/3) -Most commonly comorbid w/ ADHD, OCD Treatment: -Behavioral therapy (habit reversal training, substituting voluntary movement incompatible w/ tic), watchful waiting if not interfering w/ ADLs -Antidopaminergic agents like tetrabenazine (dopamine depleter, VMAT inhibitor), antipsychotics (receptor blockers) -Alpha-2 adrenergic receptor agonists (clonidine, guanfacine) Pathophysiology: -Decreased caudate nucleus volume

Carotid Sinus Hypersensitivity

Presentation: -Bradycardia, hypotension, lightheadedness, syncope following carotid massage (or other stimulation, e.g. during shaving or neck extension, putting on collared shirt), minimal tactile stimulus of carotid sinus causing a >3 s ventricular pause & >50 drop in SBP -Autonomic prodromal sx -Risk factors: elderly, male, h/o atherosclerotic CVD Diagnosis: -Carotid massage testing Treatment: -Permanent pacemaker Pathophysiology: -Carotid sinus baroreceptors become overly sensitive -> exaggerated vagal response -> bradycardia + vasodilation -> transient decrease in cerebral perfusion -> syncope, presyncope

Epidural Hematoma

Presentation: -Brief LOC followed by lucid interval of mins-hrs, followed by sudden somnolence/coma -Hematoma expansion leads to increased ICP (headache, vomiting, impaired consciousness, Cushing triad w/ bradycardia, HTN, irregular respirations), possible uncal herniation (ipsilateral pupillary dilation due to CNIII compression & contralateral hemiparesis due to ipsilateral crus cerebri compression) -Most commonly follows high-impact head trauma (e.g. MVC) in older children Diagnosis: -Biconvex density (lens-shaped) on CT that does not cross suture lines (vs. subdural) Treatment: -Urgent surgical evacuation by craniotomy for symptomatic pts -If small & asymptomatic w/o significant neuro dysfxn, close observation & serial CTs every 6-8 hrs Pathophysiology: -Trauma to sphenoid bone w/ tearing of middle meningeal artery -> blood accumulation between dura mater and cranium

Dyspepsia

Presentation: -Burning epigastric pain, bloating/epigastric fullness after meals (postprandial discomfort), N/V, heartburn, early satiety -Alarm sx: progressive dysphagia, iron def anemia, odynophagia, palpable mass or lymphadenopathy, persistent vomiting, fam hx of GI malignancy -If H. pylori, may present w/ sx of duodenal ulcer (nocturnal pain due to circadian gastric acid secretion, pain worse w/ fasting, bloating) -Labs: positive stool guaiac Diagnosis: -Age <60 testing & treatment for H. pylori (stool antigen testing, urea breath test), upper endoscopy in high-risk pts (overt GI bleeding, significant weight loss, >1 alarm sx) -Age 60+ upper endoscopy Pathophysiology: -Most commonly functional (75%), but may be associated w/ malignancy (gastric, esophageal), peptic ulcer, NSAID use, H. pylori infection, GERD

Duodenal Ulcers

Presentation: -Burning epigastric postprandial pain (usually 2-5 hrs later or at night, when food is absent and cannot buffer acid) improved by eating (due to alkaline fluid secretion into duodenum), N/V (when obstruct gastric outlet) -Vs. gastric ulcers, which tend to present w/ worse pain after eating (increased acid secretion) -Intermittent melena Diagnosis: -EGD Treatment: -PPIs (omeprazole, pantoprazole), H2 inhibitors -Abx for H. pylori (amoxicillin + clarithromycin) -Sucralfate -Selective vagotomy only in refractory cases (removal of vagal input to stomach -> decreased gastric acid production/release) Pathophysiology: -Usually caused by H. pylori infection or NSAID use; gastric ulcers more likely to be malignant

Tarsal Tunnel Syndrome

Presentation: -Burning pain, numbness, cramping, and/or paresthesias involving posteromedial ankle, heel, distal plantar sole, toes, may radiate into calf -Pain worsened by weight bearing & better w/ rest, Tinel sign--pain reproduced by percussing nerve in posterior medial malleolar area, worse w/ dorsiflexion-eversion & plantarflexion-inversion due to nerve compression -Cramping of arch -May be associated w/ trauma (fx of calcaneus/medial malleolus, dislocation), overuse injury (poor biomechanics w/ repetitive microtrauma, tendinopathy, tenosynovitis), inflammatory disorders (e.g. RA) -Risk factors: increased tensile stress on nerve (e.g. pes planus, other lower extremity deformities), fxs, posttraumatic scar tissue Diagnosis: -Clinical, nerve conduction studies, X-ray & MRI may be used to rule out structural deformities Treatment/Management: -Activity modification, orthotics (soft heel inserts), NSAIDs/corticosteroid injections, surgical release in refractory cases Pathophysiology: -Compression of posterior tibial nerve under flexor retinaculum at medial ankle

Blepharitis

Presentation: -Burning/itching of eyelids, discharge (may be associated w/ crusting of eyelashes in morning), foreign body sensation in eye -Redness, swelling, scaling of lid margins (often bilat) w/ possible inflammation of adjacent conjunctiva, possible lipoid plugs at meibomian glands, eyelash changes (e.g. depigmentation, loss, misdirection) -May be associated w/ seborrheic dermatitis, rosacea, allergic disorders, bacterial infection (esp Staph), viral infection (e.g. HSV), Demodex mite infestation Treatment: -Supportive w/ warm compresses, gentle scrubs, lid massage -Topical or oral abx (Staph coverage) if sx persist Pathophysiology: -Inflammation of eyelid margin, usually most prominent at the opening of the meibomian glands

Tricyclic Antidepressant Poisoning

Presentation: -CNS: mental status changes (drowsiness, delirium, coma), seizures, respiratory depression -CV: sinus tachycardia, hypotension, prolonged QRS/PR/QT intervals, arrhythmias (e.g. VTach, VFib) -Anticholinergic: dry mouth, blurred vision, dilated pupils, urinary retention, flushing, hyperthermia, ileus -Labs: mild acidosis Treatment/Management: -IV sodium bicarb for QRS widening (>100) or ventricular arrhythmia -If refractory to bicarb, adjuvant magnesium or lidocaine -Activated charcoal for pts within 2 hrs of ingestion (unless ileus present) -Supportive care: supplemental O2, intubation, IV fluids, telemetry, benzos for seizures -Contraindicated: physostigmine, class IA & IC antiarrhythmics Pathophysiology: -Affinity for many receptors -> inhibitory effects at muscarinic, adrenergic (alpha 1), cardiac (fast Na+ channels), histaminic, & presynaptic neurotransmitter reuptake (norepi, serotonin) receptors -Inhibition of cardiac fast Na+ channels in His-Purkinje system & myocardium -> decreased conduction velocity, increased duration of repolarization, prolonged absolute refractory period -> QRS prolongation, ventricular arrhythmias

Unstable Angina/NSTEMI

Presentation: -CP w/ exertion & at rest -CXR w/ no ST elevations, may show other changes (e.g. T wave inversions, ST depressions) -Labs: significant troponin elevation within 6-12 hrs distinguishes NSTEMI from UA Treatment/Management: -Nitrates (caution w/ hypotension, RV infarction) -Cardioselective beta blockers (e.g. metoprolol, atenolol) IV in hypertensive pts, contraindicated in HF & bradycardia -Antiplatelet therapy (aspirin, P2Y12 inhibitor like clopidogrel or ticagrelor) -Anticoag therapy (unfractionated heparin, enoxaparin, bivalirudin, fondaparinux) -High intensity statin therapy (e.g. atorvastatin, rosuvastatin) -Coronary reperfusion by PCI within 24 hrs

Chronic Stable Angina

Presentation: -CP/discomfort that predictably occurs w/ exertion & is relieved by rest Diagnosis: -ST depression w/ exertion treadmill/pharmacologic stress test Surgical Treatment: -Coronary revascularization Medical Treatment: -Beta blockers: first line, decrease myocardial contractility & HR -Nondihydropyridine CCBs: second-line alternative to BB, also decrease myocardial contractility & HR -Dihydropyridine CCBs: added to BB when needed, cause coronary artery vasodilation & decreased afterload by systemic vasodialtion -Nitrates: short-acting (e.g. nitroglycerin), long-acting (e.g. isosorbide dinitrate) added for persistent angina, decrease preload by dilation of capacitance veins -Ranolazine: alternative therapy for refractory angina, decreases myocardial calcium influx -> decreased myocardial O2 demand & ventricular wall stress Pathophysiology: -Mismatch of myocardial oxygen demand & supply

Diabetic Autonomic Neuropathy

Presentation: -CV: tachycardia, impaired exercise tolerance, postural hypotension w/ loss of diurnal BP variation -Peripheral: dry skin, pruritus, callus formation, foot ulcers, poor wound healing, Charcot arthropathy (increased fx risk w/ resultant secondary ulceration) -GI: gastroparesis w/ delayed gastric emptying, esophageal dysmotility w/ possible dyspepsia, intestinal involvement w/ possible diarrhea, constipation, or fecal incontinence -GU: erectile dysfxn & retrograde ejaculation in men, decreased libido & dyspareunia in women, decreased ability to sense bladder filling leading to incomplete emptying & decreased urination, eventual recurrent UTIs and/or overflow incontinence (e.g. dribbling, poor urinary stream) -Risk factors: poor glucose control, other vascular risk factors (e..g HTN, elevated triglycerides, smoking, obesity) Diagnosis: -High PVRs

Acute Hypocalcemia

Presentation: -Can occur w/ neck surgery (parathyroidectomy, thyroidectomy), pancreatitis, sepsis, tumor lysis syndrome, acute alkalosis, chelation (blood/citrate transfusion, EDTA, foscarnet, lactate) -Presents w/ muscle cramping, paresthesias (esp perioral, hands/feet), hyperreflexia/tetany, seizures, laryngospasm, encephalopathy, HF, QT prolongation -Trousseau sign--carpal spasm (forceful wrist flexion & thumb abduction) -Chvostek sign--facial spasm w/ tapping over zygomatic arch -Risk factors: liver disease Diagnosis: -Ionized calcium level Treatment: -IV calcium gluconate/chloride Pathophysiology: -Citrate in transfused blood binds ionized calcium; uncommon in transfusion of pt w/ normal hepatic fxn b/c liver quickly metabolizes citrate, but may occur in pts w/ liver disease, ischemic liver injury, renal failure, hypothermia, shock

Osteomyelitis

Presentation: -Can present as chronic, indolent infection in diabetics w/ neuropathic ulcers w/ sinus tracts or exposure of underlying bone -Acute: may be asymptomatic or present w/ fever, bone pain, elevated ESR, sinus tract drainage (purulent) -Chronic: persistent pain/swelling, chronic nonhealing wound, sinus tract formation, nonunion of fx (even after surgical fixation) -Increased risk w/ positive probe to bone test (palpation of bone from outside w/ sterile instrument), large ulcer >2 cm^2, ulcer lasting 1+ wk -Labs: elevated ESR/CRP, leukocytosis -Risk factors: T2DM (peripheral neuropathy, hyperglycemia, peripheral arterial disease), ulcers, surgical hardware/instrumentation Diagnosis: -X-ray w/ lytic lesion, loss of cortical & trabecular bone, bone necrosis w/ surrounding sclerosis & periosteal thickening -If following fx, X-ray may show ragged fx edges, bone fragmentation, nonunion -If X-ray negative, MRI preferred (high NPV) -Bone biopsy & culture is most specific (preferable before starting abx); superficial wound cultures usually not reliable Treatment: -If ulcer complicated by osteo, surgical debridement of necrotic tissue & prolonged abx -If d/t DM, glucose control, weight off-loading, revascularization if needed -Amputation is last resort when medical management fails or significant complications (e.g. extensive necrosis, ischemia) Pathophysiology: -If ulcers, may result from contiguous (nonhematogenous) extension of infection from ulcer to underlying bone -If other source, may result from hematogenous seeding of bone or direct inoculation (e.g. compound fx, bite wound) -Usually due to S. aureus, coag-negative Staph, or polymicrobial infection -Chronic osteomyelitis is common complication of high-velocity tibial fxs b/c bone fragments are exposed & blood supply often interrupted by trauma

Enteropathy-Associated T-cell Lymphoma (EATL)

Presentation: -Can present w/ untreated celiac disease -Abd pain, B sx (weight loss, fatigue, fever), dark stools -Labs: positive occult blood -Complications: bowel obstruction, bowel perforation, GI bleeding Treatment/Management: -Usually present w/ late disease, poor prognosis w/ mean survival of 10 mos Pathophysiology: -Aggressive hematologic malignancy primarily affecting proximal jejunum

Clostridium Septicum

Presentation: -Can rarely present w/ bacteremia & invasive infection (e.g. gas gangrene, tissue necrosis) -Risk factors: colonic malignancy (present in ~30% of infections) Diagnosis: -Colonoscopy to screen for malignancy Pathophysiology: -Gram+, spore forming colonic bacterium -Tumor cells in colonic malignancy frequently undergo anaerobic glycolysis -> allows germination of spores -Tumor cells also damage colonic mucosa -> allows translocation of sporulated bacteria into bloodstream

Restrictive Cardiomyopathy

Presentation: -Cause of HFpEF, presents w/ s/s of CHF (e.g. JVD, bibasilar crackles, pulm vascular congestion), biatrial enlargement, pHTN -Less common than dilated/hypertrophic CM, may be associated w/ infiltrative disorders (e.g. sarcoidosis, amyloidosis), storage disease (e.g. hemochromatosis), endomyocardial fibrosis

Arrhythmogenic Right Ventricular Cardiomyopathy

Presentation: -Cause of SCD in young athletes -ECG findings including precordial T-wave inversions Pathophysiology: -Heart muscle is replaced by fibrous scar and fatty tissue; right ventricle most likely affected

Purulent Cellulitis

Presentation: -Cellulitis w/ purulent drainage -May present as folliculitis (infected hair follicles), furuncles (folliculitis extension into dermis -> abscess), or carbuncle (multiple furuncles) Treatment/Management: -Empiric abx w/ activity against MRSA (clindamycin, TMP-SMX, doxycycline) -I&D of furuncles/carbuncles Pathophysiology: -Most commonly due to MSSA or MRSA infection (commonly methicillin resistant)

Dementia with Lewy Bodies

Presentation: -Central features (required for dx): progressive cognitive decline, dementia -Core features: visual hallucinations, spontaneous parkinsonism (rigidity, bradykinesia, gait disturbance), fluctuating cognition, REM sleep behavior disorder, dysautonomia, variations in attention/alertness -Suggestive features: severe antipsychotic sensitivity (can lead to worsening of confusion, parkinsonism, autonomic dysfxn), SPECT/PET showing low dopamine transporter uptake in basal ganglia -Supportive features: repeated falls, syncope or near-syncope, severe autonomic dysfxn, delusions, depression/anxiety -Typically presents ~75 yo, 4x more common in men, second leading cause of dementia after Alzheimer, avg survival after diagnosis of 8 yrs -DLB less likely if neuroimaging shows cerebrovascular disease, appearance of parkinsonism first w/ dementia later Diagnosis: -2+ of core features Treatment/Management: -Cholinesterase inhibitors for cognitive impairment, carbidopa-levodopa for parkinsonism, melatonin for REM sleep behavior disorder -CAREFUL W/ ANTIPSYCHOTICS; if necessary for hallucinations/delusions, should use low-potency 2nd gen antipsychotics like quetiapine (avoid risperidone, 1st gen agents) Pathophysiology: -Alpha-synuclein neurodegeneration (round, eosinophilic, cytoplasmic inclusions in neurons of substantia nigra)

Cytomegalovirus Retinitis

Presentation: -Central vision loss, blurry vision, scotomata (blind spots), floaters, photopsia (sensation of flashing lights) -Sx usually present subacutely (wks), unilaterally, w/o pain -Risk factors: chronic immunosuppression (HIV, transplant), medication noncompliance, CD4 <100 Diagnosis: -Ophtho exam w/ yellow-white fluffy/granular retinal lesions/exudates adjacent to fovea & retinal vessels, associated hemorrhages Treatment: -Oral antiviral therapy (valgancyclovir) -If lesions near fovea/optic nerve, concomitant intravitreal injections to reduce risk of blindness & retinal detachment d/t retinal scarring -If not already started, antiretroviral therapy (usually ~2 wks after starting anti-CMV therapy)

Spinocerebellar Ataxia Type 1

Presentation: -Cerebellar ataxia, CN dysfxn (e.g. dysarthria), intention tremor (tremor that worsens in amplitude at end of movement), dysdiadochokinesia (inability to perform rapid alternative movements), dysmetria (overshooting finger-to-nose), scanning speech (pauses b/t syllables of words) Pathophysiology: -AD inheritance

Factitious Disorder

Presentation: -Characterized by intentional production of false physical or psychological signs/sx to assume the sick role -E.g. Evasiveness in providing history, dramatic/overt presentation, frequent medical visits, inconsistent/vague hx, hx of extensive medical evaluation w/ negative results, refusal to sign release of info for medical records, eager acceptance of further testing and invasive diagnostic procedures, atypical course of illness, poor response to standard treatments, evidence of tampering w/ test samples -Vs. malingering, no secondary/external gain (e.g. financial compensation), motivated by internal factors -Patients often knowledgeable about which diagnoses/sx warrant admission, may resort to extreme and dangerous measures (e.g. self-inflicted trauma, manipulating lab tests, injecting fecal matter) to generate signs of disease -Risk factors: more common in healthcare workers, women Diagnosis: -Direct observation or discovery of medical supplies (e.g. syringes, meds) -Confirmatory testing (e.g. sulfonylurea screen, stool laxative screen for factitious diarrhea) -Medical record review

Caustic Ingestion

Presentation: -Chemical burn or liquefaction necrosis injury from ingesting toxin (e.g. oven cleaner, other cleaning supplies) -Affects lips, tongue, oral mucosa, esophagus (e.g. bleeding, ulceration, erythema, gray pseudomembranes if extensive necrosis) -May develop chest & abd pain, vomiting w/ occasional hematemesis, dysphagia/odynophagia, hoarseness, airway compromise/stridor, difficulting managing secretions -Often conscious & alert but in severe pain -Child w/ crying, drooling, blood-tinged oral secretions, swollen/erythematous lips/face -Complications: esophageal perforation -> mediastinitis, peritonitis, esophageal strictures, esophageal SCC Treatment/Management: -Prehospital: decontamination (remove contaminated clothing, brush off visible chemical, irrigate exposed skin), do not induce vomiting or administer charcoal or attempt to neutralize alkaline substances w/ vinegar (can cause exothermic damage) -ED: confirm decontamination, chest/abd X-rays (identify signs of perforation), laryngoscopy for airway evaluation if respiratory distress, endotracheal intubation if significant oropharyngeal injury, consider gastric lavage if nasogastric tube is placed -Inpatient: endoscopy within 12-24 hrs if hemodynamically stable & without respiratory distress or perforation, serial X-rays to rule out perforation, tube feedings & surgical intervention for severe injury Pathophysiology: -Acidic substances -> coag necrosis (protein denaturation) -> eschar that prevents further acidic penetration/injury -Alkaline substances -> liquefactive necrosis (cell membrane dissolution) -> leads to deeper penetration of tissues & more severe injury

Spontaneous Pneumothorax

Presentation: -Chest pain, dyspnea, tachypnea, tachycardia, cyanosis, hypoxemia -Decreased breath sounds & tactile fremitus, decreased chest wall movement, increased transillumination of affected side, hyperresonance to percussion -If intubated, may see increased peak & plateau pressures on ventilator -Primary: no preceding event or lung disease, often tall/thin/young men -Secondary: underlying disease (e.g. COPD, CF, respiratory distress syndrome in premature infants) -Risk factors: premature, mechanical ventilation, meconium aspiration syndrome, underlying disease like COPD (bullae/blebs that can rupture), CVC placement, smoking, Marfan, thoracic endometriosis Diagnosis: -CXR w/ visceral pleural line, absent lung markings beyond pleural edge, inspiratory CXR -If acute setting, bedside US Treatment/management: -If small (2 cm or under), observation & oxygen administration (enhances speed of resorption)-If large and unstable, needle aspiration (stable) or chest tube (unstable) -If lung fails to expand after above (<90% expansion) w/ persistent air leak, may place one-way valve or use blood patch or video-assisted thoracoscopic surgery (VATS)/pleurodesis Pathophysiology: -Positive pressure ventilation can cause pulm barotrauma -> alveolar rupture (esp in pts w/ underlying pulm disease)

Vertebral Osteomyelitis

Presentation: -Chronic (>6 wks), insidious, progressive back pain that is unrelieved by rest -Tenderness to gentle percussion over spinous processes of involved vertebrae -Only presents w/ fever in 50% of cases, night sweats -Labs: leukocytes can be normal or elevated, thrombocytosis, elevated ESR -Risk factors: IV drug use, sickle cell anemia, immunosuppression, other recent distant site infection (e.g. UTI) Diagnosis: -Spinal MRI w/ vertebral body destruction, collapse of disc space -Confirmation w/ open or CT-guided bx -Tagged leukocyte scan if metal implants preclude MRI Treatment: -IV abx +/- surgery Pathophysiology: -Most commonly due to S. aureus in IV drug users

Bronchiectasis

Presentation: -Chronic cough w/ daily production of copious mucopurulent sputum (>100 mL/day), inadequate mucus clearance, hx of recurrent respiratory infections -Rhinosinusitis, dyspnea, hemoptysis, crackles, wheezing, digital clubbing -Often associated w/ Pseudomonas infection -Exacerbations usually d/t bacterial infection vs. viral infection in chronic bronchitis -Risk factors: smoking, recurrent RI, CF (often upper lobe involvement), alpha-1-antitrypsin (usually lower lobe) Diagnosis: -HRCT of chest needed for initial dx (shows bronchial dilation, lack of airway tapering, bronchial wall thickening) -Ig quantification, CF testing, sputum culture (for bacteria, fungi, mycobacteria) -PFTs w/ obstructive pattern -CXR may show linear atelectasis, dilated/thickened airways, irregular peripheral opacities Treatment: -Chest physiotherapy to aid in sputum clearance -Abx for bacterial exacerbations Pathophysiology: -Irreversible dilation, thickening, & destruction of bronchi, often following infectious insult w/o complete resolution due to impaired clearance -> cycle of further airway damage & remodeling & recurrent infection -Chronic airway inflammation & edema -> rupture of superficial blood vessels -> hemoptysis -May be associated w/ malignant airway obstruction, rheumatic disease (e.g. RA, Sjogren), toxic inhalation, chronic or prior infection (e.g. aspergillosis, Mycobacteria), immunodeficiency (e.g. hypogammaglobulinemia), congenital causes (e.g. CF, alpha-1-antitrypsin def) -Excessive elastase production in response to enhanced neutrophil recruitment results in bronchial airway damage in CF & alpha-1-antitrypsin def

Rosacea

Presentation: -Chronic erythematous rash most prominent on convex areas of face -Flushing, skin sensitivity may be precipitated by hot/spicy foods, EtOH, sun exposure, high ambient temps, stress -Erythematotelangiectatic rosacea: flushing/burning discomfort, erythema on nose & medial cheeks (w/ nasolabial fold involvement), telangiectasias, roughness/scaling, usually in fair-skinned individuals >30 yo, usually intermittent but can lead to permanent skin flushing -Papulopustular rosacea: small papules & pustules on central face -Phymatous rosacea: irregular thickening of skin -Ocular rosacea: burning/foreign body sensation, blepharitis, keratitis, conjunctivitis/conjunctival hyperemia, corneal ulcers, lid margin telangiectasias, recurrent chalazion Treatment: -General measures: avoidance of sun exposure (sun protection), hot/spicy food, EtOH, use of gentle cleansers & emollients -Erythematotelangiectatic: topical brimonidine (vasoconstrictive alpha-2 agonist), laser/intense pulsed light therapy -Papulopustular: 1st lines include topical metronidazole, azelaic acid, ivermectin, benzoyl peroxide; 2nd lines include PO tetracyclines & metronidazole -Phymatous: oral isoretinoin, laser therapy/surgery -Ocular: lid scrubs, ocular lubricants, topical or systemic abx (metronidazole, macrolides) Pathophysiology: -Not completely known, may be chronic inflammatory rxn to cutaneous microorganisms, UV damage, vasomotor dysfxn -Papulopustular: overgrowth of sebaceous glands

Osteoarthritis (OA)

Presentation: -Chronic joint pain/stiffness/swelling, tender to palpation but no erythema/warmth (no synovitis), worse w/ weight bearing & activity/joint loading, better w/ rest, possible brief morning stiffness (<30 min) -Stable ligament testing, exam w/ crepitus, periarticular bony enlargement -Active & passive ROM limited due to pain -Heberden/Bouchard nodes if involving hands (DIPs & PIPs), commonly also involves hips, knees, 1st carpometacarpal joint -Modifiable risk factors: obesity, occupational joint loading, obesity, T2DM, physical inactivity, EtOH -Nonmodifiable risk factors: age >40, prior joint injury, abnormal joint alignment (e.g. varus deformity), female sex, fhx Diagnosis: -X-ray w/ joint space narrowing, periarticular osteophytes, subarticular sclerosis/cysts -Effusion aspiration w/ bland analysis (noninflammatory w/ <2000 WBCs, no organisms) Treatment/Management: -1. Nonpharmacologic (weight loss, exercise w/ regular moderate activity, PT w/ quadriceps strengthening for knee OA), if sx persist ->-2. Topical or oral NSAIDs as needed (diclofenac), other pharmacologic options (duloxetine, tramadol, topical capsaicin) ->-3. Injectable glucocorticoids, hyaluronic acid ->-4. Surgery (if candidates for arthroplasty), chronic pain management (if not candidates for surgery) Pathophysiology: -For knee OA, quadriceps strength declines w/ age, pain w/ decreased activity -> atrophy of quadriceps complex -> abnormal loading of articular cartilage, accelerated joint degeneration -Involves progressive destruction of articular cartilage due to altered expression of matrix metalloproteinases, accelerated chondrocyte death

Autoimmune Metaplastic Atrophic Gastritis (AMAG)

Presentation: -Chronic postprandial epigastric abd pain (crampy, intermittent, nonradiating), bloating, nausea, other sx of dyspepsia (e.g. heartburn, regurg) -Abd tenderness to palp w/o rebound, guarding, distention -Labs: macrocytic anemia, iron def anemia (hypochlorhydria reduces iron bioavailability), hypochlorhydria, elevated serum gastrin levels -Risk factors: autoimmune illness (e.g. autoimmune thyroid disease, T1DM), female Treatment/Management: -Routine surveillance endoscopy d/t increased risk of gastric adenocarcinoma & neuroendocrine tumors Pathophysiology: -Production of antibodies against parietal cells -> atrophy & metaplasia of gastric corpus, hypochlorhydria, uncontrolled gastrin production -Production of antibodies against intrinsic factor -> B12 deficiency

Chronic Bronchitis

Presentation: -Chronic productive cough (often frothy sputum) for 3+ mos in 2 successive yrs w/ or w/o airflow limitaiton, hemoptysis -CXR w/ prominently thickened bronchovascular markings (reflect airway inflammation) w/ some diaphragmatic flattening -Risk factors: smoking Diagnosis: -Clinical, supported by PFTs w/ obstructive pattern, normal DLCO Treatment: -Mucus clearance therapy (e.g. chest percussion) -Anti-inflammatory therapy (inhaled corticosteroids) Pathophysiology: -Due to irritant-induced airway mucosal inflammation, respiratory epithelium metaplasia, mucus hypersecretion

Idiopathic Pulmonary Fibrosis

Presentation: -Chronic progressive dyspnea, nonproductive cough, fatigue -Fine "velcro" inspiratory crackles, digital clubbing -Avg survival of 2-3 yrs -Risk factors: male, >60 yo, smoking, polymorphisms in genes regulating epithelial regeneration -Not associated w/ connective tissue disorders (normal joint/skin exams) or exposures (e.g. asbestos, pneumotoxic drugs) Diagnosis: -High resolution CT w/ subpleural/lower lobe honeycombing, reticular opacities (UIP--usual interstitial pneumonia) -Restrictive pattern on LFTs, reduced DLCO Treatment/Management: -Antifibrotic therapy (pirfenidone, nintedanib; block TGF-beta signaling to reduce fibroblast proliferation & collagen production, can slow rate of decline but not restore lost lung fxn) -Smoking cessation, treatment of GERD, supplemental O2, pulm rehab -Lung transplant -Hospice care when life expectancy of 6 mos or shorter Pathophysiology: -Microscopic alveolar epithelial injury (smoking, GERD, silent aspiration) -Inappropriate repair by fibrosis instead of epithelial regeneration

Cystic Fibrosis

Presentation: -Chronic rhinosinusitis, nasal polyposis, digital clubbing, barrel chest, recurrent PNA & bronchiectasis (sinopulmonary infections) -Biliary cirrhosis, pancreatic exocrine insufficiency (malabsorption -> poor growth, fat-soluble vitamin deficiencies, steatorrhea aka bulky/greasy stools), CF-related DM -Inspissated stool, meconium ileus -Absent vas deferens, male infertility -Normal birth measurements w/ subsequent failure to thrive (weight deceleration crossing 2+ major percentiles), deceleration in linear growth velocity Diagnosis: -Elevated sweat chloride concentration, CFTR mutation on genetic testing, abnormal nasal potential difference Treatment: -Intranasal glucocorticoids for nasal polyps -Nutritional support (exocrine enzyme supplementation) -Airway clearance -Abx (S. aureus, Pseudomona aeruginosa) Pathophysiology: -Most commonly due to AR F508 mutation of CFTR gene

Inducible Urticaria

Presentation: -Chronic urticaria induced by environmental stimuli including: -Dermographism: induced by scratching -Cholinergic: induced by elevated core body temp (e.g. hot showers, exercise) -Delayed pressure: induced by sustained mechanical pressure -Aquagenic: induced by water -Solar urticaria: induced by sun exposure -Typically affects pts 20-40 yo, usually resolves within 10 yrs -1-4 mm wheals develop after exposure, eventually coalesce to form large areas of erythema, usually starts on torso & spreads peripherally to face & extremities, burning/tingling sensation -After removal of trigger, rash typically resolves in 1-2 hrs Diagnosis: -Clinical present of urticaria following stimulus exposure -Provocation test if diagnosis unclear Treatment/Management: -Trigger avoidance -H1 blocker

Postthrombotic Syndrome

Presentation: -Chronic venous insufficiency following acute DVT, usually within 2 yrs of thrombus episode -Leg edema, fatigue, pain, superficial venous dilation, venous stasis ulcers, trophic skin changes -Sx often worse at end of day Diagnosis: -Doppler US to rule out DVT Treatment/Management: -Exercise (ankle flexion, walking) -Compression (bandages, stockings) Pathophysiology: -Due to venous HTN distal to site of previous thrombus

Persistent Vegetative State

Presentation: -Chronic wakefulness (e.g. spontaneous eye opening) without awareness (e.g. blinking on command) -Non-purposeful limb movements & no comprehension/expression of language Pathophysiology: -Sequela of diffuse cortical injury

Open Angle Glaucoma

Presentation: -Chronic, insidious loss of peripheral vision (may lead to near-miss MVCs, tripping over objects) -> tunnel vision -Fundoscopy w/ optic cupping (enlargement of optic cup & increased cup:disc ratio, optic disc rim thinning), late finding -Risk factors: topical or systemic steroid use (e.g. prednisolone eyedrops for uveitis), African American, T2DM, fhx glaucoma -If steroid-induced, may develop early central blurriness from corneal edema, can eventually cause subcapsular cataracts Diagnosis: -Tonometry (measures deformation of cornea in response to applied pneumatic/mechanical pressure) w/ increased IOP -Automated visual field testing to assess for peripheral vision Treatment/Management: -Prostaglandin analog eye drops (latanoprost, carboprost, bimatoprost) to increase aqueous humor drainage through uvoscleral pathway -If prostaglandins ineffective, addition of topical beta blockers (e.g. timolol), use w/ caution in pts w/ comorbid asthma -Other options: topical carbonic anhydrase inhibitors (e.g. dorzolamide) -If pharm intervention ineffective or not tolerated, surgery (laser trabeculoplasty) Pathophysiology: -Decreased outflow of aqueous humor via drainage channels in anterior chamber -> increased intraocular pressure -> atrophy of optic nerve head -> loss of peripheral vision -Atropine contraindicated: pupillary dilation can lead to angle narrowing and precipitate angle closure glaucoma

Bladder Cancer

Presentation: -Chronic, painless gross and/or microscopic hematuria throughout micturition -Chronic irritative voiding sx (vs. UTI, >4 wks) e.g. frequency, urgency, dysuria, nocturia -Possible bladder neck/urethra obstruction -Dull, nonradiating suprapubic pain -Hydronephrosis/CVA tenderness (bladder outlet or ureter obstruction) not attributed to infection (negative UA apart from hematuria) -> acute renal insuff -Labs: UA w/ hematuria and w/o pyuria or other signs of UTI -Risk factors: smoking, exposure to industrial carcinogens, age, M>F, h/o pelvic radiation or exposure to aniline dyes Diagnosis: -Flexible cystoscopy w/ biopsy (gold standard), urine cytology, CT urography to evaluate for metastases -Staging w/ transurethral resection of bladder tumor (TURBT), upper urinary tract imaging (IVP, MRI, CT) Treatment/Management: -If no muscle invasion, TURBT & intravesical immunotherapy -If muscle invasion, radical cystectomy & systemic chemotherapy -If metastatic, systemic chemotherapy & immunotherapy Pathophysiology: ->90% due to urothelial carcinoma -Tumors may protrude into bladder & cause detrusor overactivity -> subacute/chronic voiding sx -Suprapubic pain usually indicative of advanced tumor penetration into muscle, invasion of surrounding soft tissues & nerves -Neovascularization of tumor causes painless hematuria throughout micturition (tumors at bladder neck occasionally present w/ terminal hematuria)

Male & Female-Pattern Hair Loss

Presentation: -Chronic, progressive thinning of hair -Men: loss @ vertex, frontal hairline, temporal areas -Women: loss @ vertex, center of scalp (sparing of hairline) Treatment: -Men: finasteride, topical minoxidil (vasodilator that increases scalp blood flow) -Women: topical minoxidil Pathophysiology: -Polygenetic inheritance, hormonal factors -Men: primarily driven by excess androgens -Women: replacement of terminal hairs by smaller vellus hairs (follicular miniaturization), less driven by androgens (can worsen hair loss but usually normal in most pts w/ female-pattern hair loss)

Pulmonary Tuberculosis

Presentation: -Chronic/mild/productive cough (worse in morning d/t pooling of secretions overnight), hemoptysis, fever, weight loss, hilar/cervical lymphadenopathy, night sweats, muscle wasting -(Upper lobe) cavitary lesion, older individuals may have multifocal infiltrates -Risk factors: workplace hazard (e.g. prison, healthcare facility like nursing home), immigrant from endemic area (usually presents as reactivation of latent infection), CKD -Labs: anemia (of chronic disease), hypergammaglobulinemia (elevated total protein), hypoalbuminemia (late finding), monocytosis (late finding, indicative of increased macrophage production, important for granulomatous immune response), positive tuberculin skin test (can be falsely negative in HIV, deficiency of cell-mediated immunity, e.g. in CKD, liver failure, inherited immunodeficiency) Diagnosis: -CXR -Acid fast bacilli microscopy smear: low cost & rapid in mins-hrs, low sensitivity b/c requires high organism burden for detection (cannot be used to rule out TB), cannot differentiate TB from non-TB mycobacteria -NAAT: higher cost but slightly less rapid, higher sensitivity, can differentiate TB from non-TB mycobacteria -Sputum culture: gold standard, quantitative & allows for drug sensitivity testing, very slow (3-8 wks) -Tuberculin skin testing and IFN-gamma release assay cannot differentiate b/t active & latent disease Treatment/Management: -Airborne isolation, empiric therapy (2 mos w/ RIPE, 4 mos w/ RI) esp if high degree of suspicion, even if AFB microscopy is negative Pathophysiology: -Preferentially infects lung apices d/t higher O2 tension, slower lymphatic outflow -> permits accumulation/proliferation

Delayed Sleep-Wake Phase Disorder (DSWPD)

Presentation: -Circadian rhythm sleep-wake disorder w/ sleep-onset insomnia & excessive morning sleepiness -"Night owls," problems going to sleep at conventional time -Often occurs beginning in adolescence Treatment/Management: -Light/behavioral therapy Pathophysiology: -Occurs when internal clock regulating sleep & wakefulness is misaligned w/ pt's desired sleep time or social/professional schedule

Pheochromocytoma

Presentation: -Classic triad of episodic pounding HAs, sweating, tachycardia/palpitations -Hyperadrenergic spells (nonexertional palpitations, pallor) -Vision changes (e.g. blurriness), normal BMI, weight loss -Resistant HTN, onset of HTN at young age, or HTN accompanied by unexplained increase in glucose, paroxysmal HTN (higher supine > upright, orthostatic) -Increases in intraabd pressure (e.g. tumor palpation, position changes), surgical procedures, certain medications (esp anesthetics) may incite paroxysms w/ pallor, HTN, sinus tachycardia -May have family hx of syndrome (MEN2, NF1, VHL) -Labs: hyperglycemia Diagnosis: -Urine/plasma catecholamines/metanephrines (24-hr fractionated urine) -Confirmatory abd imaging (CTA) for increased metanephrines -If above elevated, imaging w/ CT/MRI abdomen (usually >4-4.5 cm w/ high tissue density on CT, larger tumors w/ central necrosis & calcification) -Testing also indicated if adrenal incidentaloma found on imaging, pressor response during surgery/anesthesia, or idiopathic dilated CM found Treatment/Management: -Preop alpha blockade (e.g. phenoxybenzamine, terazosin) prior to beta blockade (nonselective beta blockers create state of unopposed alpha adrenergic stimulation -> paradoxical HTN) just prior to surg, usually w/ propanolol -Do not start alpha blockers until after confirming diagnosis; prior to testing these can cause falsely elevated catecholamine/metanephrine levels -Laparoscopic or open surgical resection (adrenalectomy) -MIBG scan if tumor >5 cm & suspicion of extra-adrenal disease -Conduct genetic testing Pathophysiology: -10% bilateral, 10% extraadrenal, 10% malignant -Neuroendocrine tumor arising from chromaffin cells of adrenal gland medulla or extra-adrenal paraganglia, release excess catecholamines (dopamine, epi, norepi) into bloodstream -Excess catecholamines inhibit insulin secretion -> hyperglycemia -During surgery, catecholamine surge can precipitate hypertensive crisis, pheochromocytoma crisis (labile BP, high fever, multiorgan failure), fatal arrhythmias; thus alpha blockade started 7-14 days prior to surgery, beta blockade 2-3 days before surgery

Acute Iron Poisoning

Presentation: -Classically presents <6 yo w/ unintentional ingestion of prenatal vitamins, supplements or as suicide attempt in adults -Abd pain, vomiting, diarrhea, bleeding (hematemesis/coffee ground emesis, melena), shock, liver necrosis (within 1-2 days of ingestion) -Vomitus/stool may be black/green from tablets -Due to GI losses +/- vasodilation +/- myocardial injury, can develop hypovolemic/distributive/cardiogenic shock -> hypotension, tachy, prerenal azotemia -Anion gap metabolic acidosis -> tachypnea (compensatory resp alkalosis) -Radiopaque opacities in GI tract on X-ray -Complications: liver failure, shock, death, bowel obstruction wks after (d/t GI scarring) Diagnosis: -Serum ion levels, anion gap metabolic acidosis Treatment: -IV deferoxamine chelation therapy, whole bowel irrigation -Hemodialysis if deferoxamine fails Pathophysiology: -Iron directly damages GI mucosa by free radical production & lipid peroxidation -> upper & lower GI bleeding

Acute Promyelocytic Leukemia (APML)

Presentation: -Clinically unique form of AML that presents in young and middle-aged adults -Anemia (dyspnea on exertion, fatigue), thrombocytopenia (bleeding complications), neutropenia (infections) -Considered medical emergency due to high risk of pulm/cerebrovascular hemorrhage from tumor-induced consumptive coagulopathy (DIC) -Labs: pancytopenia Diagnosis: -Smear w/ atypical myeloid cells (often w/ intracytoplasmic violet granules) w/ Auer rods -Cytogenic analysis for specific translocation Treatment: -Urgent treatment w/ all-trans retinoic acid (promotes differentiation of atypical promyelocytes) Pathophysiology: -Fusion of promyelocytic leukemia gene & retinoic acid receptor alpha gene t(15;17) (RARA) -Exact mechanism unclear, thought to promote bleeding diathesis due to activation of tissue factor (-> DIC) & increased generation of plasmin (primary hyperfibrinolysis)

Photoaging

Presentation: -Coarse, deep wrinkles on rough skin surface -Skin often marked w/ actinic keratoses, telangiectasias, brown "liver" spots -Risk factors: UV light exposure, tobacco use Treatment: -Tretinoin (all-trans-retinoic acid) emollient cream Pathophysiology: -Phenomenon that arises from intrinsic aging and damage caused by UV light; contrasts intrinsic aging effects (fine wrinkles on smooth skin surface)

Brain Death

Presentation: -Comatic on exam, clinical/imaging evidence of devastating cause, hemodynamic stability on exam (not hypothermic) -Absent brainstem/cerebral reflexes (pupillary, corneal, oculocephalic, oculovestibular, corneal, jaw jerk, cough, gag) -Absent brain-originating motor responses including decorticate/decerebrate posturing, purposeful movement, facial grimacing, sucking, swallowing -Apnea test (no respiratory response to PaCO2 >60, indicating nonfunctioning medullary respiratory centers), final arterial pH of <7.28, shows evidence of no spontaneous breaths regardless of hypercarbia or hypoxemia -Movements from spinal cord & peripheral nerves may still be present (DTRs, finger flexion, truncal movements, triple flexion response, plantar reflexes, limb movements to painful stimuli, alternating flexion/extension of toes) -Sustained brain death = unresponsive after 24 hrs Diagnosis: -Defined as irreversible cessation of all cerebral & brainstem activities, legally acceptable definition of death -Clinical definition w/ neuro exam -Criteria: known cause (e.g. most commonly TBI, stroke), evidence of devastating CNS event (e.g. clinical, neuroimaging), exclusion of confounding conditions (e.g. electrolyte abnormality, intoxication, paralytics, hypothermia), core temp >36 (96.8), SBP >100 -Ancillary tests may include EEG (shows electrocerebral silence or absence of somatosensory or brainstem evoked potentials), brain imaging (transcranial Doppler, CT angio w/ absence of intracranial blood flow) -Apnea testing w/ absence of respiratory response Treatment/Management: -If preparing for harvesting organ transplantation, should mitigate risks that threaten viability of donated organs, including central DI, systemic hypotension, & hypothermia -Maintain euvolemic, normotensive, normothermic state by administering IVF & desmopressin, ionotropic or pressor support to maintain BP, warm air blankets to maintain temp

Follicular Lymphoma

Presentation: -Common form of NHL that presents in elderly pts w/ indolent, painless peripheral lymphadenopathy over mos-yrs (axillary, mediastinal, inguinal, etc.) w/o systemic signs/sx -Lymphadenopathy may wax/wane -B symptoms & lab abnormalities generally absent Diagnosis: -Excisional lymph node bx, histo w/ nodular growth of follicular lymphocytes -Immunophenotyping most commonly shows ch14;18 translocation (~85%) that leads to overexpression of BCL-2 Pathophysiology: -Overexpression of BCL-2, oncogene that prevents apoptosis

Hypomagnesemia

Presentation: -Common in alcoholics, prolonged nasogastric suction/diarrhea, diuretic use -Labs: hypocalcemia, hypophosphatemia, hypokalemia Pathophysiology: -Causes hypocalcemia by inducing resistance to PTH, decreasing PTH secretion -Cause may be multifactorial: malnutrition, urinary losses, pancreatitis, diarrhea -One of few causes of hypoparathyroidism in which phosphate is not increased (but instead decreased) -Intracellular Mg usually inhibits K+ secretion by renal outer medullary K+ (ROMK) channels; hypomagnesemia leads to K+ wasting, refractory hypokalemia

Hypophosphatemia

Presentation: -Common in pts w/ EtOH use disorder, risk factors for refeeding syndrome (e.g. prolonged malnutrition) -Weakness, rhabdomyolysis, paresthesias, respiratory failure

BK Virus Nephropathy

Presentation: -Common in renal transplants (prevalence of ~5%), usually asymptomatic or presents w/ progressive renal failure, tubulointerstitial nephritis w/ hematuria, pyuria, WBC casts -Fever & worsening renal fxn Diagnosis: -Serum PCR of BK viral DNA -Confirmation: renal bx w/ basophilic intranuclear inclusions, enlarged nuclei, mixed lymphocytic/neutrophilic infiltrate (c/w interstitial nephritis) Treatment: -Reduced immunosuppression, antiviral therapy in some Pathophysiology: -Caused by polyomavirus -Usually d/t excessive immunosuppression in renal allograft recipients

Pulmonary Contusion

Presentation: -Common sequelae of blunt chest trauma (25-35%), presents w/ localized pulmonary edema at site of injury (may take ~24 hrs after injury to develop), tachypnea, tachycardia, hypoxia -Rales or focally decreased breath sounds, CP/tenderness, hemoptysis -Injured lung parenchyma & visceral pleura may bleed into pleural space -> associated pleural effusion -Often associated w/ rib fxs but may occur in absence of fxs, esp in children Diagnosis: -CT (most sensitive) or CXR w/ irregular/nonlobular, focal, patchy opacities -CXR immediately after accident may be normal, thus should repeat imaging -Alveolar infiltrates not restricted by anatomic borders (nonlobular), often adjacent to chest wall (where trauma was greatest) Treatment/Management: -Pain control, pulm hygiene (incentive spirometry, chest physiotherapy), supplemental O2 & ventilatory support -Sx usually resolve within 3-5 days Pathophysiology: -Focal alveolar hemorrhage & edema following blunt chest trauma -Resuscitative fluid administration may worsen edema

Seborrheic Keratosis

Presentation: -Common, benign epidermal lesion in middle-aged or elderly pts, more common in fair-skinned pts -Found almost anywhere except palms/soles -Vary from nearly flat macules to raised/wart-like appearance, pink/white, brown, or black -Often have velvety or greasy appearance, well-demarcated border, stuck-on appearance -Usually asymptomatic, can also be pruritic or tender -Explosive onset of multiple lesions may indicate occult internal malignancy, esp intestinal & lung (Leser-Trelat sign) Diagnosis: -Clinical, bx usually not necessary (shows small cells resembling basal cells w/ variable pigmentation, hyperkeratosis, keratin-containing cysts) Treatment/Management: -Observation, bothersome lesions may be treated w/ curettage/shave excision, cryosurgery, electrodessication Pathophysiology: -Benign epidermal tumor d/t proliferation of immature keratinocytes

Fungal Otitis Externa (Otomycosis)

Presentation: -Commonly presents after treatment for acute otitis externa, insidious of ear itchiness & mild-moderate ear pain -Whitish fungal debris in external auditory canal w/ fruiting bodies and spores -Risk factors: chronically moist ear canals (hearing aids, warm/humid climate) Treatment: -Debridement, topical (clotrimazole) antifungals Pathophysiology: -Aspergillus species (e.g. A. niger)

Osteitis Fibrosa Cystica

Presentation: -Complication of advanced hyperparathyroidism -Mutifocal bone involvement, sx of hypercalcemia (e.g. constipation, fatigue in primary hyperparathyroidism) or chronic renal failure (secondary hyperparathyroidism) -X-rays w/ lytic lesions Pathophysiology: -Advanced hyperparathyroidism causes increased resorption in cortical bone w/ subperiosteal thinning & cystic degeneration, marrow fibrosis

Perivalvular Abscess

Presentation: -Complication of infective endocarditis progression, most commonly of the aortic valve (esp if bicuspid) -Presents w/ early diastolic murmur (aortic regurgitation) at LUSB -Suspected in pts w/ IE w/ persistent bacteremia, new cardiac conduction abnormalities (e.g. heart block) Diagnosis: -Echo (TEE) Pathophysiology: -Cardiac conduction abnormalities common b/c aortic valve ring b/t right cusp & noncoronary cusp overlies interventricular septum, which harbors the proximal conduction system

Critical Illness Polyneuropathy

Presentation: -Complication of sepsis, presents w/ weakness & hyporeflexia, LMN signs (severe, diffuse, symmetric weakness & difficulty weaning from ventilator) -Typically affects pts w/ severe encephalopathy, coma Diagnosis: -Abnormal peripheral nerve conduction & EMG Pathophysiology: -Axonal injury of peripheral nerves

Tympanosclerosis

Presentation: -Conductive hearing loss, chalky/white patches visualized on TM -Risk factors: otitis media (often chronic), previous tympanostomy tube placement Pathophysiology: -Scarring of tympanic membrane -> TM stiffening, conductive hearing loss

Toxic Metabolic Encephalopathy

Presentation: -Confusion, lethargy, neuromuscular findings (e.g. bradykinesia, asterixis), global cerebral dysfxn Pathophysiology: -Etiologies include infection, toxins, metabolic derangements (e.g. electrolyte disturbances, uremia, hyperammonemia

Pyruvate Kinase Deficiency

Presentation: -Constant hemolysis, hepatosplenomegaly, skin ulcers, pigmented gallstones Pathophysiology: -AR deficiency in gene encoding pyruvate kinase

Overflow Incontinence

Presentation: -Constant, painless dribbling of urine from incomplete emptying and chronic urinary retention/overflow -Urinary frequency, nocturia Diagnosis: -Increased postvoid volume of 150+ mL in women, 50+ in men Treatment: -Cholinergics (e.g. bethanechol) to increase bladder contractility w/ neurogenic bladder -Intermittent self-catheterization if severe -Nonpharmacologic techniques: applying suprapubic pressure, timed voids, double voiding (attempting standing void after sitting void) Pathophysiology: -Impaired detrusor contractility, esp in postmenopausal women, DM neurogenic bladder, or external compression of urethral outlet (e.g. fibroids, prolapse, BPH) impeding bladder emptying

Polyarteritis Nodosa

Presentation: -Constitutional: fever, weight loss, malaise -Skin: tender nodules, livedo reticularis, ulcers, purpura, erythema nodosum -Renal: HTN, renal insuff, arterial aneurysms -Neuro: HA, seizures, mononeuritis multiplex, polyneuropathy -GI: mesenteric ischemia/infarction, abd pain -MSK: myalgias, arthritis Diagnosis: -Negative ANCA & ANA -Mesenteric angiography w/ microaneurysms & segmental/distal narrowing, irregular arterial constrictions (mesenteric angiography for mesenteric ischemia) -Tissue biopsy w/ nongranulomatous transmural inflammation Pathophysiology: -Systemic vasculitis of medium-sized arteries -Correlation w/ underlying hep B/C (immune complexes) -Fibrinoid necrosis of arterial wall -> luminal narrowing & thrombosis -> tissue ischemia-Internal/external elastic lamina damage -> microaneurysm formation -> rupture & bleeding

Herpetic Neuralgia

Presentation: -Continued burning pain & hyperesthesia, possible anesthesia/sensory deficits in the dermatomal distribution of resolved rash from shingles (occurs in ~5% of cases) -Pain may be constant or intermittent, typically associated w/ allodynia (pain elicited by nonpainful stimuli) -Risk factors: advanced age, severe initial pain, severe rash Diagnosis: -Acute: persists 30 days or shorter from rash onset -Subacute: persists >30 days but resolves within 4 mos of rash onset -Postherpetic: persists >4 mos from rash onset Treatment: -Acute & subacute: NSAIDs & analgesics (e.g. topical lidocaine/capsaicin if mild/moderate) -Postherpetic: TCAs, gabapentin, pregabalin Pathophysiology: -Acute: hemorrhagic inflammation of dermatomal sensory nerve d/t viral replication

Cholesteatoma

Presentation: -Continued, painless ear drainage for several wks despite abx -Tympanic membrane scarring, peripheral granulation, skin debris, pearly white mass, retraction pocket in superior portion of tympanic membrane -Complications: conductive hearing loss, cranial nerve palsies, vertigo, serious infections (e.g. brain abscesses, meningitis) -Risk factors: recurrent AOM, chronic middle ear effusion, tympanostomy tube placement Diagnosis: -Referral to otolaryngologist for otologic exam, possible CT and/or surgical visualization to confirm diagnosis Treatment: -Surgical excision Pathophysiology: -Benign growth of squamous epithelium w/ accumulation of keratin debris within middle ear -Can be congenital (~5 yo) or acquired via chronic middle ear disease/inflammation (e.g. repeat infections) -> formation of retraction pocket in tympanic membrane that can fill w/ granulation tissue -> chronic otorrhea

Medial Mid-Pontine Syndrome

Presentation: -Contralateral ataxia & hemiparesis of face/trunk/limbs (ataxic hemiparesis) -Variable loss of contralateral tactile/position sense -Possible CN deficits (CNs originating in pons): trigeminal (facial paresthesias), abducens (EOM palsy), facial palsy, vestibulocochlear (vertigo) Pathophysiology: -Occlusion of paramedian branches of basilar artery

Pleural Mesothelioma

Presentation: -Cough, chest pain, dyspnea, night sweats, weight loss -Hx asbestos exposure (working in cement, tile, automobile brake pad, or shipbuilding industries prior to asbestos control initiatives); tumors arise ~15-30 yrs after exposure -Unilateral pleural abnormality w/ large pleural effusion on CXR, nodular pleural thickening, obscuring of diaphragm -Pleura most commonly affected, other mesothelial sites include peritoneum, tunica vaginalis, pericardium Diagnosis: -Chest imaging w/ pleural calcifications, thickening, mass -Thoracentesis w/ cytology (35% sensitivity); pleural fluid analysis w/ exudative effusion w/ low pH & glucose -VATS or open thoracotomy Treatment/Management: -Largely palliative, cure rates low -Surgery, chemo, radiation -Median survival 9-13 mos Pathophysiology: -Malignant neoplasm derived from mesothelial lining of thoracic cavity

Pulmonary Edema

Presentation: -Crackles on exam Diagnosis: -CXR w/ diffuse bilat interstitial infiltrates, Kerley B lines, cephalization of pulmonary veins -Bx in HF shows septal widening w/ hemosiderin-laden macrophages Pathophysiology: -May be caused by L-sided HF, volume overload due to primary renal disease, increased capillary permeability in ARDS

Raynaud Phenomenon

Presentation: -Cyanosis, numbness, tingling, ulceration of distal digits exacerbated by cold -May be independent or associated w/ other disorders (e.g. cryoglobulinemia, SLE, Sjogren, scleroderma, connective tissue disease, vascular lesions, etc.) Diagnosis: -If indicated, further workup if suspicion of systemic disease (e.g. ANA, RF, CBC, BMP, UA, complement levels) -If sx suggestive of vascular lesions (e.g. asymmetric involvement, deficient pulses), digital plethysmography, arterial doppler US, or angiography Treatment/Management: -Dihydropyridine calcium channel blockers (e.g. nifedipine, amlodipine), diltiazem -Alpha blockers (e.g. prazosin) can provide relief, but pts usually become refractory after prolonged use -Nitroglycerin can be used as adjunct to CCBs in severe pts -Avoidance of precipitating meds & environmental factors (e.g. frostbite, vibration) Pathophysiology: -Exaggerated vascular response to cold temp or emotional stress

Craniopharyngioma

Presentation: -Cystic structure w/ calcification on imaging in the sella turcica -Visual field deficits (temporal field loss) and hormonal deficiencies/endocrinopathies (e.g. growth failure in children from low TSH or GH, pubertal delay in kids or sexual dsyfxn/hypogonadism in adults from low LH and FSH, DM insipidus from low ADH) from compression of optic chiasm, pituitary stalk -Peripheral vision loss may present as frequent bumping into furniture, clumsiness -DM insipidus presents w/ dehydration, polyuria, hypernatremia, polydipsia, dilute urine -Headache, vomiting -Bimodal age distribution (5-14 yo, 50-75 yo) -Poor linear growth, worsening headaches w/ papilledema (obstructive hydrocephalus) Diagnosis: -MRI/CT w/ calcified and/or cystic suprasellar mass Treatment: -Surgical resection +/- radiation therapy Pathophysiology: -Benign, slow growing tumor derived from remnants of Rathkes pouch

Aspiration Pneumonia

Presentation: -Days after aspiration event (indolent course), presents w/ fever, cough, increased putrid sputum that is often foul-smelling, often w/ concurrent periodontal disease -CXR infiltrate in dependent lung segment (classically RML or lower lobes if upright, posterior segments of upper lobes & superior lower lobes if supine) -Can progress to abscess -Risk factors: altered consciousness impairing cough reflex/glottic closure (e.g. heavy EtOH use, dementia, drug intoxication, seizures, sedation), dysphagia due to neuro deficits (e.g. stroke, neurodegenerative disease like dementia, parkinsonism, myasthenia), upper GI disorders (e.g. GERD), mechanical compression of aspiration defenses (e.g. nasogastric & endotracheal tubes, bronchoscopy, endoscopy), protracted vomiting, large-volume tube feeds in recumbent position, PPI therapy Diagnosis: -Videofluoroscopic swallowing study, bedside swallowing study Treatment/Management: -Abx covering community-acquired PNA pathogens (e.g. CTX + azithromycin) -If empyema or lung abscess present, clindamycin (anaerobic coverage) or beta-lactam/beta-lactamase inhibitor (e.g. Augmentin) -Multidisciplinary dysphagia rehab program w/ input from dietary, nursing, speech therapy -Nutrition w/ thickened liquids, modified swallowing techniques Prevention: -Oral care, diet modification for pts w/ dysphagia (thickened liquids, chin-tuck position while eating), elevation of head of bed (30-45 degrees) Pathophysiology: -Lung parenchyma infection from aspiration of upper airway or stomach microbes (anaerobes + aerobes, mixed flora) that colonize oropharyngeal secretions

Severe Acute Pancreatitis

Presentation: -Defined as acute pancreatitis w/ signs of organ failure (e.g. respiratory, CV, renal) persisting >48 hrs, encompasses 15-25% of pts w/ acute pancreatitis -Predictors of poor prognosis include risk factors (obesity, older age >55), clinical findings (AMS, SIRS w/ leukocytes >12,000 & temp >100.4), lab findings of intravascular depletion (BUN >20, Cr >1.8, HCT >44%), CRP >150 within 48 hrs, radiologic findings (CXR w/ pulm infiltrates or pleural effusion, CT w/ severe pancreatic necrosis; indicate third spacing of fluid) -Grey Turner sign: reddish/brown ecchymoses of the flanks associated with fulminant hemorrhagic pancreatitis from retroperitoneal hemorrhage; very poor prognostic sign -Cullen sign: periumbilical bluish coloration indicating hemoperitoneum -Dyspnea, tachypnea, basilar crackles, hypotension -Complications: pseudocyst, peripancreatic fluid collection, necrotizing pancreatitis, ARDS, renal failure, GI bleed Diagnosis: -Clinical prediction scores (SIRS score, BISAP, Ranson criteria) that incorporate predictors above -BUN especially reliable in predicting mortality -CT/MRCP may show pancreatic necrosis, extrapancreatic inflammation Treatment/Management: -Aggressive fluid resuscitation, ICU admission Pathophysiology: -Intravascular volume depletion w/ hemoconcentration occurs due to third spacing of fluid from inflammatory response and increased capillary permeability -> shock, end-organ damage -Pleural effusion usually unilateral, L-sided, exudative, high amylase

Hyperkalemic Emergency

Presentation: -Defined as serum K+ of >6.5, rapid escalation in serum K+ due to tissue breakdown, or symptomatic hyperkalemia (e.g. ECG changes, arrhythmias) Treatment: -Calcium gluconate IV to rapidly stabilize muscle membranes, insulin + glucose to drive K+ into cells -Oral cation exchange agent e.g. patiromer, zirconium cyclosilicate (bind K+ in stool in exchange for Na+ or calcium, facilitate K+ excretion) -Hemodialysis if ESRD and very severe

Hemophilia A & B

Presentation: -Delayed/prolonged bleeding after minor trauma: hemarthrosis after mild injury, intramuscular hematomas, GI/GU tract bleeding, intracranial hemorrhage, delayed post-procedure bleeding (e.g. oozing from dental extraction site) -Easy bruising, family hx (X-linked) -Complications: hemophilic arthropathy (w/ recurrent hemarthrosis in same joint) Diagnosis: -Increased activated PTT (intrinsic pathway has another T IN it), normal platelet count and PT, absent/decreased factor VIII (A) or factor IX (B) activity -Hemophilic arthropathy detected by MRI (X-ray less sensitive) -Prolonged PTT corrects w/ mixing study Treatment: -Factor replacement, desmopressin for mild hemophilia A -Factor replacement can prevent hemophilic arthropathy Pathophysiology: -Factor VIII/IX deficiency -Hemophilic arthropathy = joint damage caused by intra-articular bleeding -> hemosiderin deposition within joint -> synovial inflammation -> fibrosis, destruction of cartilage & bone

Vitiligo

Presentation: -Depigmented macules on acral areas & extensor surfaces, commonly on face -Lesions may be symmetric, dermatomal, or unilateral -Most cases progress gradually, spontaneous repigmentation in 10-20% of cases -Associated w/ increased incidence of other autoimmune disease (esp autoimmune thyroid disease, also SLE, pernicious anemia, Addison, RA) Diagnosis: -Skin biopsy if uncertain (shows loss of melanocytes, often w/ scattered lymphocytes @ lesion border) Treatment: -Topical corticosteroids for limited disease -Oral corticosteroids, topical calcineurin inhibitors, PUVA for extensive/unresponsive disease Pathophysiology: -Regional destruction of melanocytes, typically autoimmune

Fungal Keratitis

Presentation: -Develops after corneal injury in agricultural workers, immunocompromised pts following corneal injury typically involving contaminated soil (e.g. gardening) -On exam, cornea w/ multiple stromal abscesses, ulcerations w/ feathery margins & satellite lesions, mucopurulent discharge, indolent course Treatment/Management: -Topical/oral antifungal therapy Pathophysiology: -May involve Candida

VIPoma

Presentation: -Diarrhea during fasting & dehydration, tea-colored stools -Labs: hypokalemia, hypochlorhydria Pathophysiology: -Pancreatic tumor producing excess vasoactive intestinal peptide (VIP)

Breast Mass in Males

Presentation: -Differential includes fat necrosis (e.g. after trauma), lipoma, breast cancer -Fat necrosis & breast cancer may demonstrate nipple involvement (e.g. nipple inversion, fixed mass) Diagnosis: -Like female breast masses, should be followed up w/ imaging (mammography, US, core biopsy if appropriate), esp w/ suspicious features (firm, nontender, fixed mass, nipple inversion) -Core biopsy may be performed w/ suspicious radiographic features (e.g. microcalcs, spiculated margins)

Acute Respiratory Distress Syndrome (ARDS)

Presentation: -Diffuse pulmonary edema (crackles on exam) w/ decompensation over hrs, increased oxygen requirement, diffuse bilat infiltrates on CXR c/w pulmonary edema -Usually takes hrs (7-62) after insult to develop -Mortality rate of 40% in hospital, primarily d/t multiorgan failure, morbidity rate of 50% w/ chronic cognitive impairment & physical debility, 25% w/ chronic pulm dysfxn -Risk factors: trauma, pulm contusion, infection, massive transfusion, acute pancreatitis Diagnosis: -New/worsening respiratory distress within 1 wk of insult -Bilat opacities on CXR c/w pulmonary edema and not due to CHF/fluid overload -Hypoxemia w/ PaO2/FiO2 ratio of 300 or lower Treatment/Management: -Mechanical ventilation w/ low TV (6 ml/kg) & plateau pressure (to prevent alveolar overdistention & barotrauma, improves mortality), high PEEP (sometimes 10+, prevents alveolar collapse at end of expiration/reduces atelectasis), permissive hypercapnia, goal SpO2 of 88-95% w/ lowest feasible FiO2 (usually weaned to <60% ASAP after intubation/stabilization to avoid O2 toxicity) -Treat underlying condition (e.g. abx for source control in sepsis) -Prevent iatrogenic harm by maintaining negative/neutral fluid balance (diuretics, renal replacement therapy, minimizing boluses, concentrating IV drips, pressors), timely extubation, minimizing sedation -Corticosteroids in select pts w/ moderate to severe early ARDS Pathophysiology: -D/t direct (e.g. PNA, inhalation, aspiration) or indirect (e.g. sepsis, pancreatitis, trauma) lung injury -Triggered by diffuse endothelial damage & inflammation -> increased capillary permeability -> capillary fluid/protein/cytokine/neutrophil leakage into alveoli -> alveolar collapse due to loss of surfactant, alveolar damage, diffuse pulmonary edema -Fluid from pulmonary edema fills alveoli -> alveolar collapse -> extreme V/Q mismatch w/ R-to-L shunting -> hypoxemia -> hypoxic pulmonary vasoconstriction -> pulm HTN, increased RV afterload -Fluid in lungs prevents air delivery, so increased FiO2 does not correct hypoxemia -Fluid in interstitium also leads to decreased lung compliance (alveolar flooding), increased A-a gradient from impaired diffusion -Vs. pathologies w/ focal intrapulmonary shunting where other alveoli can compensate w/ increased FiO2 by increasing O2 transfer (e.g. atelectasis, lobar PNA, basilar pulmonary edema, small PE), diffuse pulmonary edema in ARDS cannot be corrected by increased FiO2

Telogen Effluvium

Presentation: -Diffuse, acute, noninflammatory thinning of hair (not in patches) -Scalp & hair fibers appear normal -Slow hair growth, shedding of hair shafts -Hair pull test (shafts easily pulled out, extraction of >10-15% of hairs) -Usually responds spontaneously -Risk factors: recent precipitating event including illness, stressor, new med (beta blockers, anticoags, systemic retinoids, anticonvulsants, antithyroid meds) Treatment: -Observation, reassurance, address underlying cause -Self-limited, may take up to a yr to resolve Pathophysiology: -Due to premature shedding, often triggered by stress (e.g. major illness, fever, surgery, malnutrition, iron deficiency, emotional trauma, pregnancy/childbirth, endocrine/nutritional disorders, hormonal changes like starting/stopping OCPs) -Hair follicles normally go through 3 stages: linear growth phase (anagen, 90% of follicles), transformative phase (regression/apoptosis, catagen, <1%), rest/shedding phase (telogen, 10%) -Telogen effluvium = shair shift into telogen phase

Subacute Combined Degeneration

Presentation: -Diminished vibration & position sense, spastic paresis w/ hyperreflexia & +Babinski (damage to lateral corticospinal tracts), sensory ataxia (spinocerebellar tract damage, +Romberg/swaying w/ eyes closed or in the dark), lower extremity paresthesias (often the presenting sx), incontinence -Neuropsych sx due to defective myelin in brain (e.g. irritability, mood changes, memory deficits, dementia, delirium w/ hallucinations & bizarre behavior) -Labs: indirect hyperbili d/t ineffective erythropoiesis, signs of hemolytic anemia (high LDH, low hapto), low/normal reticulocytes -Risk factors: age, chronic PPI therapy Diagnosis: -Increased serum methylmalonic acid & homocysteine levels, low B12 (normal B12 does not exclude) -Associated w/ smear findings c/w B12 def (macrocytosis, hypersegmented neutrophils) -MCV may be borderline/normal w/ concomitant iron deficiency Pathophysiology: -B12 deficiency causes degeneration of dorsal columns, damage to myelin of peripheral nerves

Biliary Colic

Presentation: -Dull, recurrent, episodic postprandial RUQ pain or epigastric pain (worsens over hr following eating, subsides w/ gallbladder relaxation, radiates to back & R shoulder), usually lasting <6 hrs -Fatty meals may trigger episodes; pts can also develop nocturnal sx or pain independent of meals -N/V, diaphoresis -Vital signs, WBCs, LFTs all normal, no peritoneal signs/guarding -Risk factors (for gallstone disease): female, >40 yo, hypertriglyceridemia, obesity Diagnosis: -RUQ US (shows gallstones as echogenic foci w/ shadow and/or sludge as echogenic foci w/o shadow) -If typical biliary colic sx but no gallstones on imaging, further workup w/ cholecystokinin-stimulated cholescintigraphy to evaluate for functional gallbladder disorder (cholecystectomy in pts w/ low gallbladder ejection) Treatment/Management: -Acute pain management -Prophylactic cholecystectomy (usually elective & laparoscopic) -Possible ursodeoxycholic acid in poor surgical candidates or pts refusing surgery Pathophysiology: -Occurs when gallbladder contracts against a gallstone or sludge that is temporarily blocking cystic duct -Can progress to cholecystitis w/ complete obstruction

Brain Metastases

Presentation: -Duration of sx usually <2 mos -Sites of metastasis usually at gray-white junction or watershed zones -On imaging, lesions often multifocal and spherical, well-circumscribed, ring-enhancing, often surrounded by vasogenic edema -Most common type of intracranial brain tumor (most commonly from NSCLC) Diagnosis: -Contrast-enhanced brain MRI Treatment/Management: -Favorable survival outcomes: <65, good functional performance status, stable extracranial disease -If single lesion in surgically approachable location & good performance status, surgical resection followed by stereotactic radiosurgery (SRS) or whole brain radiation therapy (WBRT) to tumor bed -SRS used as alternate therapy in pts who are not candidates for surgery, have surgically inaccessible lesion, or smaller met <3 cm -WBRT or supportive care (e.g. glucocorticoids, pain control) usually recommended for pts w/ multiple brain mets or poor performance status -Brachytherapy (implantation of radioactive source directly into intracerebral mass or surgical cavity to allow higher radiation doses to be delivered directly into brain w/o affecting other organs) used in conjunction w/ surgery or for recurrence after WBRT/surgery -Chemotherapy occasionally used if met is from chemosensitive tumor (e.g. SCLC, lymphoma, choriocarcinoma) Pathophysiology: -Most common tumors involved: lung (esp small cell)/breast cancer, melanoma, RCC, colon cancer -Usually spread hematogenously

Cor Pulmonale

Presentation: -Dyspnea & fatigue on exertion, exertional angina, exertional syncope, anorexia/abd pain (d/t hepatic congestion) -JVD w/ hepatojugular reflex, peripheral edema, palpable RV heave, loud P2, RV S3, tricuspid regurg murmur (holosystolic, augments w/ inspiration), hepatomegaly w/ pulsatile liver, ascites, pleural effusion -Most commonly associated w/ COPD, may present w/ distant heart sounds -End-stage disease: hypotension, tachycardia, signs of cardiogenic shock due to decreased SV -CXR w/ enlarged central pulmonary arteries, loss of retrosternal air space d/t RVH -Labs: elevated transaminases, hypoalbuminemia Diagnosis: -Gold standard catheterization showing elevated filling pressures in R heart (elevated CVP, RV end-diastolic pressure, mean pulm artery pressure), decreased CO, pHTN, absence of L heart diseaes -RV dysfxn on Echo (pulm HTN, dilated RV, tricuspid regurg) -ECG w/ incomplete/complete RBBB, R axis deviation, RV hypertrophy, RA enlargement Treatment/Management: -Optimizing RV dynamics (preload/afterload/contractility) w/ supplemental O2, diuretics, treatment of underlying etiology, IV inotropes for severe decompensation Pathophysiology: -Impaired RV fxn due to pulm HTN that occurs as a result of underlying pulm parenchymal disease & chronic hypoxia (COPD, pulm vascular disease, OSA, CF, ILD, chest wall disorders) or pulm vascular disease (e.g. PE, pulm arterial HTN) -Absence of L-sided heart disease

Acute Pyelonephritis

Presentation: -Dysuria, frequency/urgency, pyuria, fever, CVA tenderness/flank pain, suprapubic pain, abd/pelvic pain, hematuria (grossly is abnormal), N/V -Uncomplicated: otherwise healthy, nonpregnant -Complicated: T2DM, urinary obstruction/instrumentation (e.g. h/o nephrolithiasis), renal failure, immunosuppression, hospital-acquired, complications (renal corticomedullary abscess, perinephric abscess, emphysematous pyelonephritis, papillary necrosis) Diagnosis: -UA, urine cultures -Additional urologic imaging (e.g. CTAP) if no clinical improvement w/ abx after 48-72 hrs, hx of nephrolithiasis, or w/ unusual urinary findings (e.g. gross hematuria, suspicion for urinary obstruction) Treatment: -Uncomplicated: oral fluoroquinolone or SMX-TMP, IV abx & fluids if elderly/vomiting/septic then transition to PO abx after 48 hrs stabilization & culture guidance -Complicated: IV fluoroquinolone, aminoglycoside (e.g. gentamicin) +/- ampicillin, extended-spectrum beta-lactam/cephalosporin, increased risk of abx resistance & treatment failure

Infectious Urethritis

Presentation: -Dysuria, mucopurulent/watery discharge at urethral meatus, increased urinary frequency, may also be asymptomatic -Risk factors: sexual contact, multiple partners Diagnosis: -Sterile pyuria (WBCs but no bacteria), intracellular gram- diplococci if gonococcus -Urine Gram stain & culture, negative culture & gram stain may be seen w/ chlamydia -NAAT on first-catch urine/vaginal/anal sample for confirmation Treatment: -Ceftriaxone if gonococcus alone -Ceftriaxone + doxycycline if gonococcal + chlamydial -Azithromycin if chlamydia/Mycoplasma (single dose) or 7 days doxycycline -Metronidazole if Trichomonas -In older men, fluoroquinolones (e.g. ofloxacin) Pathophysiology: -Neisseria gonorrheae, Chlamydia trachomatis, Mycoplasma genitalum, Trichomonas vaginalis, Ureaplasma Urealyticum -Leukocytes w/o visible organisms b/c C. trachomatis & M. genitalum do not have significant peptidoglycan in cell wall -In older men, usually d/t coliform organisms (e.g. E. coli)

Urinary Schistosomiasis

Presentation: -Dysuria, urinary frequency terminal hematuria -Labs: peripheral eosinophilia, hematuria, anemia -Complications: bladder cancer Diagnosis: -Urine sediment microscopy to identify eggs Treatment: -Praziquantel Pathophysiology: -Parasitic blood fluke infection affecting >200 million worldwide, w/ greatest prevalence in sub-Saharan Africa -Parasite lives in specific types of freshwater snails, humans in endemic are infected as children when bathing in contaminated freshwater -Usually d/t Schistosoma haematobium

Atrial Flutter

Presentation: -ECG w/ sawtooth flutter wave, atrial rate usually ~300/min, may have rapid ventricular response -> irregular rhythm -If constant ventricular response, may be regular rhythm -Risk factors: CAD, HTN, HF, cardiac surgery -Complications: like AFib, risk of thromboembolism Treatment: -Chronic anticoag (rivaroxaban, apixaban, dabigatran) -Rate control if needed (no rhythm control agents, can precipitate RVR) -Radiofrequency ablation of reentrant circuit or cardioversion (pharmacologic or electrical, must be preceded by anticoag if >48 hrs of flutter d/t risk of precipitating thromboembolism) Pathophysiology: -Large reentrant circuit involving cavotricuspid isthmus of RA

Alpha-1 Antitrypsin Deficiency

Presentation: -Emphysematous lung disease, chronic hepatitis (including neonatal), cirrhosis, necrotizing panniculitis (painful, erythematous nodules & plaques on thighs & buttocks) -Lungs: COPD w/ chronic dyspnea on exertion, decreased breath sounds, productive cough, wheezing, recurrent resp infections, often presents in 30s in smokers, 40s in nonsmokers (suspect in COPD in young pt, minimal/no h/o smoking, fhx of emphysema or liver disease), bullae at lung bases -Complications: HCC -Labs: slight elevation in transaminases Diagnosis: -Serum AAT levels, PFTs Treatment: -IV replacement of AAT, bronchodilators & corticosteroids as needed -Lung/liver transplant Pathophysiology: -Codominant genetic disorder

Papilledema

Presentation: -Enlarged blind spots, momentary vision loss that varies w/ head positioning (e.g. worse w/ standing up) -Can lead to permanent vision loss -Swelling of optic disc on fundoscopy (blurred disc margins, obscuration of vessels, splinter hemorrhages, serpentine engorgement of small veins) Diagnosis: -Fundoscopy, CT/MRI to exclude mass lesion or other CNS abnormalities as causes of increased ICP Pathophysiology: -Associated w/ increased ICP -Etiologies: idiopathic intracranial HTN, mass lesions, increased CSF production, decreased CSF outflow (e.g. venous sinus thrombosis)

Bullous Impetigo

Presentation: -Enlarging, flaccid bullae filled w/ yellow fluid confined to single location -Ruptured lesions w/ collarette of scale at periphery -Lesions may be tender/pruritic, may present w/ or w/o fever, pts usually appear well Diagnosis: -Clinical Treatment: -PO abx (e.g. cephalexin, clindamycin) Pathophysiology: -Superficial S. aureus infection of epidermis, usually caused by bacterial invasion through break in skin barrier (e.g. wound, eczema, burn) -S. aureus produces exfoliative toxin that disrupts cell adhesion of superficial skin layers

Acute Necrotizing Pancreatitis

Presentation: -Epigastric pain, imaging showing pancreatic edema & necrosis, usually >7 days after initial presentation of uncomplicated acute panc -Consider in acute panc pts w/ persistent abd pain & whose condition improves but then deteriorates w/ leukocytosis, fever, unstable VS -Predisposes to infected pancreatic necrosis, resultant sepsis Diagnosis: -Abd CT, may show gas if infected pancreatic necrosis Treatment: -IV abx (meropenema, fluoroquinolone + metronidazole) followed by possible debridement once necrotic collection becomes walled off Pathophysiology: -Pancreatitis -> uncontrolled release of pancreatic enzymes -> autodigestion of pancreatic parenchyma & peripancreatic tissues -> necrotic collection that is initially sterile -> may be colonized by enteric pathogens (E. coli, Pseudomonas, Enterococcus) within 7-10 days -> septic decompensation

Acute Pancreatitis

Presentation: -Epigastric tenderness/pain radiating to back, nausea, vomiting -Elevated amylase, lipase >3x upper limit of normal, ALT >150 suggests biliary pancreatitis -Can occur postop in pts w/ hypotension -If severe, may present w/ fever, tachypnea, hypoxemia, hypotension -If associated w/ hypertriglyceridemia, may have fhx of dyslipidemia or poorly controlled diabetes, +/- eruptive xanthomas -Labs: leukocytosis, elevated lipase/amylase, elevated TGs, ALT >150 & hyperbili in biliary pancreatitis -Complications: SIRS, ARDS, distributive shock, pleural effusion, ileus, pancreatic pseudocyst/abscess/necrosis Diagnosis: -Requires 2+ of: 1. epigastric pain radiating to back, 2. amylase/lipase >3x normal, 3. imaging findings c/w pancreatitis (focal or diffuse pancreatic enlargement w/ heterogeneous enhancement w/ IV contrast on CT or diffusely enlarged & hypoechoic pancreas on US; imaging not necessary if 1&2 are present) -RUQ US if suspected gallstone pancreatitis (shows biliary dilation, choledocholithiasis), ERCP if US inconclusive w/ still suspicion for biliary disease -CT showing parenchymal enhancement w/ IV contrast (in pts w/o pancreatic necrosis), pseudocyst formation, fat stranding, and/or peripancreatic fluid collections -CT not required for diagnosis, but may be used to confirm & identify complications like hemorrhage/necrosis Treatment/Management: -Supportive measures (IVF, antiemetics, pain control) -Oral/tube feeding within 24 hrs as tolerated, starting w/ soft & low-fat diet -Gallstone panc: ERCP if CBD stone present, interval cholecystectomy to prevent recurrence -Hypertriglyceridemia-induced: apheresis if severe (e.g. fever, tachycardia, leukocytosis, lactic acidosis, hypocalcemia), insulin if not severe or if apheresis unavailable, fibrates (e.g. fenofibrate, gemfibrozil) to prevent recurrence Pathophysiology: -Etiologies include chronic EtOH use (~40%), gallstone pancreatitis (~40%), hypertriglyceridemia, hypercalcemia, drugs (azathioprine, didanosine, pentamidine, valproic acid, thiazides, furosemide, sulfasalazine, metronidazole, tetracyclines), infections (CMV, Legionella, Aspergillus, HBV, HIV, HSV, ascariasis, Salmonella, Cryptosporidium), iatrogenic (post-ERCP, ischemic/atheroembolic following vascular procedure, cardiac catheterization), trauma -Hypertriglyceridemia only considered if >1000

Pancreatic Pseudocyst

Presentation: -Episode of pancreatitis in recent past (seen in ~10% of pts after acute panc, usually 4+ wks after), presents w/ progressive abd distention, epigastric fullness/tenderness, N/V -No peritoneal signs -Labs: possible increased serum amylase/lipase -Complications: spontaneous infection, duodenal/biliary obstruction, pseudoaneurysm/fistulization (from digestion of adjacent vessels & organs), pancreatic ascites, pleural effusion, GI hemorrhage Diagnosis: -Abd CT w/ round, well-circumscribed, encapsulated fluid collection in pancreatic bed Treatment/Management: -If minimal/no sx & no complications (e.g. pseudoaneurysm), expectant management used initially (symptomatic therapy, NPO) -If significant sx (e.g. abd pain, vomiting) or complications (infection, pseudoaneurysm), endoscopic drainage of pseudocyst -If evidence of pseudoaneurysm, usually embolized prior to drainage -If pseudocyst infected, IV abx Pathophysiology: -Mature, walled off pancreatic fluid collections (usually no necrosis or solid material) surrounded by thick fibrous capsule & containing enzyme-rich fluid, tissue, debris -Can leak amylase-rich fluid into circulation

Paroxysmal Nocturnal Hemoglobinuria

Presentation: -Episodes of hemolytic anemia (gross hematuria), cytopenias (due to impaired hematopoiesis), hypercoagulability (e.g. cerebral/intraabdominal venous thrombosis, hepatic venous thrombosis, etc.), fatigue, dyspnea -May be triggered by stress, infection -Often presents in 40s -Labs: reticulocytosis, CBC w/ cytopenias (hypoplastic/aplastic anemia, thrombocytopenia, leukopenia), signs of hemolysis (elevated LDH, low haptoglobin, indirect hyperbilirubinemia), hemoglobinuria on UA, negative Coombs Diagnosis: -Absence of erythrocyte CD55 and CD59 on flow cytometry testing, UA Treatment/Management: -Iron/folate supplementation -Eculizumab (mab that inhibits complement activation) Pathophysiology: -Hematopoietic stem cell disease w/ episodes of complement-mediated hemolysis -Autoimmune disorder w/ both extravascular & intravascular hemolysis -Acquired genetic defect resulting in absence of glycosylphosphatidylinositol anchor (connects CD55 & 59 to cell surface, both of which inhibit complement activation on RBCs) -> excessive formation of complement MAC -> hemolysis

Chronic Spontaneous Urticaria

Presentation: -Episodes of spontaneously resolving urticaria, sx >6 wks w/o apparent trigger -Intensely pruritic, round/serpiginous erythematous plaques/wheals that appear over min to hrs -Individual lesions lasting <24 hr +/- associated angioedema (40% of cases) -Etiologies: 80-90% idiopathic, atopic disorders, autoimmune disorders -Usually self-limited and resolves spontaneously within 2-5 yrs, >50% of pts respond to standard therapy Diagnosis: -CBC, CRP/ESR, LFTs, UA, skin bx to exclude urticarial vasculitis or mastocytosis (only if sx or labs suggest different etiology than spontaneous urticaria) Treatment/Management: -Initial therapy of 2nd gen H1 antihistamines (e.g. loratadine, ceterizine) -Avoidance of aggravating conditions (e.g. heat, NSAIDs, cold, stress) -If persistent sx despite above, may consider adding 1st gen H1 blocker at bedtime, leukotriene receptor antagonists (e.g. montelukast), H2 blockers (e.g. cimetidine), brief course of oral glucocorticoids -If still refractory, may consider immune-modulating therapies as last case scenario (e.g. hydroxychloroquine, tacrolimus, omalizumab)

Cocaine Abuse

Presentation: -Epistaxis & rhinitis & septal perforation w/ intranasal use, HA, chest pain, personality/mood changes, changes in sleep patterns, unexplained weight loss, financial difficulties -On exam, new-onset HTN, tachycardia, dilated pupils, psychomotor agitation, tremor -Features of secondary HTN (age <30, no fhx, severe/malignant HTN, resistant to 3-drug regimen, new-onset worsening after previous HTN control) -CXR may show ST segment & T wave changes c/w ischemia -Complications: seizures, MI, aortic dissection, intracranial hemorrhage, neurologic ischemia/stroke, hemorrhagic alveolitis, PTX Diagnosis: -Urine drug screen Treatment/Management: -Supplemental O2 -Benzos for HTN & anxiety -Aspirin for inhibition of cocaine-induced platelet aggregation -Nitroglycerin & CCBs for vasoconstrictive pain -Phentolamine for persistent HTN -Immediate cardiac catheterization if indicated -CT/MR angio/TEE of chest if c/f aortic dissection (e.g. arm weakness, back pain, worsening CP) -Caution w/: fibrinolysis (increased risk of ICH), beta blockers (increased risk of coronary vasoconstriction) Pathophysiology: -CP due to coronary vasoconstriction -Cocaine potentiates sympathetic activity by inhibiting norepi reuptake -> overstimulation of adrenergic receptors -> increased myocardial contractility, arterial vasoconstriction -Causes increased platelet activity & thrombus formation

Acute Paronychia

Presentation: -Erythema, edema, & tenderness of nail fold -Minimal changes to/sparing of fingernail -Often follows injury to nail fold or cuticle (e.g. overzealous manicuring, nail biting, thumb sucking) by 2-5 days -Complications: finger abscess Treatment/Management: -Warm soaks in water, Burow solution (aluminum acetate), or 1% acetic acid -Antiseptic soaks (e.g. chlorhexidine) -Topical abx (e.g. mupirocin) -If abscess, drainage -If severe or immunocompromised, oral abx Pathophysiology: -Localized bacterial infection of nail fold, usually by gram+ skin flora or oral flora (e.g. Eikenella, Fusobacterium)

Candida Intertrigo

Presentation: -Erythematous ("beefy red") plaques w/ overlying scale in symmetric "kissing" or mirror image pattern across skin fold w/ multiple satellite lesions near primary infection -Dermatitis occurring in inguinal, axillary, gluteal, and inframammary folds -Pruritic rash, especially w/ tight clothing/friction/obesity (causes skin maceration/trauma) -High risk populations include immunocompromised (e.g. systemic steroids, DM) Diagnosis: -Usually clinical, can be confirmed by microscopic visualization of pseudohyphae from skin scrapings, KOH prep Treatment: -Topical azoles (clotrimazole, ketoconazole, miconazole) or nystatin or terbinafine -Use of skin-drying agents, correction of underlying predisposing conditions to reduce recurrence risk

Seborrheic Dermatitis

Presentation: -Erythematous plaques and/or yellow, greasy scales peaking in infancy (1st yr) and adulthood (30-60 yo), mild pruritus -Located on scalp ("cradle cap," dandruff), face (eyebrows/eyelids, posterior ears/external ear canal, nasolabial folds), umbilicus, sternal area, axillae, groin, gluteal crease -Severe disease may be associated w/ CNS disorders (esp Parkinson disease, dementia) & HIV Treatment: -First line is emollients, nonmedicated shampoos, tar-based creams -Second line is topical antifungals (ketoconazole, selenium sulfide) or low-potency glucocorticoids (e.g betamethasone valerate) or calcineurin inhibitors (e.g. pimecrolimus, tacrolimus) for widespread/refractory cases -Keratolytic agents (e.g. salicylic acid) -Course is chronic & relapsing: may require intermittent retreatment w/ topical ketoconazole or ciclopirox every 1-2 wks Pathophysiology: -Colonization by Malassezia species affecting areas w/ many sebaceous glands -Chronic superficial inflammatory process predominating in areas rich in sebaceous glands

Narcolepsy

Presentation: -Excessive daytime sleepiness, may affect academic performance -Onset often in late teens to early 20s -Cataplexy: sudden loss of tone present in ~70% of pts w/ narcolepsy, either conscious (brief episodes of sudden bilat muscle tone loss precipitated by emotions such as laughter or joking) or spontaneous (abnormal facial movements w/o emotional triggers) -Hypnagogic hallucinations: associated w/ sensation of falling, occur while falling asleep (e.g. hearing people walking around, hearing one's name being called), may cause sudden, brief jerks -Hypnapompic hallucinations: hallucinations while waking up Diagnosis: -Polysomnography showing REM sleep latency of 15 min or shorter, multiple spontaneous awakenings, reduced sleep efficiency -Hypocretin-1 deficiency by CSF analysis Treatment/Management: -Modafinil: novel stimulant w/ better side effect profile than other stimulants & lower abuse potential -Nonpharmacologic interventions: scheduled naps during day, sleep hygiene measures -Cataplexy treatment w/ SNRIs (e.g. venlafaxine), SSRI, TCA, sodium oxybate (can improve nocturnal sleep & has anti-cataplectic properties but rarely used d/t abuse potential) -Methylphenidate: second-line stimulant if modafinil ineffective

Primary Polydipsia

Presentation: -Excessive thirst drive & water intake -Risk factors: psychiatric conditions, taking antipsychotics (dry mouth), CNS lesions -Labs: dilute urine (Osm <100), low serum osmolality (Osm <290), hyponatremia Diagnosis: -Water deprivation testing -> high urine osmolality reflecting appropriate concentration of urine -No change in urine osmolality in response to desmopressin Pathophysiology: -ADH-independent, due to excessive intake, kidneys appropriately dilute urine

Subclavian Steal Syndrome

Presentation: -Exercise-induced arm pain, fatigue paresthesias, coolness, color change (due to ischemia) -BP differential b/t L & R arms; lower SBP in affected arm (>15 mm) -Systolic bruit in supraclavicular fossa of affected arm, possible S4 d/t LVH from systemic HTN -Vertebrobasilar sx w/ concurrent atherosclerosis of circle of Willis (dizziness, ataxia, dysequilibrium); worsens w/ exercising affected UE Diagnosis: -Doppler US, MR angiography Treatment: -Lifestyle management (e.g. lipid-lowering interventions, smoking cessation), sometimes stent placement Pathophysiology: -Stenosis/atherosclerosis of the subclavian artery proximal to the veretbral artery -> decreased pressure in distal subclavian artery -> reversal/steal of blood into ipsilateral vertebral artery -Vertebrobasilar sx: exercising affected UE causes arterial vasodilation that further lowers distal pressure -> worsening of vertebrobasilar sx

Heart Failure with Preserved Ejection Fraction (HFpEF)

Presentation: -Exertional dyspnea, orthopnea (choking sensation/dyspnea when lying flat), paroxysmal nocturnal dyspnea (dry cough worse when lying down at night), bibasilar rales (pulm congestion), LE edema, JVD, S3 on auscultation -Normal or near-normal EF (>50%) on Echo -Risk factors: chronic HTN (concentric LV hypertrophy), obesity & sedentary lifestyle (myocardial interstitial fibrosis), CAD & related risk factors such as DM Diagnosis: -BNP (<100 can help exclude HF, though obesity can cause falsely low levels) -CXR (shows pulmonary edema beginning as interstitial edema w/ kerley B lines, can progress to alveolar edema w/ alveolar opacification) -Echo: LV ejection fraction normal, may see LA dilation, diastolic dysfxn not always detectable Treatment/Management: -Specific therapies to reduce hospitalization & possibly mortality (MRAs including spironolactone, SGLT2 inhibitors including dapagliflozin) -Control BP & HR -Address concurrent conditions (e.g. AFib, MI, CAD, OSA) -Treat volume overload w/ diuretics (loop) -Exercise training/cardiac rehab Pathophysiology: -Causes include LV diastolic dysfxn, valvular heart disease, pericardial disease, systemic disorders (high-output HF) -LV diastolic dysfxn: HTN w/ concurrent LVH, constrictive CM, HCM; all due to impaired myocardial relaxation or increased LV wall stiffness -> increased LV end diastolic pressure -> increased pulm & LA pressure -Valvular disease: aortic stenosis/regurg, mitral stenosis/regurg -Pericardial disease: constrictive pericarditis, tamponade -Systemic disorders: thyrotoxicosis, severe anemia, large AVF, beriberi, Paget disease -Degree of pulmonary edema can vary b/c pulmonary lymphatics can increase fluid outflow rate; can prevent significant alveolar edema seen in acute HF exacerbation (e.g. w/ crackles on exam)

Sjogren Syndrome

Presentation: -Exocrine features: keratoconjunctivitis sicca (dry/itchy eyes w/ gritty/burning sensation, conjunctival injection, dental caries, cough, prominent bulbar blood vessels, eye discharge), dry mouth, salivary hypertrophy, xerosis, dysphagia (to solids, chronic esophagitis), thrush -Extraglandular features: Raynaud phenomenon, cutaneous vasculitis, arthralgia/arthritis, interstitial lung disease, non-allergic rhinitis, bronchiectasis, B-cell non-Hodgkin lymphoma (neck mass, lifetime risk ~5%) -Dyspareunia due to dryness of vaginal mucosa -Can be isolated or occur in conjunction w/ other autoimmune disease (e.g. RA) -Risk factors: middle-aged female -Labs: ANA, RF may be positive Diagnosis: -Schirmer test-objective signs of decreased lacrimation (sterile strip of filter paper placed under lower eyelid) -Positive anti-Ro (SSA) and/or anti-La (SSB) abs -Gold standard, confirmatory: salivary gland biopsy w/ focal lymphocytic sialoadenitis -Classified as primary if no associated connective tissue disease, secondary if comorbid CTD (e.g. SLE, RA, scleroderma) Pathophysiology: -Autoimmune disease w/ inflammation of exocrine glands -B-cell NHL: Sjogren's results in polyclonal B cell activation & infiltration of salivary glands -> persistent lymphocyte activation can cause lymphocytic neoplasia

Scabies

Presentation: -Extremely pruritic, erythematous, papular rash in interdigital webspaces of fingers, lateral surfaces of fingers, flexor surfaces of wrist, extensor surfaces of elbows, periumbilical region, axillae, genitalia, nipples/areola (uncommon on back/head except in children) -Papules, pustules, vesicles, and burrows (vs. peeling/fissuring in ringworm), often w/ extensive excoriations -Worsening pruritus at night -Burrows look like ~1 cm long lines-Incubation period of 3-6 wks Diagnosis: -Usually clinical, confirmation w/ skin scrapings & light microscopy to identify mites & eggs Treatment/Management: -Topical 5% permethrin cream (removal by thorough washing after 8-14 hrs) or oral ivermectin -Oral antihistamines to reduce dermatitis & pruritus, topical steroids if persistent dermatitis after mite eradication -Bedding & clothing should be cleaned or placed in plastic bag for 3+ days (mites live in human skin for 2-3 days) Prevention: -Treating household contacts, decontamination of linens (e.g. hot water washing of linens & clothing) Pathophysiology: -Highly contagious infection w/ Sarcoptes scabiei mite, spread by direct person-to-person contact -Delayed type IV sensitivity to mite, feces, eggs

Herpes Keratitis

Presentation: -Eye pain, photophobia, decreased/blurred vision, tearing, redness, watery discharge, ciliary flush (conjunctival injection surrounding cornea) -Can also have decreased corneal sensation (corneal anesthesia from infection of V1) -Clear vesicles on corneal epithelium, branched dendritic corneal ulceration, corneal opacification -May have hx of prior episodes, may be precipitated by excess sun exposure, outdoor occupation, fever, immunodeficiency -In immunodeficient individuals (esp HIV), can progress to rapidly progressing bilateral necrotizing retinitis (vision loss, fundoscopy w/ pale peripheral lesions & central necrosis of retina) -Most common cause of corneal blindness in developed world Diagnosis: -Primarily diagnostic, corneal epithelial scrapings w/ multinucleated giant cells on microscopy Treatment: -Topical or oral antiviral treatment (acyclovir, valacyclovir, ganciclovir, trifluridine) Pathophysiology: -HSV, VZV infection of V1 (ophthalmic branch) of trigeminal nerve

Temporomandibular Joint Disorder

Presentation: -Facial pain that worsens w/ jaw motion, ear pain, tinnitus, HA (unilateral, worse on awakening), jaw dysfxn, neck stiffness -Normal hearing/Rinne/Weber, tender mastication muscles/TMJ on palpation when opening/closing jaw, evidence of bruxism on exam (worn teeth), crepitus/clicking w/ TMJ motion -Risk factors: joint trauma (TMJ injury, bruxism), psychiatric illness (anxiety, hx of abuse) Diagnosis: -Clinical Treatment/Management: -Education (avoidance of triggers, soft diet, warm compresses), dental splints if bruxism suspected, NSAIDs -Muscle relaxants may be added for associated muscle spasm (e.g. cyclobenzaprine) Pathophysiology: -Otalgia is referred

Myelofibrosis

Presentation: -Fatigue, anemia, hepatomegaly, splenomegaly -Classically seen in older adults Diagnosis: -Bone marrow bx w/ hypocellular, fibrotic bone marrow -Peripheral smear w/ fragmented megakaryocytes, immature leukocytes -Splenic infarction, splenomegaly d/t venous occlusion from congestion & extramedullary hematopoiesis Pathophysiology: -Collagen or reticulin deposition in bone marrow

Mobitz II 2nd Degree AV Block

Presentation: -Fatigue, lightheadedness, syncope, CP, dyspnea, risk of sudden cardiac arrest -High rate of progression to complete (3rd degree) AV block w/ potentially devastating outcomes (e.g. SCD) Diagnosis: -ECG w/ constant prolonged PR w/ randomly dropped QRS complexes w/ intermittent nonconducted P waves Treatment/Management: -Pacemaker placement (unstable rhythm) -If unstable (hypotension, confusion, CP), IV atropine Pathophysiology: -Etiologies: ischemia, electrolyte abnormalities, meds (e.g. beta blockers, nondihydropyridine CCBs), infiltrative disease (e.g. sarcoidosis), age-related fibrosis -Impaired electrical conduction across AV node

Acute Myeloid Leukemia (AML)

Presentation: -Fatigue, other B symptoms uncommon -Symptoms from cytopenias: anemia (fatigue, weakness), thrombocytopenia (easy bruising, bleeding), granulocytopenia (infection) -Most common adult acute leukemia, median age 65 -Hepatosplenomegaly/lymphadenopathy rare/atypical, risk of DIC if APML subtype -Labs: cytopenias (leukocytes may be increased, decreased, or normal), elevated LDH, peripheral smear w/ myeloblasts w/ Auer rods, if APML may present w/ DIC (prolonged PT/PTT, decreased fibrinogen) Diagnosis: -BMBx: usually hypercellular w/ myeloid blasts Treatment: -APML: all-trans retinoic acid Pathophysiology: -Clonal myeloproliferative disorder characterized by accumulation of blastic/immature myeloid cells in bone marrow & blood -APML: 15;17 RARA translocation

Primary Sclerosing Cholangitis (PSC)

Presentation: -Fatigue, pruritus, RUQ pain, previous episodes that may have resolved -Acute cholangitis presents w/ Reynolds pentad (pain, fever, jaundice, hypotension, AMS) -Associated w/ IBD, esp UC (~90% of PSC pts) -Labs: HIGH alk phos & GGT (cholestatic pattern), hyperbili, elevated transaminases, leukocytosis, high ESR, increased IgM -Complications: biliary stricture, cholangitis/cholelithiasis, cholestasis (decreased fat soluble vitamin absorption, osteoporosis), increased malignancy risk (cholangiocarcinoma, colon cancer, biliary cancer) Diagnosis: -Cholangiography/MRCP w/ multifocal stricturing/dilation of intrahepatic and/or extrahepatic bile ducts -Liver biopsy w/ fibrous obliteration of small bile ducts, concentric replacement by connective tissue in onion-skin pattern -RUQ US may be normal b/c intrahepatic bile ducts difficult to visualize, may show bile duct wall thickening & dilations -c/p-ANCA+ -Colonoscopy if no prior IBD diagnosis, annual surveillance colonoscopies due to increased colon cancer risk Treatment/Management: -Endoscopic interventions for strictures, therapy for coexisting UC, abx for cholangitis (sometimes long-term) -Ursodeoxycholic acid Pathophysiology: -Progressive biliary fibrosis & stricturing, obliteration of intrahepatic bile ducts

Colorectal Cancer

Presentation: -Fatigue, weight loss, abd pain -R-sided tumors: include cecum and ascending colon, present w/ occult bleeding & iron def anemia (hematochezia uncommon b/c blood is diluted by stool as it travels, obstructive sx uncommon b/c lumen is wide & passing stool is liquidy) -L-sided tumors: include descending & sigmoid colon, obstructive sx w/ crampy/colicky pain, change in bowel habits (constipation), visible red/maroon hematochezia -Rectal tumors: hematochezia/frank red stool, tenesmus, narrowed stools, sensation of mass in rectum -Labs: microcytic anemia, positive occult blood -Risk factors: personal/fam hx of adenomatous polyps or colon cancer, inherited syndromes (Lynch/HNPCC, FAP), ulcerative colitis, prior abdominopelvic radiation therapy, T2DM, obesity, frequent consumption of red/processed meats, tobacco/EtOH use, African American -Protective factors: NSAIDs/aspirin use, high fiber diet, caffeine, hormone replacement therapy in postmenopausal women, regular exercise Diagnosis: -Colonoscopy, abd CT for hepatic mets Pathophysiology: -Most commonly adenocarcinoma -Hyperinsulinemia -> increased IGF-1 in circulation -> inhibition of colorectal epithelial cell apoptosis -> promotion of neoplastic progression -Commonly associated w/ liver mets (blood from colon moves through portal circulation directly to liver)

Spontaneous Bacterial Peritonitis

Presentation: -Fever of 100+, abd pain/tenderness, AMS, hypotension, hypothermia, paralytic ileus w/ severe infection -Signs of cirrhosis/ascites: shifting dullness, fluid wave on exam, bilat LE pitting edema, asterixis, hepatic encephalopathy Diagnosis: -Peritoneal fluid from paracentesis w/ 250+ PMNs, positive gram stain/culture (usually GN organisms like E. coli, Klebsiella), protein <1 g/dL, SAAG 1.1+ (c/w portal HTN) Treatment/Management: -Empiric abx: third-gen cephalosporins (e.g. ceftriaxone, cefotaxime), IV albumin (decrease risk of renal failure & mortality) -FQs for SBP PPx Pathophysiology: -Thought to involve translocation of enteric bacteria across intestinal wall -> seeding of ascitic fluid within peritoneal cavity

Miliary Tuberculosis

Presentation: -Fever, cough, lethargy, cachexia, night sweats, respiratory failure, failure to thrive in infants -Numerous micronodular lesions in liver, lungs, spleen, lymph nodes, bones & marrow, CNS, adrenal glands (presents as primary adrenal insuff), etc. -"Millet-seed" appearance of diffuse & innumerable pulmonary micronodules, reticulonodular pattern -Interferon-based testing may be falsely negative due to ineffective T cell response -Labs: transaminitis from liver infiltration, anemia/leukopenia/thrombocytopenia from BM infiltration -Risk factors: incarceration, substance abuse (e.g. tobacco, IV drugs), group living, international travel, from endemic region Diagnosis: -Culture from lungs, biopsy (esp liver bx, may also perform bone marrow or transbronchial bxs), or blood Treatment: -Anti-tuberculosis regimen, possible addition of glucocorticoids if severe Pathophysiology: -Caused by lymphohematogenous spread of TB; rare but serious complication of primary TB in hosts w/ poor T cell fxn (e.g. infants, immunocompromised) -Can arise during primary infection or w/ reactivation

Sarcoidosis

Presentation: -Fever, cough, weight loss, arthritis, peripheral lymphadenopathy, parotid gland swelling (exocrine gland infiltration), hypercalcemia -Cutaneous: erythema nodosum, papules/nodules/plaques -Pulm: bilateral hilar adenopathy w/ interstitial/reticular/consolidative/fibrotic infiltrates on CXR, peribronchovascular nodules, cysts, fibrosis, cough/dyspnea/chest pain, mixed restrictive/obstructive PFTs -Ophtho: anterior/posterior uveitis, keratoconjunctivitis sicca (dry eyes, sensation of grittiness) -Neuro: facial nerve palsy (facial droop, inability to close eye, hyperacusis, disappearance of nasolabial fold), central DI, hypogonadotropic hypogonadism, seizures -CV: AV block, dilated/restrictive CM -GI: hepatosplenomegaly, asymptomatic LFT abnormalities (first cholestatic, then hepatocellular), can also cause infiltrative liver disease -Lofgren syndrome: classic triad of fever, erythema nodosum, bilat hilar adenopathy -Labs: hypercalcemia, hypercalciuria, hyperphosphatemia, elevated serum ACE, BAL w/ lymphocytosis, elvated alk phos if hepatic involvement, elevated ESR -Risk factors: young adult, African American Diagnosis: -Lesion bx (e.g. lymph node or skin lesion; easiest location to assess) w/ noncaseating granulomas that stain negative for fungi & acid-fast bacilli Treatment: -PO prednisone (~12-24 mos for pulm impairment, usually resolves & does not recur) Pathophysiology: -Granulomatous infiltration & inflammation -Macrophages in lung granulomas produce 1-alpha hydroxylase -> elevation of calcitriol -> hypercalcemia & hyperphosphatemia -> kidney stones, nephrocalcinosis, renal failure

Acute Cholangitis

Presentation: -Fever, jaundice, RUQ pain (Charcot's triad) -Reynolds pentad: Charcots triad + hypotension & AMS -Labs: elevated alk phos, direct hyperbili, mildly increased aminotransferases, leukocytosis w/ L-shift, anion gap metabolic acidosis (lactic acidosis) Diagnosis: -Common bile duct dilation, stones on RUQ US (more sensitive than CT, which can miss radiolucent gallstones) -MRCP for identifying biliary strictures or less common causes of biliary obstruction (e.g. biliary tumor) Treatment/Management: -Empiric abx coverage of enteric bacteria (e..g piperacillin/tazobactam, cipro + metronidazole) -ERCP w/ stone extraction/stent placement within 24-48 hrs -If ERCP unsuccessful or not possible d/t contraindications (e.g. hemodynamic instability, neurologic compromise), percutaneous drainage/cholecystostomy to facilitate biliary drainage Pathophysiology: -Common bile duct obstruction by stone or malignancy -> ascending biliary tract infection -Etiologies: choledocholithiasis, malignancy, biliary stenosis, PSC, PBC, biliary interventions resulting in incomplete bile drainage (e.g. postcholecystectomy migration of biliary clip)

Serum Sickness

Presentation: -Fever, joint pain, urticarial or vasculitis-like rash Pathophysiology: -Type III HS rxn w/ immune complexes, immune rxn against blood products or antigens from nonhuman species (e.g. monoclonal antibodies including rituximab, infliximab)

Pyogenic Liver Abscess

Presentation: -Fever, leukocytosis, elevated LFTs (esp elevated alk phos & bili), RUQ pain, hepatomegaly, jaundice -May be associated w/ recent biliary stenting, biliary infection, bowel perforation, or other source of infection (e.g. endocarditis, diverticulitis) -If in hepatic dome, can cause diaphragmatic irritation and sterile, right-sided pleural effusion (if ruptures, can spread through diaphragm and cause empyema) -More common in pts w/ T2DM or underlying hepatobiliary/pancreatic disease Diagnosis: -Abd imaging (abd CT, US) w/ round, well-defined hypoattenuating lesion (may be surrounded by peripherally enhancing abscess membrane, may contain internal debris or septations) Treatment: -Blood cultures, IV abx, percutaneous abscess aspiration/drainage & culture -Possible catheter placement to drain if large, surgical drainage if not amenable to percutaneous intervention -Thoracentesis if associated pleural effusion Pathophysiology: -Direct spread of bacteria to liver from biliary tract (e.g. infected biliary stenting), through portal vein (e.g. intraabd infection like diverticulitis), or via systemic circulation (e.g. endocarditis)

Acute Interstitial Nephritis (AIN)

Presentation: -Fever, malaise, N/V, diffuse maculopapular skin rash, arthralgias, leukocyturia (particularly eosinophiluria, WBCs, WBC casts), and acute renal failure, decreased UOP -Sx within 7-10 days to wks of starting drug -Labs: eosinophilia Diagnosis: -Labs showing AKI, UA w/ hematuria/pyuria/WBC casts/eosinophiluria, +/- mild RBCs & proteinuria -Renal bx shows tubulointerstitial mononuclear inflammatory infiltrate & edema Treatment/Management: -Discontinuation of the offending agent can result in full recovery of renal function +/- systemic glucocorticoids -Supportive HD if needed -Prognosis: recovery of renal fxn, but may not return to baseline & future risk of recurrence w/ exposure to offending agents Pathophysiology: -Acute inflammatory process that develops 3 to 21 days following exposure to an immunogenic drug (PPIs, sulfonamides esp TMP-SMX, penicillins & other beta-lactams, indinavir, NSAIDs, rifampin, diuretics) or infection (e.g. Legionella, Mycobacterium tuberculosis, Streptococcus, CMV) and results in injury to the renal tubules and interstitium -Can also be associated w/ rheumatologic disease (e.g. SLE, Sjogren, sarcoid)

Herpes Zoster Ophthalmicus

Presentation: -Fever, malaise, burning/itching sensation in periorbital region -Vesicular rash in distribution of cutaneous branch of first division of trigeminal nerve (ophthalmic) over forehead and eye, conjunctivitis, chemosis, corneal ulcers (dendriform ulcers, decreased vision) -Hutchinson sign (involvement of tip of nose) -Risk factors: immunosuppressed, elderly Treatment: -High-dose acyclovir within 72 hrs of eruption to reduce complications Pathophysiology: -VZV latent in trigeminal ganglion -> reactivation causes travel down ophthalmic branch (V1)

Diphtheria

Presentation: -Fever, malaise, sore throat, cervical lymphadenopathy, usually in children <15 yo -Pharyngitis w/ grey patches/pseudomembranes that bleed w/ scraping -Toxin-mediated myocarditis (2/3 of pts, if severe can cause complete heart block, HF), neuritis (peripheral/cranial neuropathies), kidney disease Diagnosis: -Culture from respiratory secretions, toxin assay (proves toxigenic strains) Treatment/Management: -Erythromycin or pen G, diphtheria antitoxin if severe Prevention: -Diphtheria toxoid vaccine Pathophysiology: -Caused by toxigenic strains of Corynebacterium diphtheriae, a gram+ bacillus transmitted by respiratory secretions -Exotoxin released by bacteria is absorbed systemically, can result in dose-dependent damage to heart, nervous system, kidneys

Serum Sickness-Like Reaction

Presentation: -Fever, skin rash (urticarial, NO MUCOSAL involvement), polyarthralgias 1-2 wks after drug exposure -Usually in response to beta-lactam or sulfa abx or hepatitis B infection -When associated w/ hep B infection, develops during prodromal/preicteric phase of acute infection & resolves w/ onset of jaundice Diagnosis: -Clinical, ESR/CRP, nonspecific hypocomplementemia Treatment: -Removal of offending agent (should relieve sx within 48 hrs), supportive care, steroids or plasmapheresis if severe -Usually self-resolves in 5-14 days; avoid culprit med in future Pathophysiology: -Similar to serum sickness in presentation (immune complex formation/type III HS rxn) but different mechanism -Complement activation by circulating immune complexes composed of hep B surface antigen (also responsible for polyarteritis nodosa & GN seen w/ hep B) -Thought to be due to genetic deficiencies in eliminated metabolic by-products of culprit meds -> hapten-mediated cytotoxic T cell injury or direct cytotoxicity

Staphylococcus Aureus Bacteremia

Presentation: -Fevers, positive blood cultures -Up to 40% develop metastatic infection, most commonly of heart valves, lungs, osteoarticular structures (e.g. vertebral osteomyelitis, epidural abscess) -Labs: persistent leukocytosis Treatment/Management: -IV vanc coverage -If back pain, spinal MRI to evaluate for osteomyelitis, confirmation w/ open or CT-guided bx

Tonsillar Herniation

Presentation: -Fixed, midposition pupils due to disruption of sympathetic & parasympathetic innervation -Coma, loss of CN reflexes, flaccid paralysis, respiratory arrest (brainstem compression) Pathophysiology: -Herniation of cerebellar tonsils through foramen magnum

Pemphigus Vulgaris

Presentation: -Flaccid bullae of mucosa (most commonly oral, mucosa usually affected first) and skin that rupture (Nikolsky sign) easily into painful erosions -Usually seen in older adults (40-60 yo) Diagnosis: -Skin bx: histo w/ intraepidermal cleavage, IF w/ net-like intercellular IgG against desmosomes -Serology for abs against desomosome components (desmogleins 1 & 3) Treatment: -Systemic corticosteroids, rituximab

Dengue Fever

Presentation: -Flu-like illness w/ fever, HA, RETRO-ORBITAL PAIN, diffuse maculopapular rash, myalgias/arthralgias -Hemorrhagic form can present w/ skin/nasal hemorrhage, positive tourniquet test (petechiae after sphygmomanometer cuff inflation for 5 min) -Warning signs: abd pain, persistent vomiting, fluid accumulation (ascites, pleural effusion), mucosal bleeding, lethargy, restlessness, hepatomegaly >2 cm, increased Hct w/ rapidly decreasing plt count -Severe dengue: some forms can infect bone marrow ("break-bone" fever), can cause renal failure & other severe organ involvement (transaminitis >1000, impaired consciousness), septic shock or resp distress from severe plasma leakage (dengue shock syndrome, usually days 3-5 illness, can rapidly progress to death), severe bleeding -Sx usually 4-7 days following mosquito bite -Labs: leukopenia, thrombocytopenia, hemoconcentration, transaminitis Pahtophysiology: -Mosquito-borne viral infection endemic to tropical & subtropical regions of Asia (e.g. India), Africa, South America, Pacific/Caribbean islands -Transmitted by Aedes mosquitoes that can also harbor yellow fever, chikungunya, Zika -Risk of dengue shock syndrome greatest if pt was previously exposed to different dengue virus serotype

Myasthenia Gravis

Presentation: -Fluctuating & fatigable proximal muscle weakness, including ocular (e.g. diplopia, ptosis), bulbar (e.g. dysphagia, dysarthria), respiratory (myasthenic crisis), facial ("myasthenic sneer"), & proximal (e.g. difficulty holding up head or lifting arms) weakness -Weakness worsens w/ activity/repetition & improves w/ rest; exam may be normal if repetitive movements not performed -Exacerbations may be precipitated by meds (abx including FQs & aminoglycosides, cardiac agents including beta-blockers, MgSO4, penicillamine, hydroxychloroquine, neuromuscular blockade e.g. rocuronium) & physiologic stress (e.g. pregnancy/childbirth, surgery esp thymectomy, infection) Diagnosis: -Bedside ice pack test (ice pack placed over eyelids leads to improvement in ptosis; cold inhibits ACh breakdown) -AChR-Ab (highly specific) -CT scan of chest for thymoma Treatment: -AChE inhibitors (e.g. pyridostigmine, neostigmine) +/- immunotherapy (e.g. corticosteroids, azathioprine, cyclosporine) -Thymectomy (associated w/ long-term clinical improvement but initial transient worsening of sx, e.g. myasthenic crisis, thus receive pyridostigmine & immunosuppressants perioperatively) Pathophysiology: -Autoantibodies against postsynaptic NM junction ACh receptor -> fluctuating weakness

Hypoactive Delirium

Presentation: -Fluctuating wakefulness and awareness Pathophysiology: -Diffuse cortical dysfxn leading to decreased consciousness

Scombroid Poisoning

Presentation: -Flushing, throbbing HA, palpitations, abdominal cramps, diarrhea, oral burning beginning 10-30 min after ingestion -On exam, skin erythema, wheezing, tachycardia, hypotension -Pts may describe a bitter taste -Self-limited rxn Pathophysiology: -Caused by ingestion of improperly stores seafood -If seafood stores at temps exceeding 15C, histidine can undergo decarboxylation to form histamine

Ischemic Stroke

Presentation: -Focal neuro deficits, vs. hemorrhagic stroke does not usually present w/ HA or impaired consciousness since ICP is not significantly increased -Risk factors: HTN (for both hemorrhagic & ischemic, 4x risk), hypercholesterolemia, T2DM, smoking, sedentary lifestyle -Complications w/ tPA: intracerebral hemorrhage, systemic bleeding (e.g. GI hemorrhage), bradykinin-mediated angioedema Diagnosis: -Noncon CT (often can be normal in early hrs <6 hr following ischemic event, as cytotoxic edema takes several hrs to manifest) to rule out intracranial hemorrhage -CT angiogram -Further workup to determine etiology: brain MRI, carotid duplex, ECG, Echo Treatment/Management: -If presentation within 3-4.5 hrs of sx onset & no contraindications (even if imaging is negative), IV alteplase, wait at least 24 hrs before initiating antiplatelet/anticoag -If >4.5 hrs & stroke w/ no prior antiplatelet therapy, aspirin -If >4.5 hrs & stroke while on aspirin therapy, aspirin + dipyridamole/clopidogrel -If stroke w/ evidence of AFib, long-term anticoag (warfarin, dabigatran, rivaroxaban) -If stroke w/ large anterior circulation artery occlusion within 24 hrs of onset, mechanical thrombectomy (regardless if pt received alteplase), then aspirin -If pt w/ intracranial large-artery atherosclerosis, aspirin + clopidogrel for 90 days, then aspirin -If HTN 185/110+, IV labetalol or nicardipine to lower BP prior to thrombolysis (decreases risk of hemorrhagic transformation, but also want to avoid hypotension to maintain perfusion to ischemic penumbra) -Primary/secondary prevention of recurrence w/ aspirin, statins -DVT ppx w/ intermittent pneumatic compression only if thrombolytics, dual antiplatelet therapy, or anticoag; add prophylactic-dose heparin if only aspirin Pathophysiology: -HTN -> elevated shearing forces on intracerebral vascular endothelium -> accelerated atherosclerosis, thrombus formation

Hemorrhagic Stroke

Presentation: -Focal neuro deficits, vs. ischemic stroke usually also presents w/ HA, N/V, impaired consciousness, & other signs of increased ICP -Noncontrast CT w/ white, hyperdense region -Risk factors: HTN, h/o TIA Diagnosis: -CT w/o contrast Treatment/Management: -Prevent further bleeding, reduce ICP to prevent herniation from brain edema by controlling BP (e.g. IV nicardipine or labetalol), reversing anticoagulation (vitamin K for warfarin, protamine sulfate for heparin), decreasing ICP w/ bed elevation, sedation, osmotic therapies (e.g. mannitol) -Neurosurgical evacuation of hematoma may be required in severe cases w/ impending brain herniation or elevated ICP refractory to medical management

Post-Intensive Care Syndrome (PICS)

Presentation: -Following prolonged ICU course: -Psych: >50% w/ major depression (sleep disturbance common), PTSD -Neurocog: decreased attention/memory, executive fxn & processing speed for yrs -Physical: >50% w/ decreased mobility & independence, most able to walk again -Prognosis: most require additional home care & never return to work -Risk factors: ICU delirium, ARDS, prolonged mechanical ventilation Treatment/Management: -Multidisciplinary post-ICU clinic, pharmacotherapy for psych sx Prevention: -Early therapy participation (e.g. PT/OT even when intubated) Pathophysiology: -CNS hypoxia, neuroinflammation & metabolic disruption

Steppage Gait

Presentation: -Foot drop (weakness in foot dorsiflexion), excessive hip & knee flexion while walking, "slapping" quality, frequent falls -Distal sensory loss & weakness Pathophysiology: -Motor neuropathy, most commonly of L5 (back pain radiating to foot, weakness of foot eversion/dorsiflexion) or common peroneal nerve (distal lateral fibular compression, dorsal foot paresthesias & sensory loss) -Weakness in foot dorsiflexion -> compensation w/ hip/knee flexion to avoid dragging toes w/ each step

Plantar Verrucae

Presentation: -Form of cutaneous warts, most often occurring in young adults or individuals w/ certain occupations (e.g. meat, poultry, fish handlers) -Appears as single or multiple hyperkeratotic papules on sole of foot -More extensive disease in pts w/ atopic dermatitis & conditions w/ decreased cellular immunity (e.g. HIV, organ transplant) Diagnosis: -Usually clinical, scrapings of hyperkeratotic debris shows thrombosed capillaries called "seeds" Treatment/Management: -Salicylic acid as initial treatment (may take 2-3 wks) -Alternatives include liquid nitrogen cryotherapy (can cause local burning, hypopigmentation in dark-skinned pts) -Topical imiquimod in pts that do not respond to or tolerate initial therapies above -Dermatologist referral, surgical excision if pts do not improve w/ medical therapy Pathophysiology: -Usually d/t HPV infection (types 1-4) -Inoculation typically by skin-to-skin contact, can have incubation period of 2-6 mos after exposure

Blepharospasm

Presentation: -Form of focal dystonia w/ periodic involuntary eye closure that may be provoked by external stimuli (e.g. bright light, irritant) -Usually seen in older, postmenopausal women (possibly triggered by dry eyes) Treatment/Management: -Botulinum toxin injections

Barrett Esophagus

Presentation: -Frequent reflux sx (e.g. heartburn), h/o chronic GERD common -Risk factors: age >50, male, Caucasian, hiatal hernia, obesity or increased waist circumference (>102 cm), current/former tobacco use, FDR w/ Barrett esophagus or esophageal adenocarcinoma Diagnosis: -If chronic GERD or frequent sx & 2+ of risk factors above, screening endoscopy (shows columnar-lined esophagus, esophageal bx w/ intestinal metaplasia) -Endoscopy shows reddish, salmon-colored mucosa w/ velvet-like texture Treatment/Management: -If ruled out w/ no dysplasia, PPI & surveillance endoscopy in 3-5 yrs -If low-grade dysplasia, PPI & surveillance endoscopy in 6-12 mos or endoscopic eradication -If high-grade dysplasia, endoscopic eradication therapy (preferred), surgical esophagectomy Pathophysiology: -Metaplastic replacement in lower esophagus of normal stratified squamous epithelium w/ columnar epithelium (normally lines intestines) in response to chronic gastric acid exposure, predisposing to development of esophageal adenocarcinoma

Proctalgia Fugax

Presentation: -Functional anorectal disorder w/ recurrent, brief episodes (s to mins, 30 min or shorter) of rectal pain unrelated to defecation, no pain b/t episodes -May be precipitated by stress, sexual intercourse, sitting, or may be spontaneous -Normal physical exam (rectal/pelvic/prostate), no laboratory abnormalities -Risk factors: female, other functional pathologies (e.g. IBS), psychosocial stress, anxiety Treatment/Management: -Reassurance, nitroglycerin cream +/- biofeedback therapy for refractory sx Pathophysiology: -Spastic contraction of anal sphincter, possible intermittent pudendal nerve compression

Eosinophilic Esophagitis

Presentation: -GERD-like sx, w/ chest/epigastric burning pain, dysphagia, reflux/vomiting refractory to PPIs -May experience acute/intermittent food impaction (drooling, hypersalivation, inability to tolerate liquids, substernal/upper abd pain, can lead to esophageal perforation) -Often associated w/ atopy (e.g. asthma, eczema, seasonal allergies), most common in young men -Nonspecific findings on endoscopy including circular rings, strictures, esophageal furrows, may also be normal Diagnosis: -Endoscopy w/ esophageal bx showing 15+ eosinophils per hpf -Manometry may show esophageal hypercontractility Treatment: -Dietary modification, +/- topical glucocorticoids (fluticasone, budesonide) -If acute food impaction, may require disimpaction by endoscopy -Stricture dilation if no response to medical therapy Pathophysiology: -Chronic, immune-mediated esophageal inflammation -Stricture formation can predispose to food impaction

Lactose intolerance

Presentation: -GI distress after dairy intake, w/ periumbilical abd pain, flatulence/bloating, watery diarrhea (bulky, malodorous, but not usually bloody/mucoid) -Hypertympanic abd on exam -Worse w/ foods w/ high lactose content (ice cream, milk > cheese, yogurt) -Can be primary (onset usually early childhood) or secondary (transient, wks-mos, associated w/ GI illness, celiac, etc.) -Risk factors: Asian/African/Hispanic, h/o congenital/developmental lactase deficiency, h/o small intestinal infection/inflammation Diagnosis: -Resolution of sx on dairy-restricted diet, +lactose breath hydrogen test -Increased stool osmotic gap, low stool pH Treatment/Management: -Dietary restriction of lactose, lactase replacement if dairy ingested -Vit D & calcium supplementation Pathophysiology: -Primary: acquired, persistent lactase deficiency -Secondary: small intestinal epithelial damage due to infection (e.g. gastroenteritis) or inflammation (e.g. celiac, Crohn disease) -Carbohydrate malabsorption due to lactase deficiency -Lactase def -> undigested lactose metabolized by colonic bacteria -> release of H2 & other gases that cause bloating, flatulence

Celiac Disease

Presentation: -GI: abdominal pain, distention, bloating, diarrhea, malabsorption (bulky, foul-smelling, floating stools), borborygmi, late complications of ulcerative jejunitis, enteropathy-associated T cell lymphoma -General: failure to thrive, weight loss, short stature, delayed menarche/puberty -Oral: enamel hypoplasia, atrophic glossitis (smooth/red tongue w/ burning pain) -Dermatologic: dermatitis herpetiformis (grouped papulovesicular rash on extensor surfaces like elbows/knees & buttocks, erosions & excoriations due to pruritus), hyperkeratosis (vit A def) -Hematologic: iron deficiency anemia (microcytosis, low ferritin), easy bruising (vit K def) -Neuro: peripheral neuropathy, mood disorders (e.g. anxiety, depression) -MSK: arthritis, osteomalacia/rickets due to vit D malabsorption, loss of muscle mass (d/t poor absorption) -Endocrine: vit D def, secondary hyperparathyroidism -Risk factors: FDR w/ celiac, Down syndrome, autoimmune disorders (T1DM, autoimmune thyroiditis), increased age, Northern European ancestry -Labs: elevated stool osmotic gap, microcytic anemia, iron deficiency -Complications: if untreated, osteopenia, severe anemia, increased malignancy risk (enteropathy-associated T-cell lymphoma) Diagnosis: -Increased tissue transglutaminase IgA levels (anti-transglutaminase, anti-endomysial abs); may be negative in IgA deficiency (if suspected measure total IgA, conduct IgG testing instead) -Proximal intestinal/duodenal biopsy (villous atrophy/effacement, crypt hyperplasia, intraepithelial lymphocytosis) -Skin bx shows microabscesses at tips of dermal papillae, deposits of IgA abs against epidermal transglutaminase in dermis -D-xylose testing of small bowel absorption shows poor absorption d/t villous destruction Treatment/Management: -Gluten-free diet -Address dietary deficiencies w/ supplementation (iron, calcium, vit D, folic acid, thiamine, B12) -Dapsone for dermatitis herpetiformis -DEXA at diagnosis d/t increased risk for osteopenia & osteoporosis, repeat DEXA 1 yr later if osteopenia present -Pneumococcal vaccination (d/t associated hyposplenism) -Dietary counseling w/ dietician, access to advocacy group -Serology can be used to assess treatment progress & dietary compliance (anti-TGA, anti-gliadin) Pathophysiology: -Atrophy of small intestinal villi in proximal small bowel d/t exposure to gluten-containing wheat products -> malabsorption -Immune rxn to gliadin

Acute Liver Failure

Presentation: -Generalized sx (fatigue, lethargy, anorexia, N/V), RUQ abd pain, pruritus/jaundice d/t hyperbilirubinemia -Labs: thrombocytopenia, hypoglycemia, renal insufficiency -Complications: acute renal insufficiency, cerebral edema (can lead to coma, brainstem herniation, most common cause of death) Diagnosis: -Requirements include: severe acute liver injury (ALT & AST often >1000), signs of hepatic encephalopathy (e.g. confusion, asterixis), synthetic liver dysfxn (INR of 1.5+) Treatment: -Liver transplantation (only ~50% survive w/o transplant, 1 yr survival after liver transplant is ~80%) Pathophysiology: -Etiologies include viral hepatitis (hep A/B/D/E, HSV, CMV), drug toxicity (e.g. acetaminophen toxicity, idiosyncratic), ischemia (e.g. shock liver, Budd-Chiari syndrome), autoimmune hepatitis, Wilson disease, malignant infiltration

Prostate Cancer

Presentation: -Generally asymptomatic, commonly discovered on prostate exam (nodule, vs. symmetric enlargement in BPH) or w/ PSA testing (markedly elevated vs. mildly elevated in BPH) -Asymmetrically enlarged & firm prostate w/ nodule(s)/induration, inguinal lymphadenopathy -Generally grows at periphery of gland, so urinary sx (e.g. urgency, frequency, etc.) and hematuria are uncommon -Rare sx include systemic signs (fatigue, weight loss), bone pain from metastases (esp rib, lower back from vertebral mets), lower UTI sx -Risk factors: biggest is AGE (>40), African American, family hx, diet high in animal fat & low in vegetables/fruits -Labs: markedly elevated PSA Diagnosis: -Transrectal biopsy, UA -Mets: radionuclide bone scan, PSA Treatment/Management: -Surgical resection (radical prostatectomy), radiotherapy (brachytherapy, external beam) -Androgen deprivation therapy for disseminated cancer -If palliative & bone mets, focal external beam radiation therapy & pain control -If very low risk (Gleason score of 6 or lower w/ <3 cores affected & <50% involvement in each core, normal digital rectal exam, PSA <10), may be managed w/ active surveillance rather than definite therapy (PSA every 3-6 mos, digital rectal exam yrly, repeat bx at end of yr 1) Pathophysiology: -Most commonly adenocarcinoma (2nd most common malignancy in men worldwide) -Most commonly affect peripheral zones of prostate, but can occur anywhere -Mets to spine: unlike other metastatic lesions, purely osteoblastic, not osteolytic (normal/low serum calcium, not hypercalcemic), w/ focal/sclerotic vertebral lesions

Hyperosmolar Hyperglycemic Syndrome (HHS)

Presentation: -Gradual hyperglycemic symptoms (polyuria, polydipsia) -Pronounced AMS (confusion, lethargy, focal signs, seizures, coma) -Often severe dehydration (due to osmotic diuresis from elevated plasma osmolality) -Hyperventilation & abd pain less common than in DKA -May be precipitated by infection, major illness, dehydration, certain drugs (e.g. corticosteroids, antipsychotics, diuretics, sympathomimetic agents, beta blockers) -Labs: glucose >600 (frequently >1000), normal pH & bicarb >18, normal anion gap, negative or small serum ketones, serum osmolality >320, hyponatremia due to extracellular fluid shift -Risk factors: T2DM, advanced age Treatment/Management: -Aggressive hydration w/ NS (lowers plasma osmolality, often present w/ fluid loss of 8-10 L), IV insulin, K+ replacement if level is <5.3 Pathophysiology: -Compared to DKA, glucose more elevated (>1000 vs. 300-500), normal anion gap & pH, absent ketoacids, more severe dehydration -K+ may be elevated, but overall total body K+ deficit of 300-600 mEq (from urinary losses due to osmotic diuresis induced by hyperglycemia + urinary excretion of potassium ketoacid anion salts + hyperaldosteronism d/t volume contraction), should carefully replace as IV insulin may precipitate hypokalemia -Dehydration occurs secondary to osmotic diuresis caused by hyperglycemia -Neuro sx d/t markedly increased serum osmolality

Central Line-Associated Bloodstream Infection (CLABSI)

Presentation: -Greatest risk when central catheter has been left in place for 6+ days Prevention: -Checklist prior to catheter insertion -Proper hand hygiene prior to catheter handling -Us of all-inclusive catheter kit -Sterile barrier precautions during insertion -Chlorhexidine-based antiseptic for prep of pt's skin -Proper catheter site care -Catheter removal when no longer needed -Use of subclavian > IJ vein Pathophysiology: -Usually result of catheter contamination w/ skin organisms including coag-negative Staph, S. aureus, Candida species, aerobic gram- bacilli

Colonic Polyps

Presentation: -Grossly visible protrusions from flat mucosal surface of intestine Diagnosis: -Bx on colonoscopy Treatment/Management: -Surveillance increased for neoplastic polyps (usually every 5 yrs vs. 10 yrs) -If hyperplastic poly <10 mm, colonoscopy in 10 yrs -If 1-2 tubular adenomas <10 mm, colonoscopy in 7-10 yrs -If hyperplastic polyp 10+ mm or 3-4 tubular adenomas <10 mm, colonoscopy in 3-5 yrs -If 5-10 tubular adenomas <10 mm, tubular adenoma 10+ mm, tubulovillous or villous adenoma, or adenoma w/ high-grade dysplasia, interval colonoscopy in 3 yrs -If >10 adenomas, colonoscopy in 1 yr -If large (>20 mm) adenoma removed by piecemeal excision, colonoscopy in 6 mos Pathophysiology: -Can be neoplastic (malignant potential) or benign/nonneoplastic -Neoplastic: adenomatous polyps (esp villous/tubulovillous, w/ long glands on histo, high-grade dysplasia, and sessile/nonpedunculated), large polyps 1+ cm, 3+ concurrent adenomas -Non-neoplastic: hyperplastic polyps, hamartomatous polyps (e.g. juvenile polyps, Peutz-Jeghers syndrome), inflammatory pseudopolyps, submucosal polyps (e.g. lipomas, lymphoid aggregates)

Angle Closure Glaucoma

Presentation: -HA, acute onset ocular pain, N/V, decreased visual acuity, may report seeing light halos -Conjunctival redness, corneal opacity/edema, fixed & mid-dilated & poorly reactive/sluggish pupil, palpable firmness of eye -Usually occurs following pupillary dilation (e.g. in dark room, during times of stress, drug intake e.g. sympathomimetics, anticholinergics, SMX-TMP) -Risk factors: female, >40, Asian/Inuit populations, farsightedness Diagnosis: -Tonometry to measure intraocular pressure -Gonioscopy to measure corneal angle (shallow anterior chamber) Treatment: -Initial management: systemic therapy w/ IV acetazolamide/mannitol, combo therapy w/ multiple topical agents including timolol (reduces aqueous production), apraclonidine (decreases aqueous production, increases outflow), pilocarpine (causes ciliary muscle contraction -> opens trabecular meshwork at corneal angle) -Laser iridotomy to facilitate aqueous outflow & provide definitive management Pathophysiology: -Narrowing/closure of anterior chamber angle -> decreased aqueous outflow, increased IOP -Can be spontaneous or triggered by meds (decongestants, antiemetics, anticholinergics) -Can damage optic nerve & result in permanent vision loss within 2-5 hrs

Toxoplasma Encephalitis

Presentation: -HA, confusion, fever, focal neuro deficits/seizures -Risk factors: HIV (CD4 <100) Diagnosis: -Positive Toxoplasma gondii IgG -Multiple ring-enhancing lesions on MRI (usually w/ edema vs. PML) Treatment: -Sulfadiazine + pyrimethamine + leucovorin -Antiretroviral initiation -PPx w/ TMP-SMX if CD4 <100 (should be initiated in all HIV pts w/ positive IgG serology, may be d/c'd if CD4 >200 & adequate viral suppression) Pathophysiology: -Most cases caused by reactivation of widely prevalent intracellular protozoan

Cavernous Sinus Thrombosis

Presentation: -HA, fever, CN deficits (e.g. diplopia, ophthalmoplegia, vision loss), proptosis/periorbital swelling, eye chemosis, photophobia -Ipsilateral deficits (may also be bilat) in CN III, IV, V (ophthalmic & maxillary branches), VI -Numbness in V1/V2 distribution Diagnosis: -Magnetic resonance venography Treatment/Management: -IV heparin -IV abx if suspected infectious cause (e.g. extension from orbital cellulitis) Pathophysiology: -Infection originating in maxillary space (not mandibular), medial third of face, sinuses (ethmoidal or sphenoidal), or teeth through valveless facial venous system -> spread to infratemporal space & orbit -> spread to cavernous sinus -Abducens nerve runs through cavernous sinus

High-Altitude Illness

Presentation: -HAPE: dyspnea, cough, hemoptysis, respiratory distress, bilat pulm crackles, patchy/bilat pulm infiltrates (d/t unbalanced hypoxic vasoconstriction in lungs -> noncardiogenic pulm edema) -HACE (high altitude cerebral edema): lethargy, confusion, gait disturbance (decreased PaO2 -> increased cerebral blood flow) -Acute mountain sickness: most common, HA, fatigue, N/V Treatment: -Supplemental O2 to reduce hypoxic pulm vasoconstriction, unloading victim to reduce peripheral oxygen consumption, descent to lower altitude -Acetazolamide (for prevention, not for treatment after HAPE develops): accelerates bicarb excretion to lower pH & compensate for respiratory alkalosis Pathophysiology: -Normal physiology: reduced PiO2 at high altitude (>8,000 ft) -> stimulates compensatory hyperventilation (respiratory alkalosis, helps increase PaO2 but decreases PaCO2), increased 2,3-BPG production by erythrocytes (increases O2 unloading in tissues), increased EPO production and bicarb excretion by kidneys -HAPE: hypoxic pulm vasoconstriction d/t low PiO2 leads to increased pulmonary arterial pressure, uneven vasoconstriction in certain predisposed individuals -> fluid extravasation, noncardiogenic pulm edema

HIV-Associated Nephropathy

Presentation: -Heavy proteinuria, rapidly progressive renal failure in pt w/ advanced HIV/AIDS -Edema, hematuria, HTN -Risk factors: advanced HIV/AIDS, sub-Saharan African descent Diagnosis: -Renal bx w/ collapsing focal glomerulosclerosis -Tubuloreticular inclusions visible on EM Treatment/Management: -ART if not already started, ACEi for HTN -Poor prognosis, often develop ESRD despite therapy Pathophysiology: -Thought to be due to direct infection of renal tubular and glomerular cells by HIV -Higher risk w/ sub-Saharan African possibly due to APOL1 gene variant thought to confer resistance to trypanosomiasis

Acute Erosive Gastropathy

Presentation: -Hematemesis, abd pain -Risk factors: use of aspirin, EtOH, cocaine Pathophysiology: -Development of severe hemorrhagic lesions after exposure of gastric mucosa to various injurious agents or after substantial reduction in blood flow (e.g. aspirin decreases protective prostaglandin production, cocaine causes vasoconstriction, EtOH & aspirin disturb normal protective barriers including secreted mucins, bicarbonate, allowing acid & other luminal substances like proteases & bile acids to penetrate lamina propria)

Renal Vein Thrombosis

Presentation: -Hematuria, nausea, flank/abd pain (constant/dull/nonradiating), enlarged kidney on imaging (renal US, CT), varicocele/scrotal edema (similar presentation to RCC but more acute) -No dysuria/pyuria, fever, other signs of infection -Flank & CVA tenderness on exam -Labs: hematuria, elevated LDH (from necrosis) +/- AKI -Risk factors: hypercoagulability (nephrotic syndrome, malignancy esp renal, OCPs), volume depletion (infants), trauma Diagnosis: -CT/MR angiography, renal venography -Doppler US low sensitivity but may show reversal of diastolic flow Treatment: -Anticoag, thrombolysis/thrombectomy if AKI present Pathophysiology: -Thrombosis -> markedly increased glomerular pressure -> renovascular congestion, capsular stretch (flank pain), hematuria -May occur w/ nephrotic syndrome due to loss of anticoag proteins

Beta Thalassemia

Presentation: -Hemolysis, unconjugated hyperbili, microcytic anemia -Fatigue, pallor, jaundice, splenomegaly, dark urine -Asymptomatic in infants, which predominantly produce fetal Hgb (alpha2/gamma2); become symptomatic ~6 mos w/ increased production of adult Hgb (alpha2/beta2) -Target cells on smear -Common in pts of Mediterranean ancestry (e.g. Greek) Diagnosis: -Hemoglobin electrophoresis w/ no Hb A, elevated Hb A2, predominantly Hb F Treatment: -Chronic transfusions, iron chelation therapy Pathophysiology: -Decreased beta globin chain production -If untreated, can lead to skeletal abnormalities due to extramedullary hematopoiesis

Hereditary Spherocytosis

Presentation: -Hemolytic anemia, jaundice, splenomegaly, scleral icterus, esp following viral illness or other insult, dark urine, pigment gallstones (calcium bilirubinate, can cause acute cholecystitis) -Family hx of anemia (AD inheritance, most common in Northern European populations), splenectomy, jaundice -Smear w/ anisocytosis, spherocytes, polychromatophilia -In infants, may present w/ jaundice refractory to traditional phototherapy Diagnosis: -Eosin-5-maleimide (EMA) binding test (flow cytometry) demonstrating RBC fragility OR acidified glycerol lysis test -Lab evidence of Coombs-negative hemolytic anemia (indirect hyperbilirubinemia, reticulocytosis, low hemoglobin), elevated mean corpuscular hemoglobin concentration (MCHC, due to membrane loss in spleen & RBC dehydration), normal or slightly low MCV, normal or slightly high RDW Treatment: -Folic acid supplementation, therapeutic transfusion, splenectomy if hemolysis severe or gallstones Pathophysiology: -Due to heritable RBC membrane defect (spectrin, ankyrin; link RBC membrane to cytoskeleton) that results in extravascular hemolysis as RBCs pass through splenic circulation -> unstable, round RBCs that become trapped in red pulp of spleen -> chronic hemolysis, splenomegaly -Elevated MCHC due to cellular dehydration and membrane loss

Sickle Cell Disease

Presentation: -Hemolytic anemia, vaso-occlusive pain crises, functional asplenia -Susceptible to sepsis w/ encapsulated bacteria (Strep pneumo most common, Neisseria meningitidis, H. flu) -Susceptible to osteomyelitis w/ Salmonella Diagnosis: -Labs: low Hct, reticulocytosis, sickled RBCs (& Howell-Jolly bodies if asplenic) on smear, increased HbS & HbF, decreased HbA on Hgb electrophoresis Treatment/management: -Acute pain crises: hydration, analgesia (NSAIDs, opioids), +/- transfusion -Vaccination (e.g. meningitis, pneumococcal), prophylactic penicillin until age 5, folic acid supplementation, hydroxyurea for patients w/ recurrent vaso-occlusive crises (recurrent pain crises, hx of acute chest syndrome, severe symptomatic anemia) -Hydroxyurea increases levels of fetal HGB (often >15%), decreasing proportion of sickled HGB, major side effect of myelosuppression (neutropenia, anemia, thrombocytopenia) Pathophysiology: -AR beta globin mutation (glutamic acid -> valine)

Type 2 Heparin-Induced Thrombocytopenia (HIT)

Presentation: -Heparin exposure >5 days ago and: platelet count reduction >50% from baseline, arterial/venous thrombosis, necrotic skin lesions at heparin injection sites, acute systemic (anaphylactoid) reactions after heparin administration -50% risk of arterial or venous thrombosis in untreated HIT -Labs: thrombocytopenia Diagnosis: -Serotonin release assay is gold standard confirmatory test, high-titer immunoassay (HIT antibody testing) -Start treatment in suspected cases prior to confirmatory tests Treatment: -Stop all heparin products (should be avoided for life if h/o type 2 HIT) -Change to alternative anticoag: direct thrombin inhibitor (e.g. argatroban, bivalirudin) or fondaparinux (synthetic pentasaccharide) -Platelet transfusion usually only necessary if pt is overtly bleeding -Initial treatment w/ warfarin contraindicated due to further increase in thrombotic risk, but may be used as maintenance therapy after using alternative anticoag and once platelet count is >150,000 Pathophysiology: -Life-threatening complication of heparin therapy (esp unfractionated) in which heparin induces conformational change in platelet factor 4 (platelet surface protein 4), exposing a neoantigen to which HIT antibodies form -> IgG abs bind platelet surfaces -> platelet aggregation, thrombocytopenia, prothrombotic state -Thrombocytopenia results from reticuloendothelial system removing antibody-coated platelets -May occur earlier than 5-10 days if pt previously exposed to heparin

Familial Adenomatous Polyposis (FAP)

Presentation: -Hereditary cancer syndrome associated w/ colorectal cancer (almost universal if untreated), brain tumors, desmoids, osteomas -May present w/ >1000 polyps Diagnosis: -Germline gene mutation testing Treatment/Management: -Increased screening: annual screening sigmoidoscopies for children 10-12, switch to annual colonoscopies once colorectal adenomas are detected or pt is 50+ yo -May screen less frequently and later (25 yo, every 1-2 yrs) if attenuated form of FAP -Elective proctocolectomy (performed in pts presenting initially w/ CRC or adenomas w/ high-grade dysplasia, other indications including severe sx from neoplasia like hemorrhage, increase in polyp number in short interval) usually in 20s if no indications prior to then Pathophysiology: -Alteration of tumor suppressor gene APC (adenomatous polyposis coli)

Epidermolysis Bullosa

Presentation: -Hereditary disorder w/ epithelial fragility (bullae, erosions, ulcers) triggered by minor trauma -4 main subtypes: simplex, junctional, dystrophic, Kindler syndrome -Simplex: most common, presents in children & young adults w/ friction-induced blisters of palms/soles that usually heal w/o scarring, some experience chronic thickening of skin, infants present w/ oral blisters w/ bottle feeding Diagnosis: -Blister bx w/ IF microscopy, genetic testing Treatment/Management: -Careful wound care, supportive measures Pathophysiology: -Mutations of proteins involved in intraepiderma and dermoepithelial adhesion complexes in basement membrane zone

Protein C/S Deficiency

Presentation: -Hereditary thrombophilia that predisposes to warfarin-induced skin necrosis (protein C deficiency) Pathophysiology: -Decreased inactivation of factors Va & VIIIa

Staphylococcal Toxic Shock Syndrome

Presentation: -High fever (>102), hypotension (SBP 90 or lower), diffuse macular rash/erythroderma eventually involving palms/soles, headache, mucous membrane hyperemia/ulceration -Multisystem involvement: altered mentation w/o focal neuro signs, multiorgan failure (e.g. crackles, decreased urine output), GI (vomiting/diarrhea), muscular (myalgias, elevated CK), renal (elevated BUN & Cr), hematologic (thrombocytopenia), liver (elevated AST, ALT, bili) -Desquamation 1-3 wks after sx onset Treatment: -Supportive therapy w/ extensive fluid replacement -Removal of foreign body (e.g. vaginal/nasal packing, tampon) -Abx (vanco/oxacillin/nafcillin + clinda) -Pressors only if hypotension persists despite fluid resuscitation Pathophysiology: -Tampon use/nasal packing/surgical or postpartum wound infection (e.g. recent lac repair)/sinusitis/septorhinoplasty -> infection by S. aureus -> toxic shock syndrome toxin-1 (TSST-1) exotoxin release -> acts as superantigen (can activate T cells directly w/o needing to be processed by antigen recognition cells) -> widespread T cell activation & cytokine release -> leukocytosis, multiorgan failure, shock

Mitral Regurgitation

Presentation: -Holosystolic murmur best heard at apex w/ radiation to axilla -If acute onset (e.g. chordae tendinae rupture, infective endocarditis), may present w/ new onset exertional dyspnea, pulmonary congestion/edema (e.g. dry cough), holosystolic murmur, fatigue, syncope, hypotension, cardiogenic shock, JVD, hyperdynamic precordium, absent murmur d/t equalization of LA & LV pressures in up to 50% -If chronic, may develop S3 from LV volume overload (d/t eccentric hypertrophy & cavity dilation) + displaced apical impulse, can lead to eventual LA enlargement & associated AFib (palpitations), subacute/chronic dyspnea & signs of HF -If associated w/ MVP, mid-systolic click (may not be audible if severe) Diagnosis: -Echo -If acute, LA/LV volumes remain normal b/c have not had time to compensate, LVEF is increased (CO overall decreased, but increased forward + backward SV), increased preload, decreased afterload -If compensated chronic, LA/LV volumes are increased w/ normal/increased LVEF, normal CO -If decompensated chronic, LA/LV volumes increased w/ decreased LVEF (60% or less) & CO Treatment/Management: -If acute, emergency surgical intervention -For primary chronic: surgery regardless of sx if LVEF 30-60%, consideration of surgery if success likely (asymptomatic and LVEF >60%, symptomatic and LVEF <30%), repair favored over replacement (necessitates lifelong anticoag, repeat replacements) -If LVEF >60% & not surgical candidates, may refer for exercise program, repeat echo in 6 mos -For secondary: surgery rarely indicated, medical management typical w/ angiotensin receptor blockers (e.g. losartan), beta-blockers (e.g. metoprolol), cardiac resynchronization therapy Pathophysiology: -Can be primary: intrinsic defect in mitral valve apparatus (e.g. leaflets, chordae tendinae, MVP most common cause in developed world, mitral annular calcification w/ age) -Secondary: due to other cardiac disease, e.g. MI/ischemic CM, dilated CM, endocarditis, HCM, rheumatic heart disease -If acute, usually d/t rupture mitral chordae tendinae (e.g. MVP, infective endocarditis, rheumatic heart disease, trauma) or papillary muscle displacement/rupture d/t MI -LVEF for severe mitral regurg overestimates LV fxn b/c regurgitant flow accounts for large amt of stroke volume

Subarachnoid Hemorrhage

Presentation: -Horrible "thunderclap" headache ("worst headache of my life," worst intensity within 1 min, sudden onset, usually lateralized, different in quality from typical HA pattern), somnolence, sx of meningeal irritation (vomiting, photophobia, neck pain/stiffness, low-grade fever), brief loss of consciousness, seizures , N/V -Focal neuro deficits (uncommon): can involve CN III (pupillary dilation/anisocoria, ptosis, down/out eye deviation, rupture of PCA), CN II (vision loss, rupture of ACA/internal carotid), CN IV/VI (rupture of SCA or AICA) -Complications: rebleeding (first 24 hrs), vasospastic cerebral ischemia (within 8 days of bleed), aqueductal stenosis, normal pressure hydrocephalus, seizures, hyponatremia (usually due to SIADH) -Risk factors: HTN, smoking, EtOH use, AVMs, ADPKD Diagnosis: -Noncontrast brain CT w/ blood (hyperattenuation) in basilar cisterns & cerebral sulci, hemorrhage b/t arachnoid & pia mater -If CT unremarkable but still high suspicion for SAH, LP for spinal fluid analysis (elevated opening pressure, increased RBCs, slightly increased protein, xanthochromia of CSF--pink/yellow tinge from Hgb degradation products, may appear as early as 2 hrs after SAH & persist for wks) -CT angiography to identify bleeding source, vasospasm Treatment: -Nimodipine shown to improve outcomes, vascular stenting -Cerebral angiography, surgical repair (craniotomy w/ aneurysm clipping) or endovascular repair (coiling/stenting of aneurysm) Pathophysiology: -Usually due to rupture of saccular/berry cerebral aneurysm (usually in posterior or anterior communicating arteries) -Blood in CSF causes meningeal irritation -> fever, meningismus

Wound Botulism

Presentation: -Hx injection drug use, presence of cutaneous abscesses, presents ~10 days after inoculation -Symmetric, descending paresis starting w/ CNs (III, IV, VI -> diplopia, ptosis, mydriasis, blurry vision, IX & X -> dysphagia, suppressed gag reflex) -Progressive resp compromise from diaphragmatic paralysis -> acute hypoxemic resp failure -Autonomic dysfxn (ileus, orthostatic hypotension, urinary retention) -Sensory abnormalities & confusion rare b/c affects motor synapse (though may have lethargy from resp failure) -Fever, leukocytosis Diagnosis: -Isolation of C. botulinum in culture or identification of toxin in serum -EMG can suggest diagnosis Treatment: -Equine botulinum antitoxin, respiratory support, wound debridement, abx Pathophysiology: -C. botulinum spores contaminate a puncture wound, germinate in anaerobic environment, and produce botulinum toxin in vivo

Human Rabies

Presentation: -Hx of animal bite (bat bites may occur during sleep and go unnoticed), sx present 1-3 mos after exposure w/ initial nonspecific prodrome (fever, chills, sore throat, malaise) -Pain, tingling, numbness of wound site -Encephalitis subtype: hydrophobia (fear of liquids, refusal to drink) & aerophobia due to pharyngeal spasm, autonomic instability (e.g. drooling), spasticity (e.g. opisthotonos, grimacing), agitation, AMS (lethargy, confusion, aphasia) -Paralytic subtype: ascending flaccid paralysis -Prognosis: coma, respiratory failure, death within wks Treatment: -Post-exposure prophylaxis w/ rabies immunoglobulin & vaccine series (only effective in preventing disease prior to sx manifestation) -If vaccinated prior, post-exposure prophylaxis w/ only vaccine (no Ig) -After disease onset, treatment is primarily palliative; most progress to coma & death within wks & if pt survives, expected to have long-term neurologic deficits Pathophysiology: -Reservoirs in US include bats (most common), raccoons, skunks, foxes -Reservoir in developing world most commonly dogs -Neurotropic virus replicates at inoculation site, then travels centrally to spinal cord -Can also potentially contract via scratch or inhalation of aerosolized viral particles (e.g. spelunking/cave exploration)

Squamous Cell Lung Cancer

Presentation: -Hx smoking, hemoptysis, hilar mass, weight loss, anorexia, fatigue -Cough, dyspnea, wheezing -Paraneoplastic syndrome (PTHrP production) causes hypercalcemia of malignancy (constipation, anorexia, nephrogenic DI w/ diabetes insipidus/polydipsia, MSK pain) Diagnosis: -CXR, whole body scan, serum PTHrP -On imaging, tumor w/ heterogeneous density, possible central cavitation Pathophysiology: -Most commonly arise in central lung -> erosion of airway mucosa -> hemoptysis -Accounts for 25-30% of all lung cancers

Tumor Lysis Syndrome

Presentation: -Hyperkalemia, hyperuricemia, elevated LDH, AKI w/ acidosis from high tumor cell burden, rapid replication, and/or massive tumor cell lysis -Nausea, vomiting, myalgias, fatigue, arrhythmias (e.g. QRS widening from hyperkalemia), diarrhea, muscle cramps, tetany (secondary to electrolyte disturbance), low urine output, seizures -Labs: hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia, AKI (unremarkable UA w/o cells/casts) -Risk factors: tumor w/ high cell burden or rapid turnover (e.g. lymphomas, leukemias, bulky lymphadenopathy), cytotoxic chemotherapy/immunotherapy initiation, esp combination therapy Treatment: -Acute: aggressive IV hydration, uric acid reduction w/ rasburicase (urate oxidase analogue, metabolizes uric acid to allantoin), correction of electrolyte abnormalities, continuous telemetry -PPx: IVF, xanthine oxidase inhibitor (allopurinol, febuxostat) or rasburicaase Pathophysiology: -Tumor cell lysis -> release of nucleic acids into bloodstream -> metabolized to uric acid -> precipitate in kidney as uric acid stones -> AKI, renal tubular obstruction, direct cellular injury (acute nephrocalcinosis) -Tumor cell lysis -> release of phosphate into circulation -> precipitation of calcium-phosphate stones -> AKI, obstruction -Tumor cell lysis -> release of K+ into circulation -> hyperkalemia, myocardial calcium-phosphate deposition -> cardiac arrhythmias -Seizures may result from neuronal hyperexcitability caused by hypocalcemia (secondary to calcium-phosphate formation)

Actinic Keratosis

Presentation: -Hypertrophic form: pre-malignant lesions w/ dry, rough, scaly, flat papules w/ erythematous base, most commonly in sun-exposed areas (face, scalp, ears, upper chest, dorsal hands/forearms) -Atrophic form: soft, red macules w/o scales -Can also be associated w/ cutaneous horn (straight or curved cone of keratin) -May appear on skin w/ other signs of actinic damage (e.g. dyspigmentation, atrophy, telangiectasia, wrinkling, thinning, diffuse surface involvement aka field cancerization) -Prognosis: can be chronic/persistent, progress to SCC (1-20%), or may undergo spontaneous resolution Diagnosis: -Bx if features of possible SCC (e.g. size 1+ cm, rapid growth, tenderness, induration, ulceration, tenderness) Treatment: -Cryotherapy, surgical excision, curettage for isolated lesions -Photodynamic therapy -For diffuse lesions, field therapy w/ topical fluorouracil, imiquimod, tirbanibulin, diclofenac

Kallman Syndrome

Presentation: -Hypogonadotropic hypogonadism w/ anosmia (congenital GnRH deficiency); primary amenorrhea w/ delayed adrenarche/pubarche/thelarche but normal uterus & ovaries in females, short stature -Delayed puberty/adrenarche in males, microorchidism/microphallus -X-linked disorder usually (can also be sporadic, AR, AD) Diagnosis: -Low/absent FSH & LH -Karyotype/phenotype c/w appearance Pathophysiology: -Disorder of migration of fetal olfactory & GnRH-producing neurons -> rhinencephalon hypoplasia, hypogonadotropic hypogonadism

Beta Blocker Toxicity

Presentation: -Hypotension, bradycardia (cardiogenic shock), bronchospasm/wheezing, AMS (e.g. delirium), seizures 2-6 hrs after ingestion -Labs: HYPOGLYCEMIA, ECG w/ prolonged PR interval, heart block, bradycardia Treatment/Management: -Secure airway, GI decontamination -IV fluid boluses, IV atropine, IV glucagon -Other therapies: IV calcium, catecholamine vasopressors (EPI, norepi), high-dose insulin & glucose, IV lipid emulsion therapy (used for poisoning w/ lipophilic agents like beta-blockers) Pathophysiology: -Blockade of sympathetic activity -> decreased hepatic glucose production, decreased glycogen breakdown -> hypoglycemia

Methotrexate-Induced Lung Injury

Presentation: -Idiosyncratic (not dose-dependent) hypersensitivity pneumonitis -Exertional dyspnea, hypoxia, pulm infiltrates -Onset within 1-12 mos of starting MTX, progresses from pneumonitis to fibrosis (restrictive PFTs) -Risk factors: RA, parenchymal lung disease Diagnosis: -PFTs: restrictive pattern -BAL w/ lymphocytosis, peripheral blood w/ eosinophils -CT w/ variable patterns including ground glass opacities, consolidation, fibrosis (reticulation) Treatment: -Trial of MTX cessation (diagnostic + therapeutic), usually resolves on discontinuation -Corticosteroids if respiratory status does not improve w/ cessation Pathophysiology: -Like hypersensitivity pneumonitis, causes granulomatous lymphocytic lung inflammation + organizing PNA, rapidly progressive pulmonary fibrosis (reticulation, honeycomb changes)

Hypermagnesemia

Presentation: -If mild, can result in decreased DTRs -If severe, can cause complete loss of DTRs & muscle paralysis -> flaccid quadriplegia, decreased respiration, eventual apnea

Syndrome of Inappropriate Antidiuretic Hormone (SIADH)

Presentation: -If mild/moderate hyponatremia, nausea, dizziness, forgetfulness -If severe hyponatremia, confusion, seizures, coma -Euvolemia (moist mucous membranes, no edema or JVD) -May be associated w/ CNS disturbance (e.g. stroke, hemiplegia, trauma, cerebral salt wasting from increased ICP), meds (e.g. carbamazepine, SSRIs, NSAIDs, desmopressin), lung disease (e.g. PNA), ectopic ADH secretion (e.g. SCLC), pain/nausea (e.g. postoperative) -Labs: hyponatremia, serum osmolality <275 (hypotonic), urine osmolality >100, urine sodium >40 Diagnosis: -Serum electrolyte measurement Treatment/Management: -Fluid restriction +/- salt tablets, hypertonic saline (3%) for severe hyponatremia (e.g. <120) w/ close regulation of correction (max of 8 mEq/L over 24 hrs) to prevent osmotic demyelination syndrome -Demeclocycline, vaptans (V2 antagonists), lithium as second lines for persistent severe hyponatremia resistant to above Pathophysiology: -ADH-mediated water resorption initially causes increase in extracellular fluid, but increased natriuretic peptide release causes increased sodium/water excretion, normalizing extracellular volume (thus euvolemic) -Acute development of severe hyponatremia can cause cerebral edema, risk of brainstem herniation, seizures

Vertebral Compression Fracture

Presentation: -If osteoporotic, may occur w/ minor trauma (e.g. coughing, sneezing, twisting, bending, lifting, fall from standing height) -Acute: severe/midline low back pain, decreased spinal mobility, focal tenderness to spinal/vertebral palpation at affected level, pain increased w/ standing, walking, lying on back (at night), coughing -Chronic: gradual & often painless, w/ progressive kyphosis & loss of stature (esp w/ multiple/recurrent fxs) -Complications: increased risk for future fxs, hyperkyphosis (can lead to protuberant abdomen, early satiety, weight loss, decreased respiratory capacity) -Risk factors: trauma, osteoporosis/osteomalacia, bone metastases, metabolic diseases (e.g. hyperparathyroidism), Paget disease, postmenopausal women and/or >65, osteomyelitis, chronic corticosteroid use Diagnosis: -X-ray Pathophysiology: -Most commonly due to decreased bone mineral density w/ age, osteoporosis -Spinal cord or nerve root compression rare, so usually does not present w/ neurologic deficits -Almost always thoracic/lumbar, very rarely cervical

Acute Graft-Versus-Host Disease (GVHD)

Presentation: -Immune-mediated, multisystem inflammatory disorder that occurs in 35-50% of pts that undergo allogeneic hematopoietic stem cell transplantation (HSCT), usually presents within 100 days of initial transplant -Profuse, watery diarrhea w/ secretory pattern (persistent, unrelated to eating, occurs at night), often w/ crampy abd pain, hematochezia, N/V -Maculopapular rash of palms/soles/face that is painful, may become confluent (similar appearance to SJS) -Liver inflammation w/ damage to biliary tract epithelium -> hyperbili, high alk phos, elevated transaminases Diagnosis: -Colonoscopy w/ biopsy after ruling out infections like C. diff, CMV, cryptosporidium Treatment: -Systemic glucocorticoids Pathophysiology: -Donor T cells (esp cytotoxic T cells/CD8+) identify antigens (minor/major HLA antigens) on host cells as foreign -> cell-mediated proinflammatory response

Ventilator-Associated Pneumonia (VAP)

Presentation: -In mechanically ventilated pt, increased resp secretions (may be purulent), worsening oxygenation & increased requirement for ventilatory support, fever, tachycardia, leukocytosis, new pulm infiltrates +/- effusion -Hospital-acquired PNA usually occurring 48+ hrs after intubation -Signs of worsening resp status (hypoxia, decreased TV, increased inspiratory pressure, increased respiratory rate) -Risk factors: acid suppression (PPI, H2 blockers, antacids), supine position, pooled supraglottic secretions, paralysis & excessive sedation (prolongs length of intubation, inhibits gag reflex), excessive pt movement while intubated, frequent ventilator circuit changes Diagnosis: -CXR first (may show alveolar infiltrates, air bronchograms, silhouetting of adjacent solid organs) -Noninvasive (endotracheal aspiration) or invasive (BAL) sampling of lower resp tract w/ culture & microscopy -Moderate/heavy growth of 1+ organisms generally diagnostic -CT if suspect complications (abscess, empyema), unresponsive to abx Treatment/Management: -Abx w/ broad coverage of gram+, possibly Pseudomonas & gram- (e.g. pip-tazo), possibly MRSA -Narrow coverage w/ +cultures & clinical improvement, change abx if +cultures but no improvement Pathophysiology: -Usually due to aspiration of oropharyngeal & gastric secretions around endotracheal tube cuff -Nosocomial PNA usually involving aerobic gram- bacilli (Pseudomonas, E. coli, Klebsiella) and/or gram+ cocci (MRSA, Strep pneumo) Prevention: -Elevation of bed to 30-45 degrees, continuous/intermittent suctioning of secretions, minimization of pt transport, limited use of gastric acid inhibitors (reduces burden of microorganisms in gastric secretions)

Peritoneal Dialysis-Related Peritonitis

Presentation: -In pt on peritoneal dialysis, abdominal pain (~80%), nausea (~30%), high fever often absent, diffuse abd tenderness +/- rebound, cloudy peritoneal fluid Diagnosis: -Confirmed by 2+ of: 1. consistent clinical presentation, 2. fluid neutrophils >50% (usually w/ leukocytes >100), 3. positive gram stain or culture Treatment/Management: -Empiric vancomycin + antipseudomonal agent (e.g. gentamicin, cefepime); intraperitoneal abx administration preferred w/ continued peritoneal dialysis during treatment, may start w/ empiric IV abx & narrow based on culture/fluid results -IV abx continued in minority of pts w/ clear evidence of sepsis (e.g. hypotension, high fever) d/t bacteremia that intraperitoneal abx would not treat -2-3 wks of therapy tailored based on fluid culture -Catheter removal for fungal or refractory bacterial infection Pathophysiology: -touch contamination of catheter lumen, extension of catheter site skin infection -Most commonly: gram+ bacteria (~65%, most commonly coag- Staph), gram- bacteria (~30%), fungal organism (~5%)

Catheter-Related Bloodstream Infection (CRBSI)

Presentation: -In pt w/ external catheter site, presents w/ fevers, chills, malaise, signs of septic shock/bacteremia (lactic acidosis, confusion, hypotension) -Esp w/ tunneled catheters, there may be no evidence of infection around external catheter site (e.g. erythema, purulence), no localizing manifestations to a particular site in the body -Occur ~1x annually in pts w/ tunneled catheters, much high rate than AV fistula Diagnosis: -Blood cultures (one from catheter site, one peripheral, faster growth from catheter sample is suggestive of CRBSI) Treatment/Management: -Hemodynamic support, empiric abx (e.g. vancomycin + ceftazidime, vanc + cefepime, vanc + gentamicin) against common skin pathogens -Add on caspofungin w/ any of the following risk factors: TPN, prolonged use of broad-spectrum abx, hematologic malignancy, solid organ transplant, femoral catheterization, Candida colonization at multiple sites -Catheter removal (eliminate nidus of infection) if any of the following present: severe sepsis, hemodynamic instability, evidence of metastatic infection (e.g. endocarditis), purulence at exist site of catheter, continued sx after 72 hrs of empiric abx -If none of the above, wait for culture results; remove catheter if blood culture shows e/o S. aureus, Pseudomonas, or fungi (e.g. Candida) -In some cases w/ very poor vascular access, antibiotic lock therapy may be used w/ catheter exchange over guidewire several days later (once afebrile & clinically stable, w/ no evidence of tunnel tract involvement) Pathophysiology: -Migration of skin flora through catheter lumen into bloodstream (coag-negative Staph, S. aureus, Enterococci, Candida, etc.) -Most commonly caused by coag- Staph or S. aureus, infections w/ gram- bacilli also common

Stomal Stenosis

Presentation: -In pt w/ h/o roux-en-y gastric bypass, surgical complication in which GJ anastomosis progressively narrows -> gastric pouch outflow -Progressive sx of nausea, postprandial vomiting, GERD, dysphagia -May progress from not tolerating solids to not tolerating solids + liquids Diagnosis: -EGD Treatment/Management: -Balloon dilation of stenosis during EGD -May require surgical revision if dilation fails Pathophysiology: -Thought to involve post-surgical tissue ischemia/ulceration

Mitral Chordal Rupture

Presentation: -In pt w/ hx MVP, presents as new onset severe pulmonary edema, holosystolic murmur at cardiac apex, hypotension w/ hyperdynamic precordium -May also be associated w/ endocarditis, rupture following MI Diagnosis: -Echo w/ flail mitral valve leaflet Pathophysiology: -Myxomatous degeneration in pts w/ MVP predisposes to spontaneous rupture

Spondyloarthropathies

Presentation: -Includes apophyseal (spinal) joint arthritis, ankylosing spondylitis, RA, IBD-associated arthritis, psoriatic arthritis, reactive arthritis -Chronic, progressive pain that is worse at night and w/ prolonged rest, prolonged morning stiffness, improves w/ activity, usually presents <40 yo in men Pathophysiology: -Associated w/ HLA-B27 -All involve ligamentous inflammation & destruction of articular cartilage -> stiffness, pain

Fecal Impaction

Presentation: -Incontinence of small volume soft stools w/ h/o chronic constipation (encopresis) -May often be associated w/ concurrent urinary incontinence -Can develop rectal bleeding d/t ulceration caused by stool in rectum -Risk factors: elderly, chronic constipation, impaired mobility, inadequate fluid or dietary fiber intake, decreased sensation of stool in rectal vault (e.g. spinal cord injury, dementia) Diagnosis: -Digital rectal exam, abd X-ray to detect impaction in more proximal rectum (not required for diagnosis, usually shows normal bowel gas pattern w/ excessive stool) Treatment/Management: -Manual disimpaction to break up hard stools, followed by suppository or enemas (mineral oil, tap water) to dislodge fecal fragments -Bowel regimen after disimpaction: laxatives (e.g. PEG, lactulose), dietary alterations (increased fluid/fiber intake) Pathophysiology: -Obstruction of fecal flow in rectum causes backup of stool proximal to impaction, passage of soft stool around impaction leads to incontinence -Often associated w/ constipation & age-related changes including slowing of fecal transit, decreased sensation of stool in vault, reduction in anal sphincter tone

COPD Exacerbation

Presentation: -Increased dyspnea, cough (more frequent/severe), sputum production (e.g. change in color/volume) -May be triggered by URI (e.g. presentation w/ low-grade fever) -Physical exam w/ wheezing, tachypnea, prolonged expiration, use of accessory muscles, distant breath sounds, JVD (esp w/ inspiration d/t increased intrathoracic pressure) Diagnosis: -CXR w/ hyperinflation, ABG showing hypoxia & CO2 retention (chronic and/or acute) -Initial dx of COPD: PFTs w/ low FEV1 (<40% = severe obstruction), increased RV Treatment/Management: -Supplemental O2 (target SpO2 88-92%) -Inhaled bronchodilators (beta-2 adrenergic agonist, anticholinergics), systemic glucocorticoids (oral prednisone or IV methylprednisolone) -Abx if 2+ cardinal sx or if mechanically ventilated (NPPV or intubation), empiric coverage w/ macrolide (azithromycin), resp FQ (levofloxacin, moxifloxacin), or penicillin/beta-lactamase inhibitor (amoxicillin-clavulanate) for 3-7 days -Oseltamivir if evidence of influenza -Noninvasive PPV if ventilatory failure, tracheal intubation if NPPV failed/contraindicated

Myasthenic Crisis

Presentation: -Increased generalized & oropharyngeal weakness, bulbar weakness (e.g. difficulty coughing up sputum) in pt w/ myasthenia gravis -Respiratory insufficiency/dyspnea -May be precipitated by infection, surgery, childbirth/pregnancy, tapering of immunosuppressive drugs, meds (e.g. aminoglycosides, FQs, beta-blockers, azithromycin, CCBs, Mg), thymectomy Treatment: -Intubation for deteriorating respiratory status (declining sats, decreasing vital capacity) -Intubation, hold/discontinue AChE inhibitors (e.g. pyridostigmine) to reduce excess airway secretions & risk of aspiration -Plasmapheresis or IVIg + high-dose steroids, followed by bridge to steroid-sparing agents (e.g. azathioprine) -Other immunomodulatory drugs may be used if corticosteroids ineffective (e.g. mycophenolate mofetil, azathioprine)

Mycoplasma Pneumonia

Presentation: -Indolent headache, malaise, fever, persistent dry cough, nonexudative pharyngitis, macular/vesicular rash, sore throat -Atypical pneumonia w/ dyspnea, diffuse bilateral reticulonodular/patchy pulmonary infiltrates (looks worse than clinical severity of sx), bilat crackles & wheezing -Pt often able to continue normal activities -"Walking pneumonia" Diagnosis: -Bilateral interstitial infiltrate on CXR (often w/ small serous pleural effusion), cold agglutinins/subclinical hemolytic anemia, normal leukocyte count Treatment: -Usually empiric PNA coverage -Macrolide (azithromycin) or respiratory fluoroquinolone Pathophysiology: -Spread by respiratory droplets, commonly in close quarters among young people (e.g. school, military), fall/winter -No cell wall, so beta-lactam abx are ineffective

Cytomegalovirus (CMV)

Presentation: -Infection common in immunosuppressed (e.g. transplant, HIV) -Associated w/ colitis/enteritis (<50 CD4, fever, malaise, weight loss, vomiting, abd pain, multiple/large/shallow colonic ulcerations or erosions, bloody & frequent/small-volume diarrhea, toxic megacolon) -Tissue-invasive disease: can also precipitate pancreatitis, hepatitis, meningoencephalitis, pneumonitis (dyspnea, nonproductive cough, low-grade fever, patchy/diffuse ground-glass opacities) -Labs: cytopenias due to bone marrow involvement, atypical lymphocytes on peripheral blood smear Diagnosis: -Tissue biopsy of organs for tissue invasive disease, serum PCR -If colitis, bx shows eosinophilic intranuclear & basophilic intracytoplasmic inclusions -Should receive ocular exam to rule out retinitis if HIV Treatment/Management: -Ganciclovir (IV indicated w severe disease--pneumonitis, meningoencephalitis, high viral loads, significant GI disease) -Foscarnet if drug-resistant CMV -Reduction of immunosuppressant meds, if HIV antiretroviral therapy Pathophysiology: -Widely prevalent herpesvirus that establishes latent infection after initial inoculation; most carriers are asymptomatic unless immunosuppressed -> CMV reactivation, viremia and/or tissue invasive diseases

Acute Dysentery

Presentation: -Infectious diarrhea w/ cramping, blood/mucus in stools, fever -Fecal leukocytes Diagnosis: -Stool studies: culture, Shiga toxin assay, fecal leukocytes -Abd CT if suspicion of ischemic colitis (e.g. hypoperfusion) -Endoscopy if suspicion of IBD (e.g. progressive, waxing/waning course) Treatment/Management: -Fluid repletion (oral preferred) -Empiric abx (unless STEC/EHEC suspected; abx can worsen risk for HUS) for severe disease, elderly, immunocompromised Pathophysiology: -Etiologies include bacterial infection (e.g. STEC/EHEC, Shigella, Campylobacter, Salmonella), intestinal amebiasis, IBD, ischemic colitis

Nonfunctioning Pituitary Adenoma

Presentation: -Infertility, irregular menstrual cycles, sx of sellar mass (headache, loss of peripheral vision) -Can be complicated by apoplexy (spontaneous hemorrhage into pituitary, most commonly in patients w/ adenoma); presents w/ acute headache, visual field defects, decreased visual acuity -Fewer signs associated w/ functional adenomas (e.g. galactorrhea, acromegaly, etc.) Diagnosis: -Increased alpha-subunits, suppressed LH & FSH -Prolactin may be mildly increased (>5-20) but much lower than prolactinoma (>200) Treatment/Management: -Transphenoidal surgery -Radiation (field therapy or stereotactic radiation) can be used as adjuvant/second-line therapy in pts w/ incompletely resected adenomas Pathophysiology: -Pituitary adenoma usually arising from gonadotrophs in pituitary gland -Gonadotrophs usually secrete LH & FSH (dimeric hormones consisting of common alpha-subunit & different beta-subunit), but dysfunctional cells in adenoma secrete primarily just common alpha-subunit -Prolactin mildly elevated d/t compression of pituitary stalk that blocks normal hypothalamic inhibition of prolactin secretion

Polychondritis

Presentation: -Inflammation of cartilage, most commonly of the pinna

Tenosynovitis

Presentation: -Inflammation of tendon & sheath, most commonly in hands/wrists -May be associated w/ overuse or following bite/puncture wound -Presents w/ pain/tenderness along tendon sheath, esp w/ flexion/extension movements

Chronic Paronychia

Presentation: -Inflammatory condition of finger lasting >6 wks, associated w/ retraction or loss of cuticle & dystrophic nail changes Treatment/Management: -High-potency topical corticosteroids Pathophysiology: -Variant of contact dermatitis

Diabetic Retinopathy

Presentation: -Initially asymptomatic until late in disease course -Floaters, decreased visual acuity, patchy focal visual field defects, acute monocular vision loss Diagnosis: -Fundoscopy w/ microaneurysms, hard or soft exudates, retinal dot/blot hemorrhages, neovascularization, cotton wool spots -Start screening 5 yrs after T1DM diagnosis, at time of diagnosis for T2DM Treatment/Management: -Laser photocoagulation Pathophysiology: -Proliferative: vascular proliferation (neovascularization) w/ or w/o vitreous hemorrhage, macular edema, retinal detachment -Can predispose to retinal detachment

Cyanide Poisoning

Presentation: -Initially presents w/ catecholamine-induced compensatory HTN & tachycardia to increase O2 delivery, but degenerates within mins to bradycardia & hypotension, coma, seizures, cardioresp arrest -Acute toxicity presents w/ neuro/cardioresp stimulation -Skin: cherry-red flushing, cyanosis (later) -CNS: HA, AMS, seizures, coma, vertigo, dizziness -CV: arrhythmias -Resp: tachypnea followed by resp depression, pulm edema -GI: abd pain, N/V -Renal: metabolic acidosis (from lactic acidosis), renal failure -Can cause anoxic brain injury -May be associated w/ administration of nitroprusside (esp in setting of renal insuff, prolonged infusion), occupational exposure in mining, pesticide use, fires -Labs: severe lactic acidosis, metabolic acidosis, very low bicarb Treatment/Management: -Supplemental O2, airway protection (intubation), IV fluids for hypotension -Decontamination: skin decontamination & removal of clothing w/ dermal exposure, activated charcoal if ingested exposure -Hydroxocobalamin preferred antidote, sodium thiosulfate as alternate -If antidote not available, nitrites to induce methemoglobinemia (cyanide binds tightly to Fe3+, sequestering away from mitochondria) Pathophysiology: -Impaired aerobic metabolism, cyanide inhibits cytochrome oxidase a3 in mitochondrial electron transport chain -> binds to Fe3+ and prevents reduction to Fe2+ -> blocks production of ATP by oxidative phosphorylation -Increased anaerobic metabolism -> lactate buildup -> depletion of bicarb, decrease in pH from acid -> metabolic acidosis, compensatory tachypnea

Stress Fracture

Presentation: -Insidious onset of sharp, local pain that worsens w/ activity, improves w/ rest, common in foot (esp 2nd metatarsal), anterior tibia -Point tenderness at fx site, swelling -Risk factors: repetitive activities (e.g. running, gymnastics, dancing), abrupt increase in physical activity (e.g. military recruits), inadequate calcium/vitamin D intake, decreased caloric intake, female athlete triad (low caloric intake, hypomenorrhea/amenorrhea, low bone density), improper footwear, biomechanical abnormalities (e.g. weak calf muscles, high arches) Diagnosis: -X-ray w/ linear fx at an angle to the cortex or hairline lucency, periosteal elevation & cortical thickening, bone sclerosis (but most commonly negative) -Possible negative X-ray in first 6 wks following injury -MRI, CT, bone scintigraphy more sensitive but usually unnecessary Treatment/Management: -Reduced weight bearing for 4-6 wks (e.g. crutches), analgesics, pneumatic splinting, graduated exercise program -Referral to orthopedic surgeon for fxs at high risk of malunion (e.g. anterior tibial cortex, 5th metatarsal) -If continued pain despite activity restriction, may use wide, hard-soled podiatric shoe Pathophysiology: -Repeated tension/compression on bone w/o adequate rest -> microfxs that coalesce within cortical bone

Morton Neuroma

Presentation: -Interdigital neuroma, presents w/ numbness/pain at distal forefoot b/t 3rd & 4th toes -Pain reproduced by squeezing forefoot across metatarsals -Clicking sensation/crepitus when palpating space & squeezing metatarsal joints (Mulder sign) -Commonly seen in runners, sx worsen w/ walking on hard surfaces, wearing tight or high-heeled shoes Treatment: -Metatarsal support w/ bar or padded shoe inserts to decrease pressure on metatarsal heads -If fail conservative therapy, surgery Pathophysiology: -Mechanically induced neuropathic degeneration of interdigital nerves

Diffuse Esophageal Spasm

Presentation: -Intermittent chest pain (secs-mins), dysphagia for both liquids & solids -Sensation of food "getting stuck," may be triggered by ingestion of hot/cold foods -Often associated w/ emotional factors, functional GI disorders -Better w/ nitrates & CCBs, worse w/ ergonovine administration (used for manometry) -Sx resembling achalasia, nutcracker esophagus Diagnosis: -Esophageal manometry w/ intermittent peristalsis, multiple simultaneous contractions of middle & lower esophagus -Barium esophagram w/ "corkscrew" pattern, nonperistaltic contractions -Normal ECG/cardiac workup, normal EGD Treatment: -CCBs, 2nd lines include nitrates, TCAs Pathophysiology: -Uncoordinated, premature simultaneous contractions of esophageal body, probably related to impaired inhibitory innervation of esophagus

Peripheral Arterial Disease (PAD)

Presentation: -Intermittent claudication (calf pain/tightness w/ exertion, generally improves w/ rest, if severe may present w/ cramping leg pain w/ rest, dangling/gravity dependence) -Pain may involve buttocks, thigh, calf, foot -No significant LE weakness -Skin hairless & shiny, cool extremities, weak pulses, delayed cap refill, may be associated w/ nonhealing ulcers (painful, at tips of digits where least perfused), gangrene, pallor w/ leg elevation, skin atrophy -Leriche syndrome: triad of LE claudication, absent/diminished femoral pulses, erectile dysfxn -Risk factors: T2DM, HTN, smoking, hyperlipidemia -Complications: acute-on-chronic limb ischemia & arterial thrombosis from plaque disruption, increased risk of ACS/CVA (20% 5 yr risk of MI/stroke, 15-30% 5 yr risk of death d/t CV cause), critical limb ischemia (1-2%) Diagnosis: -ABI of 0.9 or lower, 0.4 or lower = severe ischemia (may be falsely elevated in DM pts d/t calcified, noncompressible vessels) -Arterial duplex US used to localize site & severity of obstruction in pts w/ abnormal ABI considering interventional procedures Treatment/Management: -Supervised graded exercise program (helps reduce sx and improve max walking distance), smoking cessation, aggressive T2DM control (HbA1c <7%), BP control, low-dose aspirin & statin -Cilostazol (arterial dilator) considered in pts w/ persistent sx despite antiplatelet/exercise therapy -Percutaneous/surgical revascularization reserved for pts w/ persistent sx despite exercise and/or pharm therapy, can involve angioplasty +/- stent placement or autogenous/synthetic bypass graft -Daily intermittent pneumatic compression therapy may help some pts w/ severe PAD or critical limb ischemia if refractory to above Pathophysiology: -Atherosclerosis of LE vessels, most commonly occurring toward the proximal end of larger peripheral arteries (e.g. iliac, popliteal)

Monomorphic Ventricular Tachycardia

Presentation: -Intermittent palpitations, dizziness, chest discomfort, presyncope/syncope w/o preceding sx, often in pts w/ underlying structural heart disease (e.g. dilated CM) or prior MI -Arrhythmia usually spontaneously terminates within few min; rapid recovery from syncope -High risk of sudden cardiac death Diagnosis: -ECG w/ regular, wide-complex tachycardia demonstrating AV dissociation + fusion beats (vs. SVT w/ aberrancy) -Echo w/ evidence of underlying structural heart disease (e.g. ischemic scarring, CM w/ low EF) Treatment: -If hemodynamically unstable: synchronized cardioversion -If hemodynamically stable: IV amiodarone, if no response other antiarrhythmics (e.g. procainamide, sotalol, lidocaine) -If no longer in rhythm, admission to undergo telemetry monitoring & Echo; may warrant treatment w/ antiarrhythmics, catheter ablation, and/or placement of ICD Pathophysiology: -Etiologies: ischemia, electrolyte abnormalities, hypoxia, drug effects, HF -Ventricular in origin -> wide-complex arrhythmia

Glossopharyngeal Neuralgia

Presentation: -Intermittent, severe, stabbing pain in areas innervated by glossopharyngeal & vagus nerves (including ear)

Endophthalmitis

Presentation: -Invasive bacterial or fungal infection of the globe due to disruption of external surface of eye (e.g. trauma) -Conjunctival injection, purulent haziness of ocular contents, hypopyon (pus in anterior chamber), aching eye pain, decreased visual acuity -Risk factors: hospitalization w/ catheter (e.g. CVC), recent eye surgery Diagnosis: -Visual examination in all pts w/ candidemia Pathophysiology: -Strongly associated w/ candidemia -Occasionally may be caused by Staph epidermidis (often presents w/ concurrent endocarditis)

Stress Urinary Incontinence

Presentation: -Involuntary discharge of urine during coughing, sneezing, straining, laughing, intercourse, Valsalva, or sudden movements -May be exacerbated by compression of bladder by fibroids, anteverted/enlarged uterus, pregnancy -Due to either increased laxity of pelvic floor (urethral hypermobility) w/ age, vaginal deliveries, etc. or decreased urethral sphincter muscle tone -Pooling of urine in posterior vagina on speculum exam from retrograde vaginal voiding during pregnancy (trapping of urine in vagina due to positional changes & growing uterus) -May also present w/ cystocele -Risk factors: pregnancy (esp vaginal delivery, esp w/ macrosomia), obesity, chronic high-impact exercise Diagnosis: -US of pelvis, urodynamic testing -UA and postvoid residual volumes normal (<150 mL) Treatment: -Pelvic floor muscle (Kegel) exercises (strengthen/stabilize pelvic floor musculature), lifestyle modifications (weight loss), continence pessary, midurethral sling procedure/pelvic floor surgery -If after delivery, observation & reassurance, Kegel exercises Pathophysiology: -Urethral hypermobility from pelvic floor weakness and/or intrinsic sphincter deficiency -Urogenital mucosa atrophy from estrogen deficiency -If presents after delivery, may be due to stretch injury to pudendal nerve (innervates voluntary contraction of external urethral sphincter); usually self-limited and resolve w/ healing

Paradoxical Embolus

Presentation: -Ischemic stroke in young pt, possibly in setting of LE DVT Diagnosis: -TTE or TEE w/ bubble study to detect intracardiac shunt Pathophysiology: -Intracardiac communication like patent foramen ovale or ASD allows dislodged venous clot/embolus to travel from venous to arterial circulation (bypassing pulmonary circulation)

Orthostatic Proteinuria

Presentation: -Isolated proteinuria (no hematuria, oliguria, HTN, edema, CVA tenderness) -Presents most commonly in adolescent boys; proteinuria seen when patient is upright (during daytime) and is absent after prolonged recumbent period (e.g. in morning) -Most common cause of proteinuria in adolescents, rarely occurs after 30 yo Diagnosis: -Proteinuria first detected on UA, evaluated w/ first morning urine protein:creatinine ratio (normal ratio) -Split 24-hr urine collection (divided b/t day/night) -Distinguished from transient proteinuria by persistent proteinuria on repeat UA Treatment/Management: -Reassurance and observation, excellent prognosis & does not predict development of significant glomerular pathology in the future Pathophysiology: -Mechanisms may include exaggerated normal response to upright posture, increased glomerular capillary pressure d/t exaggerated angiotensin II response w/ standing, subtle glomerular abnormality (e.g. focal mesangial hypercellularity and/or basement membrane thickening), left renal vein entrapment

Atopic Keratoconjunctivitis

Presentation: -Itching, tearing, thick mucus discharge, photophobia, blurred vision, hyperemia, conjunctival/eye edema & injection -Severe form of allergic conjunctivitis (distinguished by more prolonged course, possible vision impairment due to corneal involvement, thickening of eyelids & surrounding skin)

Dermatitis Herpetiformis

Presentation: -Itchy papules/vesicles/bullae most prominent on elbows & forearms (also back, knees, buttocks), associated w/ celiac disease -Grouped papulovesicular rash Treatment: -Dapsone

Autoimmune Hemolytic Anemia (AIHA)

Presentation: -Jaundice from hyperbilirubinemia due to increased RBC turnover, may be triggered by URI Diagnosis: -Labs: increased reticulocyte count, spherocytes, indirect hyperbili, elevated LDH, low haptoglobin, positive Coombs test Pathophysiology: -May be primary/idiopathic or secondary (e.g. SLE) -Antibodies to RBCs

Wilson Disease

Presentation: -Kayser-Fleischer rings in eyes (golden brown/greenish rings on slit lamp exam), hepatic failure w/ hepatosplenomegaly/chronic hepatitis/cirrhosis, Coombs-negative hemolytic anemia, thrombocytopenia -Neurologic sx like chorea & labile mood, parkinsonism (tremor, hypertonia, facial stiffness, pooled saliva, dystonia), gait disturbance/ataxia, dysarthria -Psychiatric sx like depression, personality changes, psychosis, academic decline, irritability -Hepatic dysfxn predominates in younger pts, neurologic sxs predominate in older pts (adolescence) Diagnosis: -Low serum ceruloplasmin levels, elevated urinary copper, elevated AST & ALT -Increased copper content on liver biopsy Treatment: -Chelators (D-penicillamine, trientine) -Zinc (interferes w/ copper absorption) -Liver transplant curative in fulminant liver failure or drug-resistant disease Pathophysiology: -Autosomal recessive defect (APT7B) in ATP mediated hepatocyte copper transport, hepatolenticular degeneration -Can't transport copper into bile or ceruloplasmin -> copper accumulation/leak from hepatocytes -> copper deposition in tissues (basal ganglia, cornea)

Lupus Nephritis

Presentation: -LE edema, HTN, renal dysfxn, proteinuria, UA w/ active sediment (e.g. hematuria, RBC casts) in pt w/ SLE Diagnosis: -Renal bx prior to treatment in all pt w/ e/o marked proteinuria (>500 mg/day), active urinary sediment (hematuria, dysmorphic RBCs, casts) or declining renal fxn Treatment/Management: -Classes I & II: mild, often do not require therapy unless disease progresses -Classes III & IV: require immunosuppression w/ glucocorticoids (e.g. methylprednisolone, prednisone) & cyclophosphamide or mycophenolate mofetil -Class V (membranous): may require immunosuppression if proliferative lesions or nephrotic syndrome present -Class VI: advanced sclerosing disease, immunosuppression not recommended Pathophysiology: -Immune complex-mediated glomerular injury -Complexes primarily composed of anti-dsDNA antibodies, deposited in mesangial, subendothelial, subepithelial space -> neutrophil & mononuclear influx, complement fixation within glomerulus

Hemolytic Anemia Findings

Presentation: -Labs: indirect hyperbili, increased urinary urobilinogen, hemoglobinemia (increased free Hgb in circulation), hemoglobinuria, increased serum LDH, decreased haptoglobin, increased reticulocyte count, increased MCV (due to increased erythropoiesis to replace RBCs), schistocytes/helmet cells on smear Pathophysiology: -Haptoglobin binds free Hgb in circulation to promote excretion by reticuloendothelial system, w/ hemolysis Hgb exceeds binding capacity of available haptoglobin -> decreased haptoglobin -May be associated w/ destruction by prosthetic valves, microangiopathic hemolytic anemias (DIC, HUS, TTP)

Leukemoid Reaction

Presentation: -Labs: leukocytes >50,000, high leukocyte alk phos score, elevated neutrophil precursors (metamyelocytes > myelocytes, more mature forms than in CML), no absolute basophilia vs. CML Treatment: -Clinical improvement often takes >24 hrs after starting abx Pathophysiology: -Severe infection -> massive mobilization of mature & immature leukocytes from bone marrow

Sideroblastic Anemia

Presentation: -Labs: normochromic/hypochromic (dimorphic RBC populations) microcytic anemia, smear w/ ring sideroblasts (nucleated erythroblasts w/ mitochondrial iron granules around nucleus), increased serum iron & decreased/normal TIBC, high transferring saturation Diagnosis: -Bone marrow sampling can be used for confirmation (not necessary in many reversible forms) Treatment/Management: -B6 for isoniazid toxicity Pathophysiology: -Can be congenital (children, adolescents) or acquired (myelodysplastic syndrome, EtOH, malnutrition, B6 def, meds including B6 antagonist isoniazid, lead poisoning) -Due to defects in heme synthesis; in acquired types may be due to pyridoxine-dependent impairment of protoporphyrin synthesis within mitochondria

Diabetic Neuropathy

Presentation: -Large fiber neuropathy presents w/ predominantly negative sx: numbness, poor balance, diminished/absent ankle reflexes, reduced/absent vibration & light touch sensation, impaired proprioception, weakness of intrinsic muscles of feet -> foot deformities (e.g. hammer toes) -Small fiber neuropathy presents w/ predominantly positive sx: burning & stabbing pain, reduced pinprick sensation, ankle reflexes may be preserved if only small, allodynia, paresthesias -Stocking-glove distribution Diagnosis: -Tuning fork test as preliminary test -Confirmation w/ nerve conduction studies (if dx uncertain) Treatment/Management: -TCAs (amitriptyline, use w/ caution in elderly), SNRIs (duloxetine, venlafaxine), anticonvulsants (pregabalin, gabapentin, lamotrigine, carbamazepine), topical treatments for some pts (capsaicin cream, topical lidocaine) -Aggressive glycemic control in pts w/ high A1c Pathophysiology: -Large fiber neuropathy affects nerves involved in pressure, proprioception, balance -Small fiber neuropathy affects nerves involved in pain/temp sensation -Neuronal injury due to microvascular involvement, demyelination, oxidative stress, deposition of glycation end products, metformin use (may decrease B12 absorption)

Secretory Diarrhea

Presentation: -Large volumes of watery, nonbloody diarrhea that persists while fasting and at night -Risk factors: intestinal surgery/trauma Diagnosis: -Low stool osmotic gap (<50)--low SOG = low stool solute concentration -Colonoscopy for workup Pathophysiology: -Caused by secretion of water & electrolytes into intestine -Etiologies: infection/toxins (e.g. Vibrio cholera), hormones (e.g. VIPoma, gastrinoma), congenital disorders of ion transport (e.g. CF), poor bile acid reabsorption aka bile salt diarrhea (e.g. postsurgical changes w/ bowel resection, cholecystectomy), microscopic colitis,

Acute Intermittent Porphyria

Presentation: -Largely asymptomatic, occasional episodes of diffuse abd pain w/o guarding/rebound, N/V, hypoactive bowel sounds, constipation -Peripheral neuropathy (most pronounced in UEs, weakness/areflexia), dark red/brown urine (oxidizes w/ light/air exposure), autonomic dysfxn (tachycardia, diaphoresis, HTN), neuropsych sx (e.g. hallucinations, anxiety, psychosis, restlessness) -No photosensitive skin findings vs. porphyria cutanea tarda -May be precipitated/exacerbated by meds (e.g. CYP450 inducers., progesterone, phenytoin), physiologic stress (e.g. fasting, surgery, illness), EtOH/tobacco, menstruation -Labs: hyponatremia (d/t SIADH), elevated transaminases Diagnosis: -Elevated serum & urinary PBG, ALA, porphyrins Treatment: -Glucose & hemin (heme analogue) Pathophysiology: -AD partial defect in heme biosynthesis enzyme (reduced activity of porphobilinogen deaminase), low penetrance -Leads to buildup of neurotoxic heme pathway intermediates (porphobilinogen, aminolevulinic acid) -Pigmented urine due to accumulation of porphyrin & porphyrin precursors

Aortic Stenosis

Presentation: -Late-peaking, crescendo-decrescendo systolic murmur heard best at RUSB w/ radiation to R carotid (if loud, more c/w mild-moderate disease), soft & single S2 during inspiration (paradoxical splitting during expiration) -Pulsus parvus et tardus (delayed, slow-rising, weak carotid pulses) -S4 from concentric hypertrophy + stiffening of LV -If severe, sx of angina, fatigue, presyncope/syncope, dyspnea, exertional lightheadedness, decreased exercise tolerance, angina pectoris, HF -Avg survival following sx onset is 2-3 yrs w/o valve replacement Diagnosis: -Echo (shows LVH, valvular stenosis) -Severe AS defined as: aortic jet velocity 4+ m/s or mean transvalvular pressure gradient of 40+ mm Hg, valve area usually <1 cm2 but not required for diagnosis, low pulse pressure usually <25 Treatment/Management: -Indications for valve replacement--severe AS & 1+ of: onset of sx (angina, syncope), LVEF <50%, undergoing other cardiac surgery (e.g. CABG) -Perc balloon aortic valvulotomy considered only as bridge to surgical/transcatheter valve replacement (high rate of procedural complications like MI & acute aortic regurg, does not improve long-term prognosis) -If severe AS but normal LVEF, monitoring w/ serial Echos Pathophysiology: -Etiologies: calcific disease (most common, esp >70 yo, aka senile calcific aortic disease), congenital bicuspid valve (younger pts, <70), rheumatic heart disease (common worldwide but rare in developed countries) -Angina d/t insufficient O2 supply during exertion (LVH increases myocardial mass & O2 requirements) -During inspiration, closure of aortic valve is delayed in severe AS -> nearly simultaneous closure of aortic/pulmonic valves during inspiration (single S2, correlated w/ disease severity)

Chronic Venous Insufficiency

Presentation: -Leg discomfort (achy/heavy sensation), pain, swelling worse in the evening & after prolonged standing, improves w/ walking & leg elevation -Pitting edema -In severe cases, redirection of blood from deep venous system to superficial venous system -> telangiectasias, varicose veins, skin discoloration, lipodermatosclerosis, skin ulceration (characteristically on medial aspect of lower leg, medial malleolus), stasis dermatitis -Dermatitis classically along anterior shins w/ erythema, pruritus, scaling, weeping, chronic woody induration & brown discoloration, looks similar to dermatitis but is bilat -Risk factors: advanced age, obesity, fhx, pregnancy, sedentary lifestyle, previous LE trauma & venous thrombosis Treatment: -Leg elevation, exercise, compression stockings, avoidance of prolonged standing Diagnosis: -Venous duplex US (identification of venous reflux--retrograde venous flow) Pathophysiology: -Increased intraluminal pressure or loss of vessel wall tensile strength -> venous dilation & incompetence of venous valves -> venous HTN in deep venous system of legs -Increased venous pressure + vascular permeability -> extravasation of fluid, plasma proteins, RBCs -> deposition of hemosiderin -> red/brown/woody discoloration -Microvascular disease + platelet aggregation + increased expression of proteolytic enzymes -> chronic irregular ulcers

Tension Pneumothorax

Presentation: -Life-threatening, often due to trauma or mechanical ventilation -Same findings as spontaneous PTX, w/ additional findings of hemodynamic instability (hypotension, hypoxemia, distended neck veins), tracheal deviation away from affected side, absent breath sounds Diagnosis: -CXR w/ same as spontaneous PTX + contralateral mediastinal shift, ipsilateral hemidiaphragm flattening -Visceral pleural line w/ no lung parenchymal markings beyond Treatment/management: -Emergent needle decompression (needle thoracostomy) or chest tube placement (tube thoracostomy) @ midclavicular 5th intercostal space to prevent CV collapse -Decompression followed by intubation (never before compression; positive pressure ventilation can worsen PTX) Pathophysiology: -High intrapleural/intrathoracic pressure -> compression of vena cava & other mediastinal structures -> decreased venous return, impaired RV filling, decreased cardiac output -> severe distress w/ hypotension & hypoxemia -One-way valve forms in penetrating trauma--air flows into pleural space during inspiration but is trapped during expiration

Acromegaly

Presentation: -Local tumor effects: HA, visual field defects, CN defects -MSK: gigantism, frontal bossing, malocclusion of jaw, macrognathia (jaw widening, increased interdental spaces), arthritis, carpal tunnel syndrome, enlargement of hands/feet (widened joint spaces on X-ray), kyphoscoliosis -Skin: thickening, hyperhidrosis (odor), skin tags -CV: cardiomyopathy (concentric hypertrophy, asymmetrical septal hypertrophy), HTN, HF, arrhythmias -Pulm: sleep apnea -GI: colon polyps/cancer, diverticulosis -Endocrine: galactorrhea, hypogonadism, T2DM (polyuria), hypertriglyceridemia -Enlarged tongue, thyroid, salivary glands, liver, spleen, kidneys, prostate -Increased risk of malignancy (esp colon, thyroid, breast) Diagnosis: -IGF-1 levels (elevated), oral glucose test showing inadequate GH suppression -MRI to identify pituitary source, if none identified evaluate for extrapituitary sources (e.g. ectopic GH- or GHRH-secreting tumors) Treatment: -Transphenoidal resection of adenoma -If tumor unresectable, medical treatment w/ somatostatin analogues (e.g. octreotide), GH receptor antagonists (e.g. pegvisomant) Pathophysiology: -Overproduction of GH, usually by pituitary somatotroph adenoma -> overgrowth of tissues by direct effects and indirect through IGF-1 -Growth of adenoma causes decreased secretion of other pituitary hormones (e.g. gonadotropins -> erectile dysfxn, low testosterone) -Joint space widening due to hyperplasia of articular chondrocytes & synovial hypertrophy -> eventually leads to degenerative arthritis that looks similar to OA

Breast Abscess

Presentation: -Localized collection of pus in the breast tissue, area of fluctuance w/ surrounding erythema, possible expression of purulent nipple discharge, unilateral breast pain -Fever, erythema that improve w/ abx -Axillary lymphadenopathy -Risk factors: first pregnancy, tobacco use, maternal age >30 Diagnosis: -US Treatment: -Abx against MSSA (e.g. dicloxacillin, cephalexin, vancomycin), possible incision/aspiration & drainage -Continued breastfeeding to facilitate breast drainage Pathophysiology: -Often develop in setting of untreated mastitis (due to incomplete breast emptying, milk stasis) -Most commonly due to S. aureus, enters breast through nipple trauma, e.g. chafing/blistering

Mallory-Weiss Syndrome

Presentation: -Longitudinal esophageal/gastric tear -> hematemesis (bright red or coffee ground), epigastric/back pain in the setting of suddenly increased intraabd pressure (vomiting w/ retching, trauma, hiccuping, Valsalva, heavy lifting, straining to stool), melena (black/tarry stools), possible hypovolemia -Risk factors: alcoholism, hiatal hernia Diagnosis: -Upper GI endoscopy can confirm diagnosis (reveals longitudinal laceration), can be used to treat bleeding Treatment: -Acid suppression w/ PPI, most heal spontaneously -Endoscopic therapy (electrocoag/sclerotherapy, local epi injection, ligation) for persistent bleeding Pathophysiology: -Forceful retching -> mucosal tear near GEJ -> submucosal venous/arterial plexus bleeding

Aspirin/Salicylate Toxicity

Presentation: -Looks like sepsis or acute abdomen, hyperthermia, AMS, tachypnea (metabolic acidosis), tachycardia, abdominal pain, nausea, vomiting, confusion, tinnitus, dizziness, can cause arrhythmias -Noncardiogenic pulmonary edema (increased pulm vascular permeability) -Labs: paradoxically normal pH, low bicarb indicating metabolic acidosis, anion gap, lactic acidosis Diagnosis: -Obtain salicylate serum levels, ECG Treatment: -Alkalinize urine with IV sodium bicarbonate (requires large volumes of fluid to be administered); keeps salicylic acid in charged form (uncharged form can freely pass through BBB & renal tubules, leading to toxicity) -Supplemental glucose (D5W) ot prevent neuroglycopenia -Gastric decontamination w/ activated charcoal (within 2 hrs of acute ingestion, not helpful in chronic ingestion, avoid in confused/obtunded pts d/t risk of aspiration) -Hemodialysis to remove from blood if severe (if unable to tolerate IV bicarb d/t pulm edema, acute/chronic renal failure, persistent acidosis despite bicarb therapy, sx of shock, seizures, CNS dysfxn, cerebral edema) Pathophysiology: -Causes simultaneous primary anion gap metabolic acidosis + primary respiratory alkalosis, so initial pH is often normal (respiratory alkalosis is independent, not compensation for metabolic acidosis, does not meet requirements for appropriate compensation per Winter's formula) -Salicylates stimulate medullary respiratory center -> increased RR & respiratory alkalosis -Salicylates stimulate chemoreceptor trigger zone -> N/V -Cerebral tissue injury, neuroglycopenia -> cerebral edema, AMS

Anterior Cord Syndrome

Presentation: -Loss of anterior tract fxn (spinothalamic, corticospinal): bilat loss of motor control, pain, temp, crude touch sensation (e.g. pinprick testing) affecting both UEs & LEs -Flaccid paralysis initially, UMN signs may present days to wks later -Preservation of posterior tract fxn (intact dorsal columns w/ preserved vibration/proprioception/light touch) -Urinary retention/incontinence from disruption of autonomic tracts Treatment/Management: -Urinary catheterization to prevent bladder distention/injury Pathophysiology: -Often associated w/ posterior displacement of bone fragments in spinal cord trauma or anterior spinal artery injury (e.g. disc retropulsion) -May be associated w/ spinal cord infarction (complication of thoracic aortic aneurysm repair due to disruption of blood flow w/ aortic cross-clamping, can disrupt anterior spinal arterial flow)

Syringomyelia

Presentation: -Loss of pain and temperature sensation in cape-like distribution over shoulders, back, arms -Stiffness, paresthesias in UEs & neck -May be associated w/ hand weakness & flaccid paralysis (compression of ventral LMNs), UE muscle atrophy -Touch, vibration, proprioception usually unaffected if only involves ventral white commissure -May be associated w/ Chiari I malformation or trauma (mos-yrs after initial insult), sequela of meningitis/inflammatory disorders/tumors -If expansion of syrinx continues, can lead to central cord syndrome Diagnosis: -MRI w/ intramedullary fluid cavity in cervical/thoracic spine Treatment: -Surgical intervention (usually shunt placement) Pathophysiology: -Fluid-filled cavity in spinal cord due to disruption of CSF drainage from central canal -Disruption of crossing spinothalamic tracts -> loss of pain & temp sensation

Sensory Ataxia

Presentation: -Loss of proprioception -> wide-based, high stepping gait -Positive Romberg (swaying w/ eyes closed) Pathophysiology: -Seen w/ lesions involving peripheral nerves, dorsal roots, and/or posterior columns

Obstructive Sleep Apnea (OSA)

Presentation: -Loud snoring w/ periods of apnea/choking/gagging (often reported by partner) -Daytime somnolence (e.g. increased risk of MVCs d/t falling asleep while driving), nonrestorative sleep w/ frequent awakenings, morning headaches, affective/cognitive sx (e.g. depression, poor judgment), impotence, tongue scalloping -Complications: systemic HTN, pulm HTN -> R-sided HF (cor pulmonale), coronary heart disease, arrhythmias (e.g. AFib) -Risk factors: obesity, increased neck girth (>17 in), M>F, age >50, HTN Diagnosis: -Overnight polysomnography Treatment: -Nighttime continuous CPAP Pathophysiology: -Relaxation of pharyngeal muscles -> closure of airway, nocturnal hypoventilation -> hypercapnia, hypoxia -> detection by chemoreceptors in carotid body & brainstem -> arousal, increased pharyngeal tone, strong catecholamine response -> frequent nocturnal awakening, sympathetic hyperactivity -> secondary HTN

Physiologic Tremor

Presentation: -Low-amplitude & high frequency (10-12 Hz), not visible under normal conditions -Acute onset w/ increased sympathetic activity (e.g. caffeine, drugs/withdrawal, hyperthyroidism, anxiety, toxic/metabolic derangements) -Usually worse w/ movement, can involve face & extremities -Medication-enhanced tremor may be suspected w/ temporal relationship to initiation of med, dose-response relationship, lack of progression of tremor, & overall symmetry of affected limbs -Postural tremor (e.g. w/ holding out hands, not seen at rest) Pathophysiology: -Enhanced physiologic tremor may occur w/ meds (beta-adrenergic agonists, SSRIs, TCAs, nicotine, caffeine, corticosteroids), physiologic conditions (stress, anxiety), & medical conditions (hypoglycemia, EtOH/opioid withdrawal, thyrotoxicosis, liver disease)

Allergic Bronchopulmonary Aspergillosis

Presentation: -Low-grade fever, cough productive of brownish mucus plugs, occasional hemoptysis, thick/mucinous sputum in context of underlying asthma, bronchiectasis (e.g. CF) -Recurrent asthma exacerbations -Imaging w/ findings of bronchiectasis & mucus plugging; CXR w/ transient infiltrates, CT w/ central bronchiectasis -Labs: elevated IgE Diagnosis: -Skin test positive for Aspergillus fumigatus -Eosinophilia >500, IgE >417 -Specific IgG & IgE for A. fumigatus Treatment/Management: -Glucocorticoids & itraconazole or voriconazole to control inflammation & prevent irreversible damage -Other options: omalizumab (esp in poorly controlled asthma) Pathophysiology: -Hypersensitivity disorder occurring in pts w/ asthma or CF involving noninvasive colonization of airways by Aspergillus

Lumbosacral Strain

Presentation: -Lumbar back pain after inciting event (e.g. heavy lifting), may radiate into buttocks, hips, thighs above knee (achy pain vs. shooting/burning pain in radiculopathy) -Increased pain w/ movement, better w/ rest -Pain in paraspinal region, no tenderness to palpation of vertebrae (vs. compression fx) -No neuro deficits, negative straight leg test -Risk factors: obesity, spinal deformity/degeneration (e.g. scoliosis, DDD), muscle weakness/deconditioning Treatment/Management: -Moderate activity/early mobilization, heat, NSAIDs, nonbenzo muscle relaxants, spinal manipulation Pathophysiology: -Strain of paraspinal muscles, tendons, intervertebral ligaments -May be d/t sudden/unbalanced muscle contraction (e.g. during heavy lifting, twisting movements)

Refeeding Syndrome

Presentation: -Malnourished patient (e.g. anorexia nervosa, advanced malignancy) receives nutrition -> cardiac arrhythmias (e.g. VTach), congestive HF w/ pulmonary/peripheral edema (& JVD), tremor, seizures, Wernicke encephalopathy & other neuro signs (paresthesias, seizures, hyporeflexia), MSK weakness & rhabdo, diarrhea, hemolysis -Tachycardia, tachypnea -Labs: hypokalemia, hypophosphatemia, hypomagnesemia, elevated transaminases -Risk factors: alcoholism, chronic malnutrition Diagnosis: -Hypophosphatemia Treatment: -Close monitoring & repletion of electrolytes, thiamine prior to starting feeds -Oral phosphate replacement preferred (IV can lead to hypocalcemia, acute renal injury, arrhythmias) Prevention: -Careful correction of serum electrolytes before sudden refeeding Pathophysiology: -Starvation: catabolic state w/ low insulin, high cortisol/glucagon -> decreased ketone body use by muscle, increased by brain -> increased glycogenolysis, lipolysis, protein catabolism -> depletion of fat, vitamins, minerals, protein, intracellular electrolytes -Refeeding: anabolic state w/ high insulin -> glycogen/protein synthesis, increased intracellular uptake of phosphorus, K+, Mg, thiamine -> further exacerbation of low serum phosphorus (in particular), K+, Mg, thiamine, increased sodium & water retention -> arrhythmias (low K+, Mg), HF, etc.-Increased phosphate uptake due to increased phosphate utilization during glycolysis (formation of ATP, 2,3-DPG, etc.) -In alcohol use disorder, phosphate chronically depleted d/t decreased vit D & phos intake, decreased intestinal phos uptake from chronic diarrhea, increased urinary phos excretion d/t secondary hyperparathyroidism from decreased vit D intake & proximal tubular reabsorption defect from EtOH use

Malignant Hypertension

Presentation: -Markedly elevated BP w/ end-organ damage, e.g. to eye (retinal hemorrhage, papilledema), kidney (nephrosclerosis), CNS (hemorrhage, encephalopathy)

Hypokalemic Hypochloremic Metabolic Alkalosis

Presentation: -May be associated w/ protracted vomiting, gastric suctioning, loop or thiazide diuretic use -Signs of volume depletion (dry mucous membranes, tachycardia) Treatment/Management: -Remove/treat initiating factor -Cl- repletion w/ NS corrects alkalosis Pathophysiology: -Gastric/renal H+ loss -> excess serum bicarb -Hypovolemia -> RAAS activation -> Na+ retention, K+/H+ excretion -> hypokalemia, low urine sodium, worsening of metabolic alkalosis -Relatively greater loss of Cl- to Na+ -> hypochloremia -Cl- depletion -> impairment of bicarb excretion at pendrin exchanger -> perpetuates metabolic alkalosis

Uncal Herniation

Presentation: -May be associated w/ rapidly expanding epidural hematoma (pt becomes unresponsive after lucid interval) or other pathology causing rapid increase in ICP (Cushing triad w/ HTN, bradypnea, bradycardia) -Compression of ipsilateral CN III -> ipsilateral, fixed, dilated pupil -> later progresses to oculomotor muscle involvement w/ ptosis, down-and-out deviation of ipsilateral eye -Compression of ipsilateral cerebral peduncle of midbrain -> damage to descending corticospinal tracts -> contralateral hemiparesis/weakness -Compression of ipsilateral posterior cerebral artery -> contralateral homonymous hemianopia w/ macular sparing -Kernohan phenomenon (false localizing sign): late worsening herniation leads to midbrain compression toward contralateral side -> compression of contralateral peduncle -> additional ipsilateral hemiparesis Treatment/Management: -Intubation Pathophysiology: -Compression of temporal lobe -> herniation of most medial portion of temporal lobe (uncus) through tentorial notch under tentorium cerebelli

Carbon Monoxide Poisoning

Presentation: -May be associated w/ smoke inhalation, defective heating systems, gas motors operating in poorly ventilated areas, motor/mobile/older homes -Suspect w/ several symptomatic individuals in shared environment -Mild/moderate poisoning presents w/ HA, confusion, malaise, dizziness, N/V, dyspnea (d/t cerebral hypoxia) -Severe poisoning presents w/ seizures, syncope, coma, MI, arrhythmias, HF -"Bright cherry lips" -MRI may show bilat hyperintensity of globus pallidus (highly sensitive to anoxic conditions, d/t hemorrhagic infarction) -Vital signs often unremarkable -Labs: anion gap metabolic acidosis, elevated lactate, polycythemia if chronic Diagnosis: -ABG w/ carboxyhemoglobin levels/cooximetry >3% in nonsmokers or >10% in smokers (pulsox usually normal b/c oximeter cannot distinguish b/t carboxyhemoglobin & oxyhemoglobin) -ECG +/- cardiac enzymes Treatment: -High-flow 100% O2 via non-rebreather mask -Intubation w/ hyperbaric O2 if severe/unresponsive Pathophysiology: -CO binds w/ >200x higher affinity than O2 to Hgb (forms carboxyhemoglobin) -> reduction in O2 carrying capacity -CO causes left shift in Hgb dissociation curve -> decreased O2 unloading in peripheral tissues -> hypoxia -> increased EPO production -> secondary polycythemia -CO also disrupts oxidative phosphorylation in mitochondria

Spinal Cord Compression

Presentation: -May be associated w/ spinal injury (e.g. MVC, disc herniation), malignancy (e.g. lung, breast, prostate, MM), infection (e.g. epidural abscess) -Gradually worsening, severe local back pain worse in recumbent position (at night), most common in thoracic (60%) & lumbosacral (30%) spine, often preceding onset of neuro sx by 6-8 wks -Early signs: symmetric LE weakness, hypoactive/absent DTRs -Late signs: bilateral Babinski, decreased rectal sphincter tone (corticospinal involvement), urinary retention/bowel dysfxn, parapareses/paraplegia w/ increased DTRs, sensory loss 2+ levels below level of injury (spinothalamic involvement) Treatment/Management: -Emergent spinal MRI -Urinary catheter -IV glucocorticoids (decrease vasogenic edema -> decreased spinal pressure, relieves pain & can restore neuro fxn, esp in malignant cases) vs. abx -Neurosurgery +/- radiation oncology consultation for decompression, possible radiotherapy if highly radiosensitive tumor, stable spine, minimal neuro sx

Autoimmune Hepatitis

Presentation: -May be asymptomatic or present w/ nonspecific sx (e.g. fatigue, lethargy, malaise, abd pain, nausea, pruritus) -Hepatocellular pattern of injury (elevated transaminases, mildly elevated/normal alk phos) that can be asymptomatic or progress to hepatosplenomegaly, cirrhosis, liver failure -Other autoimmune sx: subacute, symmetric polyarthritis of small joints, arthralgias, erythema nodosum, thyroiditis, pleurisy, pericarditis, anemia, sicca syndrome -Risk factors: other autoimmune conditions (e.g. T1DM, thyroid disease, RA, hemolytic anemias, ITP, celiac), female Diagnosis: -Liver studies w/ markedly elevated transaminases (hundreds to thousands) -Serologies: ANA, anti-smooth muscle antibody, LKM-1, LC-1 -Hypergammaglobulinemia (increased serum globulin or IgG) -Liver bx (shows piecemeal necrosis) -Rule out other causes (e.g. viral hepatitis) Treatment: -Oral glucocorticoids Pathophysiology: -Autoimmune, progressive parenchymal liver damage

Osteomalacia

Presentation: -May be asymptomatic, may present w/ bone pain & muscle weakness, muscle cramps, difficulty walking, waddling gait, pathologic fxs -Risk factors: malabsorption (e.g. IBD), intestinal bypass surgery, celiac disease, chronic liver disease, CKD Diagnosis: -Elevated alk phos & PTH -Low serum Ca, (very low) phos, 25 OH-D (decreased total body stores) -Low urinary Ca -X-rays w/ cortical thinning & reduced bone density -Bilateral & symmetric pseudofxs (Looser zones, cortical infarction/insufficiency fxs traversing part of the bone, transverse radiolucencies w/ sclerotic margins), loss of trabeculae, concave vertebral bodies (codfish vertebrae) are characteristic Pathophysiology: -Reduced mineralization of osteoid at bone-forming sites, common in malabsorptive disorders due to chronic vit D deficiency -> decreased phos & Ca absorption -> increased PTH secretion (secondary hyperparathyroidism) -> increased bone resorption & renal Ca reabsorption, increased phos excretion -> worsening hypophosphatemia

Lead Poisoning

Presentation: -May be asymptomatic, present w/ abd pain/constipation/anorexia, cognitive impairment (e.g. memory loss), behavioral problems (e.g. fatigue, irritability, insomnia), encephalopathy, microcytic anemia, peripheral neuropathy (e.g. weakness, decreased sensation in stocking-glove distribution), HA, ataxia, psychosis, HTN -If acute poisoning, may present w/ N/V, seizures -Risk factors: home built before 1978 (esp w/ paint/dust released during renovation), pica or mouthing behaviors (e.g. infants, developmental delay), low socioeconomic status, immigrant or international adoptee, lead piping, occupation that works w/ batteries or pottery, home EtOH distillation -Labs: nephrotoxicity (elevated Cr, BUN), hyperuricemia (d/t impaired purine metabolism) Diagnosis: -Obtain venous sample (if initial screening performed by capillary sample, which has high rate of false +s, must be confirmed) -Microcytic (sideroblastic) anemia, basophilic stippling on smear, elevated serum zinc protoporphyrin level -X-ray fluorescence testing used to measure bone lead concentration Treatment/management: -Environmental surveillance (identify & remove lead sources) -Notify public health department -Nutritional counseling -Chelation therapy if lead levels 45+ (dimercaptosuccinic acid aka succimer if 45-69, dimercaprol + calcium disodium EDTA for emergent levels 70+ or encephalopathy) Pathophysiology: -Exposure primarily by inhalation, ingestion of lead particles from environment, some absorption through skin -Stored predominantly in bones, released slowly to cause effects over decades -99% bound to erythrocytes, can disrupt heme synthesis

Allergic Contact Dermatitis

Presentation: -May be triggered by exposure to poison oak/ivy/sumac, nickel, rubber/latex, leather dyes, topical medications, skin care products, cleaning products, fragrances, etc. -Appears 4-96 hrs after exposure in previously sensitized pts, up to 3 wks after if not previously sensitized -Well-demarcated, erythematous lesions on exposed skin, often in shape of original exposure (e.g. belt/watch distribution for nickel) -Pruritic papules, vesicles/bullae w/ clear fluid on red/indurated plaques, chronic lichenification/fissuring over time Diagnosis: -Patch testing for persistent cases Treatment: -Avoidance of allergen, topical/systemic glucocorticoids (e.g. betamethasone, fluocinonide), topical tacrolimus in regions where glucocorticoids are contraindicated (e.g. face) Pathophysiology: -Type IV HS rxn -Poison ivy/oak/sumac: plant produces urushiol (highly allergenic resin)

Exercise-Induced Bronchoconstriction

Presentation: -May occur in isolation or w/ underlying asthma; presents w/ asthma-like respiratory sx during exercise -Decreased exercise tolerance, asthma sx appearing within 5-10 min of exercise & improving after 20 min of exercise -Risk factors: sports involving cold/dry atmospheres (e.g. ice hockey), aerobic endurance sports (e.g. soccer, running, swimming), asthma Diagnosis: -Supportive: empiric response to preexercise bronchodilator -Confirmatory: bronchoprovocation testing (e.g. spirometry before & after exercise) showing 15%+ decline in FEV1 -Resting spirometry usually normal Treatment/Management: -Improve control of underlying asthma (step-up therapy) -Premedicate before exercise: ICS-formoterol (10 min prior) preferred over SABA, LTRA 2 hrs prior -Daily ICS-beta agonist or LTRA may be needed for frequent, prolonged exercise Pathophysiology: -Hyperventilation -> incomplete heating & humidification of inspired air -> cooler, dry air triggers mast cell degranulation & bronchospasm -W/ continued exercise, depletion of preformed mediators & release of inhibitory prostaglandins give rise to refractory period of decreased bronchial reactivity (~4 hrs)

Right Bundle Branch Block (RBBB)

Presentation: -May occur in the setting of R heart strain d/t PE or pulm HTN, may also be benign -Overall nonspecific finding, does not necessarily warrant fibrinolytic therapy -ECG shows R-prime wave (M shape) in V1 and widened S (W shape) in V6 (WILLIAM MORROW mnemonic) Pathophysiology: -R side of heart is last to depolarize d/t conduction delay along R bundle -> V1 is last to show depolarization -> R prime wave in V1 (M shaped)

Subfalcine Hemorrhage

Presentation: -May occur w/ displacement by epidural hematoma -Presents w/ somnolence, contralateral lower extremity weakness (due to ipsilateral anterior cerebral artery compression) -As long is not presenting w/ other herniation syndromes (e.g. uncal, tonsillar), usually does not involve cerebellum or cranial nerves, so no pupillary involvement, respiratory changes, coma, etc. (consciousness preserved) Diagnosis: -CT Treatment: -Intubation -Lowering ICP, correction of cause of mass effect (e.g. hematoma evacuation) Pathophysiology: -Cingulate gyrus (medial gyrus of cerebral cortex just above corpus callosum) is pushed beneath falx cerebri, usually due to frontal, temporal, or parietal lesion (e.g. epidural hematoma)

Radiation-Induced Cardiotoxicity

Presentation: -May present as MI, restrictive CM w/ diastolic dysfxn, constrictive pericarditis, valvular abnormalities (mitral/aortic stenosis/regurg), conduction defects (e.g. sick sinus syndrome, heart block) following chest radiation therapy Pathophysiology: -Radiation -> diffuse fibrosis of myocardial interstitium & pericardial layers, conduction system cells, valve cusps/leaflets -Radiation -> intimal injury -> arterial narrowing involving ostial parts of coronary vessels

Community-Associated MRSA

Presentation: -May present as necrotizing secondary bacterial PNA, esp following influenza/viral respiratory infection in young pts -Rapidly progressive PNA that is often fatal, w/ high fever, productive cough w/ hemoptysis, leukopenia, multilobar cavitary infiltrates Treatment/Management: -Usually requires ICU admission w/ broad-spectrum empiric abx (vancomycin, linezolid)

Posterior Cruciate Ligament (PCL) Tear

Presentation: -Mechanism of injury usually posterior force on anterior aspect of proximal tibia with knee in flexed position (e.g. dashboard injury), fall forward on flexed knee with foot in plantar flexion -Pts usually do not complain of popping sensation, less likely to have unstable knee than ACL tear

Chagas Disease

Presentation: -Megacolon, megaesophagus, dilated CM (systolic & diastolic biventricular HF, may precipitate arrhythmias like heart block/VTach & mitral/tricuspid regurg, ventricular apical aneurysm, mural thrombosis w/ embolic complications) -Findings of RHF (JVD, ascites, edema) > LHF (e.g. dyspnea, crackles) -CXR w/ cardiomegaly -Endemic in South America -Rare acute disease can present w/ myocarditis w/ acute-onset HF & pulmonary edema -3 phases of infection: phase 1 w/ mild/nonspecific sx including fever/myalgias, stage 2 w/ serologic/parasitic e/o infection w/o s/s, stage 3 w/ CM & GI disease Pathophysiology: -Protozoan Trypanosoma cruzi, most common in Mexico, Central/South America -Fibrosis causes conduction abnormalities

Thrombotic Thrombocytopenic Purpura (TTP)

Presentation: -Microangiopathic hemolytic anemia, severe thrombocytopenia (<30) -> SOB, bleeding -Fever, (nonpalpable) purpura, petechiae, elevated Cr, neurologic findings (weakness, confusion, seizure, headache, coma, stroke), abd pain -Acute & rapidly progressive -Labs: normal coag studies vs. DIC (increased bleeding time, normal PT & PTT), helmet cells/schistocytes/triangle cells on smear c/w microangiopathic hemolysis, hemolytic findings (high LDH, low haptoglobin), renal insuff (elevated Cr & BUN) -Vs. HUS, more commonly associated w/ neuro findings & fever Diagnosis: -Peripheral smear, testing for ADAMTS13 Treatment: -Plasmapheresis (PEX)--remove recipient plasma & replace w/ donor plasma (to remove autoantibodies), mortality w/o is 90% -Glucocorticoids, rituximab Pathophysiology: -Consumptive thrombocytopenia caused by deficiency (due to acquired antibodies or hereditary cause) of a vWF cleaving metalloprotease (ADAMTS13) that triggers formation of numerous platelet thrombi within small vessels -Low ADAMTS13 -> uncleaved vWF multimers accumulate on endothelial walls -> platelet trapping & activation in areas of high shearing force -> small vessel thrombi -> platelet consumption, RBC damage, end-organ damage -Often idiopathic but may be triggered by infections (e.g. HIV), malignancy, meds

Anemia of Chronic Disease

Presentation: -Microcytic anemia, may be associated w/ malignancy, chronic infection, rheumatic disease, obesity, DM, CHF, etc. -Labs: elevated ferritin, slightly microcytic/normocytic anemia, low serum iron/iron-binding capacity/reticulocyte count, mildly decreased transferrin saturation, increased bone marrow iron, lower than expected EPO for degree of anemia Treatment/Management: -Treat underlying condition causing inflammation (e.g. infliximab in RA), iron supplementation -If unresponsive to above & low or inappropriately normal EPO, can consider EPO or darbepoietin treatment (if persistent Hgb <10, requires concomitant iron supp) -pRBCs are last resort Pathophysiology: -Increased inflammatory cytokines (e.g. hepcidin--small peptide produced by liver in response to inflammation or bacterial lipopolysaccharide) -Inhibition/destruction of ferroportin on reticuloendothelial enterocytes & macrophages by hepcidin -> decreased iron absorption, increased iron sequestration (inhibition of reticuloendothelial system, which scavenges senescent erythrocytes, provides 95% of daily iron) -Reduced circulating iron -> impaired erythropoiesis

Lacunar Stroke

Presentation: -Microinfarctions; due to hyaline arteriolosclerosis (hypertension, diabetes) -Neuro deficits over mins to hrs, may appear in stuttering fashion -May involve internal capsule, resulting in acute contralateral hemiparesis -Absence of cortical signs (aphasia, agnosia, hemineglect, apraxia, hemianopia), seizure, mental status changes/impaired consciousness (e.g. stupor, coma) -Common syndromes: pure motor hemiparesis (most common), pure sensory stroke, ataxic hemiparesis, dysarthria-clumsy hand syndrome -Risk factors: HTN, age, HLD (high LDL), smoking, T2DM Diagnosis: -Imaging w/ small (<15 mm) subcortical infarct or no findings Pathophysiology: -Chronic HTN -> lipohyalinosis (hypertensive arteriolar sclerosis) of small penetrating branches of major cerebral arteries (branches of ACA, MCA, basilar arteries), microatheroma formation & occlusion -Typically affects deep brain structures/subcortical white matter (basal ganglia, thalamus, internal capsule, corona radiata), pons

Mitral Valve Prolapse (MVP)

Presentation: -Mid-systolic non-ejection click (snapping of mitral chordae as valve cusps extend into atrium during systole) + mitral regurgitation murmur in young patient -Timing of click varies depending on LVEDV & therefore position; w/ increased venous return (e.g. squatting, supine leg raise), LVEDV is high & click is heard late in systole or disappears, while w/ decreased venous return (e.g. standing, valsalva), LVEDV is low & click is heard earllier -Risk factors: female, young age, connective tissue diseases Pathophysiology: -Myxomatous valve degeneration

Type 1 Heparin-Induced Thrombocytopenia

Presentation: -Mild thrombocytopenia (rarely plts <100), usually within 2 days of heparin initiation -Does not require intervention, no symptoms, resolves w/o cessation of heparin Pathophysiology: -Nonimmune-mediated, heparin-induced platelet aggregation/clumping

Alcohol Withdrawal Syndrome

Presentation: -Mild withdrawal (6-24 hrs): anxiety, insomnia, tremors, diaphoresis, palpitations, GI upset, intact orientation -Seizures (12-48 hrs): single or multiple generalized tonic-clonic, may occur in rapid succession -Alcoholic hallucinosis (12-48 hrs): visual/auditory/tactile (esp of insects/animals), intact orientation, stable vital signs -Delirium tremens (48-96 hrs): rapid-onset confusion, agitation, autonomic instability (fever, tachycardia, HTN), diaphoresis, hallucinations (may be combative) Treatment: -Benzos (lorazepam used in pts w/ possible liver disease d/t no active metabolites compared to longer-acting benzos like chlordiazepoxide) -Nutritional/electrolyte resuscitation (IV fluids, thiamine, folate) Pathophysiology: -Chronic EtOH -> insensitivity to GABA (excess GABA, downregulation of GABA receptors)-Sudden cessation of EtOH -> excess excitatory tone

Acute Hemolytic Transfusion Reaction

Presentation: -Minutes to hrs after blood transfusion, fever, chills, flank pain, hemoglobinuria (dark brown/pink urine) -Pain at IV site, hypotension -If severe, can cause DIC (e.g. oozing from IV site, prolonged PT & PTT), acute renal failure (oliguria, anuria, acute tubular necrosis) -Positive Coombs test, elevated LDH, schistocytes on blood smear, pink plasma (plasma free Hg 25+ mg/dL) Diagnosis: -Repeat type & crossmatch confirming mismatch Treatment: -Immediate cessation of transfusion, supportive care -Aggressive hydration w/ IV NS to achieve hemodynamic stability and prevent renal injury Pathophysiology: -ABO incompatibility (often clerical error) -Reaction to incompatible blood antigens -> intravascular hemolysis, complement fixation -> toxic hemoglobin buildup in kidneys -> acute renal failure, dark urine

Mitochondrial Encephalopathy with Lactic Acidosis and Stroke-Like Episodes (MELAS)

Presentation: -Mitochondrial myopathy presenting w/ acute neurological changes (e.g. hemiparesis), stroke-like episodes, seizures, muscle weakness, hearing loss, lactic acidosis -Maternally inherited/mitochondrial: condition passed from affected mother to both M&F offspring, only transmitted by females Diagnosis: -Muscle biopsy confirmation, labs (serum pyruvate/lactate, creatine kinase) Pathophysiology: -One of most common mitochondrial myopathies -Lactic acidosis is d/t mitochondrial dysfxn -Brain lesions d/t defective mitochondrial energy production

Drug-Induced Acne

Presentation: -Monomorphic papules/pustule w/ lack of comedones/cysts/nodules -Location & age of onset may be atypical for acne, commonly involves upper back/shoulders/upper arms -Common triggers: glucocorticoids (steroid-induced folliculitis/steroid acne), androgens, immunomodulators (e.g. azathioprine, EGFR inhibitors, cyclosporine), anticonvulsants (e.g. phenytoin), antipsychotics, antituberculoids (e.g. isoniazid) Treatment/Management: -Discontinue offending agent -Standard acne therapy unlikely to be effective

Acute HIV Infection

Presentation: -Mononucleosis-like syndrome (fever, arthralgias/myalgias, sore throat, diffuse nontender lymphadenopathy, weight loss, HA) -GI sx (diarrhea, nausea, cramps) -Mucocutaneous lesions (painful oral ulcerations, usually shallow/discrete w/ white base), generalized maculopapular rash (oval/pink/red macules lasting ~1 wk, usually involving palms/soles), seborrheic dermatitis (e.g. dandruff, scaly facial rash) -Typically presents 2-4 wks after exposure -Labs: leukopenia, thrombocytopenia Diagnosis: -HIV viral load (usually markedly elevated >100,000 copies) -HIV antibody testing may be negative (not yet seroconverted), CD4 count may be normal Treatment/Management: -Combination antiretroviral therapy, partner notification (consider secondary ppx) -Start early ppx w/ vaccinations against: HAV (esp MSM, IV drug use, liver disease), HBV (if no immunity), HPV (if 11-26), influenza (only inactivated form), meningococcus (all pts 11-18, living in close quarters, w/ asplenia or complement def), pneumococcus, Tdap (repeat if pregnant, Td every 10 yrs), varicella (if no immunity), hold off on live vaccines if CD4 <200 -TB screening (PPD often falsely negative, IFN gamma testing preferred)

Primary Biliary Cholangitis (PBC)

Presentation: -More common in women (~90%), often middle-aged (30-65), Northern European -Pruritis (bile salts), fatigue, anorexia, painful hepatomegaly (can lead to portal HTN, cirrhosis, HCC), jaundice, weight loss, cutaneous xanthomas & eyelid xanthelasmas, hyperpigmentation, steatorrhea, inflammatory arthritis -Often associated w/ other autoimmune disorders (e.g. thyroid disease) -Labs: cholestatic pattern of liver injury (elevated alk phos, mildly elevated transaminases & bili), severe hypercholesterolemia (esp HDL) -Complications: malabsorption, fat-soluble vitamin deficiencies, metabolic bone disease (osteoporosis, osteomalacia), hepatocellular carcinoma Diagnosis: -Antimitochondrial antibodies -Biopsy w/ florid duct lesion Treatment: -Ursodeoxycholic acid (delays progression, improves overall & transplant-free survival) -Liver transplant for advanced disease -Calcium & vit D supplements, bisphosphonates if osteoporosis Pathophysiology: -Antimitochondrial antibodies, non suppurative granulomatous destruction of small and medium INTRAhepatic bile ducts by T-lymphocytes, fibrosis in lobule starting from portal zones -Metabolic bone disease likely due to malabsorption of vitamin D

Atypical Acute Coronary Syndrome

Presentation: -More common in women, elderly, & pts w/ T2DM (w/ visceral neuropathy) -May have no overt CP, may have pain in other locations (e.g. neck) -Often present w/ dyspnea, nausea, vomiting, epigastric pain

Angiodysplasia

Presentation: -Most common >60 yo, presents w/ painless hematochezia or occult bleeding w/ iron def anemia -Can occur anywhere in colon, most commonly in R colon -Increased risk of bleeding/more common in pts w/ ESRD/CKD, aortic stenosis, von Willebrand disease Diagnosis: -Endoscopy (may be easily missed, shows small & cherry-red lesions) Treatment: -Cautery of symptomatic bleed on endoscopy Pathophysiology: -Abnormal dilated & tortuous intestinal blood vessels/AVMs/vascular ectasias that are thin-walled and lined by endothelium w/ or w/o small amt of smooth muscle -Thought to form due to recurrent, intermittent obstruction of distal venules in muscularis propria -> proximal arterial damage, vascular congestion, formation of dysplastic arteriovenous collaterals -Bleeding in distal duodenum, jejunum, or ileum, which are not well visualized on standard endoscopy -Aortic stenosis -> destruction of von Willebrand factor multimers as they pass through damaged valve -> increased risk of bleeding; may remit after valve replacement -ESRD -> uremia -> platelet dysfxn -All causes above associated w/ bleeding diathesis that leads to more frequent GI bleeding from malformed vessels

Hodgkin Lymphoma (HL)

Presentation: -Most common cancer occurring in late adolescence (15-35 yo), bimodal age distribution w/ 2nd peak >60 yo -Systemic B sx (prolonged fever, night sweats, weight loss), generalized pruritus -Painless, firm lymphadenopathy (esp supraclavicular, cervical), severe nodal/chest pain w/ EtOH consumption -Mediastinal mass -> dysphagia, dyspnea if large, nonproductive cough, retrosternal pain/heaviness -Risk factors: EBV infection (esp in immunosuppressed) -Labs: eosinophilia, lymphopenia, high LDH, normocytic anemia Diagnosis: -Excisional lymph node biopsy w/ Reed-Sternberg cells (large, multinucleated lymphocytes) -CXR to look for mediastinal mass, hilar/mediastinal lymphadenopathy -PET scan showing uptake in hilar/mediastinal lymph nodes Treatment: -Combination chemotherapy & radiotherapy -> 85% cure rate w/ treatment (but increased risk of solid organ malignancy, CV disease, pulm fibrosis/bronchiectasis, hypothyroidism) Pathophysiology: -Typically begins in single lymph nodes & spreads to continguous nodes via lymphatic & thoracic ducts -EtOH consumption -> vasodilation in lymph nodes -> capsular distention, pain -Eosinophilia b/c tumor secretes cytokines that generate eosinophils

Alzheimer Disease

Presentation: -Most common cause of dementia, usually >65 -Early, insidious, short-term anterograde memory loss, e.g. for recent events, distant memories preserved -Other early signs: visuospatial deficits (e.g. getting lost in one's own neighborhood), language difficulties (e.g. difficulty finding words), cognitive impairment w/ progressive decline, impairment in ADLs (e.g. preparing meals, cleaning) -Late signs: apraxia/dyspraxia (difficulty performing learned motor tasks), later personality changes (e.g. apathy, disinhibition, suspiciousness, agitation, social isolation), neuropsychiatric/psychotic features (hallucinations, delusions, esp w/ paranoid theme, wandering, restlessness, sundowning), lack of insight regarding deficits, urinary incontinence (due to severe cognitive dysfxn), noncognitive neurologic deficits (e.g. pyramidal & extrapyramidal motor signs, myoclonus, seizures), primitive reflexes (e.g. grasp, snout) -Usually associated w/ death within 10 yrs Diagnosis: -Imaging to rule out other causes may reveal temporal lobe atrophy (most prominent in hippocampi & surrounding medial temporal lobe in late stages) Treatment: -Donepezil/galantamine/rivastigmine (cholinesterase inhibitors), memantine (NMDA antagonist) -Vit E has some evidence of modest benefit in slowing disease progression in pts w/ mild to moderate AD -SSRIs for agitation/paranoia, antipsychotics for severe aggression/psychosis -Nonpharm interventions: structured routine, regular exercise, sleep hygiene Prevention: -Insufficient evidence to conclude that any dietary or vitamin supplements reduce risk Pathophysiology: -May involve degeneration of locus coeruleus (predominantly norepinephrine-producing neurons), impaired acetylcholine synthesis -Cerebral amyloid deposition

Herpes Simplex Encephalitis

Presentation: -Most common cause of fatal sporadic encephalitis -Acute onset (<1 wk) fever, HAs, seizures, AMS (confusion, agitation, bizarre/psychotic behavior), +/- focal neuro findings (e.g. hemiparesis, CN palsies, ataxia, exaggerated DTRs), anosmia, gustatory hallucinations -Behavioral syndromes: hypomania, Kluver-Bucy syndrome (hyperphagia, hypersexuality), amnesia -Classic signs of meningeal irritation (e.g. photophobia, nuchal rigidity) usually absent -Can progress to complete unresponsiveness or coma Diagnosis: -CSF w/ slightly increased protein, increased WBCs (lymphocytic pleocytosis), increased RBCs (hemorrhagic temporal destruction), normal glucose, HSV DNA on PCR, normal opening pressure on LP -Brain MRI (preferred over CT) w/ unilateral temporal lobe hemorrhage/edema/lesions w/ mass effect -Focal EEG w/ prominent/intermittent high amplitude slow waves over temporal/frontal lobes in >70-80% of cases Treatment: -IV acyclovir Pathophysiology: -Most commonly HSV-1 beyond neonatal period -May also be associated w/ EBV, varicella

Benign Paroxysmal Positional Vertigo (BPPV)

Presentation: -Most common cause of vertigo -Short vertigo episodes (usually <1 min), often elicited by changes in head position (+Dix-Hallpike maneuver), fatigable nystagmus inhibited by gaze fixation, preserved ambulation, no other CNS sx (e.g. HA, diplopia, dysarthria) Treatment: -Canalith repositioning maneuvers (e.g. Epley, series of maneuvers to move particles out of the posterior semicircular canal & into the utricle) Pathophysiology: -Crystalline/calcium debris (otolith movement, canaliths) in semicircular canals -> disruption of normal flow of endolymph -> contradictory signaling from corresponding canals on each sign, interpreted by brain as spinning sensation

Bicuspid Aortic Valve

Presentation: -Most common congenital defect seen in adults, can cause aortic stenosis from premature valvular calcification in 40s/50s -If early/uncomplicated, may present w/ ejection murmur & click at LLSB -Systolic murmur at RUSB, narrowed or paradoxical splitting of S2 due to delayed closure of aortic valve -Eventual aortic stenosis can present w/ exertional syncope & chest pain, pulsus parvus et tardus (slow-rising and low-amplitude pulse) -May occur as isolated lesion or in association w/ other congenital CV abnormalities including aortic coarctation, sub/supravalvular aortic stenosis, VSD, sinus of valsalva aneurysm -Epidemiology: affects 1% of population, M>F, present in 30% of pts with Turner syndrome, AD with incomplete penetrance or sporadic -Complications: infective endocarditis, severe regurgitation/stenosis, aortic root or ascending aortic dilation, aneurysm, dissection -CXR may show AV calcification, aortic enlargement, rib notching Treatment/Management: -Fup Echo every 1-2 yrs -Eventual balloon valvuloplasty if symptomatic or asymptomatic & planning to become pregnant or participate in competitive sports (criteria: aortic stenosis, no significant calcification or regurgitation, peak gradient >50 mm Hg) -Surgery (valve & ascending aorta replacement) w/ severe stenosis/regurgitation w/ sx of LV dysfxn Diagnosis: -Screening Echo for pt & FDRs

Gilbert Syndrome

Presentation: -Most common in males, most common inherited disorder of bilirubin glucuronidation -Pt w/ no apparent liver disease, mild unconjugated hyperbili (usually <3) provoked by classic triggers (e.g. stress including febrile infection, fasting, vigorous exercise, surgery, dehydration, menstruation), otherwise recurrent asymptomatic & mild jaundice (scleral icterus) -Indirect hyperbilirubinemia Diagnosis: -Unconjugated/indirect hyperbilirubinemia on repeat testing -Normal CBC, blood smear, reticulocyte count, AST, ALT, alk phos Treatment: -None; benign condition Pathophysiology: -AR or AD mutation in UGT1A1 gene -> decreased UDP-glucuronosyltransferase activity -> increased unconjugated bilirubin

Staphylococcal Scalded Skin Syndrome (SSSS)

Presentation: -Most common in newborns & infants <5, presents w/ fever, irritability, rash that begins w/ diffuse erythema -> flaccid blisters in flexural areas exposed to mechanical pressure (axilla, groin), generalized erythema & blisters over entire body -Perioral crusting, other mucosal surfaces usually spared, facial edema -Positive Nikolsky sign (epidermal shedding) -Prompt treatment due to risk of septic shock -Sx usually resolve in 1-2 wks, low mortality rate Diagnosis: -Blood culture Treatment: -Oxacillin, nafcillin, or vancomycin (antistaphylococcal abx) -Wound care Pathophysiology: -Exfoliative toxins disrupt keratinocyte adhesion in superficial epidermis -In neonates, source of infection is often umbilicus or circumcision site -Other sources include nasopharyngeal colonization, primary skin lesions (e.g. pustule) -Intact bullae usually sterile

Temporal Lobe Epilepsy

Presentation: -Most common type of recurrent seizure disorder -Usually presents as focal seizure w/ impaired awareness -Often have preceding auras -Common automatisms: hand/mouth movements (e.g. lip smacking, fumbling hand movements) -Usually 30-90s in duration, followed by postictal confusion Diagnosis: -EEG showing epileptiform discharges over temporal region

Basal Cell Carcinoma

Presentation: -Most common type of skin cancer -Nodular type: pearly, flesh or pink-colored or translucent papule or plaque w/ small central telangiectasias, +/- rolled borders, +/- central ulceration ("rodent ulcer") on sun-exposed regions, most commonly on head/neck & extremities -Superficial type: reddish/pink patch/macule/plaque or irritated area, sometimes w/ atrophic-appearing center & peripheral small, pearly papules -Morpheaform/infiltrative type: least common, presents w/ pale/scar-like indentations -Persistent open sore that bleeds, oozes, or crusts, or pale scar-like area w/ poorly defined borders -Slow growing, locally invasive, rarely metastatic -Low risk: <2 cm on trunk or extremities -High risk: any lesion on face/ears/neck/hands/feet (esp 1+ cm), any lesion 2+ cm including trunk/extremities, lesions w/ aggressive histology (e.g. infiltrative type) & poorly defined borders -Risk factors: sun/UV light exposure, fair skin, light hair/eye color, ionizing radiation, chronic arsenic exposure Diagnosis: -Shave, punch, or excisional bx Treatment: -Surgical excision w/ narrow margins (3-5 mm) -Mohs micrographic surgery for delicate/cosmetically sensitive areas like the face (sequential removal of thin skin layers w/ microscopic inspection to ensure margins are clear) -Electrodesiccation & curettage for low-risk LE lesions -Occasionally topical salicylic acid + 5-fluorouracil or imiquimod for low-risk lesions (excision preferred) Pathophysiology: -Nodular BCC most common subtype (~80%) -Rarely metastatic (<1% of cases) but can be locally invasive (nerves, bone, cartilage, orbit if eyelid -> blindness, exenteration) -Thought to originate from basal keratinocytes of hair follicles & interfollicular epidermis

Secondary Adrenal Insufficiency

Presentation: -Most commonly caused by chronic glucocorticoid therapy, sometimes by infiltrative disease of hypothalamus (e.g. sarcoidosis) -Less severe sx than Addison disease, usually euvolemic w/ minimal electrolyte disturbance, no skin hyperpigmentation -Labs: low cortisol, low ACTH, normal aldosterone, mild hyponatremia -Risk factors: glucocorticoid therapy for 3+ wks, cushingoid features (facial plethora, supraclavicular fat pad) Pathophysiology: -W/ abrupt glucocorticoid discontinuation, takes 6-12 mos for pituitary gland to normalize and increase ACTH secretion -Mild hyponatremia b/c cortisol def can increase ADH secretion -Differentiated from PAI by normal aldosterone levels (production primarily driven by angiotensin II, not ACTH)

Paget Disease of Bone

Presentation: -Most commonly presents w/ asymptomatic elevation in alk phos -Focal/asymmetric bowing, fxs, and arthritis of adjacent joints, bone pain, often asymptomatic -Bone pain & deformities: HA/hearing loss/frontal bossing/head enlargement/dizziness/CN palsies w/ skull involvement, spinal stenosis/radiculopathy/bone pain w/ spinal involvement, bowing deformities of large bones w/ increased fx risk -Labs: elevated alk phos (but normal other LFTs), elevated bone turnover markers (PINP, urine hydroxyproline, CTx, NTx), calcium/phosphorus usually normal (can be elevated w/ fx or immobilization) -Complications: increased risk giant cell tumor (usually benign), osteosarcoma Diagnosis: -X-rays w/ osteolytic or mixed lytic-sclerotic (osteolytic/osteoblastic) lesions & thickening of cortical/trabecular bone -Bone scan w/ focal increase in radiotracer uptake; more sensitive than X-ray & helpful to document extent & location of skeletal involvement -Histology w/ abnormal osteoclasts, disorganized mosaic pattern of lamellar bone Treatment: -Bisphosphonates (inhibit osteoclasts, suppress bone turnover), calcitonin for pts who cannot tolerate bisphosphonates -Calcitonin/bisphosphonates may slow progression of hearing loss but cannot reverse loss Pathophysiology: -Excessive, disordered bone formation -Increased bone turnover from osteoclast dysfxn -> bone breakdown + compensatory increase in disordered bone formation, defective osteoid formation, focal hypertrophy of skull, clavicles, pelvis, long bones -Hearing loss may result from temporal bone enlargement & encroachment on cochlea -Early-stage lesions begin w/ osteoclast-predominant phase -> mixed osteoclastic/osteoblastic phase -> predominant osteoblast phase -> residual sclerotic phase

Iodine-Induced Hyperthyroidsm

Presentation: -Most often develops in pts w/ preexisting nodular thyroid disease, esp in setting of dietary iodine deficiency -Sx of hyperthyroidism, thyrotoxicosis following trigger exposure -Common triggers: radiocontrast agents, amiodarone, topical antiseptics, kelp-based dietary supplements -No extrathyroidal manifestations of Graves disease -Labs: suppressed TSH, negative thyrotropin receptor antibodies, US w/ increased vascularity & possible nodules Treatment/Management: -Beta blockers for adrenergic sx control, antithyroid meds (e.g. methimazole) for prolonged hyperthyroidism (>1-2 mos), severe thyrotoxicosis, older pts w/ cardiac disease -Discontinue causative agent; usually self-limited if removed -No use of radioactive iodine ablaiton or iodine-containing solutions; unlike to be effective since gland is already saturated w/ iodine Pathophysiology: -Pts w/ nodular thyroid disease can develop areas of autonomously functioning thyroid tissue d/t chronic TSH stimulation; following iodine load, iodine can serve as substrate for increased thyroid hormone formation in autonomous tissue (Jod-Basedow phenomenon)

Parvovirus B19

Presentation: -Most pts asymptomatic or flu-like sx -In adults may present w/ acute, polyarticular, symmetric arthralgias/arthritis/synovitis of hands/feet/wrists/knees that may resemble RA w/ brief morning stiffness & joint swelling; self-limited & usually resolves in 1-2 mos -Can present w/ circumoral pallor, reticulated/lace-like rash -Can present as erythema infectiosum (fifth disease) more commonly in children w/ fever, nausea, "slapped-cheek" rash -Causes transient pure red cell aplasia, aplastic crisis in pts w/ underlying hematologic disease (eg. SCD) -Labs: normal ESR Diagnosis: -Acute infection: B19 IgM abs in immunocompetent pts, NAAT for B19 DNA in immunocompromised pts -Previous infection: B19 IgG ab (documents immunity) -Reactivation of previous infection: NAAT for B19 DNA Treatment: -Symptomatic, primarily w/ NSAIDs Pathophysiology: -Nonenveloped, ssDNA virus that primarily infects RBC precurosrs

Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Presentation: -Most pts asymptomatic until 30-40 yo, develop ESRD b/t 55-60 yo, ~50% require renal replacement therapy by 60 -Flank pain & fullness (bilateral), hematuria, (refractory) HTN, palpable abd masses (usually bilateral), CKD, nephrolithiasis common, UTIs -Polyuria/nocturia, polydipsia d/t urinary concentrating defect -May first present after cyst rupture, triggered by activities involving bending over/exertion -Extrarenal features: CEREBRAL ANEURYSMS, hepatic/pancreatic cysts, mitral valve prolapse, aortic regurgitation, colonic diverticulosis, ventral/inguinal hernias -Fhx in 75% of cases -Labs: elevated BUN & Cr, kidney disease progresses to ESRD over time (usually 55-60), low specific gravity on UA, elevated Hgb possible d/t increased EPO production Diagnosis: -US (or abd CT) showing multiple renal cysts -Genetic testing for PKD mutations if dx unclear by US Treatment/Management: -Aggressive control of risk factors for CV & CKD (e.g. statins) -ACEi preferred for treating HTN (target secondary hyperaldosteronism) -Hemodialysis, renal transplant for ESRD -Vasopressin receptor antagonists (e.g. tolvaptan) to slow progression in some pts -Family member screening w/ renal US Pathophysiology: -Due to AD mutations in PKD1 or PKD2 genes (polycystin proteins) -> cystic degeneration of renal parenchyma, impaired vascular integrity in many vessels -HTN is early disease manifestation resulting from cyst expansion -> localized renal ischemia -> increased renin secretion -> increased RAAS activation -> secondary hyperaldosteronism -> increased water/Na+ retention -> severe HTN -Begin developing urinary concentrating defect early in disease due to renal tubular destruction -> mild nephrogenic DI (insensitivity to ADH d/t tubular damage of receptors) w/ increased vasopressin levels (encourages renal cyst growth, thus use of tolvaptan)

Nonalcoholic Fatty Liver Disease (NAFLD)

Presentation: -Mostly asymptomatic, associated w/ metabolic syndrome (obesity, T2DM, HLD), may present w/ steatohepatitis (AST/ALT <1), hyperechoic texture of liver on US (hepatic fatty infiltration) -May present w/ hepatomegaly or mild RUQ pain -Defined as hepatic steatosis on imaging/biopsy in absence of significant EtOH use and w/ exclusion of other causes of fatty liver disease -Steatohepatitis develops as disease progresses, can lead to fibrosis & cirrhosis -Risk factors: T2DM, obesity, dyslipidemia, metabolic syndrome, HTN Diagnosis: -US, may be confirmed by liver bx (ranges from bland steatosis to inflammation w/ necrosis/steatohepatitis, fibrosis, cirrhosis) -Elevated AST and ALT (often 2-5x normal), ALT>AST (vs. EtOH), mildly elevated alk phos, normal bili/albumin Treatment: -Diet & exercise, consider bariatric surgery if BMI of 35+ Pathophysiology: -Hepatocellular injury in absence of viral hepatitis or autoimmune disease, d/t increased transport of free fatty acids from adipose tissue to liver or decreased clearance of FFA from liver (d/t decreased VLDL production) -Related to peripheral insulin resistance leading to increased peripheral lipolysis, triglyceride synthesis, hepatic uptake of fatty acids -Hepatic FFA -> increased oxidative stress, increased production of proinflammatory cytokines (e.g. TNF-alpha)

Gonococcal Proctitis

Presentation: -Mucopurulent anal discharge, occasional rectal bleeding, tenesmus, constipation, pruritus, rectal pain & fullness -Risk factors: multiple sexual partners, unprotected intercourse, h/o other gonococcal infection Diagnosis: -NAAT of rectal swab Treatment: -Ceftriaxone + doxycycline (to cover chlamydia) Pathophysiology: -Transmission via receptive anal intercourse or direct spread from vagina

Neurocysticercosis

Presentation: -Multifocal brain & muscle cysts, seizures, ICH (HA, vomiting, AMS, papilledema) -Prolonged incubation (mos to yrs) -Most common in Central/South America, sub-Sahara Africa, Asia Diagnosis: -CT/MRI w/ cysts (hypodense, w/ enhancement/edema, may be calcified) Treatment: -Seizure/intracranial HTN management (e.g. anti-epileptics) -Antiparasitic therapy (albendazole) -Corticosteroids Pathophysiology: -Fecal-oral transmission of tapeworm -GI infection acquired by eating contaminated pork w/ Taenia solium -> GI infection (malabsorption) -> pass eggs in feces, eggs hatch in small intestine -> invade bowel wall -> spread hematogenously to cause cysticercosis -Drinking contaminated water containing eggs -> neurocysticercosis affecting brain (seizures) & cerebral ventricular system (intracranial HTN, hydrocephalus) -Increased ICP due to obstruction of CSF outflow

Necrobiosis Lipoidica Diabeticorum (NLD)

Presentation: -Multifocal pretibial lesions w/ brownish hue, dilated blood vessels, & central epidermal atrophy -May precede the onset of DM Diagnosis: -If no known h/o DM, screen w/ HbA1c or fasting blood glucose

Generalized Convulsive Status Epilepticus

Presentation: -Multiple generalized convulsive seizures w/o interval recovery of consciousness OR single continuous seizure for 30+ min -MRI may show cortical hyperintensity on DWI c/w cortical infarction/necrosis -Risk factors: nonadherence to anti-epileptic drugs, structural brain abnormalities (e.g. tumor, stroke), metabolic abnormalities (e.g. hyponatremia, hypoglycemia), infection (e.g. meningitis), drug withdrawal (e.g. EtOH, benzos), other causes of decreased seizure threshold (e.g. head injury, sleep deprivation) Diagnosis: -5+ min of generalized convulsive seizure OR 2+ generalized convulsive seizures w/o interval recovery of consciousness Treatment: -ABCs -1st: Benzodiazepines (repeat administration of lorazepam until termination of seizure activity) -2nd: start antiepileptic drugs to prevent recurrence (e.g. levitarecetam, fosphenytoin, phenytoin, valproate) -Typically regain responsiveness within 10-20 min of starting above; lack of responsiveness may be due to sedation from benzos, ongoing seizure activity w/o physical manifestations (nonconvulsive status), use EEG to distinguish -EEG monitoring for refractory status or failure to regain consciousness; if still seizure activity, then may start continuous benzo infusion or use propofol, phenobarbital -After stabilization, should perform CT/MRI to evaluate for structural abnormality, hemorrhage, ischemia -If fail to regain consciousness after medical therapy, continuous EEG to rule out nonconvulsive status Pathophysiology: -Prolonged seizure activity can lead to neuronal cell death, esp >5 min (increased risk fo permanent injury d/t excitatory cytotoxicity) -Associated w/ cortical laminar necrosis, which can lead to persistent neuro deficits & recurrent seizures

Oral Hairy Leukoplakia

Presentation: -Multiple painless, white mucosal lesions w/ corrugated/folded/wrinkled texture on lateral surfaces of tongue that cannot be scraped off -Fatigue, weight loss, cervical adenopathy, other signs of HIV infection Diagnosis: -HIV testing Treatment: -HART if HIV confirmed Pathophysiology: -Complication of EBV, highly associated w/ HIV

Hyperphosphatemia

Presentation: -Muscle cramps, bone pain, pruritus

Rhabdomyolysis

Presentation: -Muscle pain & weakness, dark urine, decreased urine output -Labs: AKI (from myoglobin accumulation), possible eventual ATN (days later), elevated BUN & Cr, dark urine (myoglobinuria/pigmenturia), +blood on UA but no/few RBCs on microscopy b/c pigmentation is due to myoglobin, pigmented casts, elevated K+ & phosphate, hypocalcemia/hyponatremia, AST>ALT, metabolic acidosis Diagnosis: -Serum CPK/CK >1000 (often >10,000) -UA/microscopy concordance Treatment/Management: -Aggressive IV fluid resuscitation (w/ isotonic saline), sodium bicarb in some cases -Monitor for acute compartment syndrome Pathophysiology: -Skeletal muscle lysis/necrosis due to crush injury or prolonged immobilization (ischemic compression, may present w/ mottled skin over back/buttocks/posterior thighs), intense muscle activity (e.g. seizure, exertion, esp in heat), drug/medication toxicity (e.g. PCP, statins, EtOH, fibrates, cocaine, amphetamines, opioids, benzos, colchicine), toxin exposure, infection -Drug/med toxicity may be due to direct myotoxicity, vasoconstrictive ischemia (e.g. cocaine, amphetamines), prolonged immobilization (compression ischemia) -Muscle cell lysis -> intravascular fluid shift into damaged muscle tissue -> intravascular volume depletion -> kidney damage (+direct tubular toxicity of heme from myoglobin) -Colchicine inhibits microtubule function -> myocyte waste product accumulation -Hyperkalemia/hyperphosphatemia due to cell lysis, hypocalcemia due to calcium deposition in damaged muscle, AST>ALT due to higher concentration of AST in skeletal muscle

Polycythemia Vera

Presentation: -Myeloproliferative disorder w/ increased production of RBCs, WBCs, & platelets -Hepatosplenomegaly, avg age 60 yo -Often asymptomatic, may present w/ aquagenic pruritus (itching after warm bath/shower w/o rash), headaches, bleeding, transient neuro sx (e.g. sensory neuropathy, visual disturbances), erythromelagia (burning cyanosis of hands/feet), thrombotic complications (e.g. portal vein thrombosis), gout attacks (~40%) -Facial plethora (ruddy cyanosis) -Complications: thrombosis (e.g. Budd-Chiari), myelofibrosis, acute leukemia -Labs: thrombocytosis, leukocytosis, low serum iron Diagnosis: -Elevated Hct (>50%), low serum EPO -Testing for JAK2 mutation may be used as confirmatory test (present in almost 100% of pts w/ PV, absent in pts w/ secondary polycythemia, most accurate test) Treatment: -Phlebotomy -Hydroxyurea if increased risk of thrombus -Allopurinol to prevent gout attacks Pathophysiology: -May be associated w/ JAK2 mutations resulting in constitutive activation of JAK2 tyrosine kinase (usually activated by EPO; thus constitutive activation causes decreased EPO vs. secondary polycythemias) -Increased cell turnover d/t clonal hyperproliferation of all 3 primary bone marrow lineages

Posthypoxic Myoclonus (PHM)

Presentation: -Myoclonus following prolonged hypoxic event (e.g. cardiac arrest) -Myoclonus: sustained, involuntary muscle contraction/relaxation resulting in limb/joint movement -Myoclonus status epilepticus: acute form of PHM that develops within 24 hrs of initial hypoxic insult while pt is unconscious, characterized by generalized & often symmetric myoclonus involving axial/limb/facial muscles, often w/ intermittent eye opening, upward gaze deviation, & swallowing movements; if persistent is indicative of poor prognosis -Lance-Adams syndrome: chronic form of PHM that presents days to wks after insult once pt has regained consciousness, focal in nature & exacerbated by action, may involve negative myoclonus (relaxation), leading pts to drop objects or fall Diagnosis: -EEG Treatment: -Anti-epileptic agents (e.g. clonazepam, levetiracetam), supportive care

Medication Overuse Headache

Presentation: -Near-daily HA for 3+ mos in setting of regular use of acute HA medications, usually in pt w/ preexisting HA disorder (e.g. migraines), may cause underlying HA disorder to worsen -Sx may mimic migraine or tension-type HAs -Usually present upon awakening, sx relief from analgesics for only short periods, quick rebound necessitating further meds -Most common w/ butalbital-containing products, acetaminophen, & opioids Treatment/Management: -Cessation of culprit drug, possible concomitant initiation of preventive therapy Pathophysiology: -May involve genetic predisposition, central pain sensitization w/ alteration of neuronal excitability & receptor downregulation, behavioral disorders (e.g. substance dependence)

Carotid Stenosis

Presentation: -Neck bruit -Symptomatic: occurrence of TIA or stroke in distribution of affected artery in last 6 mos, generally involves 70-99%, high grade stenosis -If internal carotid stenosis, may be associated w/ transient cerebral/ocular ischemia (e.g. amaurosis fugax, limb weakness) -Asymptomatic: carotid atherosclerosis w/o recent TIA or stroke -Risk factors: HTN, smoking, T2DM, obesity Diagnosis: -Carotid duplex US Treatment/Management: -If symptomatic & high-grade stenosis (70-99%), carotid endarterectomy -If asymptomatic but high-grade stenosis (80-99%), carotid endarterectomy -If asymptomatic but lower-grade (<80%), medical treatment (antiplatelet like aspirin, statin, careful BP control, management of risk factors like smoking & hyperglycemia)

Glucagonoma

Presentation: -Necrolytic migratory erythema: erythematous, pruritic papules that coalesce to form large, indurated plaques w/ central clearing on face/groin/extremities; presenting feature in ~70% of pts -Weight loss, new-onset DM/hyperglycemia (usually mild, rarely requires insulin), GI sx (diarrhea, anorexia, abd pain), flushing -Increased risk of VTE events, neuropsych sx (depression, psychosis) -Labs: hyperglycemia, normochromic/normocytic anemia Diagnosis: -Markedly elevated plasma glucagon levels (>500) -Abd imaging (abd MRI or CT) to localize tumor & identify metastases Treatment/Management: -Surgical excision Pathophysiology: -Pancreatic neuroendocrine tumor characterized by unregulated release of glucagon -> increased amino acid oxidation, catabolism -> hyperglycemia -Often malignant w/ metastasis to liver -Other clinical manifestations (e.g. diarrhea, flushing) d/t secretion of other peptides including VIP, calcitonin, GLP1 -Anemia may be d/t anemia of chronic disease and/or direct effects of glucagon on erythropoiesis

IgA Nephropathy

Presentation: -Nephrotic syndrome (>3.5 g/24 hrs, edema, hypoalbuminemia, hyperlipidemia, urine lipids) or gross hematuria (often reucrrent) several days following URI or spontaneously -Flank pain, low-grade fever, HTN, may be asymptomatic -Most common in young adults Diagnosis: -UA w/ +protein, RBCs, RBC casts -Normal C3 & C4 (vs. complement low in PSGN), AKI w/ increased Cr Pathophysiology: -Flank pain common & thought to be d/t stretching of renal capsule -Serum complement levels nl b/c of weak complement-fixing activity of IgA

Pernicious Anemia

Presentation: -Neurologic signs of subacute combined degeneration: ataxia, loss of proprioceptive/vibratory sensations, severe spasticity, weakness, LEs>UEs -Glossitis -Can be associated w/ autoimmune metaplastic atrophic gastritis (AMAG) -Risk factors: other autoimmune disorders (e.g. Hashimoto, Graves, Addison) -Labs: macrocytic anemia Diagnosis: -Autoantibody testing (most commonly anti-IF & anti-parietal) -If anti-IF negative, can consider Schilling test (radio-labeled cobalamin) -On endoscopy, may see atrophy of gastric body/fundus w/ loss of rugae Pathophysiology: -Autoimmune destruction of parietal cells that secrete intrinsic factor (binds dietary B12, absorbed in terminal ileum) -> decreased production of IF, achlorhydria, atrophic gastritis -> B12 deficiency -Anti-intrinsic factor antibodies prevent B12 absorption -AMAG: caused by autoimmune aggression against gastric mucosa, mainly directed against oxyntic cells & IF

Pufferfish Poisoning

Presentation: -Neurologic sx including perioral tingling, incoordination, weakness

Left Bundle Branch Block (LBBB)

Presentation: -New LBBB often presents w/ ACS -ECG shows widened S (W shape) in V1 & R-prime wave (M shape) in V6 (WILLIAM MORROW mnemonic) Treatment/Management: -Coronary reperfusion Pathophysiology: -L side of heart is last to depolarize d/t conduction delay along R bundle -> V6 is last to show depolarization -> R prime wave in V6 (M shaped)

Aminoglycoside Ototoxicity

Presentation: -New onset hearing loss (bilateral but not necessarily symmetric), imbalance, sensation of objects moving (oscillopsia) w/ aminoglycoside abx -Positive head thrust test (inability to maintain visual fixation during forced, rapid head movement; test of vestibuloocular reflex, which is affected in peripheral but not central vestibulopathies) -Risk factors: increased risk w/ bacteremia, hepatic/renal dysfxn, combination w/ another potentially ototoxic med Diagnosis: -Formal audiogram screening Treatment: -Immediate discontinuation of aminoglycoside Pathophysiology: -Vestibular sx that predominate are imbalance/oscillopsia b/c of bilateral involvement of vestibular systems, vs. vertigo (usually occurs w/ unilateral involvement) -Aminoglycosides may damage hair cells of cochlea and/or vestibular system

Methemoglobinemia

Presentation: -New onset hypoxia by pulsox, perioral/digital cyanosis (when ~10% of total Hgb), sx of hypoxia (lethargy, HA, >20%), severe sx (AMS, seizures, resp depression, 50+%) -"Chocolate" colored blood -Risk factors: certain medications (topical anesthetics like benzocaine/lidocaine, dapsone, nitrates in infants) Diagnosis: -Pulse oximetry shows lower saturation than ABG (large oxygen saturation gap) Treatment: -Discontinue causative agent, administer methylene blue to reduce iron to original state Pathophysiology: -Iron component of hemoglobin becomes oxidized -> formation of methemoglobin -> cannot bind O2, higher affinity for O2 results in decreased O2 delivery to tissues

Bronchial Mucus Plug

Presentation: -New-onset hypoxia & SOB/respiratory distress w/o chest pain, cough, hemoptysis/dark-colored sputum -Dullness to percussion, decreased breath sounds on affected side, decreased tactile fremitus -Risk factors: surgery, smoking Diagnosis: -CXR showing pronounced atelectasis (e.g. entire lung if lodged in mainstem bronchus), mediastinal shifting towards affected side, narrowed rib spacing on affected side Treatment: -Chest physiotherapy often used for minor atelectasis -If large volume, may require bronchoscopy for removal Pathophysiology: -Large airway obstruction -> distal air trapping in alveoli -> air absorbed slowly into bloodstream, no additional air can enter -> eventual collapse of alveoli (atelectasis) -> creates vacuum that pulls mediastinal structures toward it (carina, heart)

Asymptomatic Bacteriuria

Presentation: -No fever, abdominal pain, hematuria, dysuria, suprapubic/flank tenderness, AMS, other sx of UTI -Common in elderly women (~20% of women >80) -Usually transient & resolves within 2 wks -Labs: pyuria, >100,000 CFUs in clean catch urine culture Treatment/Management: -Reassurance & observation -Treatment recommended only for pts that are pregnant, undergoing urologic procedures, or within 3 mos of kidney transplant

Brain Abscess

Presentation: -Nocturnal headaches not relieved by NSAIDs, worse at night due to increased ICP when supine -Morning vomiting due to cerebral edema & activation of area postrema, medullary vomiting center -Fever, focal neurologic deficits (50% of pts), seizures (25% of pts), AMS, neck pain -May be a complication of mastoiditis -Papilledema from increased ICP in late disease -Risk factors: otitis media, sinusitis, mastoiditis, dental infection, cyanotic heart disease (e.g. ToF), endocarditis Diagnosis: -Contrast-enhanced CT or MRI (MRI better for detecting early cerebritis) -Ring-enhancing lesion (thick & diffuse contrast enhancement indicating inflammatory capsule from BBB breakdown) w/ air-fluid levels, central necrosis, & surrounding edema -CT-guided aspiration or surgical bx w/ Gram stain & culture of tissue -NO LUMBAR PUNCTURE-can precipitate brain herniation Treatment: -Needle aspiration and/or surgical excision -Prolonged administration of parenteral abx (empiric vanc, metronidazole, ceftriaxone) Pathophysiology: -Most commonly d/t Strep viridans or S. aureus -Pts w/ cyanotic heart disease at higher risk due to hematogenous spread of bacteria (R->L shunting allows bacteria to bypass pulm circulation, where bacteria may be filtered out and trapped by macrophages)

Leukocytoclastic Vasculitis

Presentation: -Non-blanching, painful, raised, nonblanching 1-3 mm petechial or violaceous/purpuric papules that may cluster/coalesce into plaques -Older lesions tend to be brown/red, new lesions tend to be violaceous Pathophysiology: -May be associated w/ infection, malignancy, meds, inflammatory conditions -Most common cutaneous vasculitis, involves neutrophil-predominant inflammation of dermal vessels

Central Vertigo

Presentation: -Nystagmus may have any trajectory, is not inhibited by gaze fixation, is not fatigable (>1 min duration), no latency period -Hearing loss/tinnitus absent, often associated w/ severe postural instability -Other CNS sx usually present (e.g. HA, diplopia, weakness/numbness of face or limbs, dysarthria) Pathophysiology: -Etiologies are central (e.g. cerebellar stroke, MS, basilar migraine)

Obesity Hypoventilation Syndrome

Presentation: -Obesity w/ BMI of 30+, awake daytime hypercapnia (PaCO2 >45), no alternate cause of hypoventilation -SOB/dyspnea w/ exertion, fatigue, pulm HTN, often w/ coexistent OSA or cor pulmonale -Labs: polycythemia from chronic hypoxemia, respiratory acidosis w/ compensatory metabolic alkalosis (baseline bicarb elevated) -Complications: acute perioperative hypercapnic/hypoxic respiratory failure Diagnosis: -ABG (hypercapnia, normal A-a gradient), no intrinsic pulm disease on CXR, restrictive pattern on PFTs, normal TSH, polysomnography Treatment/Management: -Nocturnal PPV is first-line -Weight loss (bariatric surgery in select cases) -Avoidance of sedative meds -Respiratory stimulants (e.g. acetazolamide) as last resort Pathophysiology: -Obesity -> reduced chest wall & lung compliance -> decreased tidal volumes & total lung capacity, increased airway resistance -> requires increased ventilatory drive to maintain normocapnia, increased CO2 retention -> respiratory acidosis -> compensatory reabsorption of bicarb at kidney (at bicarb-chloride exchangers in intercalated cells) -> compensatory metabolic alkalosis, hypochloremia -Hypoxic vasoconstriction in lungs -> pulmonary HTN -> eventual cor pulmonale

Benign Prostatic Hyperplasia

Presentation: -Obstructive urinary sx: weak/slow/split urinary stream, intermittency, sensation of incomplete emptying, overflow incontinence, postvoid dribbling, hesitancy, straining to void -Storage/irritative urinary sx: urgency, frequency, nocturia, incontinence -Increased risk of recurrent UTI -Can present w/ acute urinary retention (inability to micturate, bladder distention, suprapubic pain), esp in setting of bladder/urethral infection, GU trauma, & use of certain meds (e.g. muscle relaxers, anticholinergics) -Nontender, nonindurated, smooth, uniformly enlarged prostate on digital exam -Risk factors: men >50 -Labs: PSA may be elevated (not as elevated as in prostate cancer) -Complications: bladder outlet obstruction, acute urinary retention Diagnosis: -UA to rule out other causes (e.g. UTI), PSA unless remaining life expectancy is <10 yrs Treatment: -If presenting w/ acute urinary retention, urethral/suprapubic catheterization -Alpha blockers (tamsulosin, terazosin): first-line, relax smooth muscle in bladder neck, prostate capsule, prostatic urethra; adverse effects include orthostatic hypotension/dizziness -5-alpha reductase inhibitors (finasteride, dutasteride): inhibit conversion of testosterone from dihydrotestosterone -> decreased prostate size, 6-12 mos to be effective; adverse effects include decreased libido & erectile dysfxn -Phosphodiesterase 5 inhibitors -Antimuscarinics (tolterodine) for overactive bladder sx (frequency, urgency, incontinence); adverse effects include urinary retention, dry mouth -If refractory: transurethral resection of prostate (TURP) procedure, radiofrequency ablation; may recur even after resection from tissue growing back Pathophysiology: -Hyperplasia of glandular tissue (usually central, transitional zone), no increased risk of prostate cancer (usually peripheral & nodular) -Enlargement occurs under influence of androgens -Periurethral prostatic enlargement impinges on urethra, prevents complete bladder evacuation -> increased risk UTI, bacterial ascent w/ urinary stasis

Silicosis

Presentation: -Occupational exposure (quarry work, stone cutting/blasting, glass/ceramic manufacturing), e.g. miners, sandblasters, foundry workers, masons -Slowly progressive dyspnea on exertion, pulm HTN, cor pulmonale 10-30 years after exposure -Clubbing, auscultatory findings of pulm fibrosis (crackles, squawks worse in upper lobes) -CXR w/ parenchymal nodules -CT w/ annular/eggshell calcifications of hilar/mediastinal nodes -If simple silicosis, shows small, nodular fibrotic opacities in upper lung zones, emphysema/hyperinflation in lower lobes -If progressive massive fibrosis (PMF), imaging w/ coalescent fibrotic masses w/ severe respiratory impairment -PFTs w/ restrictive pattern (decreased TLC) -Associated w/ RA (Calpan syndrome), scleroderma, mycobacterial disease, lung cancer, chronic bronchitis Treatment/Management: -Smoking cessation, pulm rehab, supp O2 -Lung transplant Prevention: -Respiratory protection (minimize exposure, NIOSH respirator) Pathophysiology: -Respired silica crystals -> inflammatory activation of alveolar macrophages -> maladaptive fibroinflammatory response -> in end stage, areas of scarring coalesce to cause PMF -Lymphatic injury -> mediastinal/hilar lymph node mineralization -> eggshell calcifications (annular densities outlining nodes) -Occupational interstitial lung disease w/ upper lobe involvement reflecting inhalational distribution

Psoriatic Arthritis

Presentation: -Occurs in 5-30% of pts w/ psoriasis -Often involves DIPs/PIPs, may be asymmetric oligoarthritis or symmetric polyarthritis (similar to RA, w/ morning stiffness >30 min, improves w/ activity), arthritis mutilans (deforming/destructive arthritis), spondylarthritides (sacroiliitis, spondylitis) -Soft tissues & nails: enthesitis (inflammation at site of tendon insertion into bone), dactylitis ("sausage digits" of fingers/toes), nail pitting/hyperkeratosis/splinter hemorrhages, onycholysis (separation of nail bed), swelling of hands/feet w/ pitting edema -Skin: arthritis precedes skin involvement in 15% of pts, may be present but not yet diagnosed in 15% of pts -Sx often episodic/cyclical rather than chronic/slowly progressive -Pencil-in-cup deformity: X-ray w/ narrowing of proximal phalanx & erosion of distal phalanx w/ new bone formation Treatment: -NSAIDs, methotrexate, anti-TNF agents

Exercise-Associated Hyponatremia

Presentation: -Occurs in individuals participating in prolonged exercise (e.g. marathon, triathlons) -Ranges from mild (lethargy, nausea) to severe (seizures, confusion) -Risk factors: female, low body weight, extended duration of exercise Pathophysiology: -Ingestion of large quantities of hypotonic fluid (e.g. water, sports drinks) combined w/ SIADH (results in inability to excrete dilute urine, d/t nonosmotic causes including exertion, pain, hypoglycemia, nausea)

Scleroderma Renal Crisis

Presentation: -Occurs in up to 20% of pts w/ diffuse cutaneous systemic sclerosis -Presents w/ sudden onset renal failure, malignant HTN (HA, blurry vision, retinal hemorrhage, N/V, encephalopathy), edema -Labs: may be c/w DIC, microangiopathic hemolytic anemia, thrombocytopenia, elevated serum Cr & BUN Diagnosis: -UA: mild proteinuria, may be normal -Peripheral blood smear: microangiopathic hemolytic anemia, DIC w/ schistocytes & thrombocytopenia Treatment: -ACEi (usually captopril) -Beta blockers avoided d/t risk of worsening Raynaud's & other vasospasm Pathophysiology: -Renal vascular injury (partially d/t collagen deposition) -> renal ischemia -> activation of RAAS -> increased BP -> further damage to renal vasculature -> continued cycle

Cytomegalovirus Esophagitis

Presentation: -Odynophagia, retrosternal CP, no dysphagia -On endoscopy, large & linear ulcers in distal esophagus -Risk factors: HIV (esp if CD4 <100), transplant Diagnosis: -Culture, bx w/ intranuclear/intracytoplasmic inclusions

Herpes Esophagitis

Presentation: -Odynophagia, retrosternal chest pain, fever -Most commonly in immunocompromised (HIV, organ transplant) -Often associated w/ concurrent herpes labialis or oral ulcerations -On endoscopy, vesicular or ulcerative round/ovoid "volcano-like" lesions Diagnosis: -Culture, bx w/ multinucleated giant cells, eosinophilic intranuclear inclusions, ballooning degeneration

Necrotizing Fasciitis

Presentation: -Often antecedent hx of minor trauma to skin -Most commonly affects perineal region and extremities -Presents w/ erythema of overlying skin, swelling, edema, pain out of proportion to exam findings, systemic sx (fever, hypotension) -Crepitus of skin in 50% of cases -Progresses over time to rapid discoloration of affected site, purulent discharge, bullae, necrosis -Risk factors: peripheral vascular disease (e.g. T2DM) Diagnosis: -Imaging (CT) w/ gas in soft tissue (should not delay treatment) Treatment: -Emergent surgical debridement (fasciotomy), broad spectrum abx -Abx: pip/taxo or carbapenem to cover GAS & anaerobes, vanc to cover S. aureus, clinda added to inhibit toxin formation by Strep/Staph -Narrow abx w/ culture results Pathophysiology: -Bacteria spread rapidly through subcutaneous tissue & deep fascia, undermining skin -> extensive tissue necrosis & shock -May involve group A strep (most common), S. aureus, Clostridium perfringens, polymicrobial -Type I: usually seen in pts w/ underlying DM & PVD, usually polymicrobial (S. aureus, Bacteroides fragilis, E. coli, GAS, Prevotella) -Type II: h/o laceration, blunt trauma, or surgical procedure, most commonly caused by GAS, no concurrent medical illness

Immune Thrombocytopenic Purpura (ITP)

Presentation: -Often asymptomatic -Petechiae in dependent areas, ecchymoses, purpura, usually following viral infection -Mucosal bleeding (e.g. epistaxis, hematuria, GI bleeding, menorrhagia), severe hemorrhage rare -Usually self-resolves within 3 mos, but if persists >1 yr is considered chronic ITP -In children, usually seen b/t 2-5 yo -Labs: normal coag tests Diagnosis: -Dx of exclusion (rule out drug-induced causes, liver disease, hypersplenism, microangiopathic disorders, bone marrow defects) -Isolated thrombocytopenia <100,000 -Few platelets (size normal to large) on smear, morphologically normal -For chronic ITP, workup w/ bone marrow examination, serum testing for infection (HIV, HCV, CMV) and autoimmune disease (SLE, thyroid disease) Treatment: -Children: observe if cutaneous sx only, otherwise treat w/ glucocorticoids, IVIg, or anti-D (if Rh+ and Coombs-) if bleeding -Adults: observe if cutaneous sx and platelets of 30,000+, otherwise treat w/ glucocorticoids, IVIg, platelet transfusion, and/or anti-D if bleeding or platelets <30,000 -If refractory to treatment, second line therapies include rituximab, thrombopoietin receptor agonists, splenectomy (may be curative) -Platelet transfusion reserved for life-threatening hemorrhage; may be rapidly cleared despite plt transfusions -May consider hormonal therapy (e.g. IV estrogen) if heavy menses Pathophysiology: -Platelet autoantibodies (IgG) against surface glycoproteins (e.g. GPIIb/IIIa)-> increased platelet clearance in spleen, inhibition of megakaryocytosis -> commonly acquired form of thrombocytopenia -May be primary or secondary--e.g. due to preceding viral infection or comorbidity (HIV, HCV, CLL, SLE) -Anti-D may be used to saturate Fc receptors on macrophages, reducing ability to clear platelets

Splenic Vein Thrombosis

Presentation: -Often asymptomatic -Splenomegaly, GI variceal hemorrhage, ascites -Usually occurs in setting of portal HTN from liver disease, chronic/acute pancreatitis, or pancreatic cancer -Chronic SVT can lead to left-sided portal HTN, ascites, congestive splenomegaly d/t isolated pressure increases in splenic portion of portal system -Labs: signs of hypersplenism/GI hemorrhage (anemia, thrombocytopenia) Diagnosis: -On endoscopy, isolated gastric varices -Confirmed by imaging (contrast-enhanced CT, doppler US, MRI) Treatment: -Splenectomy Pathophysiology: -Splenic vein runs along posterior surface of pancreas, can become damaged/compressed d/t pancreatic inflammation (e.g. pancreatic masses/pseudocysts) -Gastric varices form when venous blood is redirected from splenic vein to collateral gastroepiploic system & short gastric veins

Ocular Melanoma

Presentation: -Often asymptomatic for prolonged periods, may be diagnosed incidentally during fundoscopic exam -If presents w/ sx, usually presents as blurry vision or progressive/painless visual field abnormalities (floaters, occasional pain if tumor impinges on nerves) Diagnosis: -Eye US -MRI to document extrascleral extensions for staging/treatment decisions Treatment/Management: -If asymptomatic & small lesion w/ diameter <10 mm & thickness <3 mm, observation w/o treatment, repeat exam 3 mos after first noted & every 6 mos thereafter -If large (10+ mm diameter, 3+ mm thickness), radiotherapy (brachytherapy or external beam radiotherapy) -If very large, extrascleral extension, and/or severe pain, enucleation or radiotherapy

Chronic Lymphocytic Leukemia (CLL)

Presentation: -Often asymptomatic, B symptoms, prominent lymphadenopathy (cervical, supraclavicular, axillary), hepatosplenomegaly -Median survival of 10 yrs, median age of 70, worse prognosis if multiple chain lymphadenopathy, hepatosplenomegaly, thrombocytopenia/anemia -Labs: profound leukocytosis (often >100), mild thrombocytopenia/anemia ("bicytopenia") -Complications: infection, AIHA (warm agglutinin disease, +Coombs), secondary malignancies (e.g. Richter transformation) Diagnosis: -Peripheral smear w/ smudge cells, severe lymphocytosis -Flow cytometry (shows clonality of mature B cells) -Lymph node/bone marrow bx generally not needed Treatment: -Rituximab (anti-CD20) +/- fludarabine Pathophysiology: -Despite lymphocytosis, immune system abnormal w/ hypogammaglobulinemia & defects in cell signaling that increase risk/severity of infections

Large Local Reaction (LLR)

Presentation: -Often due to bites from Hymenoptera species (bees, ants, wasps) -Significant (~10+ cm) localized swelling, erythema, and warmth contiguous w/ site of sting -Develops within 24 hrs, peaks within 24-48 hrs, resolves in 5-10 days Treatment: -Cold compresses, oral/topical corticosteroids (e.g. topical clobetasol), antihistamines, anti-inflammatory meds Pathophysiology: -Exaggerated, IgE-mediated, local allergic response observed in ~10% of people -Associated w/ possible increased risk of future systemic reactions -Lesions contain fibrin, eosinophils, neutrophils, lymphocytes

Posterior Uveitis

Presentation: -Often painless, associated w/ floaters & reduced visual acuity -Often associated w/ systemic inflammatory disorders (e.g. IBD, ankylosing spondylitis), infection (e.g. CMV) Pathophysiology: -Choroidal inflammation

Acute Bronchitis

Presentation: -Often preceded by respiratory illness (>90% viral, sore throat, nasal congestion, etc.) -Cough for >5 days to 3 wks +/- yellow/purulent/blood-tinged sputum, absent systemic findings (fever, chills), wheezing or rhonchi/crackles that clear w/ cough (easily mobilized secretions, unlike PNA), chest wall tenderness Diagnosis: -Clinical, CXR only when PNA suspected -Symptomatic treatment (e.g. NSAIDs, bronchodilators) -Abx not recommended Pathophysiology: -Yellow/blood-tinged sputum due to epithelial sloughing & damage, inflammation

Delayed Hemolytic Transfusion Reaction

Presentation: -Onset >24 hrs to 1 mo after transfusion -Often asymptomatic evidence of hemolytic anemia -May present w/ fatigue, dyspnea, tachycardia, jaundice, low-grade fever -Labs: hemolytic anemia (high indirect bili & LDH & reticulocytes, low Hgb & haptoglobin, +Coombs (must be a new + test, esp in context of diseases like SCD) -Risk factors: hx repeat transfusions (e.g. SCD) Treatment: -Supportive (e.g. fluids) Prevention: -Review transfusion hx & prior antibody screens, transfuse when necessary w/ extended-antigen cross-matched blood Pathophysiology: -Due to alloimmunization from prior exposure to foreign RBC antigen (e.g. pregnancy, prior transfusion), usually a minor antigen that causes low & often undetectable antibody response after initial exposure -> anamnestic antibody response upon subsequent exposure from memory B cells rapidly producing more abs -> abs bind donor RBCs & cause extravascular hemolysis

Candida Esophagitis

Presentation: -Oral thrush usually present -On endoscopy, white plaques throughout esophagus -Predominant sx is dysphagia, odynophagia absent or mild Diagnosis: -Bx w/ pseudohyphae Treatment: -Fluconazole

Idiopathic Orthostatic Hypotension

Presentation: -Orthostatic hypotension w/o signs of parkinsonism, cerebellar involvement, etc. Pathophysiology: -Degeneration of postganglionic sympathetic neurons

POEMS Syndrome

Presentation: -POEMS: polyneuropathy, organomegaly, endocrinopathy, M-proteins, Skin changes Diagnosis: -SPEP

Ankylosing Spondylitis (AS)

Presentation: -Pain & progressive limitation of back movement, pain worse in morning/night/with rest & improves throughout day & w/ exercise, chronic/insidious onset -Most common in young men <40, peak incidence 20-30 yo, chronic sx >3 mos -On exam, arthritis (sacroiliitis), reduced chest expansion & spinal mobility (reduced forward flexioni on Schober test), enthesitis (tenderness @ tendon insertion sites, e.g. bilat heel pain, chronically can lead to fibrosis/calcification), dactylitis (swelling of fingers/toes), uveitis, spinal/SI tenderness to palpation & decreased spinal ROM -Complications: osteoporosis, vertebral fxs, cauda equina syndrome, restrictive lung disease (limited costovertebral join motion, apical pulm fibrosis), atlanto-axial subluxation -> spinal cord compression -Other associated conditions: acute anterior uveitis, cataracts, cystoid macular degen, aortic regurgitation (displaced PMI), MVP, varicocele, nonspecific ileal/colonic ulceration, IgA nephropathy, secondary amyloidosis (nephrotic syndrome) -Prognosis: no increase in mortality, no reduced life expectancy Diagnosis: -Elevated ESR & CRP, HLA-B27 association -X-ray/MRI of sacroiliac joints & spine (shows vertebral body squaring/fusion aka bamboo spine, widening of SI joint space, sclerosis of subchondral bone, erosions of ischial tuberosity & iliac crest) Treatment/Management: -Nonpharmacologic: exercise (postural, ROM/stretching/extension exercises, PT) -Initial pharmacologic: NSAIDs, COX-2 inhibitors, acetaminophen -Pharmacologic w/ treatment failure/progression: TNF-alpha inhibitors (etanercept, infliximab), anti-IL-17 abs (secukinumab) -Intraarticular glucocorticoids in some pts -Monitor for disease progression w/ X-rays of lumbar spine, cervical spine, & pelvis (possible addition of acute phase reactants like ESR) Pathophysiology: -Autoimmune destruction of vertebral/hip joints -Aortic regurgitation in up to 10% of pts due to chronic inflammation of aortic root & valve cusps -> retraction of valve cusps, impaired valve closure -> LV overload overtime from regurgitation -> compensatory eccentric hypertrophy -> displacement of PMI to L, exertional SOB, reduced exercise capacity, rarely can lead to AV block from extension of inflammation into interventricular septum -Prostaglandin E2 acts via EP4 receptor, has synergistic effect w/ inflammatory cytokines including TNF-alpha & IL-17 in promoting syndesmophyte formation (bony growth in ligament) & ankylosis (stiffening due to bony fusion); NSAIDs & COX-2i reduce prostaglandin E2 formation & slow disease progression

Coccydynia

Presentation: -Pain at coccyx, most common in women w/ obesity, may follow traumatic injury -Pain exacerbated by sitting -Rectal exam elicits tenderness to palpation of coccyx

Meralgia Paresthetica

Presentation: -Pain, paresthesias, numbness along midlateral/anterolateral thigh -No muscle weakness or DTR abnormalities -May occur w/ frequent squatting Pathophysiology: -Compression/entrapment of lateral femoral cutaneous nerve where it passes under inguinal ligament Treatment/Management: -Weight loss in obese pts, avoidance of tight fitting garments to reduce pressure

Eczema Herpeticum

Presentation: -Painful vesicular rash involving face/neck/trunk in patient w/ hx of severe eczema, fever, irritability, lymphadenopathy, possible viral dissemination -"Punched out" erosions w/ hemorrhagic crusting, monomorphic vesicles -Can be localized, can also spread to noneczematous areas, eyes (keratitis), visceral organs (e.g. hepatitis, encephalitis) Treatment: -Immediate systemic acyclovir d/t risk of disseminated infection Pathophysiology: -Herpes simplex infection of areas of skin affected by atopic dermatitis (increased susceptibility to infectious complications d/t innate skin barrier dysfxn & immune dysregulation)

Herpetic Whitlow

Presentation: -Painful, vesicular hand rash (grouped on erythematous base) w/ tingling/burning pain, epitrochlear/axillary lymphadenopathy, usually resolves in ~2-3 wks, recurrent in 20-50% -Mild systemic sx (fever, fatigue, etc.) -Risk factors: typical predisposing sexual factors (multiple partners, etc.) Treatment: -Acyclovir if immunodeficient Pathophysiology: -HSV inoculation of cutaneous defect of hand (HSV-2 usually acquired by sexual contact, HSV-1 usually acquired by exposure to infected orotracheal secretions, e.g. in healthcare workers)

Porphyria Cutanea Tarda

Presentation: -Painless blisters, bullae, scarring, hypopigmentation/hyperpigmentation, and skin fragility of sun-exposed areas (face, dorsum of hands, forearms) -Painless lesions w/ mild pruritus -Scarring & calcification similar to scleroderma, facial hypertrichosis -Labs: mildly elevated transaminases, iron overload, elevated plasma/urine porphyrin levels -Risk factors/associations: HCV, HIV, excessive EtOH consumption, estrogen use, smoking Treatment/Management: -Treatment of underlying condition (e.g. IFN-alpha for HCV), avoidance of triggering substances -Serial phlebotomy, hydroxychloroquine Pathophysiology: -Disorder of heme synthesis w/ deficiency in uroporphyrinogen decarboxylase (abnormality in late synthesis -> photosensitivity due to accumulation of porphyrinogens that react w/ oxygen on excitation by UV) -Most common type of porphyria

Ecthyma Gangrenosum

Presentation: -Painless red macules that evolve into vesicles/bullae/indurated pustules & necrotic/gangrenous/black "punched out" ulcers w/ violaceous margins over short period (12-18 hrs) -Mosgt commonly affect anogenital area, axilla, extremities -Usually febrile, but may present w/o other signs of infection -Risk factors: immunodeficiency (e.g. neutropenia, immunosuppressants) Diagnosis: -Blood/exudate cultures Treatment/Management: -Broad-spectrum abx while awaiting culture results (including antipseudomonal coverage): common regimens include antipseudomonal beta-lactam (e.g. pip-tazo) + aminoglycoside (e.g. gentamicin) Pathophysiology: -Pseudomonas infection (bacteremia/sepsis) -Organism invades vascular media & adventitia of veins/arteries -> ischemic necrosis of overlying tissues

Macular Degeneration

Presentation: -Painless, bilateral, progressive blurring of central vision (may be acute or insidious) -Peripheral fields & navigational vision classically maintained, affects driving/reading first since they require fine visual acuity (provided by macula) -Age-related form seen >50 yo -Exudative/wet form presents w/ early straight line distortion (straight lines appear wavy), fundoscopy w/ central scotoma & growth of abnormal vessels in retinal space -Leading cause of blindness in industrialized nations -Risk factors: age, smoking -Complications: retinal detachment Diagnosis: -Fundoscopy w/ subretinal drusen (cellular debris seen in early dry AMD), pigment anomalies Treatment/Management: -Oral eye vitamins, possible intravitreal therapy for wet AMD -Cataract surgery if comorbid (can increase risk of AMD progression) Pathophysiology: -Degeneration & atrophy of central retina (macula), retinal pigment epithelium, Bruch's membrane, & choriocapillaries -Wet form: choroidal neovascularization (blood vessels grow up from the choroid behind the retina), predisposes to retinal detachment

Paroxysmal Supraventricular Tachycardia (PSVT)

Presentation: -Palpitations, dyspnea, dizziness/presyncope/syncope, chest pain, most commonly in adolescents & young adults -Sudden onset/offset, regular, narrow-complex tachycardia w/ regular ventricular response -If hemodynamic instability, can present w/ hypotension, diaphoresis, cool extremities -May occur in association w/ WPW syndrome Diagnosis: -ECG w/ narrow-complex tachycardia w/ regular RR intervals, P waves usually inverted (d/t retrograde atrial conduction) & buried within Treatment: -Adenosine (slows AV nodal conduction to interrupt reentry pathway & terminate rhythm), if stable and still fails to improve, IV beta blockers or CCBs -Carotid sinus massage (vagal maneuvers--slow AV nodal conduction & can sometimes terminate paroxysmal tachycardias) -Other vagal maneuvers: bearing down/Valsalva, squatting, breath holding, cold water immersion, eyeball pressure -Synchronized cardioversion if hemodynamically unstable -Definitive treatment by cardiac ablation of secondary conduction pathway Pathophysiology: -A dysrhythmia originating in an ectopic focus anywhere above the bifurcation of the bundle of His. P wave is often hidden in preceding T wave, but if seen may have an abnormal shape. The PR interval may be shortened or normal, and QRS is usually normal. -Includes rhythms like atrioventricular nodal reentrant tachycardia (AVNRT, abnormal circuit within AV node) and atrioventricular reentrant tachycardia (AVRT, abnormal circuit outside AV node); both due to presence of 2 conduction pathways (slow & fast) w/ predisposition to reentry mechanism in which slow & fast pathways form looped circuit w/ impulses traveling in antegrade fashion through slow pathway & returning through fast pathway -Vagal maneuvers -> increased parasympathetic tone -> temporary slowing of conduction through AV node & increase in AV node refractory period -> termination of reentry pathway

Hairy Cell Leukemia

Presentation: -Pancytopenia due to bone marrow fibrosis: granulocytopenia (infections), anemia (fatigue, weakness, pallor), thrombocytopenia (bleeding, bruising) -Splenomegaly (from infiltration), early satiety (due to gastric compression), hepatomegaly/lymphadenopathy/B symptoms uncommon -Seen in middle-aged/older adults -Labs: may have mild leukocytosis Diagnosis: -Peripheral smear w/ hairy leukocyte cells -Bone marrow bx w/ flow cytometry Treatment: -Chemotherapy if moderate/severe -Life expectancy often near-normal Pathophysiology: -Clonal B-cell neoplasm, often associated w/ BRAF mutations

Small Cell Lung Cancer

Presentation: -Paraneoplastic syndromes include ACTH production, SIADH -Labs: normovolemic hyponatremia -Risk factors: smoking (almost exclusively in smokers) Diagnosis: -Imaging w/ bulky hilar/mediastinal mass

Pinealoma

Presentation: -Parinaud syndrome (limitation of upward gaze, favor downward gaze, light-near dissociation, upper eyelid retraction aka Collier sign), pupillary abnormalities (e.g. reactive to accommodation but not light, aka light-near dissociation, loss of pupillary reaction), loss of optokinetic nystagmus -Signs of increased ICP (papilledema, headache, vomiting, ataxia, etc.) from obstructive hydrocephalus -Most common in children 1-12 yo Pathophysiology: -Germ cell tumors account for most pinealomas, can be germinomas that secrete hCG -> precocious puberty in prepubertal males -Parinaud syndrome (dorsal midbrain syndrome) due to pressure on pretectal region of midbrain near superior colliculus and CN III (innervates levator palpebrae superiorus -> eyelid retraction) -Blocks flow of CSF in aqueduct of Sylvius -> obstructive hydrocephalus

Uric Acid Stones

Presentation: -Passage of small crystals ("sand") in urine, colicky flank pain, hematuria, hydronephrosis -Risk factors: increased uric acid excretion (gout, myeloproliferative disorders, hemolytic anemia), increased urine concentration (hot climates, dehydration), low urine pH (chronic diarrhea w/ bicarb loss, metabolic syndrome/T2DM), obesity, poor ammonia secretion Diagnosis: -Radiolucent stones not visible on X-ray but visible on CT/renal US, rhomboid uric acid crystals on microscopy, urine pH <5.5 Treatment: -Alkalinization of urine to pH 6.0-6.5 w/ sodium bicarb/sodium citrate, potassium citrate (citrate = stone inhibitor, reduces crystallization) -If alkalinization not sufficient, may add allopurinol -Interventions like ureteroscopic extraction usually not necessary Pathophysiology: -Acidic urine favors formation of uric acid (insoluble) over urate (soluble) -Supersaturation of urine w/ uric acid precipitates crystal formation

Peripartum Cardiomyopathy (PPCM)

Presentation: -Peripheral/pedal edema, dyspnea, cough, hemoptysis, holosystolic mitral regurgitation, third heart sound, tachycardia -Hyperventilation, respiratory alkalosis -Typically during last mo of pregnancy (>36 wks) or within 5 mos postpartum -CXR w/ cardiomegaly, pulm vascular congestion, possible pulm effusions (often also seen in late normal pregnancy) Diagnosis: -Echocardiogram w/ LV systolic dysfxn, w/ LVEF <45% -Diagnosed in absence of other causes of HF, w/ absence of heart disease prior to final mo of pregnancy Treatment: -Urgent/emergent delivery only for pts w/ advanced HF or hemodynamic instability Pathophysiology: -Cause of dilated CM

Lymphedema

Presentation: -Persistent LE swelling (usually not associated w/ significant pain/allodynia), heaviness, discomfort -Begins w/ soft skin & pitting edema -> firm/dry/thickened skin w/ nonpitting edema (due to deposition of subcutaneous collagen & adipose) -> may look warty in advanced cases -Stemmer sign: inability to lift skin on dorsum of 2nd toe -May follow proximal LE trauma Diagnosis: -Clinical, duplex US to rule out other causes Treatment: -Weight loss, limb elevation -Compression bandages, physiotherapy (massage) -Diuretics contraindicated Pathophysiology: -Physical obstruction of lymphatic drainage, e.g. due to lymphadenectomy, radiation, malignant obstruction, chronic inflammation (e.g. recurrent cellulitis, connective tissue disease), parasitic infection (e.g. filariasis), congenital (e.g. Turner), severe chronic venous insufficiency, obesity

Arteriovenous Malformation (AVM)

Presentation: -Persistent hx of recurrent focal headaches in young pt (typically diagnosed <40 yo), seizures, focal neuro deficits (due to compression) -Rupture presents w/ signs of intracerebral hemorrhage & increased ICP (N/V, decreased level of consciousness) that progress over mins to hrs Diagnosis: -Brain MRI w/ dark flow voids, little surrounding edema Pathophysiology: -Artery directly anastomoses w/ veins w/o interposing capillary bed -> high-pressure system predisposed to aneurysm formation and/or spontaneous bleeding -Usually congenital but can form de novo

Dyspnea of Pregnancy

Presentation: -Physiologically normal SOB during pregnancy due to progesterone-induced hyperventilation & stimulation of respiratory drive -Labs: respiratory alkalosis (increased PaO2, decreased PaCO2), increased minute ventilation (primarily by increased tidal volume), decreased RV & FRC, normal vital capacity & FEV1 Physiology: -Due to changes in pregnancy, diaphragm is elevated, compressing lower lung fields -> basilar atelectasis -> decreased RV & FRC, slight decrease in TLC -Compensation w/ increased minute ventilation, larger tidal volume -> normal vital capacity

Waldenstrom Macroglobulinemia

Presentation: -Plasma cell neoplasm particularly associated w/ hyperviscosity syndrome (blurred vision, diplopia, tinnitus, confusion, HA, dizziness, vertigo, ataxia, rarely stroke/coma) -Peripheral neuropathy, bleeding, hepatosplenomegaly, lymphadenopathy, cryoglobulinemia, renal insufficiency -Fundoscopy w/ "sausage-link" retinal changes (dilated, segmented, tortuous retinal veins) -Labs: elevated ESR, cytopenias from bone infiltration Diagnosis: -Smear w/ rouleaux formation, erythrocyte agglutination -Large gamma gap (difference b/t total protein & albumin) -SPEP w/ monoclonal IgM peak (M-spike) -Bmbx w/ >10% clonal B cells (lymphocytic infiltration w/ positivity for certain immunophenotypic markers) -Serum viscosity testing Treatment: -Urgent plasmapharesis Pathophysiology: -B-cell (lymphoplasmocytic) malignancy w/ excessive production of monoclonal IgM -Large collections can clog microvasculature -> hyperviscosity syndrome

Hepatic Hydrothorax

Presentation: -Pleural effusion (usually right-sided) in the setting of decompensated, severe cirrhosis -Association w/ findings of cirrhosis: LE edema, abd distention w/ shifting dullness d/t ascites Diagnosis: -Transudative pleural effusion w/ fluid to serum total protein ratio 0.5 or lower, fluid to serum LDH 0.6 or lower, fluid LDH 2/3 upper limit of normal or lower Treatment/Management: -Thoracentesis for temporary relief; effusion usually recurs -Treatment of ascites: sodium restriction, loop & K+ sparing diuretics -Consider liver transplantation or TIPS in refractory cases Pathophysiology: -Passage of peritoneal ascites through the diaphragm, usually on R b/c R hemidiaphragm is thinner & more likely to have porous defects

Acute Pericarditis

Presentation: -Pleuritic chest pain (decreased when sitting up, better when leaning forward, worse w/ deep inspiration, retrosternal pain that often radiates to L shoulder or posteriorly to scapulae, bilateral trapezius bridges, worse when supine, may be worse when swallowing), +/- fever, pericardial friction rub (highly specific, high-frequency grating/scratching or squeaking sound, often triphasic in atrial systole, ventricular systole, early ventricular diastole) -Labs: troponin may be elevated, may be undetectable -Risk factors: delayed coronary reperfusion following ST-elevation MI (>3 hrs from sx onset), recent viral infection Diagnosis: -ECG w/ diffuse ST-segment elevation, PR-segment depression -> diffuse T-wave inversion -Echo w/ pericardial effusion (>50% of cases) Treatment/Management: -NSAIDs (ibuprofen, indomethacin) + colchicine for viral/idiopathic etiology (colchicine decreases rate of recurrence) -Steroids if NSAIDs contraindicated or refractory to NSAIDs/colchicine (but not in PIP) -High dose-aspirin for peri-infarction pericarditis (PIP); NSAIDs avoided as they can impair myocardial healing and increase risk fo ventricular septal or free wall rupture -Variable for other etiologies, largely supportive -NSAIDs avoided in peri-infarction pericarditis due to increased risk of free wall rupture, may use narcotic analgesics if refractory to aspirin/colchicine -If uremic: HD!!! (BUN usually >60) Pathophysiology: -Etiologies: viral, idiopathic, autoimmune disease (e.g. SLE), uremia (acute/chronic renal failure, esp w/ BUN >60), post-MI (early peri-infarction pericarditis <4 days after MI, later Dressler syndrome) -Viral causes include adenovirus, coxsackievirus, echovirus, influenza, HIV

Diabetes Insipidus (DI)

Presentation: -Polyuria/nocturia, polydipsia, dilute urine -Failure to concentrate urine (osmolality <300 mOsm, urine specific gravity <1.006) after water deprivation testing -Nephrogenic: intact thirst mechanism, serum Na+ usually ~145 -Central: no thirst mechanism, serum Na+ often >150 -Labs: hypernatremia, high serum osmolality (>250), low urine osmolality (<300, 300+ if partial), low urine specific gravity (<1.006) Diagnosis: -If DI, fail to concentrate urine after 2-3 hrs of no water -If nephrogenic DI, fails to concentrate urine even after desmopressin administration -If central DI, able to concentrate urine after desmopressin administration -After determining which type, may use imaging (head MRI, renal US) to localize cause Treatment: -Central: desmopressin replacement (intranasal preferred) -Nephrogenic DI: HCTZ, amiloride (if lithium-induced), NSAIDs -Salt restriction, discontinuation of offending agent if relevant Pathophysiology: -Renal concentrating ability impaired due to either renal resistance (nephrogenic DI) to ADH or deficient ADH production (central DI) -Central etiologies: head trauma, infection, intracranial hemorrhage/tumor -Nephrogenic etiologies: meds (lithium, demeclocycline, foscarnet, cidofovir, amphotericin), hypercalcemia, severe hypokalemia, tubulointerstitial renal diseases -In children, hereditary nephrogenic DI is most common cause of DI

Diabetic Ketoacidosis (DKA)

Presentation: -Polyuria/nocturia, polydipsia, polyphagia, vomiting, abd pain, weight loss, fatigue, Kussmaul respirations (deep, rapid breathing), dehydration (dry mucous membranes) -May be precipitated by infection or other acute stressor -Relative to HHS, not as pronounced AMS, more rapid onset of sx, hyperventilation & abd pain more common -Complications: cerebral edema -Risk factors: most commonly seen in T1DM, younger age Diagnosis: -Serum glucose >200 (250-500), anion gap metabolic acidosis w/ bicarb <15 and anion gap >14, pH <7.3, serum/urine ketones, serum osmolality <320 Treatment/Management: -IV: 10 mL/kg isotonic fluid bolus (NS) over 1 hr, then add dextrose 5% when serum glucose is 200 or lower -Insulin: start continuous IV regular insulin infusion until anion gap normalizes, hold if K+ <3.3, halve rate if glucose <200 but still anion gap, then switch to SQ (basal bolus) insulin if 1. able to eat, 2. glucose <200, 3. anion gap <12 & serum bicarb 15+ (overlap SQ & IV for 1-2 hrs on initiation) -K+: add IV K+ if serum <5.3, hold if 5.3+ (nearly all pts are K+ depleted, even w/ hyperkalemia) -Bicarb: consider for pts w/ pH 6.9 or lower -Phos: consider for serum phos <1, cardiac dysfxn, or resp depression -Monitor serum Ca frequently, measure glucose hourly, measure anion gap & electrolytes & venous pH every 2-4 hrs -Most accurate measures of resolution are beta-hydroxybutyrate and anion gap Pathophysiology: -Infection triggers increased catecholamine, glucagon, & cortisol release, can precipitate acute presentation -Osmotic diuresis of excess serum glucose is accompanied by net renal loss of K+ -> depletion of total K+ stores; correction w/ insulin causes inward shift of K+ (relative hypernatremia), which can precipitate hypokalemia, requires careful correction of K+ levels -AG metabolic acidosis due to accumulation of unmeasured ketoacids acetoacetate (measured by urine ketone testing) & beta-hydroxybutyrate (not measured by urine ketones)

Achilles Tendon Rupture

Presentation: -Popping sensation & acute pain following rapid acceleration or direction change -Positive Thompson test (absence of plantar flexion on calf squeeze, most sensitive/specific test), inability to walk on tips of toes (plantar flexion), pain w/ passive dorsiflexion -May be associated w/ tendinopathy (e.g. fluoroquinolone use) -May present w/ ankle edema, hematoma Diagnosis: -MRI (not performed in cases w/o rupture, useful in setting of high clinical suspicion w/ negative Thompson or if partial tendon suspected) Pathophysiology: -Achilles/calcaneal tendon connects soleus and gastrocnemius to calcaneus, permits plantar flexion -Accessory muscles of plantar flexion (e.g. plantaris, flexor hallucis longus, digitorum longus) insert directly into foot; allows pt to maintain ambulation/some plantar flexion even w/ tendon rupture

Hepatic Cirrhosis

Presentation: -Portal HTN -> esophageal varices, splenomegaly, ascites, caput medusae, anorectal varices -Hepatic synthetic dysfxn -> ecchymoses, edema (hypoalbuminism) -Hyperestrogenism -> hypogonadism, gynecomastia, spider angiomata, loss of sexual hair, testicular atrophy, palmar erythema -On exam & imaging, liver characteristically shrunken/fibrotic, occasionally normal sized or enlarged w/ accompanying splenomegaly Diagnosis: -Imaging for confirmation (abd US) -If ascites, diagnostic paracentesis to rule out infection & confirm etiology (SAAG, cell count/differential, total protein); should be straw-colored, have SAAG of 1.1+, low total protein <2.5 Treatment/Management: -Nonselective beta blocker (e.g. propanolol, nadolol) to decrease progression of varices, reduce risk of hemorrhage -If contraindications to beta blocker therapy or larger varices, may use endoscopic variceal ligation -Endoscopic sclerotherapy & octreotide if varices actively bleeding -Treatment of ascites w/ furosemide + spironolactone, sodium restriction, EtOH abstinence -Avoid ACEi, ARBs, NSAIDs -If ascites refractory, large-volume paracentesis or TIPS -Preventive care: vaccinate for HAV & HBV unless already immune -Screening: endoscopy for varices, US +/- serum AFP every 6-12 mos to screen for HCC, frequent clinical assessment for ascites/encephalopathy Pathophysiology: -Hypogonadism due to primary gonadal injury or hypothalamic-pituitary dysfxn -Elevated estradiol is due to increased conversion from androgens, impaired hepatic metabolism of estrogens -Decreased synthesis of binding proteins for thyroid hormone (thyroid-binding globulin, transthyretin, albumin, lipoproteins) -> decreased serum T3 & T4 but overall euthyroid status (normal TSH) -Portal HTN due to increased resistance to portal flow at sinusoids -> increased pressure at portosystemic collateral veins in anterior abdomen, rectum, distal esophagus

Dumping Syndrome

Presentation: -Postgastrectomy complication in up to 50% of pts, presents w/ sx 15-30 min after meals -GI sx (nausea, diarrhea, abd cramps), fatigue -Vasomotor sx (palpitations, diaphoresis, hypotension, tachycardia, dizziness/syncope, confusion) Treatment/Management: -Small/frequent meals, slow eating, replacement of simple sugars w/ complex carbs, incorporation of high-fiber & protein-rich foods, drinking fluids between rather than during meals -Sx usually resolve over time -If refractory sx, may try trial of octreotide, may require reconstructive surgery (uncommon) Pathophysiology: -Loss of normal action of pyloric sphincter due to injury or surgical bypass -> rapid emptying of hypertonic gastric contents into duodenum & small intestine -> fluid shifts from intravascular space to small intestine -> hypotension, stimulation of autonomic reflexes, release of intestinal vasoactive polypeptides, can precipitate hypoglycemia

Helicobacter Pylori Infection

Presentation: -Postprandial abdominal pain -May present w/ sx of nonulcer dyspepsia/gastritis & GERD or w/ signs of ulceration -Risk factor for peptic ulcer disease (duodenal > gastric ulcers), gastric cancer (adenocarcinoma or lymphoma), mucosa-associated lymphoid tissue lymphoma -Labs: microcytic anemia, positive occult blood stool testing, immunehtrombocytopenia -Risk factors: childhood in low-income country, low socioeconomic status, international travel, poor hygiene (e.g. handwashing) Diagnosis: -Breath test, stool antigen testing -Definitive testing by bx on endoscopy (breath test first for pts <60 w/ low risk features) -For repeat breath or stool antigen testing to confirm elimination, must be off abx & PPI for 4-6 wks -Repeat testing to confirm eradication indicated in pts w/ persistent sx, H. pylori-associated ulcer on endoscopy, evidence of H. pylori-associated malignancy (e.g. MALT lymphoma) -Repeat surveillance endoscopy indicated to confirm healing of gastric ulcers (not duodenal) d/t high risk for malignancy Treatment/Management: -If no penicillin allergy & no previous macrolide use, standard triple therapy (PPI + clarithromycin + amox for 10-14 days) -If penicillin allergy, no previous macrolide/metronidazole use, modified triple therapy (PPI + clarithromycin + metronidazole for 10-14 days) -If high macrolide or metronidazole resistance or treatment failure after 1 course of therapy, quad therapy (PPI + bismuth + metro + tetracycline for 10-14 days) -Sucralfate may be used for symptomatic relief (no specific role in eradication) -Advise reduction/elimination of NSAID use until completion of treatment, dietary changes (reduce acidic intake) Pathophysiology: -Responsible for 70-80% of duodenal ulcers & ~50% of gastric ulcers

Diabetic Gastroparesis

Presentation: -Postprandial bloating & vomiting (usually) in diabetic pt, epigastric abd pain -Early satiety, impaired nutrition & weight loss, chronic diarrhea -Epigastric distention, succussion splash on auscultation -Risk factors: long-standing DM (esp type 1), chronic poor control, labile blood glucose (may report episodes of hypoglycemia b/c of premature insulin administration relative to delayed intestinal absorption of glucose, other s/s of microvascular disease like autonomic neuropathy w/ postural dizziness, sweating, constipation, etc.) Diagnosis: -Nuclear gastric emptying study showing delayed transit to duodenum -Exclude obstruction by upper endoscopy or barium swallow +/- CT/MR enterography Treatment/Management: -DM control, dietary modification (small, frequent meals, decreased fat/fiber intake, soluble fiber only) -Promotility drugs (metoclopramide, erythromycin, domperonide) -Gastric electrical stimulation and/or jejunal feeding tube for refractory sx Pathophysiology: -Etiologies: DM (autonomic neuropathy), meds (e.g. opioids, anticholinergics), traumatic/postsurgical injury (e.g. vagus nerve injury), neurologic (e.g. MS, spinal cord injury), idiopathic/postviral -Autonomic neuropathy due to destruction of enteric neurons -> failure of fundus relaxation, uncoordinated peristalsis -Delayed intestinal absorption of glucose makes timing of insulin difficult, can lead to labile glucose levels and hypoglycemia -Impaired parasympathetic control over stomach & decreased functionality of interstitial cells of Cajal (ICC, gastric pacemaker cells) -> failure of coordinated muscle contraction

Hemolytic Uremic Syndrome (HUS)

Presentation: -Preceding bloody diarrhea (e.g. after family cookout, consuming undercooked meat) affecting multiple people -Most commonly in children <5 yo -Triad of microangiopathic hemolytic anemia, thrombocytopenia, AKI (hematuria, proteinuria, secondary HTN) -Fatigue, pallor, bruising, petechiae, oliguria/anuria, edema, scleral icterus, jaundice, abd pain, vomiting Diagnosis: -Labs: hemolytic anemia (increased reticulocytes, schistocytes, increased bilirubin, decreased haptoglobin), thrombocytopenia, AKI (increased BUN, Cr, hematuria, proteinuria) -Coombs negative anemia (microangiopathic) w/ schistocytes Treatment: -Fluid & electrolyte management, blood transfusions, dialysis -Most improve within wks -Supportive care if shiga toxin producing ETEC (abx increase risk of HUS), abx possible if Shigella (azithromycin, ceftriaxone, or ciprofloxacin) Pathophysiology: -Initial insult from Shiga toxin (O157:H7 E. coli serotype, Shigella) -> vascular damage, microthrombi formation -> consumption of platelets, microangiopathic hemolytic anemia -Toxin invades & destroys colonic epithelium -> abd pain, diarrhea

QT Prolongation

Presentation: -Predisposes to torsades -Risk factors: electrolyte derangements (hypocalcemia, hypokalemia, hypomagnesemia), abx (macrolides, fluoroquinolones), psychotropics (antipsychotics, SSRIs, TCAs), opioids (methadone, oxycodone), antiemetics (ondansetron, granisetron), antiarrhythmics (quinidine, procainamide, flecainade, amiodarone, sotalol), inherited disorders (Jervell & Lange-Nielson syndrome, Romano-Ward syndrome) Pathophysiology: -Delayed ventricular repolarization due to impaired fxn of voltage-gated K+ channels Prevention: -ECG prior to administration of QT-prolonging drugs

Bacillus Cereus Gastroenteritis

Presentation: -Predominantly vomiting 1-6 hrs after eating reheated rice or other starch (after reheating or being left at room temp for extended period), abd cramping -Diarrhea & fever may occur, less common -Self-limited, usually resolves within 24-48 hrs Pathophysiology: -Ingestion of preformed Bacillus cereus enterotoxin

Common Variable Immunodeficiency (CVID)

Presentation: -Presents in adolescence or early adulthood (20-40 yo), not as severe as Bruton -Recurrent sinopulmonary infections (sinusitis, PNA, otitis), esp by encapsulated bacteria (e.g. H. flu, Strep pneumo) -Recurrent GI infections (e.g. Salmonella, Campylobacter, Giardia) -Associations: atopic conditions (e.g. asthma, eczema), predisposition to autoimmune disease (RA, thyroid disease, hemolytic/pernicious anemia), pulmonary disease (bronchiectasis, fibrosis/ILD, restrictive/obstructive lung disease), GI disease (chronic diarrhea, IBD-like conditions), increased risk of non-Hodgkin lymphoma, granuloma development, failure to thrive Diagnosis: -Low IgG, IgA, and/or IgM -Poor antibody response to vaccines -Normal leukocytes (both B and C cells) Treatment: -Ig replacement therapy, management of chronic lung disease Pathophysiology: -Opsonization by Ig required for clearance of encapsulated bacteria, IgA required for clearance of mucosal infections -Abnormal differentiation of B cells into plasma cells -> hypogammaglobulinemia -Usually d/t sporadic mutation, usually no fhx

Cerebral Amyloid Angiopathy

Presentation: -Presents in elderly pts (>75), cause of dementia (often develops in setting of Alzheimer disease), potential cause of intracranial hemorrhage (usually lobar, may present w/ confusion, lethargy, failure to withdraw from pain on affected side, N/V, etc.) -Can lead to recurrent hemorrhage, most commonly affecting occipital (homonymous hemianopsia) & parietal (contralateral hemisensory loss) lobes Diagnosis: -In setting of hemorrhage, cranial hyperdensity on noncontrast CT (most commonly involves occipital/parietal lobes, sparing ventricles & deep brain structures vs. hypertensive hemorrhage) Treatment: -Like other causes of intracranial hemorrhage, reverse anticoag, control BP, normalize ICP Pathophysiology: -2nd most common cause of intracerebral hemorrhage after HTN -Abnormal beta-pleated amyloid protein deposition in cerebral blood vessels -> arteriopathy, vessel fragility -> increased risk of spontaneous hemorrhage

Invasive Pulmonary Aspergillosis

Presentation: -Primarily in immunocompromised pts (prolonged neutropenia, post-transplant, glucocorticoids, HIV w/ CD4 <50) -Fever, pleuritic chest pain, hemoptysis, chronic productive cough, dyspnea, weight loss -Mortality rate >50%, 1 yr mortality >80% Diagnosis: -Imaging (CT, CXR) w/ fungus ball within a cavity, nodules w/ surrounding ground glass infiltrates (halo sign) and/or cavitations w/ air-fluid levels (air crescent sign), segmental infiltrates -Positive sputum stain/cultures, positive cell wall biomarkers (galactomannan, beta-D-glucan) -Bronchoscopy w/ BAL and/or biopsy if above inconclusive Treatment: -Voriconazole +/- caspofungin (echinocandin) -Reduction of immunosuppressive regimen for transplant pts, surgery if needed Pathophysiology: -Ubiquitous fungus, conidia inhaled into lungs -> convert to pathogenic hyphae if immunocompromised (immunocompetent have macrophages, neutrophils, T cells that rapidly neutralize & clear)

Vascular Dementia

Presentation: -Progression of dementia in stepwise fashion w/ consecutive ischemic events -Early executive dysfxn (impaired ability to plan, solve problems, think abstractly, d/t medial frontal disease), focal neuro deficits, aphasia (parietal), anterograde amnesia (medial temporal), urologic disturbances & personality changes (subcortical) -Cerebral infarction and/or deep white matter changes on neuroimaging -Narrow-based, shuffling gait, frequent falls, unsteadiness -Risk factors: HTN, stroke, small vessel cerebrovascular disease, smoking, T2DM, HLD Pathophysiology: -Cortical or subcortical-predominant symptoms clusters depending on involved regions -Can result from large artery or cerebral infarctions (cortical/subcortical), small artery infarctions in subcortical areas (lacunae), and/or chronic subcortical ischemia (may present w/ focal motor deficits like reflex asymmetry, urinary sx, psychiatric sx like depression, more gradual declining course than cortical type) -2nd most common cause of dementia after Alzheimer disease

Otosclerosis

Presentation: -Progressive conductive hearing loss in younger (early to mid-30s) Caucasian people, F>M, tinnitus, no dizziness/vertigo/ear pain, usually unilateral -Paradoxical improvement in speech discrimination in noisy environments (paracusis of Willis) -Normal TM on exam +/- reddish hue behind TM (excess bony resorption can expose underlying blood vessels in middle ear) -Risk factors: family hx (AD w/ incomplete penetrance), pregnancy (may progress during pregnancy) Treatment/Management: -Amplification (hearing aids) -Surgery (stapes reconstruction) Pathophysiology: -Bony overgrowth of stapes footplate due to imbalance of bone resorption & deposition -> stapes stiffening & fixation of ossicular chain

Achalasia

Presentation: -Progressive dysphagia to both solids & liquids, regurgitation of undigested food Diagnosis: -Barium swallow showing aperistalsis, poor emptying, dilation of prox esophagus due to retention of food, "bird beak" narrowing at GEJ Pathophysiology: -Degeneration of ganglia of myenteric/Auerbach plexus in esophagus (esophageal ganglion cells) -> prevents LES from relaxing, tonic LES contraction -Secondary achalasia can also be caused by infection w/ Trypanosoma cruzi (Chagas)

Interstitial Lung Disease

Presentation: -Progressive exertional dyspnea or persistent dry, nonproductive cough -Lung exam w/ fine/dry "velcro" crackles during mid-inspiration (audible long before imaging findings), possible digital clubbing -Exertion associated w/ hypoxemia (due to V/Q mismatch) -May be associated w/ smoking (>50%) or other pulm conditions (e.g. silicosis, connective tissue disease, asbestosis) -Can progress to cor pulmonale w/ signs of LE edema, hepatomegaly Diagnosis: -CXR w/ reticular/nodular opacities, high resolution chest CT w/ fibrosis, honeycombing, traction bronchiectasis -PFTs w/ normal or increased FEV1/FVC ratio, decreased DLCO, TLC, RV, FEV1, FVC -Resting ABG can be normal or show mild hypoxemia, increased A-a gradient -Lung bx if diagnosis unclear (shows alveolar spaces filled w/ fibroblasts) Pathophysiology/Etiologies: -Sarcoidosis, amyloidosis, alveolar proteinosis, idiopathic pulm fibrosis, interstitial PNA, cryptogenic organizing PNA -Vasculitides (e.g. granulomatosis w/ polyangiitis) -Infections (e.g. fungal, TB, viral PNA) -Occupational & environmental agents (e.g. silicosis, hypersensitivity pneumonitis, asbestos, beryllium) -Connective tissue disease (e.g. SLE, scleroderma, RA) -Drugs (e.g. amiodarone, bleomycin, nitrofureantoin), radiation therapy -Excessive collagen deposition in ECM surrounding alveoli -> scarring, increased elastic recoil of airways -> restrictive lung disease pattern

Glucocorticoid-Induced Myopathy

Presentation: -Progressive proximal muscle weakness & atrophy w/o pain or tenderness -LE muscles more commonly involved -May be associated w/ exogenous glucocorticoid administration, Cushing syndrome/disease -Labs: ESR & CK normal -Risk factors: high-dose therapy, long-term therapy Treatment: -Cessation of glucocorticoid therapy, recovery may take wks-mos Pathophysiology: -Weakness due to catabolic effects of cortisol on skeletal muscle -> muscle atrophy w/o pain -Atrophy thought to be mediated by inhibition of Akt-1 (intracellular signaling molc w/ serine/threonine kinase activity), possible IGF-1 signaling, suppression of muscle cell differentiation & protein synthesis

Presbycusis

Presentation: -Progressive, bilateral sensorineural hearing loss, usually associated w/ aging (affects >50% of adults by 75 yo) -Difficulty understanding speech, esp w/ competing background noise, better speech discrimination in quiet room -Affects high frequencies first, may present as difficulty hearing high voices (women, children) -Tinnitus (continuous ringing/rushing/buzzing) -Pts may withdraw from social lives & stop leaving home to avoid conversations Diagnosis: -Audiometry, whispered voice test Treatment/Management: -Family education -Amplification devices (hearing aids) Pathophysiology: -Cochlear hair cell loss, cochlear neuron degeneration -Age-related brain atrophy may also contribute to increased information processing times -> difficulty w/ speech discrimination

Cataracts

Presentation: -Progressive, gradual, bilateral, painless blurring of vision & loss of acuity, glare, "halos" around lights due to light scattering, difficulty w/ night driving (due to glare), myopic shift (decreased difficulty w/ close vision, e.g. decreased need for reading glasses), muting of color vision -On exam, opacification of lens, reduced clarity of retinal detail, red reflex may be intact in early disease but is lost in later disease, decreased visual acuity of both central & peripheral vision -Risk factors: advanced age, sunlight exposure, smoking, T2DM, glucocorticoid use, HIV Treatment: -Indicated when loss of vision impairs daily living; definitive treatment w/ lens extraction & artificial lens implantation -Post surgical complications include posterior capsule opacification (thickening of capsule holding artificial lens, causes cloudy vision and can be treated w/ laser therapy), recurrence of cataracts uncommon Pathophysiology: -Usually related to oxidative damage to lens w/ aging -> opacification -Conversion of excess glucose into sorbitol in T2DM increases risk of lens opacification by deposition leading to increased cellular osmotic & oxidative stress

Asbestosis

Presentation: -Prolonged asbestos exposure (plumbers, electricians, carpenters, construction workers, shipbuilders, insulation workers, miners) -Sx usually develop 20+ yrs after initial exposure, w/ dyspnea, basilar fine end-inspiratory crackles, clubbing, cough, chest tightness, wheezing -Complications: lung cancer/bronchogenic carcinoma (e.g. cavitary lung mass, 59x increase in risk when combined w/ smoking), pleural mesothelioma, cor pulmonale (peripheral edema, hepatojugular reflex, JVD, RV heave) Diagnosis: -Pleural plaques on chest imaging in 50% (CXR or chest CT) -Imaging (bibasilar reticulonodular infiltrates, honeycombing, subpleural linear densities, parenchymal fibrosis), PFTs, histology c/w pulm fibrosis -PFTs: decreased DLCO, restrictive pattern (normal or elevated FEV1/FVC, decreased lung volumes)

Priapism

Presentation: -Prolonged, painful erection in absence of ongoing sexual stimulation -Usually idiopathic -Risk factors: spinal cord injury (autonomic dysfxn, cauda equina syndrome), altered blood viscosity (SICKLE CELL, blood dyscrasias like thalassemia, blood malignancies like CML, multiple myeloma), meds/drugs (phosphodiesterase inhibitors incl sildenafil, intracavernosal injection w/ alprostadil, trazodone, stimulants e.g. cocaine/methylphenidate, SSRIs), surgery (perineal/genital trauma) -In SCD, may be preceded by prior episodes that resolved, may be precipitated by triggers including fever, cold, dehydration, cocaine, alcohol, testosterone -Complications: erectile dysfxn Diagnosis: -Blood gas analysis of corporeal aspirate -Doppler US to distinguish b/t ischemic & nonischemic causes Treatment/Management: -If <4 hrs, may be terminated by simple interventions (e.g. urination, cold compresses), but if >4 hrs: -Aspiration of corpora cavernosa followed by cold saline irrigation -Intracorporal injection of alpha-1 adrenergic agonists (e.g. phenylephrine, causes contraction of cavernous smooth muscle to facilitate venous emptying) -If nonischemic (uncommon), angiographic embolization Pathophysiology: -Ischemic/low-flow priapism most common -Impaired venous outflow from corpora cavernosa -> increased cavernosal pressure -> ischemia, hypoxia, acidosis -Altered blood viscosity or abnormal autonomic/vascular tone -> decreased outflow through emissary veins -In SCD, sickling of blood in corpora cavernosa, increased viscosity of blood precipitates episode -Nonischemic & high-flow priapism uncommon, often d/t traumatic fistula from cavernosal artery

Lambert-Eaton Syndrome

Presentation: -Proximal muscle weakness: difficulty standing from chair, combing hair, reaching up -Hyporeflexia (diminished/absent DTRs) -Repeated isometric contraction can lead to temporary improvement of muscle contraction, reappearance of absent DTRs -May have autonomic dysfxn (e.g. dry mouth, erectile dysfxn), CN involvement (e.g. ptosis) Diagnosis: -Serum autoantibody testing -Electrophysiologic testing (repetitive nerve stimulation testing shows progressive improvement in amplitude of complex motor APs w/ repetition) -Evaluation/imaging for unidentified malignancy Treatment: -Guanidine, 3,4-diaminopyridine (increase presynaptic ACh levels) -Immunologic therapies: IVIg, immunosuppressants (e.g. corticosteroids, azathioprine) Pathophysiology: -Autoantibodies to presynaptic voltage-gated calcium channels, may be due to paraneoplastic syndrome (~50% associated w/ underlying malignancy, most commonly small cell lung cancer)

Bed Bugs

Presentation: -Pruritic, small punctae & maculopapules in linear groups/tracks/clusters ("breakfast, lunch, & dinner" pattern) on unclothed skin, central hemorrhagic punctum -Bites on palms/soles uncommon b/c skin thick -Small, red/brown, blood-feeding insects that hide in clusters in surrounding environment and feed at night Treatment: -Supportive (topical corticosteroids, oral antihistamines) -Bedbugs can live for up to yr w/o feeding; must be definitively eradicated w/ insecticides & heat, freezing/laundering of contaminated objects Pathophysiology: -Transmission within physical living space or via colonized objects (e.g. luggage), rarely person-to-person -Salivary proteins can inhibit coagulation -> central hemorrhagic punctum

Hemineglect Syndrome

Presentation: -Pt neglects nondominant side, only responds to stimuli on dominant side (usually R side), e.g. shaving, cleaning, combing, recognizing objects on side -May be accompanied by anosognosia (unawareness of condition) -When asked to lift nondominant side, will lift Diagnosis: -Clock test Pathophysiology: -Lesion of nondominant (right) parietal lobe, responsible for spatial organization

Cystic Fibrosis Pulmonary Disease

Presentation: -Pulmonary exacerbations w/ hypoxia and respiratory distress (nasal flaring, retractions, grunting) -In childhood years, colonization by S. aureus > Pseudomonas (esp may be susceptible w/ concurrent influenza infection) -In adult yrs (~20 yo), colonization by Pseudomonas > S. aureus -Recurrent PNA causes irreversible bronchiectasis, chronic hypoxia, progressive respiratory failure -Barrel chest, digital clubbing Treatment: -For exacerbation, empiric therapy consists of S. aureus coverage + 2 drugs effective against Pseudomonas -Oseltamivir for influenza, cefepime/ceftazidime for MSSA & Pseudomonas coverage, vancomycin for S. aureus coverage (and coverage for methicillin resistance), amikacin/tobramycin also effective against Pseudomonas -Ciprofloxacin for Pseudomonas coverage in adults -Alternative agents include carbapenems, some fluoroquinolones, aztreonam, colistin Pathophysiology: -Methicillin resistance associated w/ recurrent treatment w/ abx

Sudden Cardiac Arrest (SCA)

Presentation: -Pulselessness, sudden collapse Treatment/Management: -CPR, defibrillation for shockable rhythm -Survival dependent on factors including elapsed time to effective resuscitation -Epi only in pts w/ asystole, pulseless electrical activity (PEA), refractory ventricular arrhythmia Pathophysiology: -Most common rhythm is sustained ventricular tachycardia/fibrillation d/t acute MI (pulseless cardiac arrest w/ shockable rhythm)

Neurosyphilis

Presentation: -Pupillary abnormalities (Argyll-Robertson pupils), sensory ataxia, lancinating pains, dementia -Early stages (secondary stage): meningitis (HA, confusion, N/V, nuchal rigidity), ocular (posterior uveitis, retinitis, optic neuritis, decreased visual acuity), meningovascular (infectious CNS arteritis causing ischemia/infarction), otosyphilis (hearing loss, tinnitus), cranial neuropathies -Late stages (tertiary): general paresis, progressive dementia, tabes dorsalis (uncommon, posterior column/dorsal root disease, pupillary defect, diffuse neuro signs, may have normal CSF) -Risk factors: HIV w/ CD4 <350 Diagnosis: -Reactive VDRL of CSF (LP) Treatment: -Aqueous penicillin G, 3-4 million units IV q4hrs for 10-14 days

Thyroid Lymphoma

Presentation: -Rapid increase in size of thyroid/goiter over wks-mos -> mass effect sx of difficulty swallowing, positive Pemberton's test -Inability to palpate inferior edge of thyroid may suggest substernal extension -Risk factors: Hashimoto disease -Labs: positive for anti-TPO abs

Acute Mesenteric Ischemia

Presentation: -Rapid onset of severe, poorly localized/diffuse periumbilical pain, nausea, vomiting, urge to defecate -Pain is worse w/ eating due to need for increased intestinal blood flow; waxing and waning quality -Pain out of proportion to physical exam findings, usually benign abd exam w/ mild distention -Hematochezia (late complication) -If bowel infarction occurs, may develop more focal abd tenderness & distention, peritoneal signs (e.g. guarding, rebound tenderness), rectal bleeding, sepsis, absent bowel sounds -Labs: leukocytosis, elevated amylase & phosphate, anion gap metabolic acidosis (elevated lactate), hemoconcentration, positive fecal occult blood -Complications: perforation, sepsis -Risk factors: atherosclerosis, embolic source (e.g. thrombus, cardiac vegetation, AFib, esp w/ recent MI, arrhythmias, HF), hypercoagulable disorders, CHF, advanced age, hypovolemia (e.g. trauma, hemodialysis) Diagnosis: -CT (preferred) or MR angiography show focal/segmental wall thickening, small bowel dilation, mesenteric stranding, pneumatosis intestinalis (portal vein gas), mesenteric thrombus -Mesenteric angiography if diagnosis unclear Treatment: -Nasogastric decompression, IV fluids, anticoag, broad-spectrum abx, occasionally surgery Pathophysiology: -Abrupt arterial occlusion of superior mesenteric artery due to cardiac embolic events (e.g. AFib, valvular disease, CV aneurysms) or acute thrombosis (e.g. PAD, low cardiac output states, atherosclerotic disease) -Nonocclusive ischemia d/t hypoperfusion to splanchnic circulation & vasoconstriction -May also occur w/ superior mesenteric vein occlusion (usually occurs in hypercoagulable states, portal HTN, abd infection)

Polymyalgia Rheumatica

Presentation: -Rapid onset, bilateral pain/stiffness in shoulders and hips/pelvic girdle >1 mo, morning stiffness that improves after 1-2 hrs, gelling (stiffness w/ inactivity) -Involvement of 2+ of: neck/torso, shoulders & proximal arms, proximal thigh/hip, constitutional (low-grade fever, malaise, weight loss) -Decreased active ROM in shoulders, neck, hips, normal passive ROM, muscle tenderness to palp, normal strength -Often associated w/ temporal arteritis (~10%) -Risk factors: female, >50 -Labs: ESR >40, sometimes >100, elevated CRP, sometimes normocytic anemia, ~20% have normal studies, CK normal Diagnosis: -Elevated acute phase reactants (ESR, CRP) -Temporal artery bx if symptoms of GCA (HA, jaw claudication, vision loss, temporal tenderness) -Response to steroids Treatment: -PO corticosteroids (e.g. low-dose prednisone) -If GCA, high-dose prednisone Pathophysiology: -Inflammatory disorder of proximal joints, bursae, and tendons (contrary to name, muscle itself is unaffected so strength & CK are normal)

Multifocal Atrial Tachycardia

Presentation: -Rapid, irregular pulse & ECG w/ 3+ P wave forms & atrial rate >100/min (rate distinguishes from wandering atrial pacemaker), irregular RR intervals -Often asymptomatic or may be perceived as palpitations, rarely causes hemodynamic instability -May be associated w/ exacerbation of pulm disease (e.g. COPD), electrolyte disturbances (e.g. hypokalemia), catecholamine surge (e.g. sepsis) -Usually asymptomatic apart from palpitations, presents w/ sx of underlying disease -Risk factors: elderly (>70), underlying pulm disease Treatment: -Correct underlying disturbance (e.g. electrolyte replacement esp K & Mg, bronchodilators, corticosteroids, noninvasive ventilation, etc.) -AV nodal blockade (e.g. verapamil) only if persistent despite treatment or if RVR associated w/ CHF or MI Pathophysiology: -Supraventricular tachyarrhythmia that likely occurs due to atrial conduction abnormalities triggered by disturbances including RA enlargement, catecholamine surge, electrolyte disturbance

Creutzfeldt-Jakob Disease

Presentation: -Rapid-onset dementia, myoclonus (startle-induced involuntary muscle spasm), cerebellar signs (e.g. ataxia w/ wide-based gait, nystagmus), UMN signs (e.g. hyperreflexia, upgoing Babinski), extrapyramidal signs (e.g. hypokinesia, dystonia), akinetic mutism, behavioral changes (e.g. mood/sleep disturbances, hypersomnia, personality change), seizures -Sporadic onset may occur in pts in their 60s -Once sx begin (often after incubation for yrs), pts experience inexorable decline (e.g. loss of speech, ADLs, coma) until death in <12 mos after dx Diagnosis: -Periodic sharp wave complexes on EEG (sharp, triphasic/biphasic, synchronous discharges) -MRI w/ widespread atrophy of cerebrum/cerebellum (may not be apparent until later disease), cortical enhancement/ribboning (hyperintensity of cerebral cortex), enhancement of putamen & caudate head ("hockey stick" sign) -CSF w/ normal routine testing (no WBCs, normal glucose, normal/slightly increased protein), 14-3-3/tau protein titers, positive RT-QuIC test (real time quaking induced conversion test, shows amplification/propagation of abnormal folding) -Histo of neural tissue w/ spongiform degeneration w/o inflammation Treatment/Management: -No effective disease-modifying therapy, fatal in <12 mos -Symptomatic treatment, counseling, social services referral Pathophysiology: -Prion disease, fatal transmissible spongiform encephalopathy -Prion: abnormally folded protein that can induce similar conformational changes in other proteins -> accumulation of neurotoxic fibrils -> spongiform, noninflammatory neurodegeneration w/ widespread loss of neurons in both cerebrum & cerebellum -Most commonly sporadic, but can also be hereditary, iatrogenic (e.g. contaminated transplant or surgical instruments), associated w/ oral consumption of contaminated neural tissue -Long incubation period of ~10 yrs

Transverse Myelitis

Presentation: -Rapidly progressive myelopathy w/ early flaccid paralysis (LMN) & late spastic paralysis (UMN), hyporeflexia -Possible dull back pain -Clearly identifiable sensory spinal level w/ severe sensory deficits (vs. GBS) of all somatosensory domains (pain, temp, vibratory, proprioception) below level -If associated w/ quadriplegia, weakness in UEs = LEs -Bowel & bladder dysfxn common, sexual dysfxn -No CN involvement -Prognosis: usually incomplete recovery, increased future risk for MS Diagnosis: -MRI w/ focal enhancement of spinal cord region on T2 (reflecting increased water content) & no compressive lesion, CSF w/ pleocytosis +/- increased IgG index, EMG & nerve conduction studies mostly normal Treatment: -High-dose IV glucocorticoids, plasmapheresis Pathophysiology: -Infiltration of inflammatory cells into segment of spinal cord leading to neuron & oligodendrocyte cell death & demyelination -Occurs w/ segmental demyelination of spinal cord in MS, severe HSV infection, systemic disorders (e.g. sarcoidosis) -Often post-infectious (e.g. gastroenteritis, URI), immune-mediated destruction of spinal cord d/t molecular mimicry

Desmoid Tumor

Presentation: -Rare benign & locally aggressive tumor, usually presents as deeply seated painless or sometimes painful mass in trunk/extremity, intraabdominal bowel & mesentery, or abdominal wall -Variable clinical course ranging from slow to rapid growth, variable in size -If abdominal, can cause intestinal obstruction & bowel ischemia, have high rate of recurrence even after aggressive surgery/resection -Risk factors: increased in pts w/ familial adenomatous polyposis (Gardner syndrome) Diagnosis: -CT/MRI to evaluate size of mass -Bx for histologic diagnosis Treatment/Management: -Surgery is definitive therapy for symptomatic tumors, tumors that cause risk fo adjacent structures or cosmetic issues, and/or recurrent tumors -If not surgical candidates, pts can undergo radiation therapy -Close observation in pts that are asymptomatic, have stable masses, or have intraabd masses seen in Gardner syndrome Pathophysiology: -Locally aggressive benign tumor arising from fibroplastic elements within muscle/fascial planes -Very low potential for metastasis or differentiation -Thought to be d/t abnormal wound healing or clonal chromosomal abnormalities causing neoplastic behavior

Perilymphatic Fistula

Presentation: -Rare, debilitating complication of head injury or barotrauma -Progressive sensorineural hearing loss from damage to cochlear hair cells by loss of endolymph -Episodic vertigo w/ nystagmus triggered by pressure changes in inner ear (Vasalva, sneezing, straining, elevation change -> acutely increased endolymph leakage) -Tullio phenomenon: loud clap near ear (increased pressure/sound conduction through ossicles) triggers nystagmus Pathophysiology: -Fistula causing leakage of endolymph from semicircular canals & cochlea into surrounding tissues

HIV-Associated Thrombocytopenia (HIV-TP)

Presentation: -Rarely associated with bleeding, asymptomatic thrombocytopenia, may present w/ splenomegaly Treatment: -Antiretroviral therapy Pathophysiology: -Thought to be d/t immune dysfunction or viral destruction of megakaryocytes

Irritant Contact Dermatitis

Presentation: -Reaction to chemical agents (e.g. solvents, detergents, soaps, acid/alkali, metals, wood, fiberglass) -Acute: erythematous papules, edema, bullae, vesicles -Chronic: scaling, fissuring, lichenification (skin thickening due to chronic scratching), hyperkeratosis -Associated w/ burning/stinging sensation, commonly affects exposed surfaces like hands -Sx usually worst where skin is thinnest (e.g. dorsum of hands, fingertips, finger webs) -Common in pts w/ underlying atopic dermatitis, healthcare workers that use gloves Diagnosis: -Clinical, patch testing if need to rule out allergic dermatitis Treatment/Management: -Avoidance of irritants, washing hands w/ lukewarm water & mild cleansers, frequent application of emollients to restore skin barrier & reduce water loss -Topical corticosteroids to reduce inflammation Pathophysiology: -Nonimmunologic rxn to chemical agents; due to physical/chemical irritation -Irritants disrupt epidermal barrier through chemical/physical microtraumatic damage to keratinocytes -> increased epidermal permeability, water loss

Rhinitis Medicamentosa

Presentation: -Rebound nasal congestion commonly associated with overuse of over-the-counter nasal decongestants, most commonly oxymetazoline (may occur within 3-5 days) -Decreased sense of smell -Beefy red nasal mucosa on exam Treatment/Management: -Cessation of nasal decongestant spray, often w/ concomitant nasal glucocorticoids to aid cessation or oral glucocorticoids in severe cases Pathophysiology: -Oxymetazoline causes rapid relief of congestion by profound vasoconstriction, repeated & prolonged use causes damage to vascular endothelium -> rebound congestion

Viral Myocarditis

Presentation: -Recent URI (fever, malaise, myalgias), fatigue, dyspnea/orthopnea, elevated JVP, peripheral edema, CP (may mimic MI), cardiomegaly in relatively young adult (<55) -Audible S3 (due to large amt of blood hitting wall of very compliant LV), bibasilar rales on exam, displacement of PMI -Pulm vascular congestion on CXR -Can rarely cause sudden cardiac death Diagnosis: -Endomyocardial bx (gold standard): lymphocytic infiltration, viral DNA or RNA (PCR) -ECG: nonspecific signs (e.g. diffuse ST segment changes) -Echo: 4-chamber dilation (dilated CM) -Cardiac MRI: late enhancement of epicardium Treatment: -HF pharm management (diuretics, ACEi, beta-blockers) -Temporary ventricular assist device, if needed -Heart transplant if no recovery Pathophysiology: -Etiologies include parvovirus B19, HHV 6, coxsackievirus, adenovirus, influenza, HIV -Initially inadequate immune response to viral infection allows virus to invade cardiac myocytes -> cytotoxicity, impaired myocardial contractility -> decreased systolic fxn, ventricular dilation (eccentric hypertrophy, dilated CM)

Irritable Bowel Syndrome (IBS)

Presentation: -Recurrent abd pain/discomfort 1+ days/wk for 3+ mos and 2+ of: relation to defecation (improves or worsens), change in stool frequency, change in stool form -May be constipation-predominant, diarrhea-predominant, or mixed -Bloating, belching, flatulence, nausea, passage of mucus in stool, tenesmus, usually in young women w/ psych comorbidities (e.g. anxiety, depression), worsens during times of psychosocial stress -Alarm features: older age of onset (50+), GI bleeding, nocturnal diarrhea, worsening pain, unintended weight loss, iron def anemia, elevated CRP, +fecal calprotectin or lactoferrin, fam hx of early colon cancer (<50) or IBD -Labs: normal CBC & inflammatory markers (e.g. ESR, fecal calprotectin), negative celiac serologies (IgA, antitissue transglutaminase) Treatment/Management: -Reassurance, dietary modification (fiber supplementation for constipation), loperamide if diarrhea-predominant -TCAs may be used for abd pain if refractory to above Pathophysiology: -Functional disorder, etiology poorly understood but may involve visceral hypersensitivity & abnormal GI motility

Gout

Presentation: -Recurrent attacks w/ monoarthritis that usually develops overnight or early in the morning, reaches max intensity within 12-24 hrs, most commonly of 1st metatarsal or knees (others include ankle, wrist, finger, shoulder, olecranon bursa) -"Red hot joint": joint w/ severe pain, redness, warmth, swelling, decreased ROM -Tophi (nodular deposits, most commonly in joints & helix of ear, may ulcerate) in pts w/ chronic hyperuricemia -Risk factors: meds (diuretics, low-dose aspirin, cyclosporine), surgery, trauma, recent hospitalizations, volume depletion, heavy EtOH consumption, diet (high-protein foods like meat/seafood, high-fat foods, fructose/sweetened beverages), HTN, obesity, CKD, organ transplant -Protective factors: dairy product, vitamin C, & coffee intake Diagnosis: -Aspiration of joint fluid w/ inflammatory effusion (elevated leukocytes, neutrophil predominance), needle-shaped, negatively birefringent crystals (monosodium urate, yellow when parallel to polarizing axis, blue when perpendicular) -X-rays may show punched-out erosions/lytic lesions w/ rim of cortical bone (rat-bite lesion), soft tissue opacification Treatment: -Acute: NSAIDs (indomethacin), glucocorticoids (systemic or intraarticular, option if CKD), colchicine (avoid w/ CKD) -Prevention w/ recurrent attacks or complicated disease (tophi, uric acid stones): allopurinol, febuxostat, pegloticase, probenecid -While initiating chronic therapy, use colchicine or NSAIDs to prevent flare (ppx) -If comorbid HTN or LVH, add on ARB (avoid diuretics) or ACEi Prevention: -ETOH CESSATION, weight loss, low-fat diet, decreased seafood/red meat intake, increased protein intake from low-fat dairy & vegetables, avoidance of diuretics/aspirin/beta-blockers Pathophysiology: -Etiologies related to increased urate production: idiopathic/primary gout, myeloproliferative/lymphoproliferative disorders (e.g. polycythemia vera), tumor lysis syndrome, Lesch-Nyhan (HGPRT deficiency) -Etiologies related to decreased urate clearance: CKD, thiazide/loop diuretic use

Chronic Pancreatitis

Presentation: -Recurrent bouts of abd pain (intensifies w/ meals, better w/ sitting upright or leaning forward) w/ intermittent pain-free intervals and/or sx of fat malabsorption w/ steatorrhea, vomiting, weight loss -May develop signs of fat soluble vitamin def (ADEK), DM, pancreatic ascites (serosanguineous or straw-colored, high amylase, high protein, low serum:ascites albumin gradient) -Labs: normal LFTs, low stool elastase (measure of pancreatic exocrine fxn), amylase/lipase may be normal, low serum trypsinogen, advanced disease associated w/ direct hyperbili & alk phos -Risk factors: smoking, EtOH, family hx, CF (common in children), ductal obstruction (malignancy, stones), autoimmune disease Diagnosis: -CT or MRCP w/ calcifications, dilated ducts, enlarged pancreas, possible pancreatic atrophy in advanced disease Treatment/Management: -Pain management, EtOH & smoking cessation, frequent/small meals, pancreatic enzyme supplements, fat-soluble vitamin supp -If pain not relieved by above, can consider nonopioid analgesics (e.g. TCAs, NSAIDs, pregabalin), celiac nerve block, ductal decompression therapy, extracorporeal shock wave lithotripsy for stones, surgical resection Pathophysiology: -Recurrent episodes of pancreatitis -> fibrosis, stricture of intrapancreatic portion of bile duct -> irreversible exocrine & endocrine functional damage -Reduced exocrine pancreatic enzymes (lipase, amylase, protease) -> fat malabsorption -> steatorrhea, weight loss, diarrhea -Involves patchy inflammation & fibrosis of pancreas (vs. widespread inflammation in acute pancreatitis)

Shift Work Disorder

Presentation: -Recurrent pattern of sleep interruption due to shift work causing difficulty in initiating & maintaining sleep -> daytime sleepiness -Associated w/ work schedules that are incongruent w/ normal circadian clock

Chronic Bacterial Prostatitis

Presentation: -Recurrent sx of UTI >3 mos (dysuria, frequency, urgency, suprapubic tenderness), urinary incontinence, painful/boggy/swollen prostate (may be absent & painless, normal prostate exam), pain w/ ejaculation -Sx improve w/ short course of abx but then recur -Labs: UA w/ pyuria & hematuria, urine culture/stain w/ same organism multiple times, collect UA & culture before & after prostate massage -Risk factors: young & middle-aged men, T2DM, smoking, urinary tract procedure/hardware Diagnosis: -UA before & after prostatic massage or exam of expressed prostate fluid -Pyuria & bacteriuria on UA, bacteria in prostatic fluid > bacteria in urine Treatment: -Fluoroquinolones (e.g. ciprofloxacin) or SMX-TMP for 6 wks Pathophysiology: -Coliforms enter from urethra via intraprostatic reflux -E. coli causative in >75% of cases, other causes include Enterococcus, Pseudomonas, Klebsiella, Proteus

Hypoglycemia-Associated Autonomic Failure

Presentation: -Reduced neurogenic hypoglycemia sx (e.g. tremor, arousal, sweating) -> reduced hypoglycemia awareness -Increased risk for neuroglycopenic sx (e.g. confusion, lOC) -Risk factors: long-standing T1DM, recurrent/severe hypoglycemia, beta blocker therapy Treatment/Management: -Strict avoidance of hypoglycemia (can restore awareness in 2-3 wks), careful/reduced insulin dosing, less stringent glycemic targets, modified carbohydrate ingestion (e.g. in relation to physical activity) Pathophysiology: -Recurrent/severe hypoglycemia -> reduction of epinephrine-induced hepatic glucose release & suppression of sx related to catecholamine surge -> increased risk for progressively worse hypoglycemia

Gastroesophageal Reflux Disease (GERD)

Presentation: -Regurgitation of acidic material in mouth, heartburn/burning CP, odynophagia (often indicates reflux esophagitis) -Extraesophageal manifestations: cough, hoarseness, wheezing, throat irritation -Complications: erosive esophagitis, Barrett esophagus, esophageal strictures, asthma, laryngitis -Risk factors: obesity, pregnancy, smoking, EtOH intake Diagnosis: -24 hr esophageal pH monitoring indicated if no response to PPI Treatment/Management: -Lifestyle (e.g. weight loss, smoking cessation, EtOh reduction) & dietary changes -H2R blocker (mild/infrequent sx) or PPI (frequent sx) -If alarm s/s (e.g. dysphagia, odynophagia, anorexia, weight loss, e/o GI bleeding) or failure of PPI therapy, upper endoscopy -If failure of 8 wks of high dose PPI, esophageal pH monitoring indicated -Surgery (e.g. Nissen fundoplication) optional in pts w/ partial response to high dose PPI Pathophysiology: -Decreased tone or excessive transient relaxation of LES -Anatomic disruption to GEJ (e.g. hiatal hernia)

Amyloidosis

Presentation: -Renal: heavy proteinuria w/ nephrotic syndrome (>3.5 g/day), peripheral edema -CV: restrictive CM (EF elevated, dyspnea, JVD, edema, ascites, increased ventricular thickness, predominantly R-sided HF w/ diastolic dysfxn), can progress to dilated CM over time, conduction defects (e.g. AV block), low voltage -CNS: peripheral and/or autonomic neuropathy, stroke, dementia, carpal tunnel syndrome & scapulohumeral periarthritis (ESRD) -GI: hepatomegaly, dysmotility, malabsorption, GI bleeding due to vascular fragility, early satiety -Pulm: pulm nodules, tracheobronchial infiltration, pleural effusions -MSK: enlarged tongue, shoulder pad enlargement -Skin: thickening/waxy skin, subcutaneous nodules/plaques, ecchymoses, periorbital purpura -Heme: normocytic anemia, thrombocytopenia -Labs: elevated ESR Diagnosis: -Tissue bx (e.g. fat pad, renal, endomyocardial) w/ amyloid (amorphous hyaline material that stains Congo red, green birefringence under polarized light) -Echo w/ concentric LVH & nondilated LV, esp in absence of hx of HTN, atrial enlargement Pathophysiology: -Multisystem disease due to extracellular & insoluble fibril deposition in tissues -AL amyloidosis produced by clonal plasma cells (MM, Waldenstrom macroglobulinemia) -AA produced secondary to chronic inflammation (osteomyelitis, rheumatologic conditions) -ATTR age-related or hereditary (AD inheritance), composed of breakdown products of transthyretin (prealbumin) -AB2-m dialysis-related (beta2-microglobulin), ESRD

Renovascular Disease

Presentation: -Resistant HTN (uncontrolled despite 3 drugs), malignant HTN (end-organ damage), onset of severe HTN >55 yo (>180/120), severe HTN w/ diffuse atherosclerosis, recurrent flash pulmonary edema w/ severe HTN (pulm crackles, distended neck veins, no lower extremity edema, normal EF) -Asymmetric renal size (>1.5 cm difference), abdominal bruit, unexplained atrophic kidney on imaging -Unexplained rise in serum Cr (>30%) after starting ACEi or ARB, urinalysis normal -May be seen in women w/ fibromuscular dysplasia or older pts w/ atherosclerosis, common after renal transplant Diagnosis: -Renal US w/ Doppler (preferred in pts w/ renal insuff d/t avoidance of contrast), CT/MR angiography, captopril-enhanced radionuclide renal scan Treatment/Management: -BP control (ACEi/ARBs first line w/ close monitoring of renal fxn, + CCBs, BBs, thiazides, MRAs), aggressive treatment of atherosclerotic disease (statins, smoking cessation) -Aggressive risk factor reduction (aspirin, T2DM & HLD control) -If refractory to medical therapy, renal revascularization or stenting Pathophysiology: -Renal ischemia in poststenotic kidney -> activation of RAAS (increased plasma renin) -> sodium/water retention, generalized vasoconstriction, secondary hyperaldosteronism (hypokalemia, elevated bicarb) -> persistent HTN -If renal artery stenosis unilateral, other kidney may compensate (increased renin in affected kidney renal vein, decreased renin in unaffected kidney renal vein); if bilateral, can result in progressive CKD -In renal transplant pts, may be d/t improper surgical anastomosis, acute rejection, CMV infection

Transfusion Related Acute Lung Injury (TRALI)

Presentation: -Respiratory distress, hypotension, tachycardia, fever, noncardiogenic pulmonary edema (crackles on exam), bilateral pulmonary infiltrates on imaging -Sx beginning mins to hrs after transfusion starts -Hypoxia & dyspnea/tachypnea within 6 hrs of transfusion initiation Treatment/Management: -Immediate transfusion cessation, respiratory supportive care (usually requires ventilatory support) -Some recover in 24-48 hrs, ~50% of previously critically ill pts expire Pathophysiology: -Potentially fatal transfusion rxn due to neutrophil activation by component in transfused blood (e.g. donor anti-leukocyte antibodies) -> massive release of cytokines, ROS, other inflammatory mediators -> damage to pulmonary vasculature & capillary endothelium -> inflammatory pulmonary edema -Vs. TACO, no JVD, no S3, BNP normal, EF normal

Cluster Headache

Presentation: -Retroorbital/temporal unilateral pain (excruciating, sharp/stabbing, steady) in trigeminal distribution, often beginning at night and lasting ~15-90 min (circadian periodicity), hx of multiple similar episodes (may occur up to 8x daily for 6-8 wks, 1-2 periods a yr) -Other sx include ipsilateral autonomic signs (ptosis, miosis, rhinorrhea/nasal congestion, sweating, facial flushing, lacrimation), redness of ipsilateral eye (conjunctival injection), no vision changes, restless agitation -Risk factors: M>F Treatment: -Prophylactic therapy (verapamil, lithium, topiramate) -Abortive therapy (100% O2 by nonrebreathing facial mask, subcutaneous/intranasal sumatriptan contralateral to side of HA, ergotamine 2nd line, intranasal lidocaine) Pathophysiology: -Parasympathetic hyperactivity

Solitary Pulmonary Nodule

Presentation: -Round pulmonary opacity on imaging that is 3 cm or smaller (>3 cm is considered a mass), surrounded by lung parenchyma, w/ no associated lymph node involvement/effusion/atelectasis -Risk factors for malignant potential: large size (>2 cm = >50% risk), advanced pt age (>60), female, active/previous smoking, fam/personal hx of lung cancer, upper lobe location, spiculated radiographic appearance, irregular borders on imaging, partially solid (heterogeneous) Diagnosis: -Usually found on chest CT -Compare to previous CXRs/scans, and if no previous imaging or possibility of growth from prior, obtain chest CT -If stable on prior imaging for >2 yrs, no further testing necessary Treatment/Management: -Nodules <0.8 cm unlikely to be malignant, generally do not require f/u -Nodules >0.8 cm require additional management/surveillance; if intermediate or high probability for malignancy based on features (e.g. low density, spiculated borders, eccentric/reticular/punctate calcification) or risk factors (e.g. weight loss, h/o smoking, >40 yo, prior malignancy), should undergo biopsy (percutaneous, bronchoscopy) or surgical excision (thoracotomy, VATS) -If >0.8 cm w/ low risk for malignancy on CT, should repeat chest CT in 3 mos, serial CTs for next 2-3 yrs -If intermediate risk/unclear, can use PET to assess malignant potential Pathophysiology: -Etiologies include primary lung cancer (SCC, adenocarcinoma, small cell, large cell, carcinoid), metastatic cancer (melanoma, breast, head & neck, renal cell, colon, germ cell, sarcoma), benign infectious granulomas (TB, histo, atypical Mycobacteria, coccidioides, Cryptococcus, blastomycosis), benign neoplasms (lipoma, hamartoma, fibroma), vascular (AVM) -Low risk radiographic features: popcorn (classic for pulm harmartoma), concentric, laminated, central, diffuse homogeneous calcifications

Olecranon Bursitis

Presentation: -Rounded, fluctuant, tender swelling at posterior elbow accompanied by local pain (may be absent if chronic), often "golf-ball" sized -Typically normal ROM (b/c not within joint itself) if chronic microtraumatic; may reduce ROM if significantly inflamed (e.g. septic, inflammatory) -Erythema, warmth, fever, significant pain suggest inflammatory/infectious source Diagnosis: -X-ray if fx suspected, CBC & bursal aspiration (cell count, Gram stain, culture) in select cases -If no concerning features, expectant management Treatment: -If no concerning features, elbow protection (e.g. padding to reduce friction), NSAIDs, rest, icing -If septic, antistaphylococcal abx & drainage -If inflammatory, treat underlying gout/RA/etc. Pathophysiology: -Etiologies include: chronic overuse/microtrauma, acute trauma/hemorrhage, inflammatory (e.g. gout, RA), septic (e.g. penetrating injury, spread from regional infection) -Olecranon bursa: fluid-filled synovial sac located b/t olecranon process & skin, serves to alleviate friction at bony prominence, can develop fluid accumulation w/ injury

Dilated Cardiomyopathy

Presentation: -S/s of HF (e.g. dyspnea on exertion, volume overload) -S3, holosystolic murmur loudest at cardiac apex (secondary mitral regurg) Diagnosis: -Echo demonstrating dilation & reduced EF Treatment/Management: -HF management -Stress testing & coronary angio to evaluate for ischemic causes Pathophysiology: -Can be idiopathic/primary or secondary -Most common secondary cause is ischemic cardiomyopathy (usually secondary to CAD); chronic myocardial ischemia -> reversibly depressed contractility -Other secondary causes: HTN, hemochromatosis, Graves disease, EtOH, viral myocarditis

Isolated Systolic Hypertension

Presentation: -SBP of 140+ w/ diastolic BP <90 -Most common in elderly pts (associated w/ age) Pathophysiology: -Increased stiffness or decreased compliance of aortic & arterial walls -> reduced ability of arteries to dampen SBP -> increased pulse wave velocity & pulse wave reflection in systole -> increased SBP & increased pulse pressures

High-Altitude Pulmonary Edema (HAPE)

Presentation: -SOB, fever, fatigue, HA, N/V, hypoxemia, cough within several days of arriving at high altitude >2500 m -Worsened by EtOH intake -Recent arrival at high altitude (<1 wk), marked early improvement w/ supplemental O2 -Labs: absent/mild leukocytosis (<15), normal procalcitonin -CXR w/ patchy infiltrates (noncardiogenic pulmonary edema), crackles on exam Pathophysiology: -Lungs undergo hypoxic vasoconstriction to optimize alveolar-capillary gas exchange due to low partial pressure of inspired O2 (PiO2) at high altitude -Some pts w/ genetic predisposition experience unevenly distributed hypoxic vasoconstriction -> exposure of pulmonary capillaries in relatively less vasoconstricted areas to high perfusion pressure -> regional disruption of alveolar/capillary membrane -> patchy bilat, noncardiogenic pulmonary edema -Supplemental O2 alleviates hypoxic vasoconstriction (decreased pulmonary arterial pressure), rapidly improves edema & hypoxemia

Exudative Pleural Effusion

Presentation: -SOB, focally decreased breath sounds, dullness to percussion, CXR w/ pleural effusion -If rheumatologic/connective tissue disease, may be associated w/ ILD (fine crackles) Diagnosis: -Light Criteria: pleural protein/serum protein >0.5 OR pleural LDH/serum LDH >0.6 OR pleural LDH >2/3 upper limit of normal serum LDH -Low glucose (<50) common in empyema, RA, malignancy, TB -pH usually b/t 7.3-7.45, <7.3 associated w/ empyema, tumor, pleuritis, pleural fibrosis -High amylase associated w/ pancreatitis-associated effusion, esophageal rupture -Lymphocyte predominance seen in TB (w/ elevated adenosine deaminase), chylothorax; neutrophil predominance in empyema Pathophysiology: -Inflammation, increased capillary and/or pleural membrane permeability d/t cytokine release -> translocation of cells & fluid from vasculature into pericapillary space -Etiologies: infection (TB, PNA, empyema, low pH), malignancy, connective tissue disease, rheumatologic disease (RA, SLE), PE, pancreatitis (amylase-rich), post-CABG, esophageal perf (Boerhaave, amylase-rich, low pH)

Postoperative Atelectasis

Presentation: -SOB/dyspnea post-op days 2-5 (esp after abd & thoracoabdominal surgeries), increased work of breathing, may be asymptomatic -Dense opacities at lung bases on CXR, loss of lung volume due to collapse of lung tissue -If collapse of large portion of lung, may lead to shift of mediastinal contents toward collapsed lung w/ compensatory hyperinflation of contralateral lung -Vs. PE, not associated w/ chest pain Treatment: -Incentive spirometry, pain control, post-op deep breathing exercises (encourage high tidal volume breathing to keep alveoli open), directed coughing, early mobilization -CPAP can help open collapsed alveoli -Chest physiotherapy & suctioning in pts w/ excess respiratory secretions -In some cases of atelectasis due to mucus plugging, may require bronchoscopy to remove mucus plugs Pathophysiology: -Thoracic/abd pain following surgery restrict chest expansion -> low tidal volume w/ basilar alveolar collapse (pharyngeal secretions, airway tissue edema, residual anesthetic effects may also contribute, weak cough predisposes to small airway mucus plugging) -Low TV -> hypoxemia (low PaO2, <75) from V/Q mismatch -> triggers increased RR that overcompensates for decreased TV -> respiratory alkalosis w/ decreased PaCO2 (normal 38-42)

Classic Congenital Adrenal Hyperplasia

Presentation: -Salt-wasting adrenal crisis (hyponatremia), usually presenting in infancy -Ambiguous genitalia due to excess androgens in females but normal internal genitalia -Elevated 17-hydroxyprogesterone Pathogenesis: -Due to 21-hydroxylase deficiency -> decreased cortisol + aldosterone

Aspirin-Exacerbated Respiratory Disease (AERD)

Presentation: -Samter triad: asthma (intermittent wheezing, chest tightness), chronic rhinosinusitis w/ nasal polyps (nasal congestion, frontal HAs), NSAID-induced respiratory rxns (worsening sx after OTC analgesics) -Some may have more dramatic sx including facial flushing, urticarial eruption, angioedema, anaphylactoid rxn (bronchospasm, hypotension) Treatment: -Avoidance of NSAIDs -If NSAIDs necessary, desensitization therapy -Leukotriene pathway inhibitors (e.g. zileuton, montelukast) for respiratory/nasal sx Pathophysiology: -Nonimmunologic rxn to aspirin & other NSAIDs, increased production of proinflammatory leukotrienes & decreased production of anti-inflammatory prostaglandins d/t inhibition of COX pathway w/ NSAIDs/aspirin -Leukotriene excess -> sx that mimic rhinosinusitis or asthma flares

Tinea Capitis

Presentation: -Scaly, erythematous patch of hair loss w/ inflammation, pruritus, pustules, boggy plaques -Small black dots on areas of hair loss representing broken hair shafts -May have tender lymphadenopathy (occipital, postauricular, posterior cervical) -Most common in African American children and immunocompromised pts Diagnosis: -Clinical, KOH examination of hair stubs Treatment: -Oral terbinafine or griseofulvin -Treatment of household contacts w/ selenium sulfide or ketoconazole shampoo Pathophysiology: -Local dermatophyte infection (most commonly Trichophyton tonsurans, Microsporum canis), transmission via direct contact or fomites (e.g. shared combs)

Tinea Corporis

Presentation: -Scaly, erythematous, pruritic patch w/ centrifugal spread -> subsequent central clearing w/ raised, annular border -Patches may become confluent to form "flower petal" shape -Often w/ concurrent infection of other areas including hands (tinea manuum), groin (tinea cruris), toes/feet (tinea pedis) d/t autoinoculation -Risk factors: athletes w/ skin-to-skin testing, humid environment, contact w/ infected animals (e.g. rodent), HIV, T2DM, systemic glucocorticoids, warm/moist environment (e.g. public showers, pools), wearing tight/occlusive clothing, PAD, obesity, concurrent tinea infection Diagnosis: -KOH prep of skin scrapings -If extensive disease, consider testing for HIV & other causes of immunodeficiency Treatment: -First-line/localized: topical antifungals (e.g. clotrimazole, terbinafine, tolnaftate) -Second-line/extensive: oral antifungals (terbinafine, griseofulvin, fluconazole) Prevention: -Thorough drying after bathing (e.g. desiccant powders), wearing cotton Pathophysiology: -Cutaneous dermatophyte infection, most commonly by Trichophyton rubrum

Dialysis-Related Amyloidosis

Presentation: -Scapulohumeral periarthritis: shoulder pain/hypertrophy w/ increased rotator cuff thickness, hyperechogenic deposits on imaging -Carpal tunnel syndrome: median neuropathy, w/ thenar eminence atrophy & weakness, tingling & decreased sensation in 1st 3 fingers, possible flexor tenosynovitis (finger contractures) -Bone cysts: possible pathologic fxs -Associated w/ long-term dialysis (>5 yrs) Pathophysiology: -Beta2-microglobulin deposited in tissues as amyloid due to inadequate elimination despite dialysis, has high affinity for osteoarticular structures -Glycosaminoglycans, type 1 collage, other connective tissue components participate in amyloid fibril stabilization

Supravalvular Aortic Stenosis

Presentation: -Second most common cause of aortic stenosis -Young pt w/ aortic stenosis murmur (systolic, RUSB), palpable thrill at suprasternal notch, unequal carotid pulses, BP differential b/t UEs & LEs (like aortic coarctation) -Can present w/ stable angina (CP w/ exertion) Pathophysiology: -Congenital LV outflow tract obstruction d/t discrete or diffuse narrowing of ascending aorta, often associated w/ coronary artery stenosis -Often develop LVH -CP d/t increased myocardial O2 demand from increased muscle mass, poor perfusion through stenosed vessels

Carcinoid Tumor

Presentation: -Secretory diarrhea/cramping, cutaneous flushing (usually 20-30 s, w/ hypotension & cyanosis in severe cases), venous cutaneous telangiectasias, bronchospasm, episodic pounding sensation in neck -Cardiac valvular abnormalities (R>L), most commonly tricuspid regurgitation, results in plaque-like deposits of fibrous tissue on endocardium in R heart -> regurg + R-sided HF -Risk of niacin deficiency/pellagra (dermatitis, diarrhea, dementia, glossitis, angular stomatitis) Diagnosis: -Elevated 24 hr urine 5-HIAA levels -CT/MRI of abdomen/pelvis to localize tumor -OctreoScan to detect metastases -Echo if sx of carcinoid heart disease present Treatment: -Octreotide for symptomatic pts & prior to anesthesia/surgery -Surgical intervention for hepatic mets Pathophysiology: -Neuroendocrine tumor producing excess serotonin, most commonly found in distal small intestine, prox colon, & lung -Tumors secrete products including histamine, serotonin, VIP that are metabolized by liver -Frequently metastasize to liver (liver unable to metabolize products before release into systemic circulation -> signs of carcinoid syndrome) -Serotonin synthesized from tryptophan, which is also used in production of niacin -> niacin deficiency

Superior Vena Cava Syndrome

Presentation: -Seen in lung cancer that obstructs the SVC and causes distended head and neck veins (JVD) with edema (but no edema of LEs), blue/dark/plethoric discoloration of arms and face, facial swelling, HA worse when leaning forward, neck pain, conjunctival congestion, prominently visible collateral veins -Dyspnea, chest pain, cough -Can progress to hoarseness, dysphagia, CP, syncope -Advanced disease: cyanosis, collateral veins in thorax, ocular proptosis, lingual edema Diagnosis: -CXR identifies cause in >80% of cases, abnormalities followed w/ chest/neck CT & histology Treatment: -If d/t malignancy, may require endovenous stenting + radiation therapy (primarily palliative) -Depending on type of malignancy, possible chemotherapy Pathophysiology: -External or internal compression of SVC by pulmonary mass/metastatic nodes/thrombus -> elevated upper venous pressure -Malignancy is cause of obstruction in >60% of cases (e.g. bronchogenic carcinoma, small cell lung cancer, non-Hodgkin's lymphoma), other causes include fibrosing mediastinitis (e.g. TB, histoplasmosis), thrombosis secondary to indwelling central venous devices

Papillary Muscle Rupture

Presentation: -Serious complication of MI that may present acutely after MI or within 3-5 days -Acute-onset hypotension, pulmonary edema w/ respiratory distress (dyspnea/tachypnea), possible cardiogenic shock -Soft mid-to-late systolic murmur (mitral regurgitation) best heard at apex (sometimes absent) Diagnosis: -Transthoracic or transesophageal echo w/ severe mitral regurgitation & flail leaflet Pathophysiology: -Occurs w/ inferior wall MI involving RCA occlusion -> posteromedial papillary muscle rupture (usually 3-5 days after MI, w/ peak myocardial softening predisposing to rupture, only supplied by RCA) -> severe mitral regurgitation -Murmur absent in up to 50% of cases b/c MR is so severe that LA & LV pressures equalize

Tuberculous Meningitis

Presentation: -Several wks of prodromal sx (HA, malaise, fever, N/V, neck stiffness), progressive/subacute neurologic decline w/ nuchal rigidity, CN palsies, lethargy, seizures -CSF w/ low glucose (<40), high protein (not as high as bacterial meningitis, 100-500), mildly elevated WBCs w/ lymphocytic predominance (not as high as bacterial meningitis, 100-500), elevated adenosine deaminase -Occurs in 5% of pts w/ extrapulmonary TB -Choroidal tubercles (yellow-white nodules near optic disc) on fundoscopy -Risk factors: immunodeficiency from alcoholism, substance abuse, malnutrition, immunomodulatory meds, HIV -Prognosis: high risk of long-term neuro sequelae Diagnosis: -Noncon head CT often normal, may show basilar meningeal enhancement w/ small strokes (d/t tuberculous vasculitis) -Acid-fast stain or PCR of CSF for rapid diagnosis, requires confirmation by culture Treatment/Management: -Antitubercular therapy: 2 mos of 4-drug therapy (INH, rifampin, pyrazinamide, FQ/injectable aminoglycoside), then 9-12 mos of continuation therapy (INH + rifampin) -8 wks of adjuvant glucocorticoid therapy (dexamethasone or prednisone) to reduce CNS inflammation, reduce morbidity/mortality Pathophysiology: -Particularly affects basal portion of brain -> pressure from dense gelatinous exudate -> CN palsy

Stevens-Johnson Syndrome

Presentation: -Severe blistering reaction w/ mucocutaneous involvement following administration of drug (e.g. sulfas, anticonvulsants), usually 4-28 days after exposure (or 2 days after repeat exposure) -High fever (often prodromal, influenza-like), hypotension, tachycardia, AMS, conjunctivitis, seizures, coma, painful hemorrhagic oral lesions (mucositis) -Coalescing red/purple bullae/vesicles/macules -Positive Nikolsky sign (necrosis/sloughing/desquamation of epidermis on touch) Treatment/Management: -Primarily supportive, wound care similar to that for burns, aggressive fluid resuscitation, antiseptic precautions (d/t high risk of secondary infections) Pathophysiology: -Toxic epidermal necrolysis = >30% body surface involved, SJS = <10%, TEN/SJS overlap = 10-30% -Common drug precipitants: allopurinol, abx (sulfonamides), anticonvulsants (carbamazepine, lamotrigine, phenytoin), NSAIDs (e.g. piroxicam), sulfasalazine -Other precipitants: Mycoplasma infection, vaccinations, GVHD

Acute Mastoiditis

Presentation: -Severe ear pain, facial nerve palsy, fever, mastoid process inflammation w/ outward/verticle displacement of auricle (protrusion of outer ear, postauricular erythema & tenderness) -Tympanic membrane may show evidence of AOM (erythema, bulging, perforation) or may not be visible due to swelling of external auditory canal -Complications: extracranial extension (subperiosteal abscess, facial nerve palsy, hearing loss, labyrinthitis), intracranial extension (brain abscess, meningitis) -Nocturnal headaches (severe, not relieved by NSAIDs, worse at night due to increased ICP while supine), morning vomiting (cerebral edema stimulation of area postrema) indicative of intracranial pathology like brain abscess; may precede papilledema Diagnosis: -Opacification of mastoid air cells on CT or MRI (MRI better for detecting early cerebritis) -May be confirmed by temporal CT in atypical cases -If brain abscess, ring-enhancing lesion Treatment: -IV abx, drainage of purulent material by tympanostomy, mastoidectomy Pathophysiology: -Commonly caused by Pneumococcus, infection of mastoid air cells, often a complication of acute otitis media -May also be caused by S. pyogenes, S. aureus, occasionally Pseudomonas (esp w/ recurrent abx)

Corneal Abrasion

Presentation: -Severe eye pain d/t trigeminal nerve sensory innervation (except in pts w/ trigeminal nerve dysfxn d/t tumor, trauma, prior zoster infection) -Watering, photophobia w/ reluctance to open eye, foreign body sensation Diagnosis: -Fluorescein staining to visualize epithelial defects (corneal defect appears yellow) after below tests; multiple vertical/parallel lines suggest retention of foreign body in eye, uptake of fluorescein w/ subsequent clearing in waterfall washout pattern (Seidel sign) indicates full-thickness corneal lac -Penlight test (to document pupillary fxn, inspect for foreign body) -Visual acuity testing w/ ophtho deferral if decreased (also indicated w/ ulceration, purulence, lack of healing within 3-4 days) Treatment/Management: -Evert eyelids, irrigate eye w/ saline to remove any foreign bodies -If traumatic or foreign body abrasion, topical abx (e.g. erythromycin, polymyxin/trimethoprim) -Lubricating eye drops -Cyclopegics (parasympatholytic agents that prevent light-induced miosis) may be prescribed for pain management of photophobia w/ large corneal abrasions -If c/f ocular perf, corneal infiltrate (e.g. white spot, opacity, Seidel sign), corneal ulcer, irregular pupil, retention of foreign body not easily removed -> urgent ophtho consult, urgent operative repair Pathophysiology: -Etiologies: direct trauma or foreign body lodging under lid, contact lens use leading to defects in corneal epithelium, spontaneous (no obvious foreign body or injury) -Further damage may be incurred by rubbing/washing eye repeatedly -Pain d/t innervation by V1 (ophthalmic) branch of trigeminal (lack of pain indicates possible pathology); nasociliary branch mediates afferent limb of corneal reflex

Familial Dysbetalipoproteinemia

Presentation: -Severe hypertriglyceridemia w/ striate palmar xanthomas, grossly lipemic serum (milk/opalescent blood samples), recurrent episodes of pancreatitis Treatment/Management: -Fibric acid derivatives (fenofibrate)

Nephrotic Syndrome

Presentation: -Severe proteinuria, edema, fatigue, no hematuria, hypoalbuminemia, hyperlipidemia/lipiduria -Hypercoagulability (e.g. renal vein thrombosis most common, arterial thrombosis, PE) -Complications: protein malnutrition, iron-resistant microcytic hypochromic anemia (due to transferrin loss), vit D deficiency (increased urinary excretion of cholecalciferol-binding protein), hypothyroidism (decreased thyroxine-binding protein), infection, accelerated atherosclerosis (d/t HLD, hypercoagulability) Diagnosis: -24 hr urine sample (>3 g/day), serum C3, ANA, renal biopsy Treatment/Management: -Treatment of underlying cause -Management of HLD w/ statins Pathophysiology: -Altered permeability of glomerular membrane for proteins -Pediatric causes include minimal change disease -Adolescent/adult causes include FSGS (HIV, African American, Hispanic, heroin use), membranous nephropathy (hep B, NSAIDs, SLE), membranoproliferative GN (hep B/C), amyloidosis -Hypercoagulability due to increased urinary loss of antithrombin 3, altered levels of proteins C/S, increased platelet aggregation, hyperfibrinogenemia (increased hepatic synthesis), impaired fibrinolysis

Cryptosporidium

Presentation: -Severe watery diarrhea, low-grade fever, weight loss in pt w/ CD4 <180 -In immunocompetent, can cause traveler's diarrhea (watery/brown diarrhea) w/ malaise, nausea, abd pain, possible low-grade fever, usually resolves in 10-14 days Diagnosis: -Stool w/ modified acid-fast stain reveals oocytes -Nothing detected on routine stool O&P Treatment: -Supportive care, antiretroviral therapy to control HIV infection Pathophysiology: -Most commonly spread through contaminated drinking water (cyst contamination), animal contact, person-to-person contact -Intracellular protozoan, oocytes release protozoites that penetrate intestinal epithelial cells -> altered villous architecture

Humoral Hypercalcemia of Malignancy

Presentation: -Severe, rapid-onset, often symptomatic hypercalcemia (constipation/ileus, polyuria, nausea, fatigue, poor appetite, weakness, decreased reflexes, confusion, lethargy, "stones/bones/groans/psych overtones") -Signs c/w malignancy: anorexia/cachexia, weight loss, temporal wasting, dry cough, smoking hx, local pain, mass effects -Typically seen in advanced malignancy, confers poor prognosis -ECG: shortened QT Diagnosis: -Calcium often >14 (severe >12), suppressed PTH, elevated PTHrP, low or low-normal vitamin D, hypophosphatemia -Imaging to localize & identify tumor Treatment/Management: -Initial: aggressive hydration w/ isotonic saline, d/c of agents that can worsen hypercalcemia (e.g. diuretics), calcitonin for acute reduction of hypercalcemia -Long-term hypercalcemia reduction through bisphosphonates Pathophysiology: -Most common cause of PTH-independent hypercalcemia -Due to secretion of PTHrP (PTH-related protein) by tumor cells -> increased bone resorption, increased renal reabsorption of calcium, increased excretion of phos -PTHrP not able to induced 25-vitD to 1,25-vitD to degree that PTH is, so vit D usually low or low-normal -Often associated w/ SCC (NSCLC of lung, head & neck), renal, bladder, breast, ovarian carcinomas -Non-humoral causes of HoM: lymphoma (d/t overproduction of 1,25OH-vit D), lytic bone metastases (e.g. breast, MM, lymphoma mets), rarely d/t tumors secreting IL-6 or PTH

Aortic Dissection

Presentation: -Severe, sharp, tearing chest pain radiating to back/neck, episode of syncope -New aortic regurgitation murmur (decrescendo diastolic), asymmetric BP (+/- 20 mm Hg variation in SBP b/t arms) or pulses (only present in 20-30% of cases), CXR w/ widened mediastinum -Acute/transient increase in BP (e.g. cocaine, heavy lifting) may be precipitant -Labs: elevated D-dimer, acute anemia from blood extravasation, Cr may be elevated w/ renal artery involvement -Risk factors: hx HTN, Marfan, cocaine use, preexisting aneurysm, coronary catheterization (usually in R carotid sinus), age >60 -Complications from extension: stroke (carotid artery), acute aortic regurgitation (aortic root/valve, early diastolic murmur), Horner syndrome (carotid sympathetic plexus), myocardial ischemia/infarction (coronary artery ostia), pericardial effusion/tamponade (pericardium), hemothorax (pleural cavity), renal injury (renal arteries), abd pain (mesenteric arteries), lower extremity paraplegia (spinal arteries) Diagnosis: -Gold standard CT angiography (if hemodynamically stable) or transesophageal echo (preferred if hemodynamically unstable or renal insuff), alternatively MR angiography (avoided due to risk of nephrogenic systemic fibrosis/nephrotoxicity) -CXR w/ widened cardiac silhouette, CT w/ intimal flap or double aortic lumen -ECG normal or nonspecific ST & T wave changes Treatment: -Emergent surgical repair (esp type A/ascending tears) -IV beta-blockers to lower SBP (e.g. esmolol, labetalol, propanolol) to 100-120 & decrease LV contractility to reduce aortic wall stress -Pain control (e.g. morphine) +/- sodium nitroprusside if SBP >120 Pathophysiology: -Asymmetric pulse may occur from involvement of subclavian/iliac arteries

Necrotizing Malignant Otitis Externa

Presentation: -Severe/unremitting ear pain, worse w/ chewing & at night, cranial neuropathies (CN VII, X, XI) typically in elderly, immunocompromised patients (e.g. poorly controlled DM), hearing loss -Granulation tissue and erythema/edema in external auditory canal at bony cartilaginous junction, prominent discharge/otorrhea -Labs: elevated ESR, leukocytes may be normal -Risk factors: >60, T2DM, aural irrigation (cerumen removal), HIV & other immunosuppressive disorders -Complications: can progress to TMJ involvement, meningitis, brain abscess, osteomyelitis, CN palsies Treatment: -IV antipseudomonal abx (e.g. ciprofloxacin, prolonged abx for 6-8 wks, can be converted to oral therapy once ESR & CRP normalize) +/- surgical debridement if lack of response to abx -Other effective abx: anti-pseudomonal penicillins (e.g. piperacillin), aminoglycosides, 3rd gen cephalosporins (e.g. ceftazidime) -Hyperbaric O2 can be considered as adjunctive treatment for MOE refractory to above Pathophysiology: -Pseudomonas infection of the external ear in >95% of cases, osteomyelitis of external auditory canal and skull base -Pseudomonas expresses pro-inflammatory adhesins (leads to granulation tissue formation) & tissue-degrading proteases -Pain w/ chewing may indicate spread of infection to TMJ -Other causes include other bacteria & fungi (<5% of cases)

Cervical Radiculopathy

Presentation: -Shooting neck/shoulder/back pain & paresthesias that radiates to arms/hands w/ neck movement, sensory and/or motor deficits (weakness, decreased reflexes) that extend in a dermatomal/myotomal pattern -Lateral flexion & rotation of neck cause increased impingement -> worsening/reproduction of sx (Spurling test) -Shoulder abduction relief test: shoulder abduction relieves tension on impinged root; radicular sx improve w/ placement of hand on top of head -Most pts experience gradual resolution of sx -"5/6 pick up sticks, 7/8 lay they straight"--C5/6 control biceps, C7 controls triceps, C8 controls most finger movements Diagnosis: -Neuroimaging not required for dx if mild deficits w/ low risk of malignancy or infection -MRI in pts w/ severe/progressive/bilateral neuro deficits or high concern for malignancy or epidural abscess (e.g. fever, IV drug use, weight loss, progressive neuro deficits) -Needle electromyography Treatment/Management: -Sx management w/ NSAIDs, avoidance of provocative maneuvers, maintain moderate physical activity as able -Oral glucocorticoids may be used in pts w/ severe pain -Epidural glucocorticoid injections if still refractory Pathophysiology: -Most commonly due to compression of nerve root from disk herniation (acute onset) or progressive spinal spondylosis (subacute/progressive onset) -In cervical spondylosis, impingement may be due to osteophyte formation in facets & uncovertebral joints -> neural foraminal narrowing -Neck movement worsens nerve root impingement -> shooting pain

Suprascapular Nerve Entrapment

Presentation: -Shoulder pain, weakness of shoulder abduction (supraspinatus) & external rotation (infraspinatus), passive ROM normal -Tenderness at suprascapular notch, reproduction of pain at top of scapula w/ cross-body abduction of humerus -In severe cases, can lead to sensory deficits, atrophy of supraspinatus/infraspinatus -May develop from compression at suprascapular notch from excessive use of heavy backpack, direct trauma to shoulder, repetitive motion of shoulder (e.g. weight lifting, baseball) Treatment/Management: -NSAIDs, activity modification Pathophysiology: -Suprascapular nerve innervates supraspinatus/infraspinatus, originates from brachial plexus & traverses suprascapular notch below superior transverse scapular ligament

Parkinsonian Gait

Presentation: -Shuffling, hypokinetic gait w/ short steps -Bradykinesia, resting/pill-rolling tremor, postural instability, decreased arm swing Pathophysiology: -Parkinson disease, drug-induced parkinsonism, other basal ganglia lesions

Inahalational Burn Injury

Presentation: -Signs of smoke inhalational injury: hoarse voice, singed nasal hair/eyebrows, carbonaceous sputum, oropharyngeal inflammation, blistering/carbon deposits, stridor, carboxyhemoglobin >10%, hx of confinement in burning building -Cause of 60-80% of deaths during fire incidents Treatment/Management: -Empiric treatment for cyanide & carbon monoxide poisoning (supplemental O2, hydroxocobalamin) -Endotracheal intubation indicated in pts that may develop respiratory compromise from airway edema Pathophysiology: -Smoke injury -> glottic edema from heat & airway irritation due to particulate matter found in smoke -Major products of combustion in closed spaces are hydrogen cyanide (from combustion of nitrogen-containing synthetic compounds including paint/foam/cotton/silk) & carbon monoxide

Chronic Prostatitis/Chronic Pelvic Pain Syndrome

Presentation: -Similar sx to chronic bacterial prostatitis Diagnosis: -Urine culture results are aseptic (no bacteria), AU w/ leukocytes Treatment/Management: -Ideal approach to treatment not known; most pts receive combo of therapies including meds for prostate enlargement (e.g. alpha blockers), abx, anti-inflammatories, and/or psychotherapy Pathophysiology: -Underlying cause poorly understood

Anagen Effluvium

Presentation: -Similar to telogen effluvium, associated w/ widespread, acute hair loss, often after initiation of chemotherapy (usually beginning within 1-2 wks of initiation) -Common agents: antimicrotubules (e.g. paclitaxel), alkylating agents (e.g. cyclophosphamide), antibiotic chemotherapeutic agents (e.g. doxorubicin) Treatment: -Usually temporary, can be mitigated in some cases by scalp-cooling devices that reduce blood flow to follicles Pathophysiology: -Chemotherapeutic agents target rapidly dividing cells including follicular hair cells in anagen phase (linear growth phase)

Pityriasis Rosea

Presentation: -Single annular salmon-colored macule (herald patch, slowly increases in size and develops scaling) followed by development of multiple oval lesions on trunk & extremities, often in "christmas tree" distribution -Lesions initially erythematous, then desquamate w/ pruritus -May experience prodromal sx like fever, headache, malaise (nonspecific viral prodrome) -Usually affect adolescents to early adulthood -Usually resolves in 4-6 wks Treatment/management: -Reassurance (spontaneous resolution in wks to mos) -Treatment of pruritus (antihistamines, topical corticosteroids)

Ehler-Danlos Syndrome

Presentation: -Skin: transparent & hyperextensible, easy bruising, poor wound healing, velvety w/ atrophy & scarring (wide, atrophic, "cigarette paper-like" scars) -MSK: joint hypermobility (e.g. frequent dislocations), pectus excavatum, scoliosis, high arched palate -Cardiac: MVP (can lead to acute mitral regurg w/ rupture of chordae tendinae) -Other: abd & inguinal hernias, uterine prolapse, cervical insufficiency, spontaneous organ rupture if visceral subtype, berry aneurysms Diagnosis: -Genetic testing Pathophysiology: -AD mutations in COL5A1, COL5A2 (collagen)

Hepatic Encephalopathy

Presentation: -Sleep pattern changes, AMS (e.g. confusion, slurred speech, lethargy, disorientation), ataxia, asterixis (hand-flapping tremor) in pt w/ underlying cirrhosis/liver disease -Findings of cirrhosis (ascites, thrombocytopenia, varices, etc.) Treatment: -Correct precipitating cause (e.g. fluids, abx, electrolytes) -Pharmacologic agents to decrease blood ammonia concentration (nonabsorbable disaccharides like lactulose/lactitol, rifaximin) Pathophysiology: -May be precipitated by drugs (e.g. sedatives, narcotics), hypovolemia (e.g. diarrhea), electrolyte changes (e.g. hypokalemia), increased nitrogen load (e.g. GI bleed), infection (e.g. PNA, UTI, sepsis), portosystemic shunting (e.g. TIPS procedure) -Due to ammonia neurotoxicity from impaired liver fxn -Lactulose metabolized to short-chain fatty acids (e.g. lactic/acetic acid) in intestines -> intestine acidification, acts as trap for ammonia conversion to ammonium ("ammonia trap") -Lactulose also facilitates BMs, which helps w/ fecal nitrogen excretion

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Presentation: -Slowly progressive disorder >8 wks (vs. AIDP), presents w/ motor -> sensory signs, symmetric proximal (e.g. standing up, hip girdle) & distal (e.g. buttoning shirts, hand muscles) muscle weakness -LMN signs (atrophy, weakness, hyporeflexia) -Sensory loss, may affect vibration & position sense Diagnosis: -CSF w/ increased protein, normal WBCs -Nerve conduction studies w/ decreased conduction velocity (i.e. demyelination) -Nerve biopsy showing segmental demyelination Treatment/Management: -Glucocorticoids, IVIg, plasmapheresis Pathophysiology: -Immune-mediated demyelination of peripheral nerves & nerve roots -Not dependent on nerve length, preferentially affects large & thickly myelinated fibers (e.g. vibration, position sense)

Esophageal Stricture

Presentation: -Slowly progressive dysphagia limited to solid foods, usually w/o anorexia or weight loss -Can paradoxically lead to improvement of GERD sx (by blocking reflux) -Risk factors: GERD, erosive esophagitis, Barretts esophagus, radiation, systemic sclerosis, caustic ingestion Diagnosis: -Smoothly tapering, concentric narrowing of esophageal lumen on esophagography/Barium swallow -Should be biopsied in context of GERD/Barretts to rule out adenocarcinoma Treatment: -Esophageal balloon dilation

Nonclassic Congenital Adrenal Hyperplasia

Presentation: -Slowly progressive hyperandrogenism at puberty/adolescence and abnormal uterine bleeding/amenorrhea -Virilization uncommon, normal external genitalia at birth, usually presents w/ hirsutism w/ early pubic/axillary hair growth, severe acne, oligomenorrhea (irregular menses) -Increased growth velocity & bone age -Elevated 17-hydroxyprogesterone, testosterone, and DHEA -Sx may be masked by use of OCPs Diagnosis: -Exaggerated 17-hydroxyprogesterone response on ACTH stimulation testing Treatment: -Hydrocortisone Pathophysiology: -AR partial deficiency in 21 hydroxylase -> increased androgens, normal mineralocorticoid/glucocorticoid levels (vs. classic CAH, which present early w/ salt-wasting crisis; electrolytes & BP normal in nonclassical)

Diabetic Nephropathy

Presentation: -Slowly progressive renal disease in both T1DM & T2DM, often asymptomatic for yrs (usually 5+ yrs after T1DM diagnosis, may be present at diagnosis in T2DM) -Persistent microalbuminuria (30+ mg/day) and/or decreased GFR progressing to overt albuminuria (>300 mg/day) & nephrotic syndrome -HTN -Associated w/ concurrent proliferative diabetic retinopathy (PDR) Diagnosis: -Screening w/ 24-hr urine albumin, spot urine albumin, or urine albumin/Cr ratio; start screening at time of diagnosis for T2DM, 5 yrs after diagnosis for T1DM, start treatment w/ moderately increased albuminuria (urine albumin:Cr of 30-300) -Consider renal bx & additional evaluation if any of the following present: 1. early onset (<5 yrs after T1DM diagnosis), 2. rapid progression (>5-10x increase in albuminuria in <1-2 yrs, >5 mL/min/1.73m2 decline in GFR per yr), 3. active urinary sediment (e.g. white or RBC casts), 4. comorbid conditions associated w/ glomerulonephritis (e.g. SLE) -Start screening 5 yrs after T1DM diagnosis, at time of diagnosis for T2DM Treatment: -Intensive BP control (target <130/80), preferred w/ ACEi & ARBs (blocks angiotensin II-mediated efferent arteriole vasoconstriction -> decreased GFR & glomerular hydrostatic pressure -> slowing of glomerular capillary sclerosis) -SGLT2 inhibitors may also be considered in severe albuminuria >300 mg/g Pathophysiology: -Correlated w/ degree of PDR (retinal neovascularization); both considered chronic microvascular complications of DM -In DM, afferent arteriole dilation by natriuretic peptides & prostaglandins + efferent arteriole constriction -> state of hyperfiltration (increased GFR) -> glomerular hypertrophy & glomerular capillary sclerosis -> progression to incipient DN (mesangial expansion, GBM thickening, arteriolar hyalinosis, moderately increased albuminuria, HTN) -> progression to overt DN (mesangial nodules aka Kimmelstiel-Wilson lesions, overt proteinuria, nephrotic syndrome, decreased GFR) -Chronic hyperglycemia leads to impaired glomerular fxn d/t glycation of matrix/tissue proteins, increased matrix production, mesangial cell apoptosis

Cerebral Vein Thrombosis

Presentation: -Slowly worsening HA, focal neuro deficits (e.g. hemiparesis), seizures, confusion, N/V, bilat papilledema w/ retinal hemorrhages -Signs of increased ICP (HA worst when awakening, worse w/ valsalva-like maneuvers like coughing, sneezing) -Risk factors: OCPs, hypercoagulable states like pregnancy, malignancy, infection, head trauma Diagnosis: -Brain MRI w/ magnetic resonance venography (may demonstrate hemorrhagic foci or infarctions on imaging) Treatment/Management: -LMWH to recanalize obstructed sinus & prevent thrombus propagation -If pregnant, labor induction contraindicated d/t risk of raising ICP Pathophysiology: -Formation of blood clot within the dural sinuses, which drain CSF & venous blood from brain -> increased ICP

Idiopathic Guttate Hypomelanosis

Presentation: -Small macules in sun-exposed areas, associated w/ aging

Brown Recluse Spider Bite

Presentation: -Small ulcer at site of bite (usually spontaneously resolves), often initially painless and may go unrecognized or may be painful w/ burning sensation -Ulcer/blister may develop over several days w/ erythematous halo & necrotic center that progresses to eschar -Systemic loxoscelism (fever, hemolysis, DIC) uncommon, not correlated w/ lesion size -Most common in SW, S, & midwest US esp in dry/warm areas (e.g. attics, basements), most commonly on upper arm, thorax, inner thighs -Identification of causative spider rare b/c initial bite usually painless Treatment/Management: -W/ basic wound management, usually heal w/o scarring over several wks -Cleanse wound, apply ice, supportive management of systemic sx, pain control, update tetanus immunization -Debridement avoided in early stages of necrosis but may facilitate healing once lesion is stable & well-demarcated, may entail skin grafting Pathophysiology: -Venom contains phospholipases (thought to facilitate wound necrosis), activity slowed down by cold (thus cold compresses & ice)

Pseudofolliculitis Barbae

Presentation: -Small, painful papules caused by growth of hair shaft into perifollicular hair -Common in individuals w/ curly hair, may occur in beards and other areas where hair is shaved closely -Complications: hyperpigmentation, secondary bacterial infection, keloid formation -Risk factors: African American, use of multiblade razors (cut hair below skin surface, leave angled tip that predisposes) Treatment/Management: -Discontinuation of shaving (usually improves within wks) -Adjustment of shaving routine (single blade, warm compresses prior to shaving) -Alternatives: chemical depilatories, laser hair removal, topical eflornithine (slows hair growth), hair clippers (leaves hair longer) Pathophysiology: -Penetration of hair shaft into interfollicular skin, either through lateral wall of follicle (transfollicular penetration) or by curving down into skin after exiting follicle (extrafollicular penetration)

Keratosis Pilaris

Presentation: -Small, painless papules, roughened skin texture, mottled perifollicular erythema, "chicken skin" -Can occur anywhere, but most common on posterior surface of upper arms -Usually asymptomatic, but may occasionally present w/ pustules, pruritus, may be exacerbated by cold/dry weather -May occur alone or in association w/ atopic dermatitis or ichthyosis vulgaris, asthma Diagnosis: -Clinical -If skin bx: keratin plugs, dilated hair follicles, sterile lymphocytic infiltrate Treatment: -If necessary (usually asymptomatic), emollients & topical keratolytics (e.g. salicylic acid, urea) Pathophysiology: -Benign condition characterized by retained keratin plugs in the hair follicles

Focal Nodular Hyperplasia

Presentation: -Solid mass of liver (well-circumscribed, solitary lesion <5 cm w/ central/stellate scar surrounding large congenital arterial anomaly, enhancing/hyperdense w/ contrast due to high arterial flow), most common in women 20-50 yo -Usually asymptomatic otherwise and detected incidentally during imaging for other conditions, low risk of complications Diagnosis: -Triphasic, helical CT scan w/ uniform hyperdense lesion (i.e. filled w/ contrast during arterial phase = increased arterial flow) & central scar Treatment: -Observation; generally does not grow over time, no risk of malignant transformation or rupture Pathophysiology: -Benign, hyperplastic regenerative liver nodule associated w/ hyperperfusion by anomalous arteries

Major Depressive Disorder (MDD)

Presentation: -Somatic presentation w/ headaches, neck/back pain, fatigue -Adolescents may present with irritability rather than sad affect Diagnostic criteria: -At least 2 wks of at least 5 of depressed mood + SIGECAPS (change in Sleep, loss of Interest, Guilt, loss of Energy, decreased Concentration, change in Appetite, increased/decreased Psychomotor activity, Suicide) Treatment: -Antidepressants, 1st line SSRIs/SNRIs -Psychotherapy, esp CBT -Combo of both more effective than either alone Pathophysiology: -Hyperactivity of HPA axis w/ subclinical increased serum cortisol levels implicated in depression -Also associated w/ decreased hippocampal & frontal lobe volumes, changes in sleep architecture (decreased REM sleep latency, decreased slow wave sleep)

Central Cord Syndrome

Presentation: -Spinal injury that may occur w/ hyperextension (e.g. during intubation, MVC whiplash) in setting of cervical spondylosis -Presents w/ disproportionately greater motor impairment (weakness, reflex loss, e.g. triceps), sensory/pain/temp deficits in UEs > LEs -Similar presentation to syringomyelia (capelike distribution of sensory loss) -May be associated w/ bladder dysfxn Diagnosis: -Cervical myelogram often diagnostic w/ evidence of persistent cord compression -X-ray may show nonspecific cervical spondylosis Treatment/Management: -Glucocorticoids +/- surgical repair Pathophysiology: -Stenotic cervical spinal canal in setting of degenerative spinal joint changes + hyperextension -> compression of spinal cord b/t hypertrophied ligamentum flavum + bulging disc/osteophyte complex posteriorly -> damage to central spinal cord (grey matter) -Weakness from damage to alpha motor neuron cell bodies in anterior horn of spinal cord -Lateral spinal tracts running to sacrum & LEs generally spared (no bowel/bladder/LE sx)

Conduction Aphasia

Presentation: -Spontaneous, fluent speech w/ phonemic errors -Relatively preserved comprehension, very poor repetition Pathophysiology: -Lesion of arcuate fasciculus

Trigeminal Neuralgia

Presentation: -Stabbing, burning, electric shock-like pain over distribution of maxillary or mandibular division of trigeminal nerve, usually unilateral -Pain may be triggered by light touch or vibration/wind/chewing (e.g. brushing teeth, drinking cold water), typically lasts seconds to minutes -In MS, may present bilaterally -Can rarely involve V1 (ophthalmic branch) of trigeminal nerve -> autonomic findings (tearing, rhinorrhea) Treatment: -Carbamazepine, oxcarbazapine -If pharmacologic therapy unsuccessful, surgery (nerve decompression, radiosurgery) Pathophysiology: -In MS, due to demyelination of trigeminal nerve axons or root as it enters pons, likely due to localized compression (e.g. vascular structure) -> improper signaling, paroxysms of severe pain

Typhoidal Salmonella

Presentation: -Stepwise fever, bacteremia, abdominal pain, rose spots, relative bradycardia in wk 1 -Complications: hepatosplenomegaly, intestinal bleeding & perforation (esp w/ prolonged & untreated disease) -Potentially fatal Diagnosis: -Blood culture Treatment: -Ceftriaxone, drug resistance common Pathophysiology: -S. typhi or S. paratyphi -Most common in developing countries w/ poor sanitation (e.g. unvaccinated travelers) -Associated w/ contaminated food/water (fecal-oral transmission)

Spastic Ataxia

Presentation: -Stiff/rigid gait w/ circumduction (spastic leg is abducted & advanced while in extension, internal rotation), plantar flexion of affected limb Pathophysiology: -Pyramidal or corticospinal tract lesion

Cryptococcus Meningoencephalitis

Presentation: -Subacute (~2 wks) meningitis developing over wks, usually in immunocompromised pts (e.g. HIV w/ CD4 <100, can be more acute/severe) -HA from increased ICP d/t capsular swelling, fever, malaise, neck stiffness, N/V, AMS, abducens nerve palsy (lateral gaze palsy, diplopia), umbilicated skin papules (similar in appearance to molluscum contagiosum, d/t hematogenous dissemination, most commonly on head/neck, sometimes w/ areas of central hemorrhage or necrosis) -Can progress to coma Diagnosis: -Very elevated opening pressure on spinal tap (>250-300) -Low glucose, mildly elevated protein, WBCs <50 w/ mononuclear predominance -Transparent capsule on India ink stain, cryptococcal polysaccharide antigen +, culture on Sabouraud agar -If cutaneous lesions, lesion bx w/ histo staining w/ PAS and Gomori methenamine silver nitrate showing hyperplasia of overlying dermis w/ underlying granulomas surrounding encapsulated yeasts Treatment: -Initial treatment w/ IV amphotericin & flucytosine (2 wks), followed by maintenance treatment w/ fluconazole (high dose for 8+ wks for consolidation, low maintenance dose continued up to 1 yr, until CD4 >100 w/ ART) -Intrathecal amphotericin for pts that fail systemic therapy or develop adverse effects to IV therapy -Possible serial LPs to reduce increased ICP, ventricular drains -D/t risk of immune reconstitution syndrome, ART initiation generally delayed for ~2 wks after beginning treatment for crypto Pathophysiology: -Invasive fungal infection w/ cryptococcus neoformans -Elevated pressure due to progressive blockade of CSF flow by cryptococcal proteins (replicates in CNS & clogs arachnoid villi w/ yeast components & capsular polysaccharides) -> HA, N/V, visual changes, papilledema, CN palsies, risk for brain herniation -If recalcitrant despite serial LPs, consider ventriculoperitoneal shunt -Transmission by inhalation, lifelong latent infection in most pts but few symptomatic

Lung Abscess

Presentation: -Subacute fever, night sweats, weight loss, cough w/ putrid, foul-smelling sputum that develop over 1-2 wks -May have mild hyponatremia due to SIADH -Risk factors: dysphagia, substance use (EtOH), seizures, impaired consciousness (risk of aspiration, head trauma, anesthesia), poor dentition, gingival disease Diagnosis: -Imaging (CT) w/ cavitary infiltrates w/ air-fluid levels, often in superior lower lobe or posterior upper lobe (regions that are dependent w/ recumbency) -Cultures rarely useful (b/c anaerobes) Treatment: -Prolonged course of ampicillin-sulbactam, imipenem, or meropenem -Alternate: clindamycin (beta-lactam allergy) -Surgical intervention (pneumonectomy, lobectomy) in cases refractory to abx Pathophysiology: -Usually due to aspiration of oropharyngeal/gingival anaerobes (e.g. Peptostreptococcus, Fusobacterium, Prevotella) in setting of dysphagia or impaired consciousness -Pneumonitis -> PNA -> abscess/empyema

Optic Neuritis

Presentation: -Subacute unilateral vision loss (hrs to days, sx peak at ~2 wks), unilateral eye pain that is worse w/ movement, central scotoma, afferent pupillary defect (paradoxical pupillary dilation of affected eye w/ swinging flashlight test), "washed out" color vision -Unilateral optic disc swelling/edema due to papillitis -May be initial presentation of MS, most common in women <50 yo Diagnosis: -Visual evoked potential testing, MRI of orbits/brain -Fundoscopy usually normal (inflammation occurs behind optic nerve head) Treatment: -IV steroids, 35% of cases recur Pathophysiology: -Immune-mediated/inflammatory demyelination of optic nerve

Male Breast Cancer

Presentation: -Subareolar mass, skin/nipple dimpling, induration, ulceration, often detected at advanced stage -Risk factors: family hx (BRCA1/2), abnormal estrogen/androgen ratio (Klinefelter, obesity, cirrhosis, marijuana) Diagnosis: -Mammography -FNA or core bx (invasive ductal carcinoma, hormone-receptor+ most common)

Hyperventilation Syndrome

Presentation: -Subjective SOB w/ good SpO2, CTAB, deep breathing pattern punctuated by audible sighs -Tachypnea and/or increased tidal volume (deep breathing) -Neuro signs including paresthesias, HA, lightheadedness, carpopedal spasms (involuntary muscle contractions in the hands and feet) -Presentation may overlap w/ panic attacks (also present w/ palpitations, CP, nausea, diaphoresis, intense fear, etc.) Treatment: -First-line: breathing retraining--abdominal/diaphragmatic breathing to keep chest still -Second-line: if no improvement w/ above, small dose of short-acting benzo -No breathing into paper bag: can result in hypoxia Pathophysiology: -Intermittent episodes of hyperventilation w/o obvious cardiac or pulm etiology -Sx present when alveolar minute ventilation increases enough to decrease PaCO2 (may be due to tachypnea and/or due to increased tidal volume) -> cerebral vasoconstriction, resp alkalosis-induced hypocalcemia & hypophosphatemia

Hypertensive Hemorrhage

Presentation: -Sudden focal neuro findings that gradually worsen over mins to hrs -Lacunar stroke from severe HTN affecting small/penetrating arteries, most commonly vessels perfusing basal ganglia (putamen), cerebellar nuclei, thalamus, pons, cerebral cortex -If hemorrhage expands, can cause sx of increased ICP (HA, vomiting, seizures, AMS) -Basal ganglia: contralateral hemiparesis & hemisensory loss, homonymous hemianopsia, conjugate gaze deviation toward affected side -Thalamus: contralateral hemiparesis & hemisensory loss, nonreactive miotic pupils, upgaze pasy, conjugate gaze deviation toward affected side -Pons: deep coma (disruption of reticular activating system), total paralysis in minutes, pinpoint reactive pupils -Cerebellum: occipital HA (may radiate into neck/shoulders), neck stiffness (from extension of blood into 4th ventricle), N/V, nystagmus, ipsilateral hemiataxia of trunk (vermis) or limbs (hemispheres) Diagnosis: -Noncontrast head CT Treatment: -Surgical decompression

Vitreous Hemorrhage

Presentation: -Sudden loss of vision w/ floaters/shadows, hazy vision +/- red hue -Decreased or absent red reflex -Ophthalmoscopy w/ loss of fundus details, floating debris, dark red glow -Risk factors: most commonly diabetic retinopathy Treatment/Management: -For pts w/ underlying conditions, conservative treatment (e.g. upright position during sleep, enhances settling of hemorrhage) -Photocoag or vitrectomy in some cases

Pituitary Apoplexy

Presentation: -Sudden onset severe thunderclap HA, ptosis, vision changes (bitemporal hemianopsia, diplopia, ophthalmoplegia, anisocoria, ptosis), neck stiffness -Sx of adrenal crisis/panhypopituitarism (severe hypotension, distributive shock, hypoglycemia) -May be precipitated by anticoag use or acute hypotension, but usually no clear precipitating cause Diagnosis: -Head MRI more sensitive than CT; shows pituitary hemorrhage in bleeding etiology, pituitary enlargement if infarction Treatment/Management: -Adrenal crisis: rapid fluid resuscitation, IV glucocorticoid replacement (hydrocortisone, betamethasone) -Neurosurgical intervention, surgical decompression for persistent visual changes Pathophysiology: -Sudden bleeding into or infarction of enlarged pituitary adenoma (e.g. prolactinoma) -Eye/vision dysfxn due to compression of optic chiasm/CN III -Loss of ACTH/cortisol can precipitate adrenal crisis w/ hypotension

Retinal Detachment

Presentation: -Sudden onset vision loss, photopsia (flashes of light) w/ showers of floaters (spots in visual field), progressive visual field defect as detachment progresses -May be described as "curtain coming down" from the periphery (vs. dropping vertically in retinal artery occlusion) -Risk factors: metabolic disorders (e.g. T2DM), trauma (ocular surgery), vascular disease, myopia, degeneration, age 40-70 Diagnosis: -Fundoscopy w/ gray/elevated retina w/ folds and/or a tear, vitreous hemorrhage Treatment: -Surgical correction by retinopexy/vitrectomy typically required -Laser therapy & cryotherapy to create permanent adhesions b/t neurosensory retina, retinal pigment epithelium, & choroid Pathophysiology: -Separation of the layers of the retina -Inciting event usually mos before detachment presents (e.g. myopia, trauma) -> fluid seepage b/t retinal breaks -> layer separation

Drug-Induced Hemolytic Anemia

Presentation: -Sudden onset within hrs-days of exposure to drug -Acute anemia (fatigue, pallor, dyspnea) -Hemolysis (jaundice, dark urine, back/abd pain, splenomegaly) -Labs: normocytic anemia, increased reticulocytes & LDH & indirect bili, decreased haptoglobin, spherocytes on peripheral smear, + direct Coombs test (anti-IgG, anti-C3) Treatment: -Discontinue offending drug (usually leads to resolution within days), transfusion if severe, +/- glucocorticoids & IVIg Pathophysiology: -Commonly involves penicillins, cephalosporins -Drug coats erythrocytes (acts as hapten) -> IgG binding -> splenic destruction of RBCs (extravascular hemolysis, partial destruction -> spherocytes) -Drug triggers immune complex formation -> complement-mediated RBC destruction (intravascular hemolysis)

Anomalous Aortic Origin of Coronary Artery (AAOCA)

Presentation: -Sudden syncope in young adult, exertional angina, lightheadedness -Cause of sudden cardiac death (usually d/t ventricular tachycarrhythmia), sometimes w/o any premonitory sx -Resting ECG usually unremarkable Diagnosis: -Sometime made by TTE (but can miss or inaccurately characterize AAOCA) -Best made by CT coronary angiography or coronary magnetic resonance angiography Pathophysiology: -most commonly d/t left main coronary artery originating from right aortic sinus OR right coronary artery originating from left aortic sinus -Defect creates sharp curvature of anomalous artery, making it less amenable to high-volume flow -Anomalous artery also passes b/t aorta & pulm artery, making it susceptible to external compression during exercise

Central Retinal Artery Occlusion

Presentation: -Sudden, painless loss of vision in one eye ("shade descending") w/ temporal sparing -Possible h/o of amaurosis fugax -Usually leads to permanent visual deficits, defect in afferent pupillary reflex (complete or relative) -Can cause irreversible retinal damage within 90-100 min -Ophtho emergency Diagnosis: -Fundoscopy w/ pallor of optic disc (vs. diffuse hemorrhage in central retinal vein occlusion, whitened d/t edema), cherry red fovea, boxcar segmentation of blood in retinal veins Treatment/Management: -Urgent ophtho consult w/ interventions to decrease IOP: anterior chamber paracentesis, ocular massage (to lower IOP), IV acetazolamide/mannitol -Revascularization by intraarterial thrombolytics to attempt to recover vision -Noninvasive imaging of carotids to evaluate for stenosis -Atherosclerotic management (aspirin, statins, long-term anticoag if cardioembolic in origin) Pathophysiology: -Most commonly d/t embolized plaque from ipsilateral carotid artery or cardioembolic source (e.g. AFib) -Embolus travels from internal carotid artery -> ophthalmic artery -> central retinal artery -Other etiologies: carotid dissection, clotting disorders & hematologic diseases (e.g. SCD, hypercoag), vasculitis (e.g. giant cell arteritis), small artery disease d/t DM and/or HTN

Central Retinal Vein Occlusion

Presentation: -Sudden, painless, unilateral vision loss -Fundoscopy w/ disk swelling, venous dilation & tortuosity, retinal hemorrhages, cotton wool spots, "blood & thunder" appearance (due to diffuse hemorrhage & disc edema) -Can progress to become painful (ischemic form) -Risk factors: HTN, coagulopathy, hyperviscosity, chronic glaucoma, atherosclerotic risk factors (age, T2DM, HTN) Diagnosis: -Fluoroscein angiography Treatment/Management: -If no significant macular edema or neovascularization, managed conservatively w/ close observation -If significant macular edema, intravitreal injection of VEGF inhibitors Pathophysiology: -Unlike central retinal artery occlusion, usually d/t nonembolic causes

Gallstone Pancreatitis

Presentation: -Sudden-onset epigastric pain radiating to back -Gallstones on imaging (usually US) -Elevated amylase + lipase, ALT >150 has high PPV for predicting gallstone pancreatitis -Complications: acute cholangitis Diagnosis: -RUQ US, ERCP if US inconclusive and still suspect common bile duct involvement Treatment/Management: -During acute episode, supportive care w/ pain control, IV fluids, NPO, ERCP w/ stone extraction, biliary stent placement, and/or sphincterotomy indicated if evidence of cholangitis, visible common bile duct dilation/obstruction, increasing liver enzyme levels -Due to risk of recurrent episodes, lap cholecystectomy later once medically stable (ideally within same hospitalization, within 7 days) -If severe disease w/ organ failure, interval cholecystectomy Pathophysiology: -2nd most common cause of pancreatitis (after EtOH abuse) -Occurs when gallstone passes through biliary tree and obstructs ampulla or flow from pancreatic duct, allowing bile to reflux into pancreas

Pulmonary Embolism

Presentation: -Sudden-onset pleuritic chest pain, dyspnea, tachypnea, tachycardia, cough, hemoptysis, low-grade fever, LE swelling/pain if associated w/ DVT, JVD/mildly elevated JVP, possible soft holosystolic murmur d/t tricuspid regurgitation from elevated R-sided pressures -CXR may be normal or show atelectasis, infiltrates, ipsilateral exudative pulm effusion, Westermark sign (peripheral hyperlucency due to oligemia), Hampton hump (peripheral wedge-shaped lung opacity on CXR or peripheral hemispherical consolidation on CT due to pulm infarction), Fleischner sign (enlarged pulm artery) -ECG: classic finding is S1Q3T3 (prominent S in lead 1, Q wave & inverted T wave in lead 3), may present w/ new-onset AFib (irregular RR intervals, absent P waves, narrow QRS) due to increased RA pressure, new onset RBBB, T inversions in II/III/aVF indicating R heart strain -Hypoxia & AFib are poor prognostic factors -Complications: pulm infarct -Risk factors: sedentary lifestyle (e.g. truck driver), recent immobilization/illness, HIV, hemoconcentration (e.g. dehydration) -Labs: mild leukocytosis, respiratory alkalosis Diagnosis: -CTA (shows pulmonary artery filling defect), VQ scan (2nd choice if renal impairment, morbid obesity, contrast allergy, etc.) -D-dimer if low pretest probability (cannot be used during pregnancy, not to be used if high pretest probability d/t unacceptably high rate of FNs) -Pulm artery catheterization shows increased R-sided pressures (elevated RA & pulmonary artery pressure, low/normal PCWP) reflecting pulm HTN -Bedside Echo: evidence of RV dysfxn,, decreased RV contractility, presence of RV thrombus, pulm HTN -> tricuspid valve regurg Treatment: -Anticoag w/ direct factor Xa inhibitors preferred (e.g. rivaroxaban, apixaban) -Thrombolytic therapy reserved for pts that are hemodynamically unstable (SBP <90) & low bleeding risk (other indications: severe RV dysfxn, large clot burden, e/o free R-sided cardiac thrombus) -Embolectomy (perc or surgical) if shock likely to cause death within hrs or failed thrombolysis (or thrombolysis contraindicated) w/ persistent hypotension -IVC filter if anticoag contraindicated or ineffective w/ low cardiopulmonary reserve

Frostbite

Presentation: -Superficial extremity pallor & anesthesia, blistering/eschar formation, deep tissue necrosis & mummification -Skin typically pale/grayish and stiff/waxy/hard (after thawing/reheating becomes red/purple & pliable) -Most commonly affects face, ears, or distal limbs Diagnosis: -Angiography or technetium-99m scan to assess for thrombosis if warming unsuccessful (e.g. persistent sensory loss, grayish coloration despite reheating) Treatment/Management: -Rapid rewarming in water bath at 37-39 (98.6-102.2), systemic rewarming w/ warm IV fluids, warm blankets -Analgesia & wound care -Thrombolysis in severe, limb-threatening cases (tPa, may prevent need for amputation if administered within 24 hrs of injury) -Amputation last resort if thrombolysis/angiography unsuccessful, after affected tissues have been given time to fully demarcate (wks-mos) Pathophysiology: -Freezing of tissues -> disruption of cell membranes, ischemia, vascular thrombosis, inflammatory changes

Hereditary Angioedema

Presentation: -Swelling (facial/periorbital/circumoral, extremities, genitalia) & nonpitting, asymmetric edema w/o urticaria or pruritus, usually presents in late childhood or adolescence after dental procedure, stress, or minor trauma (or w/o trigger) -Laryngeal edema -> can cause laryngospasm & airway obstruction -Colicky abd pain, vomiting, diarrhea, ascites due to bowel wall edema Diagnosis: -Low C4 level, low C1 esterase inhibitor protein or fxn Treatment: -If emergent airway obstruction, SQ epi administration (tracheostomy if resistant) -C1 inhibitor concentrate, avoidance of triggers (e.g. ACE inhibitors, can substitute ARBs b/c they do not affect bradykinin system) -Bradykinin antagonists (icatibant) or kallikrein inhibitors (ecallantide) for resistant cases Pathophysiology: -AD defect/deficiency of C1 inhibitor (downregulates bradykinin) -> excessive bradykinin -> fluid extravasation into skin & mucosal tissues (e.g. bowel wall, upper airway)

Achilles Tendinopathy

Presentation: -Swelling, warmth, pain at posterior heel, tenderness 2-6 cm proximal to tendon insertion -Risk factors: athletic activity (esp running/jumping sports), sudden increase in activity, systemic disorders (e.g. psoriasis, ankylosing spondylitis), meds (e.g. glucocorticoids, FQ abx) Diagnosis: -Clinical, US may show swelling & neovascularization -MRI Treatment/Management: -Acute: activity modification, ice, NSAIDs -Chronic: eccentric resistance exercises Pathophysiology: -Most pain occurs at region proximal to tendon insertion, where perfusion of gastrocnemius/Achilles tendon complex is lowest

Nocardiosis

Presentation: -Sx of PNA (similar to TB), w/ fever, weight loss, night sweats, malaise, dyspnea, cough, pleurisy -CNS involvement--can cause brain abscesses, seizures -Cutaneous involvement, lesion resembling bacterial abscesses (nonpainful, may form draining sinus tracts, most commonly feet/legs/back) -Lung imaging w/ nodular/cavitary lesions in upper lobes Diagnosis: -Gram+ rod (branching/beaded/filamentous), partially acid-fast, aerobic -Sputum culture often negative (& require >4 wks to incubate), bronchoscopy w/ BAL more sensitive -Susceptibility testing necessary; many strains resistant Treatment: -SMX-TMP, may be combined w/ amikacin in severe disease (requires mos of treatment, relapse common) -Surgical drainage of abscesses, carbapenems added for better coverage w/ CNS involvement -Treatment usually 6-12 mos in duration Pathophysiology: -Epidemiology: endemic in soil, contracted by inhalation or skin puncture (e.g. gardening), usually seen in elderly/immunocompromised -Branching/filamentous structure helps prevent organism phagocytosis; makes host dependent on cell-mediated immunity

Malignant Pleural Effusion

Presentation: -Sx of effusion (e.g. SOB) & malignancy (e.g. weight loss) -Very poor prognostic sign regardless of tumor type w/ median survival fo 4 mos (advanced/incurable disease) Diagnosis: -Thoracentesis w/ exudative pleural fluid containing malignant cells Treatment/Management: -Palliative care: initial management w/ large-volume thoracentesis, repeat thoracentesis if effusion reaccumulates slowly & very short expected survival, chemical pleurodesis if effusion reaccumulates rapidly (<1 mo) or if longer expected survival, placement of indwelling pleural drainage catheter (high rate of adverse events) Pathophysiology: -May be seen in NSCLC including adenocarcinoma (considered stage IV disease -> advanced, incurable)

Primary Hypoparathyroidism

Presentation: -Sx of hypocalcemia: perioral tingling/numbness, muscle cramps, tetany, carpopedal spasms, seizures, QT prolongation Treatment/Management: -Vit D supplementation (usually calciferol), calcium -Thiazide added if persistent hypocalcemia w/ high urinary calcium Pathophysiology: -Etiologies: post-surgical (most common cause during thyroidectomy or sub-total parathyroidectomy), autoimmune, congenital absence or maldevelopment of parathyroid glands (e.g. DiGeorge syndrome), defective calcium-sensing receptor, non-autoimmune infiltrative destruction (e.g. hemochromatosis, Wilson disease, neck irradiation)

Nelson Syndrome

Presentation: -Sx of pituitary macroadenoma (e.g. bitemporal hemianopsia) w/ h/o treated Cushing disease -Hyperpigmentation secondary to ACTH excess Diagnosis: -Head MRI, plasma ACTH Treatment/Management: -Surgery and/or pituitary radiation Pathophysiology: -Enlargement of existing ACTH secreting pituitary adenoma after bilateral adrenalectomy for refractory cushing disease (d/t lack of negative feedback by cortisol) -Tumor usually aggressive, rapidly growing & require resection

Orbital Cellulitis

Presentation: -Sx of preseptal cellulitis (eyelid erythema/swelling, chemosis) + pain w/ EOM, proptosis, and/or ophthalmoplegia w/ diplopia, other visual changes like poor visual acuity -Leukocytosis, fever -Complications: permanent visual impairment (optic nerve involvement), intracranial extension (infection, cavernous sinus thrombosis) Diagnosis: -Clinical, CT if diagnosis uncertain Treatment: -IV abx +/- surgery (drainage if abscess present) Pathophysiology: -Serious bacterial infection posterior to orbital septum and involving muscles of EOM, usually resulting from contiguous extension of another source of infection (e.g. sinusitis esp of ethmoid/maxillary sinuses, dental abscess of maxillary teeth, preseptal cellulitis)

Hypovolemic Hyponatremia

Presentation: -Sx/signs of hypovolemia: low water intake/output, orthostatic hypotension, dry mucous membranes, poor skin turgor, tachycardia Diagnosis: -Urine & serum Osms, urine Na+ -May be associated w/ prerenal azotemia Treatment/Management: -Infusion of NS, careful repletion to prevent osmotic demyelination syndrome Pathophysiology: -If UNa >20, renal losses (e.g. diuretics, mineralocorticoid def d/t adrenal insuff) -If UNa 20 or lower, extrarenal losses (e.g. diarrhea, vomiting, burns, pancreatitis, sweating, etc.) -Hypovolemia -> increased ADH secretion -> water retention, dilution of serum contents -> hypotonic, hypovolemic hyponatremia

Physiologic Gynecomastia

Presentation: -Symmetric, firm, bilat enlargement of breast tissue concentrically under nipple areolar complex in neonates, adolescent boys, & older (esp obese) men -Circumscribed vs. pseudogynecomastia (diffuse breast enlargement d/t fat deposition) Diagnosis: -Rule out other causes (hepatic/renal disease, meds, measure TSH, testosterone, estrogen, prolactin, LH, bCG) -Benign exam above & no biochemical findings c/w other cause Pathophysiology: -Benign glandular proliferation of male breast tissue -In older men, related to imbalance of estrogen to testosterone due to decreased testicular testosterone production & increased conversion of testosterone to estrogen by aromatase in peripheral (mainly adipose) tissue

Distal Symmetric Polyneuropathy

Presentation: -Symptoms beginning in toes/feet & progressing more proximally over time, "stocking-glove" distribution -Distal numbness, tingling, pins & needles or burning sensation (more common w/ acute causes like meds/toxins) -Loss of pain, temp, touch/vibration sense -Decreased ankle/Babinski reflexes, intact motor strength Treatment: -Treat underlying cause -Pain management w/ gabapentin, TCAs, SNRIs (duloxetine), capsaicin cream Pathophysiology: -Damage to distal sensory peripheral nerve axons -Etiologies: T2DM (diabetic neuropathy), long-standing HIV infection, uremia, meds (FQs, metronidazole, dapsone, digoxin, amiodarone, cisplatin, paclitaxel), toxins (EtOH, heavy metal exposure) -If acute onset, more likely to be d/t meds or toxins

Sick Sinus Syndrome

Presentation: -Syncopal episodes in elderly pt -Dyspnea, fatigue, dizziness/lightheadedness -Arrhythmia in which bradycardia (sinus brady, sinus pause/arrest, SA nodal exit block) alternates with tachycardia (bradycardia-tachycardia syndrome), may occur w/ atrial arrhythmias (e.g. AFib), palpitations Diagnosis: -ECG w/ sinus bradycardia, sinus pauses w/ delayed P waves, dropped P waves w/ junctional escape beats (SA nodal exit block), can lead to sinus arrest -Exercise testing demonstrating chronotropic incompetence (inadequate HR response to exercise) Treatment: -Often requires pacemaker +/- rate-controlling meds like beta-blockers (if persistent paroxysmal tachyarrhythmias after placing pacemaker) Pathophysiology: -Inability of SA node to generate adequate HR -Often largely due to age-related cardiomyocyte degeneration & fibrosis of SA node & conduction system -Other contributing factors include ischemia, infiltrative disease (e.g. amyloidosis, sarcoidosis)x

Systemic Mastocytosis

Presentation: -Syncope, flushing, hypotension, abd pain, urticaria, hepatosplenomegaly -Rash usually urticarial, can also be maculopapular/vesicular/pustular and generalized -Darier's sign: rubbing edge of rash produces wheals & erythema Diagnosis: -Elevated tryptase (mast cell biomarker) Pathophysiology: -Excessive histamine release by mast cells

Congenital Long QT Syndrome

Presentation: -Syncope, sudden cardiac death during periods of rapid HR and high sympathetic activity (e.g. exercise) -ECG w/ prolonged QTc (>460 ms) that predisposes to torsades de point & polymorphic VTach -Romano-Ward inherited AD, Jervell & Lang-Nielson inherited AR & associated w/ sensorineural hearing loss (profound bilateral deafness) Treatment: -Avoidance of exacerbating factors (hypomagnesemia, hypokalemia), QT-prolonging meds (class IA antiarrhythmics like quinidine, class III antiarrhythmics like ibutilide, sotalol), adrenergic surges (e.g. exercise, sudden arousal) -Nonselective beta blockers (propanolol, nadolol) to dampen sympathetic activity & shorten QT interval -Implantable cardioverter-defibrillator placement or L cardiac sympathetic denervation in high risk pts Pathophysiology: -Impaired fxn of voltage-gated potassium channels that control delayed rectifier K+ potential and allow for repolarization of cardiomyocyte action potential

Immune Reconstitution Inflammatory Syndrome (IRIS)

Presentation: -Syndrome that results from rapid restoration of pathogen-specific immune responses to opportunistic infections; most often occurs after starting antiretroviral therapy (days to wks after starting) -Presents w/ worsening of infections after retroviral initiation d/t worsening inflammation (often after initial improvement of presenting sx), immune system improvement -May present as worsening of initial infectious sx (e.g. dyspnea, pulm infiltrate, fever, cough) -Risk factors: concurrent TB & HIV, low initial CD4, high initial viral loads Treatment/Management: -Usually transient state; supportive care w/o alteration to antiretroviral & other infectious treatment -Short course of PO corticosteroids may be option in pts w/ bothersome sx Pathophysiology: -Renewed ability to recognize/respond to foreign antigens -> overabundant inflammatory response to ongoing infection -> paradoxical worsening of infectious/inflammatory sx

Pancreatic Adenocarcinoma

Presentation: -Systemic sx (anorexia, weight loss) in >85%, abd/back pain (80%), jaundice (56%), pruritus, pale stools, dark urine -Recent-onset atypical DM, unexplained migratory superficial thrombophlebitis (Trousseau sign) -Hepatomegaly, ascites w/ mets -Double duct sign (dilation of both common bile duct & pancreatic duct, suggests advanced neoplasia), Courvoisier sign (distended, nontender gallbladder) -Labs: cholestasis (hyperbili, high alk phos, moderately elevated transaminases), increased CA19-9 -Risk factors: SMOKING, hereditary pancreatitis (FDR w/ pancreatic cancer, germline mutations, e.g. BRCA1, BRCA2, Peutz-Jeghers), chronic nonhereditary pancreatitis (EtOH, smoking, etc.), obesity, lack of physical activity, male, African American, age >45 (mean age 55) Diagnosis: -Abd US w/ common bile duct dilation (usually performed in pts w/ jaundice) -Abd CT (if abd US is inconclusive or in pts w/o jaundice w/ suspicion of panc carcinoma) Treatment/Management: -Poor overall prognosis, palliative care usually preferred (e.g. endoscopic stent placement to relieve CBD obstruction, surgical bypass if stenting technically difficult) Pathophysiology: -If tumor in pancreatic head (60-70%)-> pancreatic & common bile duct compression (presents w/ intrahepatic and extrahepatic biliary dilation) -> painless, obstructive jaundice, steatorrhea, possible epigastric pain -If tumor in body/tail -> progressive, constant abd & back pain (insidious, "gnawing," worse at night) w/o jaundice -Pancreas innervated by splanchnic nerves (T5-T10), tumors that irritate visceral fibers can cause referred thoracic back pain -High mortality (~98%) b/c usually diagnosed late -New-onset T2DM may be due to paraneoplastic syndrome w/ adrenomedullin secretion -> beta cell dysfxn

Hepatocellular Carcinoma (HCC)

Presentation: -Systemic sx (weight loss, fever, early satiety, cachexia/temporal wasting) -Rapidly enlarging liver mass -Signs of decompensated liver failure: chronic hepatitis/cirrhosis, SHIFTING ABD DULLNESS (ascites, usually bloody), hepatomegaly (possible palpable nodule), jaundice, hypoalbuminemia, variceal bleeding -Imaging w/ dense, irregular liver mass -May metastasize to spine (multiple osteoblastic/osteolytic vertebral lesions, back pain) -Labs: hypoalbuminemia, mildly elevated LFTs, thrombocytopenia, hyperbili, alk phos elevated if metastatic spinal lesions -Risk factors: EtOH abuse, viral hepatitis (esp hep B, most common in Asia/Africa/Middle East due to higher incidence of hep B/C), NAFLD (T2DM, obesity), cirrhosis (1-8% risk per yr), environmental toxins (aflatoxin, betel nut chewing) Diagnosis: -Elevated alpha feto-protein (only in 50% of cases, cannot be used to rule out), normal CEA/CA 19-9 -Liver mass that enhances w/ contrast on triple phase arterial contrast CT (often w/ satellite lesions) -Abd US to evaluate for ascites, clots in portal/hepatic vascular system, liver masses (recommended every 6 mos in all pts w/ cirrhosis due to high risk of developing HCC) Pathophysiology: -Tumor w/ hepatocyte origin

Pericardial Effusion

Presentation: -Tachycardia, tachypnea, distant/muffled heart sounds, enlargement of cardiac silhouette on CXR -Infants may present w/ decreased appetite, vomiting -Older children may present w/ pericardial friction rub, pleuritic chest pain Treatment: -If signs of hemodynamic compromise, effusion should be drained Pathophysiology: -Inflamed pericardial cells produce fluid -If recent cardiac surgery, may be due to immune-mediated inflammatory damage to pericardium (postpericardiotomy syndrome) -Large effusions can compress heart and impair cardiac filling -> decreased cardiac output -> tachycardia, tachypnea

Plantar Fasciitis

Presentation: -Tenderness at insertion of plantar fascia (anteromedioplantar region of heel/hindfoot) -Pain w/ passive dorsiflexion of toes, active FROM of ankle w/o pain, pain nonradiating w/ negative Tinel sign (vs. tarsal tunnel syndrome) -Pain worse w/ weight bearing, esp after prolonged rest (in morning) and prolonged standing/walking -Intact sensation -Presence of heel spurs on imaging has low sensitivity/specificity (calcaneal spurs/calcifications are common incidental finding, do not usually require treatment) -Risk factors: pes planus, obesity, working or exercising on hard surfaces, middle-aged, high-impact exercise, extended periods of walking barefoot Diagnosis: -Clinical; imaging not necessary unless other pathology expected or unless pain does not improve after treatment (US/MRI may show fascial thickening) Treatment: -Activity modification (avoid walking barefoot, reduce high-impact exercise), stretching exercises/physical therapy (fascial stretching, muscle strengthening exercises), heel pads/orthotics (reduce strain on fascia) -80% recover within 1 yr Pathophysiology: -Degenerative condition of plantar aponeurosis at calcaneal insertion caused by overuse (e.g. running, prolonged standing on hard surfaces, conditions that place excessive stress on arch like obesity/flat feet) -Heel spurs (calcifications in proximal fascia) are late sign of inflammation & degeneration in plantar fascia, but not a cause of pain

Bullous Pemphigoid

Presentation: -Tense, bullous, pruritic lesions on erythematous base (may rupture to form erosions) involving trunk & flexural areas of extremities, groin, axilla, inguinal folds; usually seen in older adults (>60) -Usually preceded by prodrome of eczematous/urticarial/pruritic lesions -Mucus membrane involvement in a small portion of pts -Increased incidence in pts w/ malignancy, neuro disorders (e.g. Parkinson, MS, dementia), depression, bipolar disorder Diagnosis: -Skin bx w/ light microscopy showing subepidermal cleavage, direct IF microscopy w/ linear C3 deposits & IgG along basement membrane against hemidesmosomes -Serology w/ abs to bullous pemphigoid antigens (hemidesmosomes) Treatment: -High-potency topical glucocorticoid (e.g. clobetasol) -Systemic glucocorticoids not more effective, may be used if topical agents not practical (e.g. extensive involvement) -Doxycycline Pathophysiology: -Autoimmune disease, IgG antibodies against hemidesmosome & basement membrane zone -Ab binding -> complement activation -> inflammatory mediators -> blister formation

Dupuytren Contracture

Presentation: -Thickening of palmar fascia at 3rd, 4th, 5th digits -Discrete/hard nodules/bands along flexor tendons near distal palmar crease, puckering of skin at base of MCPs -Normal ROM in early disease, decreased digit extension at MCPs and PIPs in -Risk factors: male, >50 yo, family hx, T2DM, tobacco & EtOH use, Northern European ancestry, manual work (e.g. gardening) Diagnosis: -Clinical, no imaging Treatment/Management: -Modification of hand tools (e.g. cushion tape, padded gloves) -Needle aponeurotomy (disruption of cords) -Intralesional corticosteroid injection -Surgery for contractures or severe disease Pathophysiology: -Progressive fibrosis of palmar fascia due to fibroblast proliferation & disordered collagen deposition -> initial painless puckering of skin proximal to MCP joint -> continued fibrosis leads to pathognomonic fascial nodules along flexor tendons -> progress to palpable fibrotic cords

Antithrombin Deficiency

Presentation: -Thrombophilia that is usually acquired (e.g. DIC, cirrhosis, nephrotic syndrome), very rarely inherited

TSH-Secreting Pituitary Adenoma

Presentation: -Thyrotoxicosis, diffuse goiter, possible mass effect sx (e.g. HA, visual field defects) -Labs: elevated or inappropriately normal TSH, elevated T3 & T4, elevated alpha subunit in 85% of pts, elevation of other pituitary hormones, minimal/no suppression of TSH by exogenous thyroxine, elevated sex hormone-binding globulin, normal thyroid hormone receptor beta gene Diagnosis: -Pituitary mass on MRI Treatment: -Somatostatin analogs, transsphenoidal surgery

Ehrlichiosis

Presentation: -Tick bite followed by flu-like illness (high fever, HA, myalgias, chills), neuro sx (e.g. confusion, AMS, clonus, neck stiffness) -Rash uncommon (<30%): "RMSF without the spots" -Transmitted by lone star tick in southeastern and south central US -Labs: thrombocytopenia, leukopenia, elevated LFTs & LDH Diagnosis: -Intracytoplasmic morulae in monocytes -PCR for E. chaffeenis/ewingii Treatment: -Doxy while testing pending Pathophysiology: -Primary reservoir is white tail deer

Locked-In Syndrome

Presentation: -Total paralysis of limbs, inability to speak/swallow, retained cognition and awareness (can only communicate w/ eyes), exaggerated DTRs -Preservation of supranuclear ocular pathway -> can blink voluntarily, look vertically Pathophysiology: -Ischemic/hemorrhagic stroke of brainstem

Todd Paralysis

Presentation: -Transient, self-resolving hemiplegia following seizure activity (focal motor seizure) -Usually preceded by sx typical of seizure activity (e.g. LOC, limb jerking) -Associated w/ postictal confusion (vs. pediatric stroke) -Not associated w/ aphasia (vs. pediatric stroke) -Normal head imaging Diagnosis: -Head imaging (CT or MRI) to rule out other causes of hemiparesis, including intracranial hemorrhage & pediatric stroke Treatment: -Supportive (typically resolves within 36 hrs) Pathophysiology: -Focal weakness or paralysis after focal-onset seizure that may become generalized

Spinal Epidural Abscess

Presentation: -Triad of focal/severe back pain ("shooting," "electric"), fever, neurologic deficits (motor/sensory findings in LEs) -Focal weakness, bowel/bladder incontinence -Typical progression of sx: focal back pain -> nerve root pain (shooting, electric shock sensation) -> motor weakness, sensory changes (e.g. paresthesias, radiculopathy), bowel/bladder dysregulation -> paralysis -Labs: elevated ESR, CRP -Risk factors: compromised immunity d/t HIV, T2DM, alcoholism, etc., inoculating source such as distant infection (e.g. cellulitis, joint/bone, endocarditis), spinal procedure (e.g epidural catheter, steroid injections, epidural anesthesia), injection drug use Diagnosis: -Spinal MRI, blood & aspirate cultures -CT myelography in pts unable to get MRI Treatment: -Broad-spectrum abx (vancomycin + ceftriaxone) -Aspiration & surgical decompression Pathophysiology: -May be caused by hematogenous dissemination (e.g. IV drug abuse, cellulitis, endocarditis), contiguous spread from vertebral osteomyelitis or other infection, direct inoculation (e.g. epidural analgesia) -S. aureus in 65% of cases, may develop from S. aureus or gram- bacilli bacteremia

Plummer-Vinson Syndrome

Presentation: -Triad of iron deficiency anemia, dysphagia, esophageal web -Insidious onset of dysphagia in pt w/ underlying metabolic abnormalities (e.g. iron def) Diagnosis: -Esophageal web seen on barium esophagram or upper endoscopy Treatment: -Iron supplementation (webs usually improve w/ repletion)

Latent Tuberculosis Infection

Presentation: -Tuberculin skin test w/ 10+ mm induration at 48 hrs, negative CXR, no sx (e.g. weight loss, night sweats, chronic cough) -Considered noninfectious -Lifetime risk of advancing to active TB small (5-10%) -CXR may show e/o upper lobe fibronodular disease & calcified granulomas from prior active infection (but no signs of active infection, e.g. lymphadenopathy, cavitations, infiltrates, effusion) Treatment/Management: -Offered to affected pts w/ high risk of developing active TB (immunosuppressed) or work/high-risk congregate settings (e.g. healthcare personnel, inmates, etc.) -Isoniazid & rifapentine weekly for 3 mos under direct observation (not recommended in pts w/ HIV) -Isoniazid monotherapy for 6-9 mos -Rifampin for 4-6 mos -Pyridoxine added to prevent neuropathies in some pts taking isoniazid -No work restrictions b/c noninfectious

Pancreatogenic Diabetes

Presentation: -Typical findings of DM in pt w/ pancreatic insufficiency (e.g. CF, chronic pancreatitis, pancreatectomy) Treatment: -Exogenous insulin (conservative regimen, close glucose monitoring, emergency glucagon kit for hypoglycemia) Pathophysiology: -CF: abnormalities in CFTR -> viscous mucus -> obstruction of pancreatic duct, inflammation, fibrosis of gland -> destruction of insulin-secreting beta cells -> insulin deficiency -Additional loss of glucagon-producing alpha cells can predispose to insulin-induced hypoglycemia

Tuberculous Pleural Effusion

Presentation: -Typical signs of active TB: fever, cough, pleurisy, weight loss -Risk factors: HIV Diagnosis: -Thoracentesis showing lymphocyte-predominant, exudative effusion w/ elevated adenosine deaminase, no organisms (vs. tuberculous empyema) -Pleural fluid smear usually aseptic for acid-fast bacilli -Pleural bx often required for dx; shows pleural granulomas Pathophysiology: -Caused by hypersensitivity rxn to M. tuberculosis or its antigens (thus fluid aseptic)

Bacterial Keratitis

Presentation: -Typically presents acutely following corneal trauma (e.g. contact lenses, foreign body) -Hazy appearing cornea (opacification), central ulceration & round infiltrate, adjacent stromal abscesses, possible hypopyon, mucopurulent discharge, severe photophobia, difficulty keeping affected eye open -Medical emergency that can lead to corneal perforation, scarring, permanent vision loss Diagnosis: -Corneal opacity/infiltrate on penlight exam Treatment: -Topical broad-spectrum abx, removal of lenses/foreign body Pathophysiology: -Contact-lens associated cases usually d/t GN organisms (Pseudomonas, Serratia), can be d/t GP organisms (e.g. Staph), anaerobes

Restless Legs Syndrome

Presentation: -Uncomfortable urge to move legs w/ unpleasant sensations in legs (dysesthesias, e.g. crawling/itching sensation), onset w/ activity or at night, relief w/ movement (e.g. walking, stretching), can interfere w/ sleep quality/duration -No pain or sx of peripheral neuropathy (e.g. numbness) -Risk factors: fhx (AD inheritance of idiopathic form) -May be associated w/: iron def anemia, uremia (ESRD, CKD), T2DM, MS, Parkinson disease, pregnancy, drugs (e.g. antidepressants, antipsychotics, metoclopramide) Diagnosis: -Serum ferritin in initial assessment, polysomnography if needed to r/o other sleep disorders Treatment/Management: -Supplement iron if ferritin 75 or lower -Mild/intermittent sx: supportive measures (e.g. leg massage, heating pads, exercise), avoid aggravating factors (e.g. sleep deprivation, meds, caffeine, EtOh), carbidopa-levodopa as needed -Persistent/moderate to severe sx: dopamine agonists (e.g. pramipexole), alpha2 calcium channel ligands (e.g. gabapentin, pregabalin) Pathophysiology: -May be primary/idiopathic or secondary -Thought to be related to CNS/PNS pathology w/ impaired iron use & dopaminergic disturbance (near circadian rhythm centers) of CNS/substantia nigra, hyperalgesia mediated by A-delta fiber input

Felty Syndrome

Presentation: -Uncommon but serious complication of long-standing erosive RA w/ severe erosive joint disease, rheumatoid nodules, vasculitis (mononeuritis multiplex, necrotizing skin lesions) -Neutropenia (ANC <2000, increased risk of bacterial infection, esp of skin/sinuses), splenomegaly Diagnosis: -Anti-CCP & RF positive in >90%, markedly elevated ESR (often >85) -Peripheral smear & BMBx to rule out other causes of neutropenia Treatment: -Typical treatment of underlying RA Pathophysiology: -Formation of autoantibodies against neutrophil components & GCSF -> neutropenia, sequestration of neutrophils coated in antibodies in spleen -> splenomegaly -Pts often HLA-DR4+

Adult Still Disease

Presentation: -Uncommon inflammatory disorder presenting w/ recurrent high fevers, arthralgias/arthritis, & salmon-colored macular or maculopapular rash -Labs: elevated ESR

Carotid Artery Dissection

Presentation: -Unilateral HA/neck pain w/ ipsilateral Horner syndrome (pupillary constriction, ptosis/miosis/often w/o anhidrosis), transient vision loss, +/- tinnitus/carotid bruit -Signs of cerebral ischemia (e.g. focal weakness, TIA), cause of stroke in young pts, mean age 45 yo -Risk factors: trauma (e.g. whiplash injury, neck manipulation), HTN, smoking, connective tissue diseases, OCP use, recent infection Diagnosis: -Neurovascular imaging (e.g. CT or MR angiography) -If imaging negative but still strong suspicion, catheter angiography (gold standard) Treatment: -Thrombolysis (if within 4.5 hrs of onset) -Antiplatelet therapy (e.g. aspirin) +/- anticoagulation -Mechanical thrombectomy for large vessel occlusions (e.g. MCA) Pathophysiology: -Etiologies: trauma, spontaneous -Injury to arterial intima -> blood flow into vessel wall -> creation of false lumen, aneurysm, or intramural hematoma -> arterial obstruction, thromboembolic events -Horner syndrome due to disruption of postganglionic sympathetic fibers as aneurysmal dilation propagates down carotid resulting in compression; anhidrosis usually absent b/c sweat nerve fibers branch off at superior cervical sympathetic ganglion (above carotid bifurcation) & follow course of external carotid artery

Preseptal/Periorbital Cellulitis

Presentation: -Unilateral eyelid erythema & swelling involving entire lid, chemosis, fever, leukocytosis -No EOM involvement Treatment: -PO abx (doxycycline) Pathophysiology: -Mild infection of eyelid anterior to orbital septum, most commonly resulting from local trauma (e.g. insect bite, wound) -Commonly S. pyogenes or S. aureus

Testicular Cancer

Presentation: -Unilateral, painless, firm testicular nodule or swelling (ovoid mass) in 15-35 yo M that does not change w/ valsalva or position changes, does not transilluminate -Dull lower abd/perineal pain -Metastatic sx (e.g. cough, dyspnea, neck mass, low back pain) -Retroperitoneal lymphadenopathy, lung metastases/nodules on imaging -Risk factors: family hx, cryptorchidism, HIV Diagnosis: -Scrotal US, usually shows solid hypoechoic lesion (seminoma) or lesion w/ cystic areas & calcifications (nonseminomatous GCT) -Elevated tumor markers (AFP, beta-hCG, LDH), staging imaging (CT, CXR) -No bx d/t increased risk for lymphatic spread & recurrence Treatment: -Radical inguinal orchiectomy, chemotherapy -Cure rate ~95% Pathophysiology: -Retroperitoneal lymphadenopathy -> compression of nerve roots, psoas muscle -> lumbar back pain -Solid organ metastases to liver and lungs common -95% are germ cell tumors--seminomatous or nonseminomatous (embryonal carcinoma, yolk sac tumor, choriocarcinoma, teratoma, mixed) -5% sex cord-stromal tumors--Sertoli cell, Leydig cell

Acute Epididymitis

Presentation: -Unilateral, posterior testicular pain, improved by testicular elevation (Prehn sign) -Signs of urethritis w/ sexually transmitted infections (e.g. urethral discharge, pain at tip of penis) -Epididymal edema, dysuria, frequency (w/ coliform infection) -US shows increased blood flow to inflamed epididymis -Labs: WBCs on UA Diagnosis: -NAAT for chlamydia/gonorrhea, UA/culture Treatment/Management: -Abx (ceftriaxone + doxycycline for chlamydia/gonorrhea, levofloxacin if coliform) -Supportive care: NSAIDs, scrotal elevation Pathophysiology: -Age <35 usually sexually transmitted infection (chlamydia, gonorrhea), usually presents w/ few urinary sx, minimal or no pyuria -Age >35 usually bladder outlet obstruction (coliform infection like E. coli), presents w/ urinary sx, pyuria -Occurs when pathogens from urethra travel retrograde through ejaculatory duct -> ductus deferens -> epididymis

Stress Ulcers

Presentation: -Upper GI bleed usually following acute event, may present as melenic BMs & anemia Prevention: -IV PPI, H2 antagonists, enteral feeding -Indicated for any 1 of: coagulopathy (plts <50, INR >1.5, PTT >2x nl), mechanical ventilation >48 hrs, GI bleeding/ulceration in last 12 mos, head trauma, spinal cord injury, major burn -Indicated for 2+ of: glucocorticoid therapy, >1 wk ICU stay, occult GI bleeding >6 days, sepsis Pathophysiology: -Critically ill pts have uremic toxins & reflux of bile salts into stomach -> disruption of protective glycoprotein layer -> increased permeability of gastric mucosa -> increased risk of ulcer formation -Head trauma -> increased gastrin secretion -> parietal cell stimulation, acid secretion -Shock -> mucosal ischemia -> ulcerations (in proximal stomach in first 72 hrs, in duodenum later in hospital course)

Aortic Coarctation

Presentation: -Upper extremity HTN, lower extremity claudication (leg pain during exercise), headaches, epistaxis -Elevated UE, low LE BPs, well-developed upper body & underdeveloped LEs -Brachial-femoral pulse delays (weak/delayed pulses), left interscapular systolic (through constricted region) or continuous murmur (if collateral vessels present), possible S4 from HTN-induced LVH Diagnosis: -CXR w/ "3" sign due to aortic indentation, inferior notching of 3rd to 8th ribs (enlarged collateral vessels) -Echo for diagnostic confirmation -ECG showing LVH -UE & LE BP measurement Treatment/Management: -Balloon angioplasty +/- stent placement -Surgical repair Pathophysiology: -Usually congenital (e.g. Turner syndrome), rarely acquired (e.g. Takayasu arteritis) -Congenital narrowing of descending aorta just distal to L subclavian artery -> proximal arterial pressure load affecting upper body

Overactive Bladder / Urgency Incontinence

Presentation: -Urinary urgency, nocturia, frequency w/o dyspareunia or pelvic pain, unrelated to increases in intraabd pressure vs. stress incontinence -"Overactive bladder" -Detrusor overactivity -> urinary urgency, incontinence due to bladder -No sx of urinary retention or difficulty initiating stream -Risk factors: >40 yo, prior pelvic surgery Diagnosis: -Normal UA & postvoid residual Treatment: -Antimuscarinics (oxybutynin, tolterodine, help decrease detrusor contraction), bladder training (to increase bladder capacity), Kegel exercises, mirabegron (beta-3 adrenergic agonist, if unable to tolerate antimuscarinic therapy) -Timed voiding: scheduling bathroom breaks based on planned time intervals rather than sense of urgency

Type 4 Renal Tubular Acidosis

Presentation: -Urine pH <5.5, hyperkalemia Pathophysiology: -Due to aldosterone resistance -May be associated w/ obstructive uropathy or congenital adrenal hyperplasia -Defect in sodium/potassium exchange in distal tubule -> hyperkalemic, hyperchloremic metabolic acidosis

Type 2 Renal Tubular Acidosis

Presentation: -Urine pH <5.5, low/normal serum K+ -Normal anion gap metabolic acidosis (low bicarb, high chloride) -Failure to thrive in infant Pathophysiology: -Poor bicarbonate resorption in kidney -Associated w/ Fanconi syndrome (glucosuria, phosphaturia, aminoaciduria) -Drug-induced Fanconi: aminoglycoside antibiotics, tetracycline antibiotics, chemotherapy agents (cisplatin, ifosfamide, carboplatin), antiviral drugs (tenofovir, adefovir), and anticonvulsant agents (valproic acid), acetazolamide

Type 1 Renal Tubular Acidosis

Presentation: -Urine pH of 5.5+, low/normal serum K+ -Normal anion gap metabolic acidosis (low bicarb, high chloride) -Failure to thrive in infant -Hypercalciuria -> nephrolithiasis (calcium phos stones), nephrocalcinosis, bone demineralization Treatment: -Oral bicarb to normalize serum bicarb levels Pathophysiology: -Poor hydrogen secretion into urine -Associated w/ genetic disorders, medication toxicity, autoimmune disorders (e.g. RA, Sjogren)

Factitious Thyrotoxicosis

Presentation: -Use of exogenous hormone, e.g. for weight loss -Sx of hyperthyroidism Diagnosis: -Decreased RAIU, low TSH & low thyroglobulin (vs. high in thyroiditis), elevated T4

Familial Hypocalciuric Hypercalcemia (FHH)

Presentation: -Usually asymptomatic -Complications: pancreatitis, chondrocalcinosis -Labs: elevated/inappropriately normal PTH, hypercalcemia (usually mild & <12), low 24 hr urine calcium (<100 mg), urine calcium/creatinine clearance ratio (UCCR) of <0.01 (vs. >0.02 in primary hyperparathyroidism), normal bone density Treatment/Management: -Observation, reassurance Pathophysiology: -Autosomal dominant, caused by inactivating mutations of Ca2+ sensing receptors (CaSR) that regulate PTH secretion -> decreased urinary Ca2+ excretion and increased serum Ca2+ -Higher calcium concentrations required to suppress PTH release than in normal pts

Hepatic Adenoma

Presentation: -Usually asymptomatic (incidentally found) -Solid, benign hepatic mass (occasionally multiple) usually in R lobe, may cause episodic dull/aching RUQ pain, hepatomegaly -Young/middle-aged women on long-term OCPs -Labs: elevated GGT & alk phos (from biliary compression), other LFTs usually normal -Complication: mass effect/compression from growth, rupture/hemorrhage, malignant transformation (HCC, ~10%) -Risk factors: OCPs, women, pregnancy, anabolic androgen use Diagnosis: -Centripetal enhancement on triphasic CT (moving from periphery to center) -Well-demarcated & hyperechoic liver lesion on US -Biopsy not recommended due to risk of bleeding Treatment/Management: -Surgical excision if symptomatic or >5 cm -Stop OCPs if asymptomatic and <5 cm -D/t risk of malignant transformation, serial imaging & AFP levels Pathophysiology: -Benign epithelial tumor

Primary Hyperparathyroidism

Presentation: -Usually asymptomatic, but can present w/ mild/nonspecific sx (fatigue) and signs of hypercalcemia (constipation, stones/bones/groans/psych overtones); renal stones, bone pain, abd pain, neuropsych sx (e.g. depression, poor sleep), HTN, muscle weakness, polyuria, polydipsia -Labs: hypercalcemia (usually <12), high or inappropriately normal PTH, elevated 24-hr urinary calcium excretion, hypophosphatemia -Complications: CKD, osteoporosis, nephrolithiasis, CV complications (arrhythmias, LVH, vascular/valvular calcification) Diagnosis: -Sestamibi nuclear imaging, neck US Treatment: -Parathyroidectomy directed by imaging -Indications for surgery: age <50, symptomatic hypercalcemia, complications (nephro, osteoporosis, CKD), high risk of complications (calcium 1+ mg/dL above normal, urinary calcium excretion >400 mg/day) -Bisphosphonates if decline surgery & symptomatic -Observation if near-normal calcium & normal bone density (w/ fup on serum Ca, Cr, DXA testing) -Vitamin D supp if deficient Pathophysiology: -Parathyroid adenomas (most common, ~80%), hyperplasia, carcinoma may all hypersecrete PTH -> increased renal reabsorption, increased release of calcium from bone, increased excretion of phosphorus, increased GI absorption of calcium -HTN thought to be due to renin secretion, sympathetic hyperresponsiveness, peripheral arterial vasoconstriction

Wolff-Parkinson-White Syndrome (WPW)

Presentation: -Usually asymptomatic, may have episodes of "fluttering"/palpitations w/ paroxysmal SVT, AFib w/ RVR -> syncope (may be precipitated by EtOH), possible deterioration to VFib -Can cause sudden cardiac death if untreated (AFib degeneration to VFib) Diagnosis: -ECG w/ short PR interval, slurred & broad upstroke of QRS (delta wave), QRS interval widening, ST/T wave changes Treatment: -Cardioversion if hemodynamically unstable, vagal maneuvers & adenosine for AVRT -If stable, rhythm control w/ procainamide, ibutilide -Catheter ablation of accessory pathway Pathophysiology: -Accessory atrioventricular pathway through bundle of Kent that bypasses AV node (type of AVRT) -Faster conduction through accessory pathway can cause ventricular preexcitation -Can develop re-entrant supraventricular tachycardia if retrograde conduction from ventricles to atria occurs (regular rhythm, inverted P waves immediately following QRS complexes)

Sickle Cell Trait

Presentation: -Usually asymptomatic, no change in overall life expectancy -Family hx of sickle cell disease -Complications: hematuria, papillary necrosis (esp w/ use of analgesics), hyposthenuria (inability of kidneys to concentrate urine, nocturia, polyuria), splenic infarction (esp at higher altitudes, dehydration due to EtOH consumption), VTE, priapism, exertional rhabdomyolysis, UTI during pregnancy -Labs: normal Hgb, reticulocyte count, RBC indices and morphology, Hb A > Hb S on Hgb electrophoresis Diagnosis: -Hgb electrophoresis Pathophysiology: -Carrier of 1 copy of sickle cell mutation -Most common in pts of African, Middle Eastern, Mediterranean ancestry -Sickling may occur in setting of physiologic stress, esp w/ low O2 tension (e.g. high altitude), dehydration (e.g. summer heat), acidosis

Stress-Induced Ulcer

Presentation: -Usually develop within hrs to days of severe physiologic stress -Vs. peptic ulcer disease, presents more acutely & may lack gnawing abd pain -Labs: positive occult blood (painless GI bleeding, may be occult or frank), decreasing Hgb/anemia -Risk factors: acute illness (esp requiring ICU care) including shock, sepsis, coagulopathy, mechanical ventilation, traumatic spinal cord/brain injury, burns, high-dose steroids Diagnosis: -Endoscopy for clinically significant bleeding Treatment: -PPIs Prevention: -Acid suppressive agents (PPIs, H2 blockers) in high-risk pts (e.g. bleeding diathesis, prolonged mechanical ventilation, recent GI bleeding) Pathophysiology: -Multifactorial, involving splanchnic hypoperfusion, reflux of bile salts, accumulation of uremic toxins that impair formation of the protective mucosal layer of stomach, allowing mucosal injury/bleeding

Factitious Diarrhea

Presentation: -Usually female, often healthcare workers, hx of multiple hospitalizations -May be associated w/ laxative abuse, presents w/ frequent water, nonbloody diarrhea, nocturnal bowel movements, abd pain/cramping -May be associated w/ signs of volume depletion (lightheadedness, tachycardia, hypotension), enhanced GI motility (hyperactive bowel sounds) -Labs: metabolic alkalosis, hypokalemia, hypermagnesemia (if Mg-containing laxative used) -Risk factors: psych conditions, healthcare worker Diagnosis: -Initial testing w/ stool osmolality, stool electrolytes (elevated stool Mg/phosphate in use of saline osmotic laxatives), & stool osmotic gap -Positive stool screen for diphenolic (e.g. bisacodyl) or polyethylene-containing laxatives -Colonoscopy classic finding of melanosis coli (dark brown colon discoloration w/ pale patches of lymph follicles, develops within mos of starting laxative use) -Histo w/ melanosis coli (macrophages containing pigment) Pathophysiology: -Profuse diarrhea -> hypokalemia -> impaired Cl- absorption -> impaired Cl-/bicarb exchange -> increased serum bicarb -> metabolic alkalosis

Cervical Myelopathy

Presentation: -Usually first presents w/ gait dysfxn (ataxia), clumsiness -Slowly progressive/insidious extremity weakness, stiffness, & numbness -LHERMITTE SIGN: electric shock-like pain w/ neck flexion -Radiculopathy: LMN signs in arms w/ muscle atrophy, hyporeflexia, radiculopathy-like sx -Myelopathy: UMN signs in legs w/ Babinski, hyperreflexia, stiffness, spastic gait -Decreased proprioception/vibration/pain sensation, urinary/bowel dysfxn -Usually age >55 Diagnosis: -MRI of cervical spine, CT myelogram Treatment: -Nonsurgical (immobilization), surgical decompression Pathophysiology: -Usually due to degenerative cervical spine/discs (lordosis/spondylosis) -> canal stenosis -> cord compression -> hyperreflexia below level of lesion, hyporeflexia at level of lesion -May be considered radiculomyelopathy: both radicular sx in UEs & myelopathic sx in LEs

Medial Meniscus Tear

Presentation: -Usually occurs after twisting injury to knee w/ planted foot (e.g. sudden turning while running) -Presents w/ knee pain/swelling, popping sensation at time of injury -Tenderness along medial aspect of knee, locking of knee on extension -Effusion usually takes ~24 hrs to develop, less severe than ACL/osteochondral injuries, not usually bloody Diagnosis: -McMurray's sign (snapping w/ tibial torsion & knee flexed at 90 degrees) Pathophysiology: -Bucket handle tear most common

Renal Infarction

Presentation: -Usually occurs in setting of cardioembolic disease (e.g. AFib) -Flank & abd pain, acute increase in BP due to renin release, N/V, fever -Labs: hematuria, proteinuria, no casts, leukocytosis, elevated LDH & CRP -Risk factors: AFib, renal artery trauma, hypercoagulability, endocarditis Diagnosis: -Wedge-shaped area of ischemia on imaging (CT w/ contrast or MRI)

Acute Renal Allograft Rejection

Presentation: -Usually occurs within first 6 mos of transplant, even w/ use of immunosuppressants -Usually asymptomatic, may present w/ fever, decreased renal output, graft tenderness (flank tenderness) -Labs: increased Cr, proteinuria Diagnosis: -Renal bx w/ lymphocytic infiltration of intima, inflammatory tubular disruption, intimal arteritis Treatment: -High-dose IV glucocorticoids, increased dosing of maintenance immunosuppression regimen Pathophysiology: -Predominantly T-cell-mediated response to antigens in donor kidney -Acute rejection mostly reversible w/ immunosuppression

Papillary Thyroid Carcinoma

Presentation: -Usually presents as palpable thyroid nodule Diagnosis: -FNA w/ large cells w/ clear cytoplasm, pale nuclei containing inclusion bodies, central grooving representing papillary formation -If cytology results indeterminate, may perform genetic mutation profiling (e.g. BRAF, RET mutations in papillary), but unnecessary if cytology c/w papillary thyroid carcinoma Treatment/Management: -Surgical resection is mainstay; extent of surgery depends on tumor size, extrathyroidal invasion, pt age, lymph node involvement -Small tumors <1-2 cm w/o evidence of nodal involvement or contralateral thyroid abnormalities usually managed w/ partial thyroidectomy/lobectomy -Large tumors warrant total thyroidectomy -Regional lymph node or central compartment lymph node resection usually performed if evidence of lymph node metastasis or extrathyroidal invasion -Following surgery, adjuvant therapy w/ radioactive iodine indicated for pts w/ increased risk of tumor recurrence (e.g. large tumors, extrathyroidal invasion, lymph node metastasis, incomplete resection) + adequate doses of thyroid hormone replacement to suppress TSH secretion (excess TSH can stimulate growth of occult residual/metastatic disease, thus must be suppressed & kept in low-normal range)

Alcoholic Cerebellar Degeneration

Presentation: -Usually presents over wks to mos -Cerebellar signs: wide-based gait, truncal incoordination/instability/ataxia, nystagmus, cognition usually intact, postural tremor, dysarthria, visual problems (e.g. diplopia) -Impaired tandem walking, heel-knee-shin testing, pendular knee reflex (swinging movements of limb after eliciting DTR, indicative of hypotonia & different from myoclonus) -Finger-nose testing usually normal Diagnosis: -CT/MRI w/ cerebellar atrophy Treatment: -EtOH cessation, nutritional supplements, ambulatory assistance devices (e.g. walker) -Damage usually irreversible Pathophysiology: ->10 yrs of heavy EtOH use -> degeneration of Purkinje cells in cerebellar vermis

Aortoenteric Fistula

Presentation: -Usually results from ruptured abd aortic aneurysm or after aortic surgery -Massive GI bleeding, fever -Risk factors: older age, h/o AAA, prior GI interventions, malignancy

Medullary Thyroid Carcinoma

Presentation: -Usually sporadic, 25% of cases associated w/ RET mutations -Most commonly presents as asymptomatic thyroid nodule, minority of pts have diarrhea/flushing from hormone secretion by tumor -If associated w/ MEN, may present w/ hypercalcemia; otherwise serum calcium usually normal Diagnosis: -FNA of nodule Treatment/Management: -Total thyroidectomy, post-surgical thyroid replacement therapy w/ levothyroxine -Post-surgical monitoring w/ serial calcitonin (correlated w/ risk of metastasis & recurrence), carcinoembryonic antigen -If calcitonin elevated, CT neck/chest +/- high-resolution US of neck to look for metastatic disease Pathophysiology: -Neuroendocrine tumor arising from calcitonin-secreting parafollicular C cells -Serum calcium usually normal unless associated w/ MEN, thought to be due to downregulation of calcitonin receptors -Often metastatic at time of diagnosis to cervical lymph nodes & upper aerodigestive tract -In MEN, d/t mutation of RET proto-oncogene

Brown-Sequard Syndrome

Presentation: -Usually the result of penetrating spinal trauma -Asymmetric/ipsilateral weakness & sensory loss of both UEs & LEs -Loss of motor function, vibration, proprioception, light touch on ipsilateral side below lesion level -Loss of pain/temp sensation on contralateral side 2 levels below lesion level -If occurs at cervical level, may be associated w/ ipsilateral Horner syndrome (ptosis, miosis, anhidrosis) -Ipsilateral reflexes may initially be absent from spinal shock, then proceeds to hyperreflexia (e.g. +Babinski) from UMN damage Pathophysiology: -Hemisection of spinal cord -Damage to ispilateral corticospinal tract -> ipsilateral hemiparesis -Damage to ipsilateral dorsal columns -> loss of proprioception, vibratory sensation, light touch -Damage to decussating fibers of contralateral lateral spinothalamic tract -> contralateral loss of pain & temp sensation

Dystonic Tremor

Presentation: -Usually unilateral & rhythmic, often task-specific (e.g. writing, typing), irregular -Worsens w/ arms outstretched, often disappears w/ rest, worse w/ stress -Occur w/ sustained muscle contractions associated w/ dystonia (e.g. wrist spasms, torticollis, oculogyric crisis, trismus, blepharospasm), typically in same area as tremor

Inverse Psoriasis

Presentation: -Variant of psoriasis that presents primarily in intertriginous regions w/ smooth plaques w/ minimal to no scaling

Progressive Supranuclear Palsy (PSP)

Presentation: -Vertical supranuclear palsy (inability to elevate eye to look up), vestibular dysfxn -Dementia, postural instability, parkinsonism Pathophysiology: -Considered a Parkinsonian-plus syndrome

Kaposi Sarcoma

Presentation: -Violaceous/pink/red/brown/dark/vascular papular lesions most commonly affecting face, lower extremities, oral mucosa, genitalia -No pruritus/skin necrosis -May be associated w/ lymphedema from vascular obstruction by tumor or due to cytokine production -> LE swelling -Lesions may also involve GI tract & other organs -Can also present as plaques/nodules that are ovoid & linear in shape along skin tension lines -AIDS-defining illness -Risk factors: HIV (e.g. IV drug use, multiple sexual partners, men who have sex w/ men, etc., esp if CD4 <200), opportunistic infections leading to increased cytokine production that predisposes to infection/exacerbation of KS (e.g. thrush) Diagnosis: -Usually clinical, skin bx Treatment: -Antiretroviral therapy -In some advanced cases, systemic or intralesional chemotherapy Pathophysiology: -Human herpesvirus-8, usually coinfection w/ HIV -Violaceous color due to increased vascularity

Varicose Veins

Presentation: -Visible, palpable, tortuous superficial veins of the legs, usually on calf or medial thigh -Usually asymptomatic, but may be associated w/ leg cramping, heaviness, fatigue, swelling -Sx worst in evening w/ prolonged standing, improve w/ leg elevation Treatment/Management: -Conservative management w/ leg elevation & compression stockings (do not use if pt has underlying arterial insuff) to decrease venous pressure in LEs by direct compression -Sclerotherapy w/ or w/o anesthetic in pts w/ symptomatic, small, varicose veins that have failed at least 3-6 mos of conservative treatment (causes endothelial damage & sclerosis of involved vein, preventing further vein filling) -Surgical ligation & stripping used in pts w/ large symptomatic varicose veins w/ ulcers, bleeding, or recurrent venous thrombophlebitis

Prolactinoma

Presentation: -Visual field deficits (bitemporal hemianopsia), HA -Premenopausal women: oligo/amenorrhea, infertility, galactorrhea, hot flashes, decreased bone density, vaginal dryness -Postmenopausal women: mass effect sx (HA, visual field defects) -Men: infertility, decreased libido, impotence, gynecomastia -Labs: elevated prolactin (>200), low LH & FSH Diagnosis: -Prolactin level -Rule out renal insuff & hypothyroidism (TSH, thyroxine levels) -Head MRI Treatment/Management: -Dopaminergic receptor agonists (bromocriptine, cabergoline) to reduce tumor size -Transphenoidal/transcranial surgery rarely required

Nontyphoidal Salmonella

Presentation: -Vomiting, diarrhea +/- blood (blood more common in children), fever, invasive disease rare Diagnosis: -Stool culture Treatment: -Usually self-limited, abx (SMX-TMP) rarely needed unless severe illness (e.g. persistent fever) or risk for invasive disease (infants <1, immunocompromised) -Supportive care w/ fluids, electrolytes -If abx needed (high risk, severe disease), treat w/ ciprofloxacin, TMP/SMX, ceftriaxone Pathophysiology: -Major cause of gastroenteritis worldwide (including US), associated w/ undercooked poultry/eggs, inadequate refrigeration of food

Monoclonal Gammopathy of Undetermined Significance (MGUS)

Presentation: -Vs. other gammopathies (MM, Waldenstrom), no systemic signs or end-organ effects (e.g. anemia, lymphadenopathy, hepatosplenomegaly) Diagnosis: -M-spike <3 g of IgA, IgG, or IgD (vs. IgM more common in Waldenstrom) -BMBx w/ <10% monoclonal plasma cells

Microscopic Colitis

Presentation: -Watery/nonbloody diarrhea, fecal urgency/incontinence, abd pain, fatigue, weight loss, arthralgias -Secretory diarrhea (may occur during periods of fasting, at night) -May be triggered by smoking, certain meds (e.g. NSAIDs, PPIs, SSRIs, ranitidine) -Labs: unremarkable, normal CRP & Hgb, negative fecal occult blood, secretory diarrhea w/ decreased osmotic gap -Risk factors: smoking, autoimmune conditions (celiac, autoimmune thyroiditis), older female (>60) Diagnosis: -Grossly normal-appearing colon on colonoscopy -On colonoscopic biopsy, lymphocytic infiltration of lamina propria (+/- thickened subepithelial collagen band if collagenous subtype, high levels of intraepithelial lymphocytes if lymphocytic subtype) Treatment/Management: -Remove possible triggers (e.g. smoking cessation), antidiarrheal meds (e.g. loperamide), budesonide Pathophysiology: -Immune-mediated colitis w/ secretory diarrhea, abnormal immune response to various GI & external agents (e.g. meds)

Gastric Cancer

Presentation: -Weight loss, gastric fullness, epigastric abd pain sometimes relieved by antacids and worsened by eating -Proximal stomach tumors may also cause dysphagia, postprandial N/V -Labs: microcytic anemia, elevated LFTs/hypoalbuminemia if liver mets -Risk factors: H. pylori infection, smoking, EtOH, atrophic gastritis, pernicious anemia, consuming salt-preserved foods (damages stomach mucosa) or foods w/ nitroso compounds (e.g. kimchi, carcinogenic, esp in Eastern Asia, Eastern Europe, Andean South America) Diagnosis: -Esophagogastroduodenoscopy w/ biopsies for initial diagnosis -Staging w/ CT abd/pelvis, then PET/CT, endoscopic US, laparoscopy, CT chest, +/- paracentesis/peritoneal lavage -90% diagnosed at stage III/IV, at which resection is not possible or very complicated; thus staging very important Treatment: -If limited, surgical resection (laparotomy) -If advanced, chemotherapy +/- palliative surgery Pathophysiology: -Most commonly adenocarcinoma -Usually detected late in disease course once large enough to cause gastric obstruction, have already spread to liver

Plaque Psoriasis

Presentation: -Well-defined, hyperkeratotic, erythematous plaques w/ silvery/white scale on extensor surfaces (knees, elbows), hands, scalp, back, nail plates, trunk (e.g. gluteal cleft) -Kobner phenomenon: when found on dorsal hands, typically associated w/ minor trauma -Auspitz sign: pinpoint bleeding after scraping scales -Worsening of sx can be associated w/ infection (esp Strep pharyngitis), certain meds (antimalarials, indomethacin, propanolol), withdrawal from systemic glucocorticoids -Extradermal: nail pitting/subungual debris (+ oil spotting of nails, onycholysis/separation of nail plate from bed), conjunctivitis, uveitis, psoriatic arthritis Diagnosis: -Usually clinical, skin bx if necessary Treatment/Management: -UV light/phototherapy (for moderate to severe disease, >5% total surface area involvement) -Topical: high-potency glucocorticoids (betamethasone, fluocinonide), vit D analogs (calcipotriene), tar, retinoids, calcineurin inhibitors (tacrolimus), tazarotene -Systemic (severe cases): methotrexate, calcineurin inhibitors, retinoids, apremilast, biologics (e.g. etanercept) -Systemic glucocorticoids not used; can precipitate severe erythrodermic/pustular psoriasis Pathophysiology: -Keratinocyte proliferation, inflammation, angiogenesis -May be associated w/ HIV if sudden-onset & severe

Hypertrophic Obstructive Cardiomyopathy (HOCM)

Presentation: -Young athlete w/ sudden cardiac arrest w/ exertion; can be asymptomatic or present w/ fatigue, chest pain, palpitations, presyncope/syncope, dyspnea. lightheadedness -Crescendo-decrescendo systolic ejection murmur @ L sternal border, strong apical impulse, dual upstroke carotid pulse (due to midsystolic obstruction during contraction), possible S4 -Increased risk of AFib (d/t LA enlargement) or VTach -Family hx of sudden cardiac arrest, AD inheritance w/ variable expressivity/penetrance, more common in African Americans -Increased murmur intensity w/ maneuvers that decrease preload and decrease LV blood volume (Valsalva strain phase, abrupt standing, nitroglycerin administration, dehydration) -Decreased murmur intensity w/ maneuvers that increase LV blood volume and LV ventricle size and thereby decrease obstruction, including sustained hand grip (increased afterload), squatting (increased afterload & preload), passive leg raise (increased preload) -Complications: sudden cardiac death, HF, stroke Diagnosis: -ECG: LVH, repolarization abnormalities (inverted T waves) or depolarization (Q waves), left axis deviation -Echo: LVH, increased LVOT gradient (indicating LVOT obstruction), systolic anterior motion of mitral valve, LA dilation -Exercise testing, family genetic screening Treatment: -Avoidance of volume depletion, nitrates & other agents that decrease preload (and worsen obstruction) such as dihydropyridine CCBs, ACEi, ARBs -Negative inotropic agents (beta blockers, verapamil, disopyramide) to prolong diastole & decrease myocardial contractility, which reduces LVOT obstruction -Rate or rhythm control & anticoag if associated AFib -ICD placement if high risk for SCD (e.g. prior syncope or cardiac arrest or sustained VT, family h/o SCD in close relative <50, hypotensive BP response to exercise, extreme LV hypertrophy, EF <50%) -Surgery if sx persist (septal ablation or surgical myecotomy if significant LVOT obstruction, cardiac transplant) -Agents to reduce contractility via modulation of cardiac myosin under investigation (e.g. mavacamten) Pathophysiology: -LVH w/ normal chamber size and w/o clear etiology (e.g. HTN, aortic stenosis, infiltrative disease) -Outflow obstruction by asymmetric interventricular septal hypertrophy (d/t contact b/t mitral valve & thickened septum during systole -D/t inherited disorder of cardiac sarcomere -> myocardial disarray, interstitial fibrosis

Fibromyalgia

Presentation: -Young to middle-aged female w/ widespread MSK pain for 3+ mos, fatigue, mood/cognitive disturbances (e.g. impaired concentration, depression, anxiety) -Point muscle tenderness w/o joint or muscle inflammation/swelling, e.g. of mid trapezius, lateral epicondyle, costochondral jxn, greater trochanter -Labs: normal ESR/CRP & other inflammatory markers Diagnosis: -3+ mos of sx w/ widespread pain index or symptom severity score -Normal labs (TSH to rule out hypothyroid myopathy) Treatment/Management: -Regular aerobic exercise (e.g. exercise program w/ aerobic conditioning, stretching, strength training, water exercises), good sleep hygiene -TCAs (e.g. amitriptyline) if refractory to above, alternatives include SNRIs (e.g. duloxetine, milnacipran), pregabalin, cyclic skeletal muscle relaxants -If persistent sx despite above, may require combined drug therapy, referral for supervised rehab, pain management consultation, CBT

Anterior Cruciate Ligament (ACL) Tear

Presentation: -Common in athletic activities, mechanism often involves hyperextension of the knee -33-90% of pts endorse popping sensation -Rapid onset of significant knee effusion, unstable knee Diagnosis: -Lachman's test

Acute Tubular Necrosis

Presentation: -Muddy brown granular casts (composed of renal tubular epithelial cells) -No response to fluid challenge (minimal improvement in UOP or Cr after IVF resuscitation) vs. prerenal AK -Labs: BUN:Cr ratio of 10-15, other findings of intrinsic AKI (urine Osms of 300-350, urine Na+ of >20, FENa of >2%) -Risk factors: prolonged hypotension, sepsis, nephrotoxic meds

Thoracic Outlet Syndrome

Presentation: -Pain, weakness, paresthesias, and numbness in arm, edema, venous congestion, and digital vasospastic changes -Acute supraclavicular pain radiating down arm -Worsens when raising over head (vs. cervical radiculopathy), worse w/ exertion (d/t involvement of subclavian vessels) -Cause unknown but may be d/t compression of the brachial plexus or subclavian vessels, or both, in the anatomic space bounded by the first rib, the clavicle, and the scalene muscles. -Ddx: carpal tunnel, cervical disk disease. Diagnosis: -Plain films may diagnose bony abnormalities, and MRI can be useful in detecting cervical disease. Neurologic abnormalities may be documented by nerve conduction studies. Angiographic studies are often negative. Treatment: -Conservative management, which generally should precede surgery, consists of an exercise program to strengthen shoulder girdle muscles and decrease shoulder droop. -Operative treatment includes division of the scalenus anticus and medius muscles, first rib resection, cervical rib resection, or a combination of all three Pathophysiology: -Associated w/ brachial plexus injury associated w/ shoulder/neck trauma

Multiple Endocrine Neoplasia Type 2B

Presentation: 1 P, 2 Ms (pheo, medullary, marfanoid) mnemonic -Pheochromocytoma -Medullary thyroid carcinoma -Marfanoid habitus -Multiple neurinomas (mucosal neuromas of lips and tongue, intestinal ganglioneuromatosis) Treatment/management: -Thyroidecomy, adrenalectomy -Metanephrine screening Pathophysiology: -Mutation of RET gene

Tuberous Sclerosis

Presentation: HAMARTOMAS mnemonic -Hamarotomas in CNS and skin -Angiofibromas (facial malar erythematous papules, may be mistaken for acne) -Mitral Regurg -Ash-leaf spots (hypopigmented macules) -Cardiac Rhabdomyoma (can be asymptomatic or cause murmur/arrhythmia/HF; may be large in infancy but regress w/ age) -Tuberous Sclerosis -Autosomal dOminant -Mental retardation, intellectual disability, autism & behavioral disorders (e.g. hyperactivity) -Renal Angiomyolipoma -Seizures/epilepsy (infantile spasms, tonic-clonic), subependymal giant cell tumors in lateral ventricles (calcified on CT) -> obstructive hydrocephalus -Shagreen patches (flesh-colored plaque on back, thickened/leathery skin w/ orange peel-like texture) -Periungual fibromas (fibrous growths from skin folds) Treatment/management: -Tumor screening: regular eye & skin exams, serial brain/kidney MRIs, baseline echo, serial ECG, baseline EEG -Neuropsychiatric screening Pathophysiology: -Mutation (de novo or inherited) in TSC1 or TSC2-If inherited, AD

Renal Papillary Necrosis

Presentation: NSAID mnemonic -NSAIDs, Sickle cell disease, Analgesic abuse, Infection (pyelonephritis), Diabetes -Painless hematuria, acute flank pain -Increased renal medullary echogenicity on US -Risk factors: sickle cell disease, sickle cell trait, analgesic use -Labs: hematuria, possible renal tubular acidosis (impaired H+ secretion)

Vertebrobasilar Insufficiency

Presentation; -Episodic vertigo, dizziness, dysarthria, diplopia, numbness -Risk factors: DM, HTN, hypercholesterolemia, arrhythmias, CAD, circulatory disorders, smoking Pathophysiology: -Reduced blood flow to base of brain, usually secondary to emboli, thrombi, or arterial dissection -Commonly affects labyrinth & brainstem

Indications for Statin Therapy

Primary intervention: -Indicated w/ any established ASCVD (prior ACS, stable or unstable angina, arterial revascularization like CABG, PAD, stroke/TIA Secondary intervention: -Indicated in any pt w/ LDL of 190+, age of 40+ w/ DM, and/or established 10-yr ASCVD risk >7.5-10%

Hepatorenal Syndrome

Progressive renal failure associated with hepatic failure Presentation: -Sudden decrease in urinary output in pt w/ cirrhosis/advanced liver failure -Risk factors: advanced cirrhosis w/ portal HTN & edema Diagnosis: -Renal hypoperfusion: FENa <1% or urine Na <10, BUN:Cr often 20+ from overdiuresis -Absence of tubular injury (e.g. RBC casts, granular casts, proteinuria on UA; may have minimal hematuria & mild proteinuria) -No improvement in renal fxn w/ IV fluids (administer IVF to confirm that renal failure is not secondary to intravascular volume depletion) -US to exclude urinary obstruction Treatment: -Address precipitating factors (e.g. hypovolemia, anemia, infection) -Splanchnic vasoconstrictors (octreotide, midrodine, norepi), albumin -Liver transplant (possible bridge w/ dialysis) Pathophysiology: -Complication of end-stage liver disease that may be precipitated by: reduced renal perfusion (e.g. hypovolemia), GI bleed, vomiting, sepsis, excessive diuretic use, SBP, reduced glomerular pressure & GFR (e.g. NSAID use) -Cirrhosis -> splanchnic arterial dilation -> decreased vascular resistance -> RAAS activation -> renal vasoconstriction -> decreased perfusion & glomerular filtration

Idiopathic Intracranial Hypertension

Pseudotumor cerebri Presentation: -Obese women of childbearing age (excessive weight gain can exacerbate sx) -Positional holocranial headache worse when supine, vision loss (enlarged blind spot, obscurations worse w/ valsalva), blurry vision (due to increased pressure on optic nerves), pulsatile tinnitus ("whooshing" sound), diplopia/palsy of CN VI (abducens), papilledema (optic disc swelling), N/V -Cushing triad from increased ICP -Ophtho exam may show peripapillary flame hemorrhages, venous engorgement, hard exudates -In children, may present w/ primarily vision abnormalities; presents w/ blurry vision, enlarged blind spot, VI palsy -Risk factors: female, obese, medications (retinoids, tetracyclines, minocycline, cimetidine, danazol, tamoxifen, levothyroxine, lithium, nitrofurantoin, growth hormone, glucocorticoids), certain endocrine disorders (e.g. hypoparathyroidism, hypothyroidism, adrenal insuff, Cushing disease) -Complications: blindness (progressive optic atrophy) Diagnosis: -Neuroimaging (brain MRI w/ MR venography preferred in pregnancy, performed before LP to rule out other causes of elevated ICP like space-occupying masses), lumbar puncture (elevated opening pressure >250 mm H2O w/ otherwise normal CSF) Treatment: -Weight loss, acetazolamide to reduce intracranial pressure (inhibits choroid plexus carbonic anhydrase -> decreased CSF production) -Withdrawal of medication if meds are underlying cause -Lumboperitoneal shunting, optic nerve sheath fenestration if refractory to above (to prevent blindness), short-term steroids & repeat LP and/or lumbar drain to bridge to surgical treatment Pathogenesis: -Elevated ICP without secondary cause, impaired absorption of CSF by arachnoid villi & CSF lymphatic drainage sites -Obesity-related increased abd & intracranial venous pressure may contribute to the above

Rheumatoid Arthritis Cervical Myelopathy

RA-associated atlantoaxial subluxation Presentation: -Neck pain radiation from occipital region, slowly progressive spastic quadriparesis, painless sensory deficits in hands/feet, respiratory dysfxn (from vertebral artery compression), Lhermitte sign -On exam, protruding anterior arch of atlas, scoliosis w/ loss of cervical lordosis, UMN signs (hyperreflexia, spasticity, Babinski) -Hoffman sign--flexion & adduction of thumb when flicking nail of middle finger (indicates lesion of corticospinal tract) -Increased risk w/ increased severity of RA -May present after recent intubation (forced neck extension can worsen subluxation) Diagnosis: -Cervical spine MRI Treatment: -Stiff surgical collar, neurosurgical intervention (cervical/atlantoaxial fixation) Pathophysiology: -Inflammatory arthritis of cervical spine w/ subluxation of atlantoaxial joints, can lead to cord compression

Herpes Zoster Oticus

Ramsay Hunt syndrome Presentation: -Ear pain, facial weakness/paralysis, vesicular rash on external auditory canal/auricle/face, preherpetic neuralgia may precede rash by 1-5 days -Infection can spread to vestibulocochlear (VIII) nerve and cause auditory (hearing loss, hyperacusis, tinnitus) and vestibular (vertigo, nausea/vomiting) sx Treatment: -Corticosteroids, antiviral meds (early initiation to reduce facial nerve damage; later initiation may be associated w/ increased risk of residual facial paralysis) -Protection of eye on paralyzed side of face (e.g. artificial tears, lubricating ointment) Pathophysiology: -Geniculate ganglion reactivation of latent infection -> disruption of CN VII fxn

Constrictive Pericarditis

Rare complication of acute pericarditis Presentation: -Fatigue/dyspnea on exertion, abd distention -Right HF: elevated JVP, peripheral edema, ascites, hepatic congestion w/ hepatomegaly, eventual progression to cardiac cirrhosis -Signs: elevated JVP w/ hepatojugular reflux & prominent x & y descents, Kussmaul sign (lack of decrease or increase in JVP on inspiration), pericardial knock (early middiastolic heart sound after S2), pericardial calcifications/thickening, pulsus paradoxus -ECG may be nonspecific or show AFib or low-voltage QRS complexes -May develop protein-losing enteropathy (intestinal lymphangiectasias in setting of increased venous pressure) w/ hypoalbuminemia Diagnosis: -CXR w/ calcifications along cardiac border -Echo w/ increased pericardial thickness/calciication, abnormal septal motion, biatrial enlargement Treatment/Management: -Supportive care (e.g. anti-inflammatory agents) -Pericardiectomy for refractory cases Pathophysiology: -TB pericarditis (developing world); idiopathic, viral illness, cardiac surgery (CABG, valvular surgery), radiation therapy, connective tissue disorders (developed world), malignancy, uremia -Due to pericardial fibrosis & obliteration of pericardial space -> impaired ventricular filling during diastole -> low CO, signs of venous overload

Familial Dysautonomia

Riley-Day syndrome Presentation: -Presents at birth w/ feeding problems & low muscle tone -Gross dysfxn of autonomic NS (no tears, severe orthostatic hypotension w/ swings b/t postural hypotension & severe HTN) -Risk factors: Ashkenazi descent Pathophysiology: -AR inheritance

Acute Lumbosacral Radiculopathy

Sciatica Presentation: -Shooting/burning low back pain radiating into one or both legs/feet posteriorly, occasionally associated w/ minor motor weakness, decreased reflexes -Positive straight leg test (worsening or reproduction of pain) -Usually no other neurologic issues (e.g. saddle anesthesia, bladder/bowel incontinence), possible dermatomal sensory loss & weakness of hip dorsiflexion Diagnosis: -Usually clinical -MRI recommended if significant/progressive or bilat neuro deficits (e.g. saddle anesthesia, urinary retention, severe weakness), suspected malignancy or epidural abscess Treatment: -NSAIDs/acetaminophen, lumbar support, activity modification to lessen pain, physical therapy w/ graduated exercises if pain longer than few wks -If refractory to NSAIDs, can consider epidural/systemic glucocorticoids -Surgery if disabling sx Pathophysiology: -Most commonly d/t disc herniation resulting in impingement of 1+ lumbosacral nerve roots -Other etiologies: degenerative spondylosis, malignancy, epidural abscess, other mass lesion, vascular disorder, developmental anomaly

Systemic Sclerosis

Scleroderma Presentation: -Skin: induration & fibrosis/thickening primarily affecting hands, fingers, face; telangiectasias, sclerodactyly, digital ulcers, calcinosis cutis, pruritus, obliteration of skin appendages (e.g. hair follicles, sweat glands) -Systemic: fatigue, weakness -GI: fibrosis/atrophy of esophageal smooth muscle -> chronic, severe GERD, strictures w/ food impaction, esophageal dysmotility, dysphagia, dyspepsia, hoarseness -Renal: mild proteinuria, renal insuff, HTN -MSK: arthralgias, myalgias, contractures -Vascular manifestations including Raynauds, telangiectasias -Complications: ILD (fatigue, dyspnea, crackles), pulm arterial HTN, scleroderma renal crisis (oliguria, thrombocytopenia, microangiopathic hemolytic anemia, malignant HTN w/ papilledema), myocardial fibrosis, pericarditis, pericardial effusion -Limited cutaneous subtype: scleroderma on head & UEs, prominent vascular involvement (Raynaud, telangiectasias, PAH), CREST, better prognosis -Diffuse cutaneous subtype: scleroderma on trunk & UEs, prominent organ involvement (renal crisis, myocardial ischemia/fibrosis, ILD), worse prognosis -Risk factors: African American Diagnosis: -ANA+ (nonspecific), anti-topoisomerase I antibody (anti-Scl-70) & anti-RNA polymerase III (seen in diffuse cutaneous disease), anticentromere antibody (seen in limited cutaneous disease) -Esophageal manometry w/ hypomotility, incompetence of LES (not needed for diagnosis) -Screening w/ PFTs at diagnosis for pulm HTN, ILD Treatment/Management: -Scleroderma renal crisis: ACEi (captopril), IV nitroprusside for malignant HTN w/ CNS manifestations Pathophysiology: -Progressive tissue fibrosis, atrophy of smooth muscle, vascular dysfxn -Fibroblast dysfxn -> increased collagen & ground substance production -Abnormal collagen deposition in kidney -> thickening of vessel wall & narrowing of vascular lumen in renal arterioles -> ischemia, activation of RAAS -> HTN, malignant HTN

CHADS-VASc

Score of >/= 2 means anticoagulate *C*HF = +1 *H*TN = +1 *A*ge > 74 = +2 *D*M* = +1 *S*troke or TIA = +2 *V*ascular dz = +1 *A*ge 65-74 = +1 *S*ex, if female = +1

Cherry Angioma

Senile hemangioma (b/c increase in number w/ age) Presentation: -Small, often multiple, vascular, nonblanching, bright-red papular lesions -Always cutaneous, never found in deep tissues or mucosa -Do not regress spontaneously, may bleed w/ trauma -Most common vascular tumor in adults (starting in 30s/40s, increase w/ age) -Most common on trunk, occur only in skin & not in deep tissues/mucosa -Blanch w/ pressure, but can become fibrotic over time & lose blanching Treatment: -Benign, no intervention needed -If desired for cosmetic reasons, electrocauterization under local anesthesia for small lesions & shave excision w/ electrocauterization for larger lesions Pathophysiology: -Common benign vascular tumor seen in adults -Sharply circumscribed areas of congested capillaries & postcapillary venules in papillary dermis

Serum-Ascites Albumin Gradient (SAAG)

Serum albumin value - ascitic fluid albumin value -Value of 1.1+ is a high gradient indicative of portal HTN (causes include CHF, cirrhosis, alcoholic hepatitis, Budd-Chiari syndrome) -Value of <1.1 is a low gradient, not indicative of portal HTN (causes include peritoneal carcinomatosis, peritoneal TB, nephrotic syndrome, pancreatitis, serositis)

Ischemic Hepatitis

Shock liver Presentation: -May be associated w/ recent MI, VTach, cardiogenic/septic shock -Massive aminotransferase elevation >1000 shortly after inciting incident, sometimes bili elevated -Poor prognosis, pts usually critically ill w/ accompanying multiorgan failure -In those who survive, injury is self-limited, aminotransferases decrease within 7 hrs Pathophysiology: -Usually occurs in setting of global hypoperfusion and/or hypoxemia (cardiac insults, hypovolemic shock, septic shock, respiratory failure) -> diffuse liver injury (dual blood supply in liver so more common to have diffuse injury than infarction) -Histo shows hepatocyte necrosis (most predominant in zone 3) of liver lobule surrounding central veins (farthest from vascular supplies)

Tuberculin Skin Test Interpretation

Should treat if 5+ mm (@48-72 hrs) if: -HIV+, recent contacts of known TB case, nodular/fibrotic changes on CXR c/w previously healed TB, organ transplant recipients & other immunocompromised patients Should treat if 10+ mm if: -Recent immigrants (<5 yrs) from TB-endemic areas, injection drug users, residents & employees of high-risk settings (e.g. prisons, nursing homes, hospitals, homeless shelters), Mycobacteriology laboratory personnel, higher risk for TB reactivation (e.g. T2DM, leukemia, ESRD, chronic malabsorption syndromes), children <4 yo or those exposed to adults in high-risk categories Should treat if 15+ mm no matter circumstances (even if healthy/asymptomatic); higher cutoff for low risk pts in US b/c low pretest probability & common exposure to nontuberculous Mycobacteria (esp in South) If positive test but no signs/symptoms/CXR normal, should be treated w/ 9 mos daily isoniazid + pyridoxine OR 12 wks of wkly rifapentine, high-dose isoniazid, + pyridoxine OR rifampin for 4 mos If advanced HIV w/ CD4 count <200, may have to repeat PPD testing after starting antiretroviral therapy (may have false-negative initial test due to anergy)

Focal Seizure with Retained Awareness

Simplex partial seizure Presentation: -Focal seizure (originating in a single hemisphere) in which pt remains aware and interactive -Sx correspond to area of brain involved: sensory domains (e.g. olfactory hallucinations), psychic sx (e.g. fear), & other auras

Acrochordons

Skin tags Presentation: -Benign outgrowths of normal skin that usually appear as pedunculated, skin-colored papules, most commonly in areas of friction (e.g. axillae, neck, inframmamary/inguinal regions) -Found in up to 25% of adults -Risk factors/associations: age, obesity, insulin resistance, overt DM, metabolic syndrome, pregnancy, colonic polyps, Crohn disease (perianal skin tags) Diagnosis: -Clinical Treatment/Management: -Usually no treatment required, may be removed by snip excision or cryosurgery if associated irritation or for cosmetic reasons

African Trypanosomiasis

Sleeping sickness Presentation: -Bite from tsetse fly (trypanosomal chancre), endemic to East Africa -Fever, myocarditis, progression to CNS involvement

Jarisch-Herxheimer Reaction

Spirochetal lysis Presentation: -Seen in 10-35% of pts treated for syphilis -6-48 hrs after initiating treatment for syphilis (usually penicillin G, usually primary/secondary syphilis), presents w/ acute onset of fever, chills, myalgias, rigors, diaphoresis, hypotension rash progression in secondary syphilis (diffuse macular rash involving palms/soles) -May also be seen in treatment of other spirochetal illnesses (e.g. Lyme disease, leptospirosis) or atypical organisms (e.g. Bartonella) Treatment: -Supportive (IV fluids, NSAIDs, acetaminophen); no real effective prevention -Typically self-limited within 48 hrs of onset Pathophysiology: -Rapid destruction of spirochetes after starting abx -> release of large quantities of lipoproteins into circulation

Sporotrichosis

Sporothrix schenckii (fungus) Presentation: -Occurs after exposure to decaying vegetation (e.g. gardening, farming, landscaping), infection via direct inoculation, usually cutaneous/subcutaneous abrasion -Presents w/ nodule/papule that usually ulcerates & drains odorless, nonpurulent fluid -> additional nodular/pustular lesions that arise along proximal lymphatic chain -Subacute/chronic disease course -Distant spread & systemic sx uncommon Diagnosis: -Cultures (aspirate fluid or skin bx) shows cigar-shaped yeast w/ narrow-based budding at 37C, thin/septate/branched hyphae Treatment: -3-6 mos oral itraconazole Pathophysiology: -Nodular lymphangitis w/ low-virulence dimorphic fungus found in decaying plant matter & soil, especially sphagnum moss, hay, decaying wood

Leptomeningeal Carcinomatosis

Spread from lungs/breast/other site to meninges Presentation: -Subacute cognitive impairment, HA, CN deficits, ataxia w/ penetration of cerebellum Diagnosis: -MRI w/ enhancement of meninges

Pancoast Tumor

Superior pulmonary sulcus tumor Presentation: -Usually in heavy smokers (usually adenocarcinoma or SCC of lung) -Shoulder pain radiating into forearm, neurologic sx in ipsilateral arm (weakness/atrophy of intrinsic hand muscles, pain/paresthesias of 4th/5th digits and medial arm/forearm) -Horner syndrome (unilateral ptosis, miosis, anhidrosis, enophthalmos--posterior displacement of orbit) -Supraclavicular lymphadenopathy, weight loss, hoarseness -SVC syndrome, hemidiaphragm paralysis (involvement of phrenic) -If invasion into spinal cord, asymmetric LE DTRs, progression to paraplegia Diagnosis: -CXR, staging imaging + biopsy of lesion Pathophysiology: -Tumor in apical pleuropulmonary groove, usually pulmonary adenocarcinoma or SCC -Shoulder pain from invasion of brachial plexus or adjacent bony structures -Horner syndrome from invasion of paravertebral sympathetic chain, cervical ganglion -Arm hand pain/weakness in ulnar distribution due to invasion of C8-T2 nerve roots (inferior brachial plexus) -Hoarseness from invasion of recurrent laryngeal nerve -No pulm sx (hemoptysis, cough, dyspnea) until late in disease course b/c tumor is located at lung periphery

Acute Febrile Neutrophilic Dermatosis

Sweet syndrome Presentation: -Abrupt onset of painful, edematous papules, plaques, or nodules of head/neck/UEs -Often associated w/ hematologic malignancy, URIs, GI infections, IBD

Calcineurin Inhibitor Renal Toxicity

Tacrolimus, cyclosporine Presentation: -Prerenal AKI, new-onset HTN -Labs: BUN:Cr of >20 Diagnosis: -Renal bx unremarkable -If acute, serum inhibitor levels may be elevated Treatment: -Usually reversible w/ adjustment in dosing (unless associated w/ chronic toxicity, extensive renal ischemia) Pathophysiology: -Vasoconstrictive properties, increased serum levels (e.g. by addition of agent w/ CYP450 inhibition) -> afferent, efferent arteriolar constriction, new-onset HTN -> renal ischemia -Calcineurin inhibitors increase vasoconstrictor endothelin, inhibit nitric oxide activity, increase sympathetic outflow

Giant Cell Arteritis

Temporal arteritis Presentation: -Jaw pain exacerbated by eating (jaw claudication), worsens during chewing and improves shortly after -Thickening & tenderness & decreased pulse over temporal artery ("cord-like"), temporal HAs -Visual: risk of anterior ischemic optic neuropathy (painless vision loss, presents w/ pale/edematous disc w/ blurred margins), amaurosis fugax (transient visual field defect progressing to monocular blindness) -Fever, fatigue, malaise, weight loss, associated w/ polymyagia rheumatica (morning stiffness affecting shoulders/hips) -Arm claudication (bruits in subclavian/axillary regions), aortic wall thickening or aneurysms, asymmetric loss of distal pulses -Labs: normochromic anemia, elevated ESR/CRP -Risk factors: age >50 Diagnosis: -Temporal artery bx (after starting steroids); shows necrotizing arteritis w/ mainly mononuclear cells Treatment: -High-dose systemic IV glucocorticoids (e.g. methylprednisolone) for GCA w/ vision loss -Intermediate to high-dose PO glucocorticoids for GCA -Low-dose PO glucocorticoids for polymyalgia rheumatica only Pathophysiology: -Chronic vasculitis affecting medium & large vessels -AION due to partial or complete occlusion of posterior ciliary artery -Amaurosis fugax associated w/ atheroemboli from carotids

Male Primary Hypogonadism

Testicular hypogonadism Presentation: -Low libido, decreased body hair, testicular atrophy, gynecomastia common -Labs: high LH/FSH, low testosterone, low sperm count Diagnosis: -High LH/FSH -Karyotype Pathophysiology: -Etiologies: congenital (e.g. Klinefelter, varicocele), acquired (e.g. radiation, infection like mumps, trauma, chronic disease, meds incl alkylating agents, glucocorticoids)

Dejerine-Roussy Syndrome

Thalamic pain syndrome/thalamic stroke Presentation: -Ischemia of sensory thalamic nuclei -> contralateral loss of sensation, tingling -> as stroke sx improve presents w/ episodic, severe pain w/ ongoing paresthesias

Maintenance Therapy

Therapy given after induction & consolidation therapies (or initial standard therapy) to kill any residual tumor cells & keep patient in remission (e.g. antiandrogen therapy for prostate cancer)

Consolidation Therapy

Therapy given after induction therapy w/ multidrug regimen to further reduce tumor burden

Onychomycosis

Tinea unguium Presentation: -Thick, brittle, discolored nails (yellow), usually painless but can cause snagging of socks -Can lead to extension (tinea pedis) into interdigital spaces -> itching, erythema, scaling of skin -Can cause fissuring that predisposes to bacterial entry -> paronychia -Risk factors: age, tinea pedis, DM, PVD Diagnosis: -KOH of nail scrapings, periodic acid-schiff stain, culture Treatment: -First line: terbinafine, itraconazole -Second line: griseofulvin, fluconazole, ciclopirox -Prolonged treatment often necessary, treatment failure common -6 wks duration for fingernails, 12 wks for toenails Pathophysiology: -Chronic dermatophyte infection of nails, most commonly by Trichophyton rubrum & similar fungi

Salvage Therapy

Treatment for a disease when standard therapy fails (disease recurs)

Neoadjuvant Therapy

Treatment given before standard therapy

Adjuvant Therapy

Treatment given in addition to standard therapy

Erectile Dysfunction

Treatment/Management: -First line is phosphodiesterase-5 inhibitors (e.g. sildenafil, contraindicated w/ use of nitrates or alpha blockers) -Second lines: prostaglandin E1 (e.g. alprostadil) -May supplement testosterone Pathophysiology: -May be organic (e.g. hypogonadism, T2DM, smoking) or psychogenic -Psychogenic may be due to interpersonal conflict, performance anxiety, underlying emotional disorder -Drugs: may be associated w/ beta-blockers, thiazide diuretics, spironolactone, SSRIs, cimetidine (less common w/ ACEi/ARB/CCB) -Neurologic causes typically caused by damage to T11-T12 sympathetic and/or S2-S4 parasympathetic fibers -CV causes associated w/ decreased penile blood flow, endothelial dysfxn, vasoactive med use

Trichinellosis

Trichinella spiralis roundworm, trichinosis Presentation: -Intestinal stage (within 1 wk of ingestion): may be asymptomatic or present w/ abd pain, N/V, diarrhea -Muscle stage (up to 4 wks after ingestion): myositis (pain/tenderness/swelling esp in neck/arms/shoulders), fever, subungual splinter hemorrhages, periorbital edema & chemosis, conjunctival/retinal hemorrhages -Usually acquired by ingestion of undercooked meat (usually pork) -Severe disease can involve heart, lungs, CNS -Labs: eosinophilia (usually >20%) w/ possible elevated CK, leukocytosis Treatment: -Mild infections usually self-limited, severe infections require antiparasitic therapy (albendazole, mebendazole) w/ corticosteroids Pathophysiology: -Endemic in Mexico, China, Thailand, parts of Central Europe, Argentina -Gastric acid releases larvae within 1 wk of ingestion -> invasion of small intestine -> develop into adult worms -Female worms release larvae in intestine up to 4 wks later that migrate & encyst in striated muscle

Myotonic Dystrophy

Trinucleotide repeat disorder Presentation: -Myotonia (delayed muscle relaxation, e.g. slow opening of hands after squeeze test aka grip myotonia, esp affects distal musculature, may be described as stiffness/cramping), progressive distal muscle weakness (e.g. face, hands; ptosis, lid lag, flat affect, dysarthria, foot drop, poor finger dexterity), widespread atrophy -Cognitive (e.g. intellectual impairment) & behavioral (e.g. ADHD, mood disorders) problems in childhood form; presents ~10 yo -Hypotonia, arthrogryposis (multiple joint contractures), club foot & other foot deformities, poor feeding, respiratory failure, absence of DTRs & primitive reflexes, cataracts, inverted V-shaped upper lip in infantile form -Arrhythmias, cardiomyopathy, dysphagia, constipation, cataracts, pharyngeal weakness, hypoventilation, excessive daytime sleepiness, testicular atrophy/infertility/hypogonadism, insulin resistance, frontal balding -Poor prognosis, death usually d/t resp failure or arrhythmias b/t 40-50 yo -Labs: low testosterone, high LH & FSH Diagnosis: -Genetic testing Treatment/management: -Supportive symptomatic care Pathophysiology: -AD trinucleotide repeat disorder (CTG repeat expansion in dystrophia myotonica protein kinase gene), repeat length inversely correlated w/ age of onset -Respiratory muscle failure and cardiac arrhythmias most commonly cause of death (avg 45-55 yo) -Trinucleotide repeat in DMPK gene -> transcribed into mRNA but not translated -> deposits & becomes toxic to cellular fxn, esp in skeletal/cardiac/smooth muscle

Chemotherapy-Induced Cardiotoxicity

Type I: -Associated w/ anthracyclines (e.g. doxorubicin, daunorubicin) -Myocyte necrosis & destruction -> fibrosis -Progression to overt clinical HF -Dose-related; related to cumulative dose -Less likely to be reversible Type II: -Associated w/ trastuzumab, increased risk combined w/ anthracycline & cyclophosphamide (25% vs. 5%) -Myocardial stunning/hibernation w/o myocyte destruction -Asymptomatic LV systolic dysfxn -If HF develops, should be completely d/c'd; responds to standard HF treatment w/ beta-blockers and ACEi -More likely to be reversible, typically fully recover cardiac fxn after treatment discontinuation Monitoring: -Radionuclide ventriculography (MUGA-multigated aquisition) scan; produces highly accurate and reproducible results for quantating LV EF -Performed at baseline & before each subsequent dose of chemo -Decrease in EF by 10+ T may warrant therapy discontinuation

Torsades De Pointes

Type of polymorphic VTach Presentation: -Rate: 120 - 200 usually -P wave: Obscured by ventricular waves -QRS: Wide QRS - "Twisting of the Points" -Conduction: Ventricular only -Rhythm: Slightly irregular Treatment: -IV Mg sulfate -Temporary transvenous pacing if not responsive to Mg sulfate

Ureterolithiasis

Ureteral stone Presentation: -Ureteral colic (severe, waxing & waning flank pain radiating to groin), hematuria, N/V, possible fever/chills (signs of urosepsis) -Often present writhing in pain, unable to sit still during exam -May have intermittent episodes of high-volume urination (post-obstructive diuresis--obstruction overcome by large volume of retained urine), excess diuresis can cause K+ wasting & dehydration -> weakness, fatigue -Imaging w/ stone in ureter Diagnosis: -Abd US (esp if pregnant) or noncontrast spiral CT Treatment/Management: -If signs of urosepsis, acute renal failure, complete obstruction (anuria), or if 1+ cm, admit & refer for urology consult (possible percutaneous nephrostomy or retrograde ureteral stent insertion, lithotropsy for large stones) -If <1 cm, may be managed conservatively w/ hydration (>2-2.5 L/day), analgesics (NSAIDs), alpha blockers (e.g. tamsulosin), anti-emetics, instructions to strain urine -If <1 cm and not managed by above, uncontrolled pain, or no stone passage after 4-6 wks, admit & refer for urology consult (possible lithotropsy, ureteroscopic intervention, perc nephrolithotomy, laparoscopic stone removal) Pathophysiology: -Ureteral spasm secondary to stone impaction, antagonism of alpha-1 receptors by alpha blockers -> ureteral relaxation, decreased intraureteral pressure to facilitate stone passage

Miller-Fisher Syndrome

Variant of Guillain-Barre syndrome Presentation: -Rapid-onset ophthalmoplegia (diplopia, ptosis), ataxia (e.g. dysmetria), lower extremity weakness (weakness vs. paralysis in other GBS forms), areflexia following GI illness Diagnosis: -Anti-GQ1b antibodies -CSF w/ albuminocytologic dissociation typical of GBS (high protein/albumin, normal WBCs) Treatment: -Plasmapheresis, IVIg Pathophysiology: -Molecular mimicry, antibodies against GQ1b (ganglioside found in peripheral nerves, esp in CN III, IV, VI)

Non-Allergic Rhinitis

Vasomotor rhinitis Presentation: -Nasal congestion, rhinorrhea, sneezing, postnasal drainage, usually w/o allergic conjunctivitis (itchy eyes, injected conjunctiva) -Appears later in life (>20) w/ no obvious allergic trigger but may have behavioral triggers (e.g. cold air, eating, strong smells) -Perennial (may worsen w/ season changes) but occurs throughout yr -Erythematous nasal mucosa Treatment: -Mild: intranasal antihistamine (e.g. azelastine, olopatadine) or glucocorticoid, intranasal ipratropium bromide -Moderate to severe: combination therapy

Reflex Syncope

Vasovagal syncope (neurocardiogenic syncope): -Preceded by autonomic prodrome (nausea, sweating, warmth, lightheadedness, pallor), tunnel vision -In response to a trigger (e.g. emotional stress, prolonged standing, pain, anxiety, heat) -Spontaneous, rapid recovery of consciousness in seconds to min Situational syncope: -Occurring w/ micturition (may be worsened by BPH), defecation, swallowing, coughing, hair-combing, eating Carotid hypersensitivity: -Precipitated by tactile stimulation of carotid sinus while standing -Associated w/ advanced age, carotid atherosclerosis Diagnosis: -Upright tilt table testing sometimes indicated in uncertain cases -Normal orthostatic vitals to rule out orthostatic syncope Treatment: -Reassurance & avoidance of triggers -Counterpressure techniques for recurrent episodes (e.g. leg crossing, hand grip to improve venous return & CO) Pathophysiology: -Due to alteration in autonomic response (e.g. d/t feedback from baro/mechanoreceptors) -> cardioinhibitory, vasodepressor, or mixed response -> transient cerebral hypoperfusion -> loss of postural tone -Increased parasympathetic stimulation -> profound bradycardia, varying degrees of AV block, asystole, decreased myocardial contractility -Decreased sympathetic output -> vasodilation of venous capacitance veins & systemic arterioles, hypotension, syncope

Libman-Sachs Endocarditis

Verrucous endocarditis Presentation: -Usually asymptomatic, may present w/ signs of systemic embolization (e.g. focal weakness d/t stroke) -Occurs in pts w/ advanced malignancy or SLE Pathophysiology: -Deposition of sterile vegetation (e.g. immune complexes, thrombus) on cardiac valves

Lateral Medullary Syndrome

Wallenberg syndrome -Mnemonic: LeMI PICcup MIself, eye am tIpsi, and my other side is numb -LeMI: lateral medullary infarct, PIC: posterior inferior cerebellar artery, cup: hiccups from autonomic dysfxn, MI: miosis, eye: ptosis (Horner), tIpsi: ipsilateral limb ataxia, other side is numb: contralateral loss of pain/temp Presentation: -Vestibulocerebellar sx: vertigo, falling toward side of lesion, difficulty sitting upright w/o support, diplopia, nystagmus (horizontal & vertical), ipsilateral limb ataxia -Sensory sx: abnormal facial sensation/pain (early) e.g. stabbing sensation, loss of pain/temp in ipsilateral face (spinal trigeminal nucleus/tract) & contralateral trunk/limbs (spinothalamic tract) -Ipsilateral bulbar muscle weakness: dysphagia, aspiration, dysarthria, dysphonia, hoarseness (d/t ipsilateral vocal cord paralysis, nucleus ambiguus involvement) -Autonomic sx: ipsilateral Horner syndrome due to disruption of sympathetic fibers (ptosis, miosis, anhidrosis), intractable hiccups, lack of autonomic respiration (esp during sleep) -Diminished corneal reflex d/t involvement of spinal trigeminal nucleus -Sparing of voluntary motor fxn Diagnosis: -Brain MRI Treatment: -IV thrombolytics (tPa) Pathophysiology: -Stroke affecting vertebral or posterior inferior cerebral arteries -> damage of vestibular nuclei, inferior cerebellar peduncle

Midbrain Infarction

Weber syndrome/Benedikt syndrome Presentation: -Mnemonic: "web in my eye" -Ipsilateral CN III palsy (ptosis, impaired pupil constriction, down & out gaze, diplopia) -Dysarthria, dysphagia -Contralateral hemiparesis/weakness -Crossed signs c/w brainstem lesions (CN deficits in one side, motor weakness on other side) -Sensory deficits Pathophysiology: -Occlusion of mesencephalic branches of posterior cerebral artery or the perforating branches of basilar artery bifurcation

Granulomatosis with Polyangiitis

Wegener granulomatosis Presentation: -Fever, malaise, weight loss -Upper respiratory: recurrent epistaxis, nasal septal necrosis & destructive rhinosinusitis (saddle nose deformity), ear pain (otitis) & hearing loss -Lower respiratory: hemoptysis (chronic pneumonitis, diffuse alveolar hemorrhage), nodular lung lesions, cavitation, tracheal narrowing w/ ulceration -Renal: glomerulonephritis (RPGN) -Skin: subcutaneous nodules, palpable purpura, livedo reticularis, nonhealing ulcers, leukocytoclastic angiitis, pyoderma gangrenosum -Labs: elevated Cr, anemia of chronic disease -Risk factors: 30-50 yo, Caucasian Diagnosis: -ANCA-positive (PR3+ in 70%, MPO+ in 20%) -Bx w/ leukocytoclastic vasculitis of skin, pauci-immune/necrotizing/crescentic GN of kidney, granulomatous vasculitis of lung -CXR w/ nodules w/ cavitation, alveolar opacities Treatment/Management: -Corticosteroids, immunomodulators (e.g. methotrexate, cyclophosphamide, rituximab) Pathophysiology: -Small & medium vessel vasculitis

Mobitz I 2nd Degree AV Block

Wenckebach Presentation: -Usually asymptomatic, irregular heart rhythm d/t intermittent delay of ventricular contraction -Possible occasional bradycardia w/ fatigue, lightheadedness, angina, syncope, HF Diagnosis: -ECG w/ progressive PR lengthening followed by dropped QRS complex Treatment/Management: -Usually observation -Rarely pacemaker placement if symptomatic

Multiple Endocrine Neoplasia Type 1

Wermer syndrome Presentation: 3 Ps (pancreatic, pituitary, parathyroid) mnemonic -Pancreatic tumors (endocrine tumors, most commonly insulinoma and gastrinoma, sometimes VIPoma, glucagonoma) -Pituitary tumors (10-20%, most commonly prolactinoma w/ galactorrhea, Cushing, acromegaly, nonfunctioning adenoma, mass effects like HA & visual field deficits) -Parathyroid hyperplasia (hypercalcemia) or multiple parathyroid adenomas -Multiple cutaneous lipomas -Carcinoid tumors Treatment/management: -Parathyroidectomy, pancreatic tumor excision, transphenoidal surgery for removal of pituitary tumor -Screening w/ IGF-1 or prolactin for pituitary lesions Pathophysiology: -Altered menin protein expression

Fragile X Syndrome

X-linked dominant disorder Presentation: -Hypotonia in infancy, sensory disturbances (e.g. sensitivity to tags, autistic & ADHD features), aggression, anxiety, self-injurious behaviors (e.g. hand biting), speech & fine motor delays, intellectual delays, macroorchidism (after 8 yo), seizures -Usually start walking/talking ~20 mos -Excessive flexibility, hypermobility of joints (esp hands) -Long/narrow face, large ears, macrocephaly, prominent forehead & chin Pathophysiology: -Trinucleotide repeat (CGG) expansion of fragile X mental retardation 1 (FMR1) gene

Klinefelter Syndrome

XXY Presentation: -Breast development (gynecomastia), tall stature (increased long bone length), minimal body hair, penis and small descended testes (hypogonadism), infertility, learning/socialization difficulties (autism spectrum, intellectual disability, psych disorders) -Hypergonadotropic hypogonadism (elevated FSH & LH, low testosterone) due to testicular damage during development; delayed puberty -Normal presentation at birth, becomes evident at puberty (can develop small phallus, hypospadias, cryptorchidism) -Increased risk of male breast cancer Diagnosis: -Karyotype of peripheral leukocytes -Low testosterone, elevated LH & FSH, increased estradiol Pathophysiology: -Nondisjunction of sex chromosomes resulting in 47,XXY male -Infertility d/t testicular fibrosis w/ seminiferous tubule dysgenesis, azoospermia

Sympathomimetic Toxidrome

o Includes: amphetamines, cocaine, MDMA, ecstasy, molly, ephedra, OTC cold agents containing ephedrine o Symptoms—MATHS; agitation (not easily restrained), tachycardia, tachypnea, hypertension/hyperthermia, hyperreflexia, seizures/sweating, differentiate from anticholinergic toxicity by "wet" handshake, mydriasis not as pronounced, hyponatremia, anorexia, feelings of euphoria/intimacy o Treatment—mostly supportive with controlling agitation, HTN, hyperthermia, seizures; benzo use for agitation, hydration, treatment of complications like rhabdo

Sedative-Hypnotic Toxidrome

o Includes: benzodiazepines, barbiturates, Z-drugs (zolpidem, eszopiclone, zaleplon) o Symptoms—depressed mental status, general autonomic depression (bradycardia, hypotension, hypothermia), bradypnea/miosis less common, ataxia, slurred speech o Treatment—flumazenil (can induce seizures, compentitive benzo antagonist, contraindicated with seizure hx, ingestion of seizure-provoking substance like TCA, long-term benzo use, hypoxia, hypoventilation, head trauma)

Serotonin Syndrome

o Includes: linezolid, MAO inhibitors, cocaine, dextromethorphan, fentanyl, SSRIs/SNRIs, Provigil, ondansetron, TCAs, trazodone o Symptoms—hyperpyrexia, hypertension, hyperreflexia/tremor/myoclonus (ocular clonus--slow horizontal eye movements), autonomic instability, depressed or hyperactive AMS, cardiac arrhythmias, seizures, headaches, hyponatremia o Treatment—supportive with rapid external cooling for hyperthermia, aggressive benzodiazepine use for muscle rigidity, cyproheptadine (5-HT2 blocker)

Opiate Toxidrome

o Includes: narcotics, cocaine o Symptoms—depressed mental status, general autonomic depression (bradycardia, hypotension, hypothermia), bradypnea (RR<12), miosis (pinpoint pupils, may be normal/enlarged if coingestion), AMS, apnea, hypoxia, flash pulmonary edema, decreased bowel sounds o Diagnosis—ABG (respiratory acidosis), fingerstick blood glucose, evaluate for presence of other drugs, ECG for prolonged QTc (methadone overdose) o Treatment—naloxone (may need repeated doses, titrated to achieve RR of 12+), methadone for withdrawal, intubation, consider continuous cardiac monitoring (if QTc >500 ms)

Contamination Bias

-Occurs when members of the 'control' group inadvertently receive the treatment or are exposed to the intervention, thus potentially minimizing the difference in outcomes between the two groups

Abdominal Aortic Aneurysm Screening

-One-time duplex ultrasound screening in men 65-75 years who have ever smoked -Also considered in men 65-75 who have never smoked but have other risk factors (e.g. FDR w/ h/o AAA rupture)

Preclinical Trials

-Phase involving no human subjects

Heinz Bodies

-Small inclusions in RBCs representing aggregates of denatured Hgb -Commonly seen in patients w/ hemolysis due to G6PD deficiency, thalassemias -Become bite cells when phagocytes extract precipitate

Variceal Bleed Algorithm

-Somatostatin analogues (e.g. octreotide) inhibit release of vasodilator hormones -> splanchnic vasoconstriction, decreased portal flow -If Hgb <9, transfuse -If plts <50,000 or active bleeding, transfuse

Koilonychia

-Spoon nails, reverse curvature of nails -Associated w/ iron deficiency

Burr Cells

-Echinocytes, spiculated appearing RBCs w/ serrated edges that may be seen in association w/ liver disease & ESRD

Brain Natriuretic Peptide (BNP)

-Produced by ventricular muscle cells, elevated in HF -May also be elevated in severe obesity, pts taking angiotensin receptor-neprilysin inhibitor (e.g. sacubitril-valsartan); neprilysin normal degrades BNP

Ankle-Brachial Index

-Ratio of systolic BP in ankle to SBP of arm (SBP of dorsalis pedis or posterior tibial artery / SBP of brachial artery) -Used to confirm suspected PAD-0.9 or lower diagnostic of PAD, 0.91-1.3 normal, >1.3 suggests calcified & noncompressible vessels (other testing should be considered)

Indications for Urgent Dialysis

AEIOU -Acidosis -Electrolyte abnormalities (hyperkalemia >6.5 refractory to medical therapy or w/ sx including arrhythmias, ECG changees) -Ingestions (salicylates, theophylline, methanol, barbiturates, lithium, ethylene glycol, valproic acid, carbamazepine) -Overload (fluid) -Uremic symptoms (pericarditis, encephalopathy, bleeding, nausea, pruritus, myoclonus, asterixis)

Friedreich Ataxia

AR trinucleotide repeat disorder Presentation: -Neurologic deficits: cerebellar ataxia, dysarthria, loss of vibration and/or position sense (dorsal columns, spinocerebellar tracts), absent DTRs, spastic weakness (lateral corticospinal tracts) -Classically presents w/ gait ataxia and dysarthria in adolescents -Hypertrophic cardiomyopathy, skeletal deformities (e.g. scoliosis, pes cavus/high arches), DM -Mean survival 30-40 yo, mortality usually due to cardiac dysfxn (arrhythmia, congestive HF) Diagnosis: -Genetic testing Treatment: -Multidisciplinary supportive care (PT, psychological support) -Monitoring for complications Pathophysiology: -Loss of fxn trinucleotide repeat (GAA) in frataxin gene (frataxin highly expressed in brain, heart, pancreas)

Physician-Assisted Suicide

Aka euthanasia -Factors most often associated w/ requests for euthanasia: loss of autonomy & control of dying process, loss of dignity, loss of ability to engage in pleasurable activities, fears of future suffering -Commonly requested by patients w/ terminal cancer

Microsporidium

Aka isosporidium Presentation: -Watery diarrhea, crampy abd pain, weight loss -Fever RARE -CD4 <100

Popliteal Cyst

Baker's cyst Presentation: -Asymptomatic bulge behind knee (medial popliteal space) that diminishes w/ knee flexion -Posterior knee pain, swelling, stiffness -Risk factors: trauma (e.g. hx meniscal tear), underlying joint disease (e.g. OA, RA) -Complications: venous compression (results in leg/ankle edema), dissection into calf (erythema, edema, positive Homan sign), rupture (acute calf pain, warmth, erythema, ecchymosis) Pathophysiology: -Extrusion of fluid from knee joint space into semimembranosus/gastrocnemius bursa -Excessive fluid production (e.g. RA) and leg extension can cause enlargement of cyst

Facial Nerve Palsy

Bell palsy Presentation: -Facial droop, unilateral facial paralysis, disappearance of nasolabial fold, weakness in closing eye or raising eyebrow (vs. stroke & other central causes spare upper face) -Ipsilateral eye lacrimation, hyperacusis, decreased taste sensation over anterior 2/3 of tongue -Often develops over course of hrs, often overnight, then sx progress over 2-3 wks, gradually improve over 3-6 mos, overall good prognosis (especially if paralysis is incomplete) -Risk factors: pregnancy/postpartum Diagnosis: -If classic idiopathic presentation (acute, peripheral findings, no systemic findings suggestive of other conditions, e.g. Lyme), no further workup -If red flag signs (sparing of upper face, associated hearing loss, associated facial twitching, etc.), should undergo neuroimaging Treatment/Management: -Glucocorticoids, possibly add valacyclovir if suspect HSV -Supportive care including artificial tears, eye patching, taping of eye at night ->85% completely recover w/ high-dose glucocorticoids Pathophysiology: -Possible causes include neurologic disease (e.g. stroke, GBS), granulomatous disease (e.g. sarcoid), infections (e.g. otitis media, herpes zoster, Lyme disease), neoplasms (e.g. cerebellopontine angle, parotid) -Peripheral neuropathy involving CN VII d/t reactivation of neurotrophic virus -> inflammation & edema of facial nerve -> nerve compression, degeneration of myelin sheath -May be associated w/ HSV reactivation, Lyme disease

Anticholinergic Toxidrome

Includes: muscle relaxants (e.g. OAB medications), antispasmodics, antiparkinson agents, scopolamine, herbals like jimson weed (Datura stramonium) & belladonna, mushrooms, antihistamines, TCAs Presentation: -"Mad as a hatter, blind as a bat, hot as a hare, red as a beet, dry as a bone;" "can't see, can't pee, can't spit, can't s***;" mydriasis (dilated, nonreactive pupils, blurry vision), confusion (agitated, hallucinating, delirious, usually can be restrained,, memory impairment), dysarthria, ataxia, dry/flushed skin, dry mouth, urinary retention, shaking, tachycardia/HTN, absent bowel sounds, seizures (end-stage), hyperthermia, anhidrosis Diagnosis: -ECG to evaluate for possible QRS or QTc prolongation Treatment: -Physostigmine (anticholinesterase, for severe central & peripheral sx), not recommended in more complex overdoses where anticholinergic effects are not main cause of toxicity, bicarbonate to control some dysrhythmias, benzos for agitation/seizures, activated charcoal if awake pt <2 hrs since ingestion Pathophysiology: -Competitive inhibition of ACh receptors in CNS, ciliary body, heart, smooth muscle, secretory glands

Erythropoietin

Indications: -Treatment of CKD-related anemia (significant renal failure & Hgb of <10) -Demonstrated to improve QOL & reduce LVH (& associated HF sx) ADRs: -New/worsening HTN in up to 30% of pts within 2-8 wks of treatment initiation, risk of hypertensive crisis w/ end-organ damage (encephalopathy, seizures, etc.) -Increased risk of ischemic stroke, venous thrombosis, & all-cause mortality w/ high Hgb levels (13+)

CYP450 Substrates

Mnemonic: Always Think When Outdoors Anti-epileptics Theophylline Warfarin OCPs

CYP450 Inducers

Mnemonic: Barb's Funny Mom Refuses Greasy Carb Shakes Phenobarbital Phenytoin Modafinil Rifampin Griseofulvin Carbamazepine St. John's wort/sulfonylureas

Myelodysplasia

Myelodysplastic syndrome Presentation: -Conjunctival pallor -Malignant hematopoietic neoplasm presenting w/ normocytic/macrocytic anemia, leukopenia, thrombocytopenia -Hepatosplenomegaly/lymphadenopathy rare -Risk factors: older age, previous chemo/radiation Diagnosis: -Peripheral blood smear w/ dysplastic granulocytes (hypogranulated, hyposegmented), dysplastic RBCs (ovalomacrocytosis) -BMBx w/ hypercellular marrow Treatment/Management: -Transfusions for symptomatic cytopenias, chemo, hematopoietic stem cell transplant, EPO for anemia Pathophysiology: -Has the potential to transform into acute leukemia

Meningococcal Meningitis

Neisseria meningitidis Presentation: -Most common in young children >1 mo and young adults (e.g. college students), rapidly progressing -Initially nonspecific sx including fever, headache, vomiting, myalgias (e.g. extremity pain w/ palp), sore throat (nonsuppurative pharyngitis) -Within 12-24 hrs, petechial rash/purpura on extremities due to thrombocytopenia, meningeal signs (neck pain/stiffness, Brudzinski sign), AMS, pale/mottled skin & pallor, cold hands/feet from hypoperfusion -Complications include shock, DIC, adrenal hemorrhage (Waterhouse-Friederichson -> sudden vasomotor collapse w/ hypotension, large purpuric lesions on flank, close to 100% mortality) -If disseminated, may present w/ pustular skin lesions, tenosynovitis, polyarthritis Diagnosis: -1. Blood cultures -2. Start abx -3. LP w/ CSF culture diagnostic (neutrophilic predominance, low glucose, high protein), testing should not delay treatment w/ empiric abx (ceftriaxone, vanco to cover S. pneumo) Treatment/management: -Ceftriaxone, droplet precautions and chemoprophylaxis w/ rifampin/ciprofloxacin (single dose)/ceftriaxone (single dose) for close contacts -Empiric coverage for penicillin-resistant Strep pneumo includes vancomycin, esp in children Pathophysiology: -Signs of poor perfusion/shock may reflect myocardial depression from increased IL-6

Charcot Joint

Neurogenic arthropathy Presentation: -Acute stage presents w/ inflammatory erythema, warmth, edema of foot w/ minimal bone involvement (X-rays often normal) -Chronic: foot & ankle deformity (swelling, collapsed arch, decreased ROM), impaired ambulation, mild pain -Associated w/ skin calluses, neuropathic ulcers -Labs: blood counts, CRP, synovial fluid analysis all normal -Risk factors: DIABETIC NEUROPATHY, other peripheral neuropathies (e.g. B12 def, alcoholism), spinal cord injury, syringomyelia, tabes dorsalis (tertiary syphilis) -Complications: osteomyelitis, amputation, foot ulcers, cellulitis Diagnosis: -X-ray w/ bone & joint destruction (bone loss, osteophytes, loss of joint space), fragmentation (extraarticular bone fragments), subluxation/dislocation/sclerosis, phalangeal osteolysis ("hourglass" appearance), "sucked candy" appearance of metatarsal heads d/t partial disappearance Treatment/Management: -Mechanical offloading & correction of joint mechanics (e.g. casting in acute phase, orthotics or surgical realignment in chronic phase) -Management of infectious complications Pathophysiology: -Impaired sensation & proprioception -> altered weight bearing & recurrent trauma/mechanical stress -> acute inflammatory response & hyperemia -> bone resorption via osteoclast stimulation -> bone & ligament destruction (e.g. stress fxs, subluxation, arch collapse) -> abnormal weight bearing -Pain only mild due to underlying neuropathy

Acute Radiation Proctitis

Presentation: -8 wks or shorter after radiation, presents w/ diarrhea, mucus discharge, tenesmus, minimal bleeding Diagnosis: -Severe erythema, edema, ulcerations on colonoscopy Treatment: -Antidiarrheals (e.g. loperamide), butyrate enemas, fluids Pathophysiology: -Direct mucosal damage from radiation for prostate/colon/ovarian/cervical cancer

Strychnine Poisoning

Presentation: -"Awake seizure:" episodic contractions that look like tonic-clonic activity in fully awake pt -Powerful, uncontrollable muscle contractions Pathophysiology: -Ingredient in rodenticide & some illicit drugs that blocks inhibitory (glycine) neurotransmission in spinal cord

Facet Osteoarthritis

Presentation: -Cause of neck/back pain in older individuals -Presents w/ pain/stiffness worse w/ movement, relieved by rest & NSAIDs -Normal reflexes, no muscle weakness

Primary Progressive Aphasia

Presentation: -Dementia syndrome that primarily affects language early in disease -Imaging w/ atrophy of parietal lobes

Cryptogenic Organizing Pneumonia

Presentation: -Dry cough, systemic sx lasting mos Diagnosis: -Chest imaging w/ bilat ground-glass infiltrates, multifocal opacities

Pulmonary Hypertension

Presentation: -Dyspnea on exertion, weakness, fatigue in early disease -May progress to chest pain (exertional angina), hemoptysis (rare), syncope, hoarseness (compression of recurrent laryngeal nerve), abd distention/pain (bowel edema), early satiety -End-stage disease w/ RV failure (RV heave, loud P2, R-sided S3 and/or S4, pansystolic murmur of tricuspid regurg, L parasternal lift, JVD, tender hepatomegaly, ascites, edema, etc.), cor pulmonale, diastolic pulmonary regurg murmur if severe (Graham-Steell murmur) -CXR w/ enlargement of pulm arteries (cephalization) & rapid tapering of distal vessels (pruning), RV enlargement -ECG may show R axis deviation, RVH -PFTs may show normal lung volumes, reduced DLCO Diagnosis: -TTE to estimate pulm arterial pressure, evaluate for RV dyfxn, secondary causes of PH -Mean pulmonary arterial pressure of 25+ Hg at rest (definitive dx w/ R heart catheterization) -Further workup for etiology w/ PFTs, V/Q scan, polysomnography, chest CT, etc. Treatment/Management: -Group 1: endothelin receptor antagonists (e.g. bosentan, ambrisentan) to delay progression of disease by dilating pulm arteries & antagonizing effects of endothelin (potent vasoconstrictor produced by endothelial cells), phosphodiesterase-5 inhibitors (e.g. sildenafil, tadalafil), prostacyclin pathway agonists (e.g. epoprostenol, treprostinil, iloprost), CCBs (e.g. long-acting nifedipine) if positive vasoreactive test during R heart catheterization -Group 2: treat underlying cardiac disease, e.g. ARBs/ACEi to decrease afterload & minimize cardiac remodeling -Group 3: treat underlying pulm disease, e.g. CPAP in OSA, COPD management Pathophysiology: -Group 1: idiopathic primary PAH (or d/t drugs, HIV, CTD like scleroderma), d/t intimal hyperplasia of pulmonary arteries -Group 2: PAH d/t L heart disease -Group 3: PAH d/t chronic lung disease, hypoxemia (e.g. OSA) -Group 4: PAH d/t chronic thromboembolic disease -Group 5: PAH d/t other causes (e.g. hematologic, systemic disorders like sarcoidosis) -May be d/t narrowing of precapillary vessels, loss of pulm capillary bed, or passive backpressure from postcapillary vessels

Hypothyroid Myopathy

Presentation: -Muscle pain, cramps, weakness involving prox muscles -Delayed DTRs, myoedema, occasional rhabdomyolysis -Other sx of hypothyroidism -Labs: normal ESR, elevated CK

Multiple Sclerosis

Presentation: -Young & middle-aged women (usually 15-50 yo) -Episodic, progressive, usually asymmetric deficits in multiple sensory/motor domains (diplopia, focal weakness, bowel/bladder dysfxn (urge incontinence), vertigo, ataxia, pronator drift, intention tremor, nystagmus, etc.)--"neurologic deficits disseminated in space & time" -Positive Lhermitte sign (electrical, shock-like sensation in limbs/back) usually no LMN signs -Uhthoff phenomenon (sx that worsen w/ increased body temp, heat sensitivity) -Internuclear ophthalmoplegia (impaired adduction of lateral gaze, disorder of conjugate horizontal gaze in which affected eye ipsilateral to lesion is unable to adduct & affected eye abducts w/ nystagmus, convergence & pupillary light reflex preserved) d/t damage to medial longitudinal fasciculus (communicates b/t CN III & VI to allow for coordinated horizontal eye movements, classically bilateral in MS), afferent pupillary defect, optic neuritis (painful eye movement, monocular vision loss, afferent pupillary defect) -Can present as transverse myelitis (flaccid paralysis/spinal shock at distinct spinal level w/ bowel/bladder dysfxn -> spastic paralysis w/ pain & stiffness) -Dysphagia, gastroparesis -Risk factors: young (<50) female, Caucasian, HLA-DRB1, environmental factors (USA/Europe, cold climates, reduced sunlight exposure), low vit D levels, smoking, EBV infection, postpartum period -Different forms: relapsing-remitting (most common), primary progressive, secondary progressive, progressive relapsing Diagnosis: -Noncontiguous, well-circumscribed, multifocal, ovoid white matter lesions that are hyperintense on T2-weighted MRI (well-demarcated plaques of demyelination) in periventricular, juxtacortical, infratentorial, corpus callosum, deep white matter, spinal cord regions -Oligoclonal IgG bands, elevated IgG index on CSF analysis -Visual evoked potentials if testing equivocal Treatment/Management: -Exacerbations, disabling sx treated w/ glucocorticoids (e.g. IV methylprednisolone first if optic neuritis, transition to PO), hastens neurologic recovery -Plasmapheresis, IVIg if flare resistant to corticosteroids -Baclofen or tizanidine may be used for spastic sx, gabapentin/duloxetine for neuropathic pain, amantadine & stimulants (e.g. methylphenidate, modafinil) for fatigue, anticholinergics for urge urinary incontinence (e.g. oxybutynin, tolterodine) -DMARDs including IFN-beta & glatiramer acetate & natalizumab used for chronic maintenance therapy in pts w/ relapsing-remitting or secondary/progressive forms of MS -Pregnancy: higher rate of operative/assisted delivery, flares treated w/ IV glucocorticoids, DMARDs teriflunomide & mitoxantrone contraindicated d/t teratogenicity Pathophysiology: -Inflammatory, immune-mediated demyelinating disorder w/ plaques in brain and spinal cord, white matter lesions -Dysphagia & gastroparesis: parasympathetic underactivity d/t CNS demyelinating lesion (e.g. of dorsal vagal nucleus of the medulla, origin of the vagus nerve)

Senile Purpura

Solar/actinic purpura Presentation: -Noninflammatory disorder most common in elderly or pts w/ extensive sun exposure -Presents w/ purple discoloration, ecchymoses over vulnerable regions like dorsum of arms/hands d/t blood extravasation -Residual brown discoloration after healing d/t hemosiderin deposition -Risk factors: anticoagulants, corticosteroids, NSAIDs Treatment/Management: -Not a dangerous condition, may be seen w/ normal aging -May require careful wound care measures d/t skin fragility Pathophysiology: -Skin fragility d/t loss of elastic fibers in perivascular connective tissue -> predisposition to rupture of superficial blood vessels in vulnerable areas

Takotsubo Cardiomyopathy

Stress induced cardiomyopathy, "broken heart syndrome" Presentation: -Angina most commonly presenting in older pts (classically postmenopausal women) following significant stress (e.g. death of loved one, physical stress) -Substernal CP that mimics MI, dyspnea, decompensated HF -Typically resolves in wks w/ supportive care Diagnosis: -TTE: bulging out of the left ventricular apex ("balloon-like" appearance) with a hypercontractile base of the left ventricle often noted, apical hypokinesis + basilar hyperkinesis -ECG: may have changes including T wave inversion, ST elevation/T wave inversions/ischemic changes in precordial leads, QTc prolongation -Catheterization shows no obstructive CAD -Elevated cardiac markers (moderate troponin elevation), catheterization w/o obstructive CAD Pathophysiology: -Transient LV dysfxn in setting of intense physical/emotional stress and in absence of significant CAD -Thought to be due to catecholamine surge from recent stressor -> microvascular spasm w/ ischemia & myocardial stunning -> impaired contraction, ballooning of LV ("octopus trap" = takotsubo)