Peds EOR Objectives

Cystic Fibrosis

*Essentials* - Pulmonary problems: chronic or recurrent productive cough, dyspnea, and wheezing, recurrent airway infections or chronic colonization of the airways with H flu, P aeruginosa, Staph A, or Burkholderia; bronchiectasis and scaring on chest radiographs; airflow obstruction on spirometry - Gastrointestinal problems: pancreatic insufficiency, recurrent pancreatitis, distal intestinal obstruction syndrome, or chronic liver disease -*Pancreatitis* - Genitourinary problems: male infertility and urogenital abnormalities - Sweat chloride concentration greater than 60 mEq/L on two occasions - Presence of two gene mutations known to cause cystic fibrosis *(DeltaF508 important)*- CFTR - Abnormal nasal potential difference MC cause of severe chronic lung disease in young adults and MC fatal hereditary disorder of whites in U.S. It is autosomal-recessive Abnormalities in membrane Cl channel (CFTR) *Sx* - Suspect in adults with a history of chronic lung disease (especially bronchiesctasis), pancreatitis, or infertility -*Meconium ileus at birth* -Decreased growth rates -Recurrent REsp infections (pseudomanas and staph) - - Cough, sputum production, decreased exercise tolerance, and recurrent hemoptysisc - Chronic sinusitis symptoms, steatorrhea, diarrhea, and abd pain - Pts usually are malnourished - Digital *clubbing*, increased AP chest, hyperresonance to percussion, and apical crackles - Sinus tenderness, purulent nasal secretions, and nasal polyps *Lab* -Elevated sweat chloride -Fecal elastase testing -PFT: obstruction typically, but can be mixed - ABG studies often reveal hypoxemia & chronic compensated resp acidosis - Pulm fx studies show mixed obstructive and restrictive pattern - *Imaging* - Hyperinflation - Peribronchial cuffing, mucus plugging, bronchiectasis (*ring shadows and cysts*), increased interstitial markings, small rounded peripheral opactities, and focal atelectasis - Pneumothorax may be seen - Thin-section CT confirms bronchiectasis - *Quantitative pilocarpine iontophoresis sweat test* shows ^^^ Na/Cl in sweat (2 tests on 2 difnt days; normal doesnt exclude) *Tx* - Clearance of lower airway secretions can be promoted by postural drainage, chest percussion or vibration techniques, positive expiratory pressure (PEP) or flutter valve breathing devices, directed cough - Inhaled recombinant human DNase decreases sputum viscosity (nebulized 2.5mg) - Inhalation of hypertonic (7%) saline twice daily - Short term ABX for infections: Staph A, Pseudomonas, H flu - Long term ABX needed if positive pseudomonas -> *Zpak* 3 times/wk - *Inhaled bronchdilators* (Albuterol) considered if pts demonstrate increase FEV1 of 12% p inhaled bronchodilator tx - Inhaled steroids given if persistent asthma or allergic bronchopulmonary mycosis - *Ivacaftor* CFTR modulator drug - Lung transplant only definitive tx - Vaccination against pneumococcal infection advised; screening of family suggested

Foreign Body Aspiration

*Essentials* - Sudden onset of coughing or resp distress - Difficulty vocalizing MC lodges in supraglottic airway, triggering laryngospasm MC with small round foods such as nuts, seeds, berries, corn/popcorn, hot dogs, beans MC on RIGHT SIDE due to wider more vertical and shorter right main bronchus. Highest risk children *6mo to 4yo* *Sx* - coughing, choking, wheezing -Unilateral diminished breath sounds may be seen, localized wheezing - Onset abrupt, w/ hx of running with food in the mouth, or playing with seeds etc - Acute onset of choking PLUS inability to vocalize or cough and cyanosis w/ marked distress or drooling, stridor, and ability to vocalize *Dx* - Chest Xray may help, but may be normal up to 17% of time. Possible mediastinal shift - *Rigid Bronchoscopy is GOLD* for dx *Tx* -If clinical suspicion persits based on 2 of 3 findings: hx of possible aspiration, focal abnormal lung exam, or abnormal CXR- bronchospy indicated. *Rigid bronchoscopy under general anesthesia recommended*. Flexible bronchoscopy may be helpful in followups -Following removal of foreign body, Nebulizer tx of albuterol and chest PT recommended to help clear related mucus and tx bronchospasm. - If complete, intervene immediately - Partial obstruction, subject should be allowed to use his/her cough reflex to remove fb (if worsening, intervene) - Awake child <1yr w/ complete, place child face down and deliver back blows & chest thrusts - Child >1yr, heimlich maneuver performed w/ special care of organs - NO BLIND FINGER SWEEPS

Osteochondroma

*MC benign tumor in children* -Cartilage capped bone spur on external surface of bone -These masses result from developmental defect of growth plate -Greatest in 2nd Decade, M>F -Distal Femur>Proximal humerus —-Presentation—— -Painless mass near affected joint -When present, pain is caused by bursitis or tendinitis d/t irritation by tumor —-Radiographic imaging——- -Bone spur arising from surface of cortex -Often pedunculated, growing away from growth plate and involves medullary tissue. -Usually points away from joint —-Tx—- -Observation -Excision if it interferes with fxn, frequently traumatized, or is large enough to be deforming ——Prognosis—— -Excellent -Malignant transformation very rare.

Diaper dermatitis

- A form of primary irritant dermatitis, MC form of primary irritant contact derm seen in Peds -Caused by prolonged contact with urine and feces, which contain irritatin chemicals such as urea and intesti9nal enzymes -Develops within a few hours, peaks at 24 hours, and then disappears -In comparison to allergic dermatitis which has a delayed onset of 18 hrs, peaks at 48-72 hrs, and often lasts as long as 2-3 weeks even if exposure is discontinued. ——Sx—— -Erythema and scaling of skin in perineal area and hx of prolonged skin contact with urine and feces -In cases lasting >3 days, usually coonized with Candida albicans even before app;earnce of classic beefy red, sharply dermarcated dermatitis with satellite lesions —-Tx—- -Change diapers frequently -Area should only be washed following a bowel movement -Air drying useful -Tx of long standing cases should use barrier cream such as zinc oxide with each diaper change and imidazole cream twice per day. -Topical petroleum.

Slipped Capital Femoral Epiphysis

- Orthopedic Emergency! -Occurs in obese children aged 11-16 yrs. If seen in children before puberty, suspect hormonal/systemic disorders like hypothyroidism, hypopituitarism. ^ incidence in:African american and polynesians -RF's: endocrine disorders, obesity, coxa profunda (deep acetabular socket), femoral or acetabular retroversion. -Pathophys: physis weakened during times of rapid growing and is susceptible to shearing failure either actuely 2ry to traumatic injury or insidiously d/t chornic overload. *Head of femur usually displaced medially and posteriorly relative to femoral neck*. -Classified as stable or unstable. Considered stable if child is able to bear weight, unstable if unable to bear weight. ^ risk of avascular necrosis with inability to bear weight. ———-sx———— -Pts complain of groin, thigh, or medial knee pain and often have a limp -PE= painful ROM of hip, *limited IR*, often have a limp. Obligatory ER when hip is flexed -Often walking in External rotation ——-imaging——— -AP and frog-leg lateral films= widening of physis and epiphyseal slippage or displacement of femoral head relative to femoral neck. -*Klein's Line*: line drawn parallel to superior border of femoral neck. Epiphysis should project superiorly to the line. Should have equal sides of ice cream on both sides of the cone. ———tx——- -Immediate non-weight bearing and referral to ortho for ORIF. PT after surgery. Return to activity progressive over months -Complications: can result in permanent hip deformity and early arthritis

Exanthems

- Term for generalized cutaneous eruption associated with primary systemic reaction - Etiology: inflammatory/immune response secondary to drugs or infection Will have seperate card for each of the exanthems, many of which will also knock out the objectives for Infectious Disease portion of the Pediatric EOR topic list.

Meningitis Tx

---<1 mo--- -Amp PLUS Cefotaxime or aminoglycoside ---1 mo- 18 yo---- -Ceftri + Vanco ---18-50 yo--- -Ceftri + Vanco + Dexamethasone if d/t strep pneumo --->50 yo--- -Vanco + Ampicillin + Ceftriaxone + Dexamethasone if d/t strep pneumo ---Impaired Cellular immunity--- -Vanco + Ampicillin+ Cefepime ---Post op or post trauma--- -Vanco + Cefepime *Dexamethasone IV if known or suspect Strep Pneumo or also in children if d/t H flue type B d/t decreased hearing loss if used* Post exposure prophylaxis= Cipro 500 mg PO one dose or Rifampin

Pneumonia- older kids/adults

---Bacterial Organisms--- 1) S. Pneumo= MCC, seen following URI (g+ cocci) 2)H. Flu= 2nd MCC. Seen with underlying pulm diseases more frequently, or following URI (G- rods) 3)Myocplasma Pneumoniae= MCC of atypical (walking) pneumonia. Seen in young adults summer/fall) 4) Staph Aureus= Seen in chronic care facilities, hospital associated pneumonia, influenze epidemics, IV drug users (G+ cocci) 5)Klebsiella Pnuemoniae= seen in severe illness in ALCOHOLICS, chronic illness (diabetes), debilitated, aspirators. Associated with CAVITARY lesions. (plump g- encapsulated rods) 6) Legionella- Summer and fall. Exposure to contaminated water supplies (AC's, cooling towers, etc), contaminated contstruction sites. GI symptoms more common (anorexia, NVD, ^ LFT's, hyponatremia) 7)Anerobes: Aspiration, severe peridontid disease. Fetid sputum, pulm abscess and empyema. MC in Right lower lobe. 8) Pseudomonas: Immunocompromised (HIV, neutropenic, s/p transplant), strucutural abnormalities (cystic fibrosis, bronchiectasis) ---Viral Causes--- 1) RSV/Parainfluenza (infants/small children) 2) Influenza (MC viral cause in adults) 3) CMV (transplant recipients, AIDS pts) 4) Varicella Zoster -----Signs/Sx--- -Fever/hypothermia, tachypnea, cough +/- sputum, dyspnea, chest discomfort, sweats, rigors, SOB/DOE -Bronchial breath sounds or inspiratory crackles -^ Tactile fremitus, dullness to percussiion, egophony -Clinical evaluation is <50% sensitive compared to chest imaging, therefore a chest radiograph is essential to the eval of suspected condition -Atypical PNeumonia sx (like mycoplasma, legionella, viruses): you will typically see more of a low grade fever (rather than sudden onset seen in bacterial), dry nonproductive cough and *extrapulmonary symptoms*: myalgias, malaise, sore throat, HA, NVD (like viral syndrome). PE often normal and signs of consolidation usually not present. ---unique Qualities--- -Chlamydophilia: hoarseness, fever, then respiratory symptomas after a few days -Mycoplasma: ear pain, bullous myringitis, erythematous pharynx or TM's. persistent nonproductive cough - Legionella: Associated with GI sx, ^ LFT's, hyponatremia. Send legionella urine antigen +/- PCR ---Unique lab/ PE findings--- -Upper lube (especially RUL) with bulging fissures/cavitations=klebsiella -Abscess formation, think: S. Aureus, klebsiella, anaerobes ----Sputum Qualities--- -Rusty (blood tinged): S. Pneumo -Currant Jelly: Klebsiella -Green sputum: H flu, pseudomonas -Foul smelling: anaerobes --Dx--- -A pulmonary opacity on CXR or CT required. Keep in mind no pattern or radiographic abnormality is pathognomonic of any infectious cause -Pts with cavitary opacities should have sputum fungal and mycobacterial cultures -Sptum exam may be helpful in selected pts, but 40% cannnot produce evaluable sample and gram stain and culture lack sensitivity for the most common causes of pneumonia -Outpatients typically don't need labs done such as sputum culture, etc. as empiric abx therapy is almost always effective in this populations. -In ambulatory outpts whose presentation (travel history, expsoure) suggests etiology not covered by standard therapy (coccidiosodes) or public health concerns (e.g: Mycobacterium tuberculae, influenzea) diagnostic testing appropritate. -Urinary antigen tests available for s. pneumo and legionella species, nearly as sensitive and specific as sputum gram stain and culuture. Order for patients with leukpenia, recent travel w/in last 2 wks, asplenia, active ETOH use, severe liver disease, pleural effusions, and those requiring ICU admit. -CXR in atypical pneumonia often diffuse patchy interstiital or reticulonodular infiltrates --Labs needed for admit--- -Blood cultures (2 sets 2 different sites) -Sputum cultures if productive cough -Urine for pneumococcal/legionella antigen if appropriate. -AFB if cavitarial disease -Pleural fluid if effusion -----When to Admit---- CURB 65= Confusion, Uremia (>7 mmol/L or 19 mg/dL), Respiratory rate (>/= 30/min), BP (SBP <90 or DBP <60), Age >65 -0-1= Home tx -2= Inpatient ward ->/=3 ICU admit ---Tx: Outpatient--- 1) If previously healthy, no risk factors, no antiobiotic use in last 90 days: Macrloide (Clarithromycin or Azithromycin) or Doxycycline 2) If Patient at risk for drug resistance (ABx in last 90 days), >65 yo, comorbid illness, Immunosupressed, exposure to child in daycare: Respiratory floraquinolone (moxifloxacin) OR Macrolide plus b-lactam (HD amox or Augmentin) --Tx: Inpatient, non ICU---- 1) 1st line= Respiratory floraquinolone ( Moxifloxacin, levo/cipro) 2)If resistance or risk factors for drug resistance present: IV macrolide + IV B-Lactam -Tx: ICU--- 1) If no pseudomonas RF's present: Azithromycin or FQ PLUS anti-pneumococcal B-lactam= CEFTRIAXONE 2) If pseudomonal RF prsent: Pip-tazo, cefepime, imipimen, or miropenem PLUS FQ 3) If pseudomonal RF prsent: Cipro or aminoglycoside PLUS Azithromycin ---Prevention--- -Pneumococcal vaccine (see next card) and yearly flu vaccine)

Inguinal Hernia

---Etiology/Notes---- -Processus vaginalis remains open and peritoneal fluid or abdominal structures forced into it (indirect inguinal hernia) -M>F (9:1) -Incidence in preterm male infants close to 5% and is reported in 30% of male infants weighing 1000g or less -Incarceration more likely to occur in boys and kids <10 mos ---Signs/Sx---- - Painless inguinal swelling -Retracts when cold, active, frightened, agitated -+/-: vomiting, ab distension -Signs: Inguinal fullness with coughing or long periods of standing, or presence of firm, globular, tender swelling. --Dx--- -Suggestive histores often only criterion for dx along with the *silk glove* feels of the rubbing together of the two walls of the empty hernia sac. ---Tx--- -Manual reduction of incarcerated inguinal hernias can be attempted after the sedated infant is placed in Trendelenburg position with ice bag on affected side. -Manual reduction CI'd if incarceration has been present for more than 12 hrs or if stools are bloody -Surgery indicated if a hernia has ever incarcerated -Hydroceles frequently resolve by age 2 -Complications: more likely in boys <10 mos

Lennox-Gastaut Syndrome

--Essentials---- Manifests between ages 2-7 yrs, triad of: 1. Mental retardation 2. Diffuse slow spike and wave pattern on EEG 3. Multiple types of generalized seizures -Pts. commonly have status epilepticus ---Info--- -Electroclinical diagnosis: 1) >2 seizure types (atonic, tonic, atypical absence), 2) 1.5-2.5 Hz spike and wave -Poor development -Causative factors: multiple causes, usually resulting in diffuse neuronal damage. Hx of infantile spasms, prenatal/perinatal braine damage, viral encephalitis -EEG pattern: atypical slow (1-2.5 Hz) spike wave complexes and bursts of high voltage generalized spikes, often with idffusely slow background frequency. ---Tx--- -Difficult to tx -Valproic acid best initial tx -Most require supervision and living in group homes, helmets often prescribed -Other tx's: Topiramte, felbamate, ethosuximide, rufinamide, keto diet, vagus nerve stimulation may help -Avoid: phenytoin, carbamazepine, oxcarbazepine, gabapentin

Infantile Spasms (West Syndrome)

-3-8mo onset, rare if >2yo -*tuberous sclerosis* most commonly identified cause; others: PKU, GLUT1 -brief, myoclonic jerks 1-2s each, in clusters of 5-10 over 3-5min -Abrupt, usually but not always, symmetrical adduction or flexion of limbs with flexion of head and trunk; or abduction and extensor movements (similar to Moro reflex). Occurs in clusters upon waking. Associated irritability and regression in development -sudden arm extension or head/trunk flexion -> jackknife or salaam seizures -EEG: *hypsarrhythmia pattern (highly disorganized high amp spike and waves in both hemispheres)* -tx: ACTH, prednisone, vigabatrin -poor prognosis, will develop MR (mental retardation)

Contact Dermatitis

-85% of cases d/t excessive exposure to or additive effects of universal irritants (soaps, detergents, organic solves)= irritant contact derm. Appears red and scaly but not vesicular and occurs only in the direct sites of contact with the irritant (like me and pool towels) -MCC allergic contact derm= poison ivy or poison oaky, topically applied antimicrobials (esp bacitracin and neomycin), anesthetics (benzocaine), hair cair products, jewlery (NICKEL0, rubber, essential oils, bees, vit E, adhesive tape -*Weeping and crusting typically d/t allergic and not irritant dermatitis* —-Signs/Sx—— -Allergic contact derm= acute phase characterized by tiny vesicles and weepy/crusted lesions, whereas resolving or chronic contact derm presents with scaling, erythema, and possibly thickened skin -Lesions distrubited on exposed parts or in bizzare assymetric patterns, consit of erythematous macules, papules, vesicles -Pattern of eruption may be diagnostic= *linear streaked vesicles* on extremities in poison oak or ivy. -Location suggests cause. Scalp= hair dyes/shampoos. Face=creams, cosmetics, soaps, shaving stuff, nail polish. Neck= jewlery, hair dye. —-Dx—— -Mostly clinical -Can do gram stain and culture if suspecting impetigo -Positive patch test in allergic contact derm (after epsiode has cleared) -Consider scabies if itching is generalized —-Tx—— - Avoid irritants. Protective equipment, wet dressings (Burrow's solution), topical CS -Acute weeping:Compresses, gentle cleansing, calamine lotion/zinc oxide paste. High potency CS may help. -Subacute: mid potency to high potency CS -Chronic: High potency to superpotency CS in ointment form -Acute severe cases: can do PO prednisoe for 12-21 days

Influenza

-A associated with more extensive outbreaks -Spread primarily via airborne resp secretions; outbreaks occur mainly in fall/winter —-Signs/Sx—— -Usually abrupt onset of a wide range of sx: HA, fever, chills, malaise, URI sx, pharngitis, pneumonia. -Myalgias MC seen in legs and lumbosacral area -Usually absent are: rash, marked conjunctivitis, adenopathy, exudative pharngitis, and dehydrating enteritis -Fever, diarrhea, vomitting, and ab pain common in young children -Infants may develop a sepsis-like illness and apnea -Chest exam usually unremarkable. -Acute illness lasts 2-5 days. -Cough and fatigue may last several weeks. Viral shedding may persists for several weeks in young children —-Dx—- -Usually clinical; but in children specific lab tests recommended -PCR has highest sensitivty and specificity (close to 100%) and is rapidly becoming preferred test. -Rapid influenza test (nasal swab) or viral culture can be peformed ——Imaging—- -Not needed -CXR nonspecific and may show hyperaeration, peribronchial thickening, increased vascular markings, diffuse interstitial infiltrates. Hilar nodes not enlarged. —-Tx—- -Supportive tx is mainstay of tx in healthy pts (acetaminophen or salicylates, rest) -Antivirals usually only needed in pts with high risk of complications or hospitalized. *best if initiated within 48 hrs of onset of sx* -Neuraminidase inhibitors (works against both A and B: Oseltamivir (PO), Zanamivir (diskhaler) -Antivirals vs influenza A (widespread resistance, not realy used anymore): Amantadine, Rimantidine)

Salmonella

-A form of invasive, inflammatory diarrhea -Recent foreign travel can cause it. -Incubation period-1-3 days -Foods: Eggs, poultry, unpasteurized milk, cheese, juices, raw fruits and veggies. -Typhoidal transmission: Humans only resovoir, poor sanitation and unclean water. Street vendor food, raw fruits/veggies. -2 types: Typohidal (enteric fever) and non-typhoidal (gastroeneritis) -SX non typhoidal: Incubation 6-48 hrs, n/v, fever, diarrhea with cramping and rarely bloody, resolves 4-7 days. -Clinical features: Gradual OR abrupt onset of diarrhea (bloody and mucusy) and low grade fever. -SX typhoidal: incubation 5-21 days. Ab pain, fever chills, diarrhea in kids, constipation in adults, relative bradycardia (<50%), rose spots (30%) -Complications of typhoidal: intestinal bleeding, perforation, bacteremia with metastatic infection, endocarditis, abscess. Chronic carriers in 1-4% -Dx: culture blood, stool, bone marrow (bone marrow more sensitive than blood) -Tx for typhoid: Fluroquinolone, ceftriaxone -Tx for non-tyhoidalSupportive (no antimotility agents), fluids.No antimicrobials unless high risk or sysemic dissemination is suspected, in which case give a fluoroquinolone, 3rd gen ceph. Prolonged carriage can occur.

Perioral Dermatitis

-A papular erythematous eruption -MC in women aged 16-45 -Etiology= hormones, LONG TERM STEROID USE,, contact allergy, irritatns —-Clinical features—- -1-2 mm clustered, erythematous inflammatory papules, papulovesicles, or papulopustules around the mouth, nose or eyes -Papulopustules on an erythematous base, which may become confluent into plaques with scales. MAy have satellite lesions. -*Classicaly spares vermillion border* —-Dx—- -Clinical —-Tx—- -Topical abx in mild cases= Metronidazole or erythromycin -Oral: Tetracyclines (usually needed for 6-8 weeks to prevent recurrences)

Erythema Multiforme

-Acute self limited type IV hypersensitivity rxn; mc in young adults 20-40 yo -Skin lesions usually evolve over 3-5 days and persist around 2 weeks -Associations: Herpes simplex virus (MC), mycoplasma (esp in PEDS), Strep pneumo -Recurrent episodes associated with reactivation of herpes simplex virus -Meds: Sulfa drugs, beta-lactams, Phenytoin, Phenobarbitol, etc) ——Findings—- -Begins with papules that later develop a dark center and then evolve into lesions with central bluish discoloration or blisters and the characteristic target lesions (iris lesions) that have 3 concentric circles of color change -Targe tlesions: dull "dusty-violet" red, purpuric macules/vesicles or bullae in the center surrounded by pale edematous rim and peripheral red halo. Often febrile ——Classificaitons—- -EM Minor: Target lesions distributed acrally; *No mucosal membrane lesions* -EM Major: Target lesions with involvement of >/= 1 mucous membranes (oral, genital, or ocular mucosa) <10% BSA acrally—>Centrally. *No epidermal detachment* —-Tx—- -Stop offending agent -Oral antihistamines like cetirizine and hydroxyzine (before bed) is helpful -Cool compressess and wet dressings will relieve pruritis -Steroids have not been demonstrated to be effective -Chronic acyclovir therapy has been successful in decreasing attacks in pts with hperes-associated recurrence -Steroid/lidocaine/diphenydramine mouthwash for oral lesions

Viral Conjunctivitis

-Adenovirus MCC, swimming pool MC source -HIGHLY contagious ——Sx—— -Foreign body sensation, redness swelling -Mattering in morning but lightly throughout day -Copious tearing, might have coryza/URI -Normal vision -Often bilateral -Redness and swelling of lids common. -Copious watery disharge, scanty mucoid discharge -Preauricular lymphadenopathy -May have punctate staining on slit lamp exam ——Tx—- -Supportive (cool compress, artifical tears) -Prophylactic topical abx can be used (polytrim)

Epistaxis

-Anterior= MC -RF's= Nasal trauma (picking nose, blowing nose forcefully, etc), humidity in a hot environment (dries nasal mucosa), rhinitis, EOTH, antiplatelet meds ——Anatomy—— -Kesselbach's plexus= MC site of bleeding in anterior epistaxis -Posterior: Palantine artery is MC site (may cause bleeding in both nares and posterior oropharynx ——Tx—— -Initial: hemostasis may be achieved by having patient blow nose, spray nares with oxymetolazone, and apply direct pressure (pinch nose) and lean forward (reduce vessel pressure) -Topical decongestants/vasoconstrictors: Good adjunct. mentioned, can use oxymetolazone (Affrin). Others= phenylephrine, cocaine. Be cautious in pts with HTN ——If initial Tx don't work—— -Anteiror: Cautery with silver nitrate or electrocautery. Nasal packing until hemostasis achieved -Posterior: Balloon or foley cath, hospitalize and ENT consult, remove packing, abx prohylaxis.

Scoliosis

-Assymetry of the spine: usually lumbar or thoracic -Types: 1)Idiopathic (80%): 8-10 yrs old, F>M.... 2)Congenital (5-7%)- failure of vertebrae to form or segment. 3) Neuromuscular 4)Syndromic -Congenital types: 1)Wedge vertebrae: partial unilateral failure of formation 2) Hemivertebra: Complete unilateral failure of formation 3)Congenital Bar: unilateral failure of segmentation 4) Block vertebra: bilateral failure of segmentation. ————Sx———— Curve >10 degrees -If curve >30 degrees: Rib cage deformity or rib hump. Assymetrical waist line -If curvature >70 degrees= decreased pulmonary function in adulthood -No pulm inovvlment typically seen w/ cobb angle <35 degrees ——PE——— -Look at apperance of rib cage and waist line -Forward bending test: can detect curvatures <30 degrees -OBservation of back in standing position: levelness of pelvis observed 1st, any leg length discrepancies are corrected (compensatory scoliosis). -Adams test= slow forward bening. Used to assess symmetry. Observe from behind (thoracic area) and in front (lumbar area) ———Radiographic imaging——— -PA and lateral spine Measure Cobb Angle: Intersection of lines: -1 parallel to superior end plate of most cephalad vertebra in curve -1 parallel to inferior end plate of most caudad vertebra in curve ———-TX————- -Curvature <20 degrees= continue monitoring with serial x-rays. Treat only if curvature progresses -Curvature 20-40 degrees= Consider bracing in skeletally immature patient -Surgical intervention (Vertical expandable prosthetic titanium ribs and eventual fusion) indications= Curvature >40-60 degrees AND progressing curvature

Hereditary Spherocytosis

-Autosomal Dominant intrinsic hemolytic anemia. MC in northern Europeans -Jaundice Occurs in 1st 24 hrs of life ---Pathophys--- - RBC membrane cytoskeleton defect (spectrin)= ^ ell fragility and sphere-shaped RBC's--> ^ RBC hemolysis in spleen by splenic macrophages. -Have a defect in one of the RBC membrane proteins causing a spherical shape to the cell. This shape causes the cell to be more fragile than a biconcave cell and allows it to hemolyze more readily. The shape of the cell also makes it unable to pass easily through the splenic red pulp fenestrations which leads to hemolysis. - Aplastic crisis if infected with parvovirus B19 ----Signs/Sx--- -Anemia, jaundice -Splenomegaly -Pigmented black gllastones (clacium bilirubinate) ---Dx---- -Coombs test (-) -Reticulocytes: high -Hyperchromic microcytosis -*(+) osmotic fragility test (easy cell lysis)* -Microcytic anemia, normal HCT, spherocytes on peripheral smear -^ RDW -*^MCHC* ----Tx---- -Splenectomy is a treatment option for hereditary spherocytosis because it eliminates the most prevalent site of hemolysis. -Treatment also should include daily folic acid because of the increase in bone marrow demand.

Androgenic Alopecia

-Autosomal dominant with variable penetrance -Onset: 3rd-4th decadee ——-Pathogenesis—— -Progressive miniaturization, not destruction -Decrease duration of anagen phase -Increase percent of hairs in telogen —-Clinical manifestations—- -Patterned hair loss -Varying degrees of hair thinning and nonscarring hair loss MC affecting temporal scalp, midfront scalp, or vertex of scalp area —-Tx—— -Finasteride (beware of sexual SE's) -Minoxidil -Hairgrafting, wigs, weaves -Women: OCP, spironolactone

Obesity

-BMI >/=95th percentile for age and gender -Severe obesity= >99th percentile; ^ risk of complications -Overweight= BMI b/t 85-94th %ile -Higher rates in blacks and hispanics. -Adolescent who is overweight has 70% chance of being obese adult -Do a through review and history of pts including asking about diabetes hx, gallbaldder hx, menstrual irregularities -Associated with CV and endocrine abnormalities (dyslipidemia, T2dm), ortho problems, pulm complications (OSA) and mental health problems -An annual increase of 2kg/m^2 of BMi is almost always an indicator of a rapid increase in fat -For kids <2 yrs, weight for length >95th percentile indicates overweight and warrants further assessment, esp of energy intake and feeding bheaviors ——Screenings—— -Labs recommended as follows for kids beginning by age 10 yrs or onset of puberty -Consider testing at younger ages with severe obesity but not younger than 2 -If Overweight and no risk factors: Fasting lipid panel -If overweight but has fam hx of obesity related diseases, elevated BP and/or lipid levels, tobacco use: do FLP, AST and ALT, fasting serum glucose -If obese: FLP, AST and ALT, fasting serum glucose —-Tx—- -Most current evidence on obesity tx in Peds reccomends a 4-stage approach that includes: 1) Prevention Plus (5-2-1-0), 2) structured weight management, 3) comprehensive multidisciplinary intervention, 4) tertiary care intervention -May need to eval for OSA and PCOS if symptoms present -Wt loss goals for obese children range from weight maintenance up to 1 lb/mo weight loss for those younger than 12 to 2 lb/wk for those >12. -Very few guidelines regarding pharmacotherapy for obesity in adolescent. -Med options include: Sibutramine (SSRI, approved for 16+), Orlistat (approved for 12+) -Bariatric surgery reserved for severely obese adolescents are physically mature, who have a BMI of 50 or more (or >/= 40 with significant comorbidities) who have failed a structured 6-month weight loss program, and who are deemed by psychological assessment to be capable of adhering to long term lifestyle changes required after surgery.

Hypoglycemia

-Blood sugar <60; for preschool children, values <70 should be cause for concern -Signs/sx:hunger, weakness, shakiness, sweating, drowsiness (at an unusual time) HA, beahvioral changes.; "feel funny", confusion, slurred speech, dizziness -Sx occur at 60, brain dysfxn begins at 50 —-Tx—— -Mild: Give 4 oz juice, a sugar-containg soda, milk, and wait 10 mins; if still <60, repeat. If above 60, give solids -Moderate (person conscious but incoherant): squeeze one-half tube of concentrated glucose between gums and lips and stroking throat to encourage swalllowing -Severe/uncounscious (<40): IV bolus of D50 or Glucagon SQ injection.

Urticaria

-Can be caused by various stimuli. -MCC's= foods, infectios, meds -Chronic causes= not often found -Typically IgE mediated —-Signs/Sx—— -Itchy, red swellings of a few millimeters to many centimeters -Morphology may vary over a period of mins to hours -Typically last <24 hrs, often only 2-4 hrs duration -Angioedema is involvement of deeper SubQ tissue with swelling of lips, eyelids, palms, soles, and genitalia. No more likely to be associated with systemic complications -Cholinergic urticaria= triggered by a rise in core body temp (hot showers, exercise). Wheals are 2-3 mm in diameter with a larger surrounding red flare. -Cold urticaria= acquired or inhierted and triggered by exposure to cold wind. ——-Labs—— - Often not helpful -CBC w/ diff, ESR/CRP, TSH, and LFT's may be helpful in chronic cases -In pts with individual lesions that persists >24 hrs, skin biopsy may confirm neutrophilic urticaria or urticarial vasculitis ——Ddx—— - Insect bite urticaria often persists several days with central punctum seen -Streaked urticarial lesions may be seen in 24-48 hrs before blisters appear in acute allergic plant dermatitis (like poison ivy, oak, sumac) ——Tx—— -Determine cause -Mainstay of tx= H1-antihistamines (hydroxyzine 10 mg PO BID) -Cryoheptadine 4mg PO 4x a day may help in cold urticaria -Other options that aren't sedating= fexofenadine, cetirizine, or loratadine -CS and montelukast can also help -Doexpin may be helpful in chronic cases -Epi if anaphylactic

Multiple Myeloma

-Cancer associated with proliferation of a single clone of plasma cells=^ monoclonal antibodies (Esp IgG and IgA) -Monoclonal Ab accumulate in BM= interrupt normal production ->10% plasma cell in BM, 75% of cases- antibodies produced in abnormal amounts (light chains only). These light chains only being produced= paraproteins. -^risk: elderly (>65), AA men; disease of elderly -Clinical Manifestations: Bones BREAK *B*= bone pain: esp in spine and ribs d/t osteolytic destructive lesions and osteopenic fx *R*= Recurrent infections from leukopenia. Hyperviscosity *E*=Elevated calcium (*only heme malignancy associated with bone destruction*)- nerve problems and dehydration. *A*= Anemia- (shift from myeloid to lymphoid progenitor cells to make more b cells, over production of blasma cells in BM, failing kidney so less EPO) *K*= Kidney failure (neuro sx) (proteinuria) -DX made with serum or urine protein electrophoresis. *Serum may show monoclonal protein spike (IgG or IgA)*. Urine can show *Bence-Jones proteins(light chains peed out)*, markedly ^ ESR, spontaneous falling -Blood and urine: anemia, abnormal RBC level, ^ paraproteins, decreased normal antibodies, hypercalcemia, ^BUN, ^Scr, Bence-Jones proteins. -DX:1)monoclonal plasma cells in BM >10%, 2)monoclonal antibody in serum or urine, 3)One or more of the following: HRAL: hypercalemia, renal insufficiency, anemia, lytic bone lesions. -BM biopsy: plasmacytosis >10%/ -Bone radiographs impt for dx: CT/PET> plain radiographs. DONT USE BONE SCAN's (don't work) (lytic bone lesions can be seen) -TX: Chemo: dexamethasone, lenalidomide, AND a proteasome inhibitor -Definitive dx= autologous stem cell transplant (pts <80 y.o.)

Rubella (German Measles)

-Causative Agent: Togavirus -Transmission: resp droplets -2-3 week incubation period *3 day rash* —-Signs/sx—- -Low grade fever, cough, anorexia, lymphadenopathy (postertior cervical, auricular)—> fine, pink maculopapular rash appears and fades quickly from face, trunk, extremities in rapid progression, usually 1 day in each location (lasts 3 days) -Forchheimer sports: Small red macules or petechiae or on soft palate (also seen in Scarlet fever) -*Transient photosensitivity and joint paints may be seen (esp in young women)* -Devastating effects on fetus: Classic blueberry muffin, cataracts, deafness, psychomotor retardation, congenital heart disease —-Dx—- -Clinical -Elevated IgM (false positive can occur with CMV, EBV, parvovirus) -IgG ^ 4x, or isoloation of virus —Tx— -Supportive, anti-inflammatories. Generally no complications in kids with this, compared to rubeola —-Prevention—- -MMR

Hand-foot-mouth disease

-Causative Agent= Coxsackie A Virus (part of enterovirus family) -Spread= feco-oral and oral-orally -MC late summer/early fall ——-Signs/Sx——- -Mild fever (<101), URI sx, decreased appetite starting 3-5d after exposure -Progresses to oral exanthem: vesicular lesions with erythematous halos in the oral cavity (esp buccal mucosa and tongue)—> exanthem 1-2 days afterword: vesicular, macular or maculopapular lesions on the distal extremities *(including palms and soles)* —-Dx—- -Mainly clinical —-Tx—- -Supportive Resolves in 7-10 days

Roseola infantum (Sixth Disease)

-Causative Agent= HHV 6/7 -Transmission= respiratory droplet; ~10 day incubation period -Produce acute infection in CD4+ T lymphoctyes -MC in children <2 ——Signs/Sx—— -High fever (102-104) for up to 8 days, then rash in 20-30%; can have rash alone -Prodrome of high fever for 3-5 days—>*Fever resolves before onset of rose-pink maculopapular blanchable rash *that starts on trunk, spreads outward and coalesces (to face). Rash can last for hours to 1-2 days. -Febrile seizures common (up to 10%) -Child appears "well" and alert during febrile phase; may be irritable -*only childhood viral exanthem that starts on trunk and spreads to face* —-Dx—- -Clinical —-Tx—- -Fever control with acetaminophen; cool sponge bath -Monitor closely as this is one of, if not the, top cause of febrile seizures

Tinea Versicolor

-Causative Agent= Malasezzia Furfur -Usually upper trunk in location. -Eruption often called to pts attn by the fact that involved areas will not tan, and thus resulting in hypopigmentation that may be mistaken for vitiligo. -Hyperpigmentation common too —-Sx—- -Lesions asymptomatic, but a few pts note itching -Lesions are velvety, tan, pink, or white macules or thin papules that vary from 4-5mm in diameter to large confluent areas. -Lesions do not look scaly, but scales may be readily obtained by scrapin the area —-Dx—- -Large, blunt hypae and thick walled budding spores on KOH (spaghetti and meatballs) -Fungal culture not useful —-Ddx—- -Vitiligo usually presents with larger periorifacial and acral lesions and is also characterized by total (not partial) depigmentation -Vitiligo does not scale —-Tx—- -Topicals= selenium sulfide lotion applied daily for 7 days, ketoconazole shampoo -Ketoconazole 200mg PO daily x 1 week or 400 mg PO single dose with exercise to the point of sweating. Often not 1st line d/t risk of drug induced hepatitis. Do not shower for 8-12 hrs after taking ketaconazole. —-Prognosis—- -Alterations in pigmentation may take months to fade or fill in.

Measles (Rubeola)

-Causative Agent= Paramyxovirus; Highly contagious; 10-12 day incubation period; so exposure typically 9-14 days previous -Transmission: Respiratory droplets, person to person, airborne —-Manifestations—— -URI Prodrome initially: High fever + 3 C's: Cough, coryza, conjunctivitis -Koplik Spots: White lesions on buccal mucosa with red halo (pathognomonic) precedes rash by 24-48 hrs, lasts 2-3 days -Morbiliform (maculopapular) brick-red rash on face beginning at hairline and moving to extremities (palms and soles involvement usually seen last if it occurs) that darkens and coalesces -Rash usually lasts 7 days fading in order it appeared; fine desquamation may occur -Fever peaks when rash appears and usually falls 2-3 days thereafter ——Labs—— -Lymphopenia is characteristic; total leukcocytes may fall to <1500/uL -Dx made by detection of measles IgM and may be made later by detection of significant rise in IgG -Direct detection of measles antigen by fluorescent antibody staining of nasopharyngeal cells is a useful rapid method -PCR extremely sensitive and specific, and can dtect infection up to 5 days before sx. —-Complications—- -Diarrhea, otitis media, pneumonia, conjunctivitis, encephalitis —-Tx— -Supportive, anti-inflamatories -Vaccination prevents disease in susceptible exposed individuals if given within 72 hrs -Immune globulin will prevent or modify disease if given within 6 days -Vit A reduces mortality in all children wit this —-prognosis—- -Recovery generally occurs 7-10 days after onset of sx ——Prevention—- -MMR vaccine given once at 12-15months and once at 4-6 yrs

Erythema Infectiosum (Fifth Disease)

-Causative Agent= Parvovirus B19 -MC <10 yo -Transmission: Resp droplets; 4-14 day incubation period —-Signs/Sx—— -Typically well appearing, *slapped cheek*, circumoral pallor, and lacy maculopapular rash on trunk/limbs -Coryza,fever—>*slapped cheek* rash on face with cirumoral pallor 2-4 days—> lacy reticular rash on extremities (esp upper) *spares palms and soles*. Resolves in 2-3 weeks -*no longer contagious once rash appears* -Polyarthritis syndrome can occur in adults -Fever, malaise, HA, pruritis of palms/soles and diarrhea less common -Can temporarily decrease/halt the bodys production of RBC's causing transietn aplastic crisis -In pregnancy, can lead to fatal severe anemia, myocarditis, hydrops fetalis, or fetal death (esp if contracted b4 20 weeks gestation) —-Dx— -Clinical -Serologies —-Tx—- -Supportive, anti inflammatories

Oral Candidiasis

-Causative agent: Candida Albicans ——Signs/Sx—— -Mouth or throat pain -May see decreased feeding in child ——-Dx—— -Clinical: white curd-like plauqes *may leave behind erythema and bleed if scraped* -KOH: budding yeast/pseudohyphae ——Tx—— -Nystatin liquid (swish in mouth)= tx of choice -Can also do clotrimazole losenges or gentian violet painting -If those do not work: PO fluconazole

Varicella

-Causative agent= HHV3 —-Signs/sx—- -Prodrome of nausea, HA, muscle aches—> fever and rash -Rash: maculopapular, spreads cephalocaudal (head to toe) -Clusters of vesicles on an erythematous base: *dew drops on a rose petal* in diferent stages *Polymorphic (macules, papules, vesicles, pustules, crusted lesions) *at any given time beginning on face trunk to textremities. -Usually pruritic -Herpes Zoster: VZV reactivation along one dermatome; does not cross midline. Usually >55 yo or immunosuppressed (screen for HIV if <55). Prodrome of pain and tingling; eventually severe pain. Progression of papules—>vesicles—> pustules—->Crusts. *Dermatomal pattern* -Herpes zoster Ophthalmicus: shingles involving 1st division of trigeminial nerve. Tip of nose involved= Hutchinson Sign; heralds occular involvement -Ramsay Hunt syndrome: Facial Nerve (CN 7): otalgia, lesions in ear, auditory canal, tympanic membrane, facial palsy, auditory sx (tinnitus, vertigo, deafness, ataxia) ——Dx—- -Clinical -Confirmed with PCR (old fashioned: Tzanck smear showing multinucleated giant cells) -Infection in women <28 wks of gestation can lead to fetal harm —-Tx—- -Acyclovir x 5 days within 1st 24 hrs of rash -Ramsay Hunt: Acyclovir + steorids -Herpes zoster ophthalmicus: Acyclovir + possible topical CS —Prevention—- -Varicella Vaccine given at 12-18 months and again at 4-6 years -Do not give in pregnancy since it is a live vaccine

Mumps

-Causative agent= paramyxovirus -Transmission= respiratory droplets -Incubation= 16-18 days; infectious 3 days before or 9 days after symptoms start -^ incidence in spring ——Signs/Sx—- -Parotid tenderness/swelling, facial edema (uni or bi lateral) up to 10 days -Stenson's duct may be red/swollen. Yellow secretions may be expressed, but pus absent. -Trismus/fever can occur -Low grade fever, myalgia headache ——Other presentations/Complications—- -Orchitis (unusual in young children, but occurs in up to 1/3 of post-pubertal males; usually unilateral) -Pancreatitis -Meningoencephalitis -Others: thyroiditis, mastoiditis, deafness, infertility —-Dx—- -Clinical when parotitis is present -Commonly seen abnormalities: leukopenia with lymphocytosis. -Viral cultures or PCR's may be positive for at least 1 week after diagnosis -IgM ^ considered diagnostic. —-Tx—— -Supportive: provision of fluids, analgesics, scrotal support for orchitis -Systemic CS have been used for orchitis, but value is anecdotal —-Pt info/Prognosis—— -Entire course rarely >2 weeks -Isolation until selling subsides, 5-9 days from onset -Prevent by immunizations and prompt diagnosis to expedite isolation.

Hypervitaminosis D

-Cause is almost always d/t ingestion of excessive amounts of Vit D -Same signs and sx as seen in other hypercalcemic states -Tx depends on stage of hypercalcemia -Severe hypercalcemia requires hospitalization and aggressive intervention -Since vit D is a fat-soluble vitamin, several months of low calcium, low Vit D diet may be required

Verruca

-Caused by HPV. Typing of HPV lesions not part of standard eval except in the case of anogenital dysplasia ——Signs/Sx—- -Usually no sx -Tenderness on pressure occurs with plantar warts -Itching occurs with anogenital warts -Common and plantar warts= firm, hyperkeratotic papules between 1-10 mm with *red-brown punctations (thrombosed capillaries= pathognomonic)*. Borders +/- rounded or irregular. Common on hands -Flat warts (verruca plana): numerous, small discrete, flesh-colored papules measuring 1-5 mm in diameter and 1-2 mm in height. Typically on face, hands, shins -Genital warts (condyloma accuminata): Tiny, painless papules evolving into soft, fleshy cauliflower-like lesions ranging from skin-colored to pink or red, occuring in clusters in genital regions and oropharynx. May persist for month and may spontaneously resolve ——Dx—— -Mucosal HPV: Whitening of lesion with acetic acid application. —-Tx—— -For common warts of hand, usually can use liquid nitrogen or keratolytic agents like salicyclic acid, paring -Genital warts: Can do cryotherapy, or do podophylin, or imiquimod plus paring. —Prevention—- -HPV vaccine (gardasil) can be done ages 9-45 (but textbook may say 9-26)

Infant GERD

-Causes secondary conditions d/t all that reflux, less common than GER -Pathophys: Transient LES relxation, increased intragastric volume. Supine and/or slumping after feeding. Hiatal Hernia. PMH of ashtma, CF, esophageal fistula -Onset usually 1-4 mos of life, but can happen anytime -Common sx: dysphagia, hoarsenss or cough, arching back during feeding, regurgitation/vomitting (not projectile). Fussy crying iritable, disturbed sleep, poor appetite. -Extraesophageal sx: recurrent sinusitis or OM, wheezing, dental ersosions, faliure to thrive (spitting up things and not getting nutrients for growth) -PE to rule out other causes and confirm poor weight gain and growth. -Dx usually clinical -If warning signs present: Barium upper GI series, 24 hour esophageal GI probe monitoring, Multichannel intraluminal impedence, upper endoscopy with biopsy (to assess damage) -Tx: Trial of PPI or H2 blocker for 2 weeks in those with mild esophagitis, signficant s/s, failed milk-free diet. 3-6 mo. trial if moderate-severe esophagitis documented by biopsy. PPI>H2 -Surgery is rare indication. basically for failed medical therapy for a long period of time, dependent on persistent aggressive med therapy, non adherent to med therapy, persistent, severe respiratory complications. -Make sure to remember positiong and feeding changes may help too

Pediatric brain tumors

-Classic triad= morning HA, vomitting, papilledema (seen in <30%) -School failure and personality changes common in older children -Irritability, failure to thrive, delayed development common in very young kids -Recent-onset head tilt can result from posterior fossa tumor -25-30% of all childhood cancers -Tend to have better prognosis than adults for most part -Risk of developing astrocytomas ^ w/ neurofibromatosis or tuberous sclerosis. ^ incidence of seizures observed in relatives of kids with astrocytomas. -SX: vary based on location. *Kids <2 more commonly have infratentorial tumors*, presenting with nonspecific sx like vomitting, unsteadiness, lethargy, irritability. Signs may be few or include macrocephaly, ataxia, hyperreflexia, nerve palsies. Bc head can expand in these young kids, papilledema ofen absent.Eye findings and like difficulty tracking can be seen in optic gliomas (often associated with neurofibromatosis) -sX *older kids: MC have supratentorial tumors* associated with HA, visual sx, seizures, focal neuro deficits. Initial presentation often nonspecific. HA common but often will not be predominant in morning. -SX: older kids with infratentorial tumors characteristically present with sx of hydrocephalus: progressively worsening morning HA/vomitting, gait unsteadiness, double vision, papilledema. -SX brainstem tumors: faical and extraocular muscle palsies, ataxia, hemiparesis -Dx: *In addition to tumor biopsy, neuraxis imaging via MRI is done*.. Can do CT w/ and w/o contrast, just not as good, but quicker. Imaging entire neuraxis and CSF cytology should be done in patients with medulloblastoma, epedyoma, pinel tumors. CSF should be obatined during dx surgery (Lumbar>ventricular) -Labs: levels of biomarkers in CSF and blood like HcG, AFP, helpful, should be obtained from blood preop in ALL pineal and suprasellar tumors -----------------Types of tumors-------- 50% occur above the tentorium, 50% in posterior fossa. 2 main categories: 1) glial (astrocytomas and ependymomas) and 2) nonglial (medulloblastoma) -Astrocytoma=MC brain tumor of childhood. Most are juvenille piolocytic astrocytoma (grade 1) found in posterior fossa. Low grade can usually be curable by excision alone -Medulloblastoma= MC high grade tumor in kids. Peak incidence= age 5-10 yrs. F>M 2:1.3.. typically arise in midline cereballar vermis. If disseminated, unfavorable, esp if <4. -Brainstem tumors 3rd most frequent. If diffuse infiltration prognosis is <5%. Frequently astrocytic orgin and high grade. ------------------------------------------------- -Tx: supportive care prior to initial surgery. Dexamethasone and anticonvulsants (levitiracetem preferred) if child has had seizures or seizures likely in surgery. -Meticulous surgical removal of as much tumor as possible is generally preferred initial approach. -High dose systemic chemo often used, esp in young children with high grade tumors to avoid cranial irradiation. -For medullloblastoma: cranospinal irradiation standard of therapy in kids >3

Mycoplasma pneumoniae info

-Compared to pyogneic pneumonia, those with this kind of pneumonia tend to have more gradual onset of sx, less respiratory distress, and usually a normal WBC. -SX: vever, cough, malaise -Sore throat, otitis media, otitis externa, and *bulluous myringitis* may occur. -Serology (ELISA)or PCR tests to get dx -CXR: interstitial or bronchopnuemonic infiltrates, frequently in middle or lower lobes. -Tx=Macrolides

Hirschsprung disease

-Congenital Aganglionic Megacolon -Males>Females (4:1), Chromsomal abnormalities increased incidence (Downs 2-10%) -80% at rectosigmoid colon -Hx: Constipated since day were born, no passage of meconimum, vomitting, thin ribbony foul smelling stool, difficulty feeding, older children may present constipated. -PE: Possible fever if enterocolitis, abdominal distenstion with palpable fecal mass, prominent ab veins and persistalsis -DRE: empty rectum (stool more likely proximal) -DDx: retentive constipation, hypothyroidism, intestinal pseudo-obstruction, motility disorder -Imaging: Start with x ray normally, Barium enema gives better pic. Biopsy=confirmatory (lack of ganglion cells). Can do rectal manometric testing too. -Tx: Surgical- resect aganglion bowel -Complications: Fecal retention or incontinence, obstruction, enterocolitis, neuronal dysplasia and pseudo-obstruction

Acute Labrynthitis

-Continuous vertigo + hearing loss -Commonlyu follows viral URI —-Tx—- -Symptomatic (meclizine + compazine) -Corticosteroids to reduce inflammation

intussusception

-Defined as invagination (or telescoping) of one bowel segment into another -MC bowel obstruction in first 2 years of life -Ileocolic mc location -Pathophys: 85% idiopathic. Lymphoma MC cause after age 6. Invagination-->vascular compromise--> eventual necrosis, possible peritonitis, perforation, other bad stuff -Always keep in ddx in kid with abdominal pain -Hx: Previously healthy infant with new onset severe paroxysmal ab pain -Sx: Lethargy (glossed over look on face), vomitting, refusing food. 12 hours later: stool with blood and mucus= CURRANT JELLY STOOL -may see them in fetal position -PE: Fever if perforation, pertitonitis, etc. Abdominal tenderness and distenstion. SAUSAGE SHAPED MASS in upper middle abdomen -Imaging: Ab xray (usually do 1st to assure no perforation, not sensitive alone), Abdominal US (98-100% sensitive, Target sign or bullseye sign), Barium/air enema= diagnositc and therapeutic (if concern for perforation, use air) -Tx: Barium/air enema. If evidence of perforation, unsuccessful reduction with enema, or severely ill--> Surgical tx

Type 2 diabetes

-Dx'ed most often in persons >40 who are usually obese and initially not insulin dependent. -Rare before age 10, but has increased in frequency in older kids as a oncsequence of obesity. —-Sx—— polyuria, polydispsia, weight loss (often missed) -Typically obese -10% present in DKA (ab pain, N/V that can mimic acute abdomen; mild-mod dehydration; kussmaul respiration, somnolent, obtunded) -BG >200 in a child always abnormal -Stress induced/steroid-induced hyperglycemia can occur with illness. In a well-Child, the dx must NOT be based on a single plasma glucose test or borderline result using a glucometer; If HbA1C is normal, home monitor glucose for several days ——Dx—— -Random blood glucose >200 + symptoms -Fasting plasma glucose >126 or plasma glucose >200 2 hrs after oral glucose load confirms dx -HbA1C >6.5% is diagnostic -Impaired fasting glucose= 100-125 -Impaired 2 hour OGTT= 140-200 -HbA1c 5.7-6.4% ——Tx—— - Varies with severity of disease. -Diet and Exercise (gatorade may be helpful in preventing hypoglycemia during exercise) -If HbA1C is still near normal, lifestyle modification best -With mildly elevated HbA1C (6.2-8.0%) and no ketosis, Metformin 500 mg usually started. -PO hypoglycemic agents can be tried at a later date, particuarly if weight loss has been successful. -If severe and ketosis present, IV or SubQ insulin needed. ——Prevention——- -HTN (anything >95th percentile): ACEI's 1st line, ARBs if ACEI's not tolerated well. Exclude nondiabetic causes before starting treatment -Lipids: Generally favorable in T1DM's. Commence screening after age of 2 yrs and repeat q 5 yrs. -Eye exams: beging at diagnosis and do annually (in comparison with T1DM where you do eye exam yearly starting 3-5 years after diagnosis)

EM vs SJS/TENS

-Erythema multiforme usually presents with typical target lesions or raised, atypical, targetoid lesions that are predominantly located on the extremities.Target lesions can be seen in SJS, but typically are macular. -In EM: Bullae and epidermal detachment are usually limited and involve less than 10 percent of the body surface area (BSA). -In contrast with SJS/TEN, erythema multiforme is associated with infection with herpes simplex virus in approximately 90 percent of cases and only rarely with drugs.

Lactose Intolerance

-Etiology: Lactase deficiency or lactose malabsorption -Lactase Enzyme deficiency causes: Primary:(non persistence MC). In childhood, diet very milk based but that decreases as we get older. Secondary: bacterial overgrowth, damage to epithelium or villi from a medical condition or malnutrition -Epidemiology: uncommon <6 yrs old, more prevalent in certain ethnic groups with low milk diets. -Sx: mild mod sx seen in smaller consumptions: Bloating, ab pain (cramping, periumbilical) flatulence. Severe sx (high ingestion): vomitting, osmotic diarrhea (frothy, watery, bulky stool from unabsorbed carbs). -PE: Normal vitals, no wt loss. -Abdmoinal exam: Inspection, auscultation (+/-borborgymi), palpation (+/- tenderness), percussion, special tests will be negative -Dx testing: 1) Lactose free diet/re-challenge in 1-2 weeks (remove lactose from diet and reintroduce in 1-2 weeks). 2) Lactose hydrogen breath test- diagnostic if >20 parts per million. Other tests not used frequently anymore -Tx: Restrict dietary lactose (2 servings day, 12 g or less at a time), enzyme replacement (pretreated foods, nonprescription formulas), Calcium and Vit D supplementation (Calcium carbonate 1200 mg/day)

Cholera

-Gram negative rod -This is a noninvasive (enterotoxin) form of infectious diarrhea -Attaches and produces toxin= blocks absorption and promotes secretion of NaCl. -Incubation period= 12-72 hours -Associated foods: contaminated water, fish, shellfish, street vendor food. Outbreaks may occur during poor sanitation and overcrowding, esp if abroad. -Exotoxin causes hypersecretion of water and chloride ions=severe dehydration -Clinical features: .Copious watery diarrhea "RICE WATER STOOLS" (gray, no fecal odor, blood, pus), vomitting, pneumonia, hypovolemia, electrolyte imbalance. Can die d/t rapid hypovolemia -Dx: Stool culture on a special medium -Tx: Fluid and electrolytes (oral or IV, IV preferable) are mainstay. Abx may shorten duration (doxy)

EBV

-HHV-4 -Transmission: Saliva -Incubation: 30-50 days ----Sx---- -Fever, sore throat, posterior cervical lymphadenopathy -Malaise, myalgias, general lymphadenopathy, splenomegaly, hepatomegaly -Exam: Posterior cervical lymphadenopathy, morbiliform rash, white/shaggy tonsils, splenogmegaly, transient eyelid edema -Petechial/morbiliform rash can occur if given ampicillin ---Dx--- Heterophile agglutination antibody test (monospot) (positive within 4 weeks) -Peripheral smear= >50% lymphocytes with >10% atypical lymphocytes ----Tx---- -Supportive: rest analgesics, antipyretics -CS used only if airway obstruction d/t lymphadenopathy, hemolytic anemia, or severe thrombocytopenia -Avoid trauma/contact sports at least 1 month if splenomegaly to prevent splenic rupture.

Pediatric Screenings

-Hemoglobinopathies: At birth -PKU: At birth -IDA: 12 months (4 in higher risk groups) -Lead: Blood lead screening recommended at 12 and 24 mos. Standardized screening questions asked from 6 mos to 6 years. Positive response to any ? Indicates need for blood testing via venous (not capillary) testing -TB: All children screened for possible high-risk category at routine HCM visits after1 yr. If in high risk group, PPD test at that time. Low risk gropus don't need repeat screening unless new RF's arise. Annual repeat screening in high risk -Vision:Evaluation begins in nursery (red reflex), <3 y.o.: mainly subjective reports by parents. 5% preschoolers have visual problems (85% myopia). VA should be 20/30 to 20/40 at age 3-4 yrs. Should improve to 20/20 by age 6-7. Strabismus needs ophtho refrral, use snell/allen charts to test VA. -Hearing: Done before leaving hospital, perform no later than 1 month old. Done yearly throughout grade-school. -Nutriton: Plotted on growth chart, breast milk or formula for first 12 months, whole milk until age 2, then 2%, fat content should be at least 30-25% of total kcals. -Dental: Routine appts every 6 mos beginning at age 2-3. Flouride supplementation if not in local water supply, only for chldren deemed at risk for caries. -HTN: Screening should begin at 3. But positive family hx, obesity, acute illness that can increase BP may require more frequent and earlier screening. Preemies lack proper regulatory system and are monitored from birth. -Behavioral:Administer screening tests to asymptomatic children 9 mo, 18 mo, 2 year, 2.5 year visits.After 2.5 year visit, administer at annual health supervision visits based on developmental surveillance and clinical judgement. At 18 mo and 2 year visits, add an autism spectrum disorder specific tool. -Alcohol/Drug abuse: Dones as part of age-appropriate hx (may vary based on location, socieconomic, home life). Ask with parents out of room. CRAFFT screening can work. If positive answers, ask additonal questions. -Cervical Dysplasia: Will vary based on what the organization recommends. Look at next notecard for those exact recommendations -STD: All sexually active persons should have annual screening (PE and lab). Start pap smears at 21 on all females -Obesity: Children 6 and older should be screened to obesity. If n ormal BMI, review healthy lifestyle behaviors, normal family risk, ROS, PE. If OVerweight/obese, review like you would in Normal BMI screen PLUS add labs: lipids, glucose LFT. -Ht and Wt: beginning at 2 y.o. And calculate and plot BMI for age and gender at all health supervision visits -Cholesterol: Screen all children 9-11 (statins can be used 1st line for kids as young as 8. Lipid panels if: positive FH or premature CVD, unknown fh of premature CVD or dyslipidemia, obesity, htn, diabetes -Insulin resistance/T2DM: If fasting glucose >100, perform OGTT or repeat fasting glucose in 3 mos.

Legg-Calve-Perthes Disease

-Idiopathic avascular necrosis of the femoral head in children d/t ischemia of capital femoral epiphysis in children -*MC in children 4-8*, M4x>F, low incidence in blacks ———-sx———— -Persistent pain= MC sx (according to current) -Patient may present with limp and limited ROM (loss of abduction and IR) -May have intermittent thigh, knee, groin pain -Another presentation (according to Pance prep pearls): painless limping x weeks (worsen w/ continued activity especially at end of day) —————Imaging———- -Early findings= effusion of joint associated w/ slight widening of joint space and periarticular swelling. Decreased bone density. Necrotic ossification center apperas denser than the surrounding viable structures, and femoral head is collapsed and narrowed. -Further progression: alternating areas of rarefaction and relative density= fragmentation of epiphysis -Late: Deformity, *crescent sign (microfractures w/ collapse of bone)* -MRI: blood supply ——-dx——- -Transient synovitis of hip may be ddxed from this condition by serial radiographs ——-tx———- -Protection of joint by minimizing impact= principal tx -If joint deeply seated w/in acetabulum and normal joint ROM maintained, a reasonably good hip can result -Little benefit seen from bracing -Surgical tx controversial.

Thyroid Nodule Algorithm (short version)

-If nodule felt on exam: do TSH and thyroid ultrasound -If TSH low: do RAI uptake scan and check T4 and T3 -If TSH high, evaluate thyroid ultrasound results and based on that, decide whether to do FNA.

Evaluation of emty scrotum

-If you try to manually express palpable testicular tissue toward inguinal canal and hold in scrotum for one minute and then release, you can sort out different causes -Holding it in place for at least 1 min should fatigue the cremasteric muscle, after which a retractile testies should remain in scrotal sac -An ectopic testis, examined with same maneuver, will immediately spring back out of the scrotum. They have previously descended normally but have ultimately adopted an aberrant location, MC= superifical inguinal pouch -A true undescended testis has not completed its descent and will be palpable in inguinal canal along the typical patho of testicular descent, but cannot be brought back into scrotal sac during exam.

Short Stature

-Important to distinguish normal variants of growth (familial short stature and constitutional growth delay) from pathologic conditons -Pathologic short stature more likely in kids whose growth velocity is abnormal (crossing major heigh percentiles on the growth curve) or who are significantly short for their family -Endocrine causes of short stature usually associated with normal or excessive weight gain while children with chronic illness or nutritional deficiencies may have inadequate weight gain. —-Clinical Evaluation—- -HX and PE (hx of chronic illnesses, meds, birth wt and ht, pattern of growth since birth) ——Labs——- -Radiograph of left hand and wrist for bone age -CBC (anemia/infection) -ESR ( -UA, BUN, Scr -CMP -Stool exam for fat, serum ttg (celiac) -Karoytope (girls, turner) and/or noonan's testing -Thyroid tests -IGF-1 (IGFBP-3 alternative in malnourished or if <4)

Hyperthyroidism

-In children, most cases are d/t Graves disease, caused by antibodies direct at TSH receptor that stimulate thyroid hormone production. -Can also be d/t acute, subacute, or chonic thyroidits, TSHoma, McCune, Albright syndrome -F>M, most frequently during adolescence —-Signs/Sx—- -Cylcic, with spontaneous remissions and exacerbations. -Worsening school performance, poor concentration, fatigue, hyperactivity, emotional lability, nervousness, personality disturbance, insomnia, wt loss (despite increased appetite), palpitations, heat intolerance, ^ perspiration, diarrhea -Polyuria, irregular menses -Signs: tachycardia, systolic HTN, increased pulse pressure, tremor, proximal muscle weakness, moist warm skin. -Accelerated growth and development may also occur -Most cases of Graves associated with diffuse, firm goiter -+/- thyroid bruit and thrill -Exophthalmos may be present ——Labs—— -TSH supressed -T4, T3, and FT3 are elevated tyipcally -Presence of TSI (thyroid-stimulatin immunoglobulin) confirms dx of Graves. -TSH receptor binding antibodies (TRAB) usually elevated in GRAVES ——Imaging—— -RAI uptake scan: diffuse uptake in Graves, singular uptake in thyroid nodule(s) with diminished uptake elsewhere, and diminshed uptake in thyroiditis. -In long standing cases, bone age is increaed and can see osteoporosis ——Tx—— -BB's (B1's preferred as they are more cardioselective): adjuncts to therapy, not curative. Helps symptoms like tremors, palpitations, tachycardia, hypertension -Antithyroid agents like PTU and Methimazole used in initial tx of childhood hyperthyroidism. These drugs interfere with thyroid hormone synthesis (Typically do methimazole) -Iodine usually prodcues a rapid, but short-lived, blockade of thyroid hormone synthesis and release, only used in thyrotoxicosis (thyroid storm) pts -RAI ablation (RAIA) usually reserved for children with Graves who do not respond to antithyroid agents, develop SE's from antithyroid med therapy, can't enter remission after several years of therapy, or poor medication adherence. Some pediatric endocrinologists actually use this 1st line as there are some hepatotxic effects of antithyroid drugs -Subtotal and total thyroidectomy infrequently used. Indicated for extremely large goiters, goiters with a suspicious nodule, very young or pregnant pts, or pts reusing RAIA. Before surgery, BB given to tx sx and antithyroid agents given for several weeks. Iodide given 1-2 weeks prior to surgery to reduce thyroid vascularity and inhibit release of thyroid hormone

Acute Rheumatic Fever

-Link to pharyngeal infection with GABHS -Continues to be problem worldwide, best prevention= correct use of abx ——*Epidemiology*——— -Children b/t 5-18, M=F, virulence of strrain importnat. Peak incidence in cold mos. ———*Jones Criteria*——- To make a dx- Either 2 major Criteria OR 1 major and 2 minor 1) Major -Arthritis (migratory polyarthritis)- *licks the bone, bites the heart* -Carditis - Sydenham Chorea (*most definitive manifestation*- involuntary movments of face, tongue, upper extremities, MC in F>M, rarely seen in adults more in kids) -Erythema Marginatum -SubQ nodules (small firm nontender attached to fascia or tendon sheeths over bony prominence) 2) Minor -History of ARF -Arthralgia -Prolonged PR (reversible) -Elevated ESR, CRP -Fever —-*Dx*—— -Evidence of strep infection: Either through culture or ASO antibody (often elevated in healthy children or those w/ RA, HSP, Takayasu's) -Jones criteria: 2 major OR 1 Major and 2 minor —TX—— -Eradication of GABHS: PCN VK PO x 10 days (*1st line*), Erythromycin PO x 10 days (can be substitued for PCN). Others: Azithromycin x 5 days, Cephalosporins x 5 days -Aspirin and CS: 3 weeks or as long as inflammation (esp in arthritis sx) is present -Anticongestive therapy- as needed -Surgical repair ——-*Prevention*——- -The initial episode of this dx can be prevented by early tx of streptococcal pharyngitis with PCN. -Preferred prophylaxis= Benzathein PCN IM q 4 wks. -If pt allertgic to PCN, sulfadiazine or Erythromycin PO may be substituted, or a macrolide, or cephalopsorin (if allergy was NOT anaphylactic) -Recurrences uncommon after 5 yrs following 1st episode and in pts over 21 y.o.

Tinea Ungum

-Loosening of nail plate from bed giving a yellow discoloration is first sign. -Thickening of distal nail plate then occurs, followed by scaling and a crumbly appearnce of the entire nail plate surface. -T. Rubrum is MCC -Dx= KOH exam and fungal cultures ---Tx--- -Itraconazole and Terbafine PO. Be conscious of SE's of systmemic antifungals like hepatotoxicity. -Topical Ciclopirox can be considered as well.

Appendicitis

-MC abdominal surgical emergency -MC cause of fecolith -Most cases caused by a blockage of the opening of the appendix by a piece of stool. -Perforation and peritonitis in about 20% -Common pts 10-30 y.o. -PE: + iliopsoas sign, + obturator sign, + Rosvig sign, + McBurney sign -Sx: initially periumbillical or epigastric pain--> then localizes in RLQ, pain worse with movement, N/V/D, anorexia. -Sx may improve over time but reoccurs, think perforation -Be aware of atypical appearance if appendix is retrocecal. Pelvic appendix may cause urge to pee or poop. -Labs: B-HcG, CBC (luekocytosis, 75% neutrophils), UA (R/o infection and stones)- may have microhematuria and pyuria -Imaging: Can do abdominal US in time-crunching cases and in KIDS, but CT is Gold Standrad. MRI in pregnant ppl. -Complications: Perforation in 20%, suspect in pts with pain >36 hrs and diffuse ab pain. Can cause peritonitis and thrombic phlebitis -TX: Surgical appendectomy

Enterohemmorhagic E Coli (STEC)

-MC cause of bloody diarrhea -Incubation: 1-8 days - Foods: Undercooked beef, especially hamburger, unpasteurized milk and juice petting zoos -Unpasteuried milk/apple cider, produces ***cytotoxin***. -Clinical features: Watery—> grossly bloody diarrhea with cramping. NO FEVER.. In adults, usually self limited to 5-10 days. -Complications: HUS -Dx: Shiga toxin- producing E coli can be cultured on special medium. Or, shiga toxin assay. -TX: Fluid replacement and supportive Measures.. ***No abx since it increases toxin production and risk of renal failure through HUS... Plasma exchange may help in pts with STEC-Associated HUS******

Ingested Foreign Body

-MC in Children <4 yrs old -Food small toys, coins MC -80-90% pass spontaneously. -Sx: dysphagia, odynophagia, drooling, regurgitation, chest/ab pain, cough, vomitting -More severe sx: Ulceration, erosion, stricture formation -PE: Airway/breathing, VS, general, neck (esophageal perforation, may see swelling around neck), heart, lungs (possible wheeze/stridor), abdominal (may be able to determine obstruction or perforation) -Imaging: 1) start with AP and Lateral x-ray. Lateral x ray can tell us where it's at, esophagus vs trachea, etc. -Can also do esophagram with contrast. Can ID radiopaque things like plastic. -TX: endoscopic removal if can't pass on own. Can eval surrounding tissue with endoscopy. Try to take out w/in 24 hours. BUT IF BATTERY, REMOVE ASAP; Occasional foley catheter removal. -If past esophagus, make take 4-7 days. ----------Specific Objects------------------- -Batteries: remove asap esp if >20 mm or <5 yrs old. -Smooth FB just watch, may be fine to let pass -Assymetrically weighted (like nail or screw). Blunt end is heavier than sharp end, so when they pass normally blunt end passes first, so they can sometimes be monitored as they pass thru. -If sharp on both sides or equally weighted, they need to be taken out. Like a toothpick. Safety pins and fishhooks also remove ASAP -Magnets: 1 magnet with no other piece of metal and small and smooth may be okay. But if multiple magnets or magnets and metal-- remove ->5cm= remove bn bnv

Kawasaki Disease

-MC in children (esp <5) boys. Asians highest risk. -Thought to be d/t underlying resp agent or viral pathogen with propensity toward vascular tissue -Medium and small vessel necrotizing vasculitis including coronary arteries --------sx/dx criteria------------- "warm + CREAM"= fever + 4 of the 5: 1)C= Conjunctivitis (bilateral and nonexudative) invovement spares limbus) 2)R= Rash: polymorphus (erythematus, morbiliform, or macular) 3) E= Extremity changes: desquamation (esp perineum), edema, erythema of pallms and soles, induration of hands and feets, Beau's lines (transverse nail grooves), *arthritis* 4)A= Adenopathy: Cervical lymphadenopathy (erythematous, nonsuppurative, induration, unilateral) 5)M= Mucous membrane: pharyngeal erythema, lip swelling and fissure, strawberry tongue -Incomplete form= 2 crieteria met and acocompanied by other lab or imaging findings ---------------dx----------- -lack of specific dx test -Increased ESR/CRP, reactive thrombocytosis (increased platelets), normochromic normocytic anemia, STERILE pyruia -Echocardiogram and angiography may be needed if heart involvment suspected ------------Tx----------- -IV Ig and highdose aspirin = mainstay of tx (lowers fever, joint pain and prevents coronary artery thrombosis/aneurysm). Corticosteroids (methylprednisone) if refractory or severe -MMR should be delayed by 9 months only if IVIG waws administered -Further refractory options= inflixmab, cyclphosphamide, cyclosporine, plasmapharesis.

Pyloric Stenosis

-MC in first 3 mos of life. -SX: Hungry Vomitter (Projectile vomiting), may have dehyrdation due to this (low UO, turgor of skin) -PE: Abdomen distended, peristaltic wave. OLIVE SHAPED MASS (5-15mm) in Right uppper abdomen -Labs: CMP (Low K, Low Cl, High HCO3-, unconjugated bilirubinemia), CBC (normal or signs of dehydration) -Imaging: Ab US= procedure of choice.. Hypoechoic mucular ring >4mm thick with hyperdense center. -Barium upper GI series if PE and US don't make dx -TX: Ramstedt or Laparascopic pyloromyotomy. Make sure they are hydrated and electrolytes restored before doing surgery. -Post op: May have continued vomitting post op for a short period of time, and that is normal and will resolve on its own.

Bronchiolitis

-MC in kids <2 (exclusively seen) -MC in winter Months (Jan-March in particular) -MCC= RSV ——Signs/Sx—— -Often preceded by a 1-3 day URI prodrome -Fever, Cough -Resp distress (tachypnea, retractions, wheezing, crackles) -Pt may be: tachycardic, febrile, tachypneic, hypoxic, coughing -Can see *retractions*, nasal flaring, +/- cyanosis, -Poor feeding, vomitting, irritable -Hyperinflation or wheezing may also be seen -PE: decreased air movement, crackles, or wheezing can be seen -Factors that predict increased risk for poor outcomes: age <3 mos, gestational age <34 wks, tachypnea (RR >70) ill apperance, or inability to maintain hydration -Historica factors that increase risk of poor outcomes: congenital heart disease, chronic lung disease, immunocompromised ——-Dx—- -Clinical -No imaging typically needed unless suspecting pneumonia -Pulse ox single best predictor of disease in children (<96%= admit to hospital) —-Tx—- -MOFO (monitor, oxygen, fluids, other) -In severe cases: can do heated humidified high flow nasal cannula, continuous positive airway pressure, or intubate. -Admit if respiratory distress or high risk -Epi and albuterol can be used but doesn't alter clinical couse -A trial of bronchodilators can be attempted in moderate to severe cases, but steroids have NOT been shown to improve outcomes -Antivirals (Ribavirin) administered if severe lung or heart disease or immunocompromised pt. ——Prevention—— -Palivizumab prophylaxis may be used in high risk group -Hand washing preventative ——Copmplications—— -MC complication is superinfection with a bacterial pathogen like strep pneumo leading to pneumonia.

Norovirus

-MC overall cause of gastroenteritis in adults in North America. -A form of non infectious diarrhea (typical symptoms of this kind is N/V cramps) -HIGHLY INFECTIOUS. -Occursed in closed settings: cruises, schools, day cares, etc. -Sx: vomitting with diarrhea,systemic sx may be present (fever, malaise, myalgia, HA), self limited (48-72 hrs), may be prlonged in IC. DX criteria: vomitting in >50% of affected persons, mean incubation 24-48 hrs, median illness duration=12-60 hrs, lack of bacterial pathogen identified. -Can dx by PCR stool; lack of fecal leukocytes; hypokalemia and metabolic acidosis - Responsible for 20% of all diarrhea in both children and adults. -Often associated with outbreaks in places like cruise ships, hospitals, restaurants. -Tx: Supportive -Can return to work 48-72 hours after symptom resolution. -Prevention: Proper hygiene.

Viral Pneumonia in Kids

-MC seen in kids <2 -RSV, parainfluenza, influenza, and human metapneumovirus are MCC's -Severity of disease, severity of fever, radiographic findings, and characteristics of cough or lung sounds do not reliably differentiate viral from bacterial pneumaonias; they can coexist -Substantial pleural effusions, pneumatoceles, abscesses, lobar consolidation with lobar volume expansion, and "round" pneumonias are generally inconsistent with viral disease ——Signs/Sx—- -Frequently precedes viral lower respiratory infections -Although wheezing or stridor may be prominent in viral disease, cough, signs of respiratory distress, and physical findings may not be distinguishable from those in bacterial pneumonia ——Labs—— -CBC: WBC can be normal or slightly elevated and is not useful in distinguishing viral from bacterial disease -PCR or other rapid viral diagnostic methods should be performed on nasopharyngeal secretions to confirm dx in high risk pts and for epidemiology or infection control -Rapid dx of RSV does not preclude the possibility of concomittant infection with other pathogens ——Imaging—— -CXR's frequently show perihilar streaking, increased interstital markings, peribronchial cuffing, or patchy bronchopneumonia -Lobar consolidation or atelectasis may occur, however -Hyperinflation of lungs may occur when small airway involvment is prominent ——Ddx—— -Pts with prominent wheezing may ahve asthma -Airway obstruction caused by foreign body aspiration, acute bacterial or viral tracheitis, or parasitic disease ——Complications—— -Bronchiolitis obliterans or severe chronic respiratory failure may follow adenovirus pneumonia -Bronchiectasis, chronic ILD, and unilateral hyperlucent lung may follow measles, adenovirus, and influenza pneumonias —-Tx—— -Supportive care -Admit pending on condition -Pts at risk for life threatening RSV infections (those with BPD or other severe pulm conditions, congenital heart disease, or immunocompromised) should be hospitalized and ribavirin should be considered -All children with influenza should be appropriately treated for specific type of influenza

Rhabdomyosarcoma

-MC soft tissue sarcoma occurring in childhood and account for 10% of solid tumors in childhood -70% dxed before ag 10, second smaller peak seen in adolescents with extremity tumors; M>F -Sx:disturbance of normal body function d/t tumor growth. For example, pts with orbital present with proptosis, whereas pts with it in bladder present with hematuria, urinary obstruction, or pelvic mass -MC site of METs= lungs -Imaging: Plain radiograph and a CT and/or MRI should be obtained to determine extent of tumor and lymph node involvement. Chest CT to rule out pulmonary METS. Skeletal survey and bone scan to determine if bony METs present. -Bilateral BM biopsies and aspirates obtained to r/o BM infiltration -TX: Complex combined modality therapy. When feasible, excision should be done. Chemo can convert inoperable tumor to operable one. Radiation pretty much given to all (exception: localized tumor that was completely resected) -Prognosis: Children with localized disease have 70-75% 3 year disease free survival rate. Children with METs have 39% 3-yr disease free survival.

RSV

-MCC of bronchiol.itis -Two types: A and B. A associated with disease severity -Prematurity= major risk factor

Acute Otitis Media

-MCC's= S. Pneumo (MC), H. Flu, S. Pyogenes, M. Catarrhalis (same organisms seen in acute sinusitis) -Peak age= 6-18 mos —-Pathophys/predisoposing factors—— -Eustachian tube dysfxn: fluid sits and becomes infected -Bacterial colonization -Viral URI -Smoke exposure -Impaired host defenses -Bottle feeding -Time of year, daycare attendeance, genetics ——-Signs/sx—- -Fever, otlagia , ear tugging, conductive hearing loss -If perforationZ: Rapid relief of pain + otorrhea (usually heals in 1-2 days) -Bulging erythematous TM with effusion. Loss of landmarks -Decreased TM mobility on pneumatic otoscopy -If bullae seen suspect Myocplasma pneumoniae -If conjunctivitis also seen, suspect H. Flu as cause ——Dx—— -Clinical -2 findings are critical in establishin dx: 1) a bulging TM, 2) a middle ear effusion proven by otoscopy or tympanometrys ——Tx—— -Amoxicillin 1st line. -Augmentin good choice in those who recenently had amoxicillin in the last 30 days, or is clinically failing after 48-72 hrs on amoxicllin -If PCN allergic: Bactrim, Marcolides, Clindamycin -Tympanostomy if recurrent (>/=3 in 6 months, or 4 in 12 months) or persistent condition -In pts with tympanostomy tubes with uncomplicated acute otorrhea, ototopical abx (fluroquinolone eardrops) are 1st line therapy -Prophylactic abx use can also work if recurrent: Bactrim

Bacterial Pneumonia-Kids

-MCC= Strep pneumo -Usually follows a viral lower respiratory tract infection ——Signs/sx—— -Fevers (>39 C typically), tachypnea, cough are all hallmarks -Chest ascultation: crackles or decreased breath sounds in setting of consolidation or an associated pleural effusion. -May see extrapulm findings: meningismus or abdominal pain; vomiting possible ——Labs—— -CBC: elevated WBC with a left shift may be seen; low WBC can be ominous finding -Blood cultures if admitting the kid -Sputum cultures helpful in older kids capable of providing satisfactory sample -Invasive procedures (bronchial washing, lung puncture, biopsy) should be only done in critically ill pts —-Imaging—— -CXR: consolidation, +/- associated pleural effusion -A diagnostic (and possible therapeutic) thoracocentesis should be done in a child with a pleural effusion —-Complications—— -Empyema can occur frequently with staph, pneumococcal, and GABHS -Distal sites of infection- meningitis, otitis media, sinusitis, and septicemia- may be present particularly with disease due to H flu or Strep pneumo ——Tx—— -* All Children <3 mos of age should be admitted* -Mod-Severe respiratory distress, apnea, hypoxemia, poor feeding, deteriorating clinically and associated complications (large effusions, empyema, or abscess) indicate need for immediate hospitalization in older children -Careful outpt followup within 12 hrs to 5 days often indicated in those not admitted. -Children <4 wks of age: Ampicillin and Aminoglycoside -Infants 4-12 wks of age: IV ampicillin x 7-10 days -Children 3 mos-5 yrs of age: PO Amoxicillin x 7-10 days -Children >5 yrs: Marcolide OR Amoxicillin OR Pen G pending on causative agent.

RH Alloimmunizztion

-Maternal antibodies that bind to fetal RBC's--> neonate hemolytic disease. -If mother is Rh (-) and father's Rh (+), there is a 50% chance baby will be Rh (+) -Less common, more severe, and more predictable than ABO incompatibility ----Pathophysiology---- -Occurs if Rh (-) mom carries and Rh positive fetus with exposure to fetal blood mixing (examples: during C section, placental abruption/previa, amniocentesis, vaginal delivery, etc). -The mixing causes maternal immuniztaion--> maternal anti-Rh IgG antibodies -*During subsequent pregnancies, if she carries another Rh (+) fetus, the antibodies may cross the placenta and attack fetal RBC's--> hemolysis of fetal RBC's* ---Presentation---- -Hemolytic anemia, jaundice, kernicterus, hepatosplenomegaly -Fetal hydrops can occur -CHF can occur -Erythroblastosis fetalis= life-threatening anemia, generalized edema, and fetal or neonate heart failure. Without antenatal intervention, fetal or neonate death often results ----Dx---- -Coombs test (+) - Fetus monitoring in 2nd trimester: if present--> amnitoic fluid (^ bili). U/S of middle cerebral artery (increased flow 2ry to decreased viscosity of blood in anemia.) -Percutaneous umbilical blood sampling shows decreased HCT ---Tx--- -Preventative in mother: Rhogam given to mother at 28 weeks gestation andwithin 72 hours of delivery of Rh (+) baby or after any potential mixing of blood. ----Tx: Erythroblastosis fetalis---- -The cornerstone of antenatal management= transfusion of fetus with Rh(-) cells, either directly into umbilical vein or fetal ab cavity. -Photherapy usually started in these infants upon delivery with exchange transfusion usually needed. -Ongoing hemolysis occurs until all maternal ab's are gone, therefore these infants require monitoring for 2-3 months for recurrent anemia severe enough to require transfusion

Acute Bacterial Meningitis

-May have sinusitis or pneumonia prior to development -Pts usually seek medical attn w/in 1-2 hr of sx onset --Causative Agents--- -Strep pneumo= MC in adults, also seen in kids -N meningitidis= MC in Kids, teens- associated with petechial skin rash (not always seen tho) -L. monocytogens= seen in more immunocompromised -Staph aureus= in post op or post trauma pts -H Flu= another known consitive agent in kids ---Signs/Sx--- -Fever/chills -Meningeal sx: HA, nuchal rigidity, photosensitivity, N/V, AMS/seizures can happen as progression occurs --PE findings--- -(+) Kernig sign: Inability to straighten knee with hip flexion -(+)Brudzinski sign: neck flexion produces knee and hip flexion ---Dx/Labs--- -CT scan performed before LP if: space occupying lesion suspected on basis of papilledema, seizures, focal neuro findings. Or if immunocompromised, mod severe impaired LOC -LP with CSF exam= definitive dx -DO NOT WAIT FOR LP TO ADMINISTER ABX. EARLY ABX THERAPY HAS BEEN SHOWN TO ^ SURVIVAL RATES -Classic traid of fever, stiff neck, AMS= 44% sensitive --Tx--- -Abx (view table) -If viral: supportive therapy --Ddx-- -viral typically more beningn and not as severe -Viral meningitis typically has normal cerebral function, unlike encephalitis

Drug Eruptions

-Medication induced changes in skin and mucous membranes. Most are hypersensitivty reactions. -Most cutaenous drug rxns are self limited if offending drug is discontinued -Triggers: Antigen from foods, insect bites, drugs, environment, exercise, infection —-Pathophys—— 1) Type 1: IgE mediated, ex: urticaria and angioedema. Immediate 2) TYpe 2: CYtotoxic, ab=mediated 3) Type 3: Immune antibody-antigen complex (ex: drug-mediated vasculitis and serum sickness) 4) Type 4: Delayed (cell-mediated): morbiliform rxn (erythema multiforme) 5) Nonimmunologic: Cutaneous drug rxns due to genetic incapability to detoxify certain meds (ex: anticonvulsants and sulfonamides) —-Clinical manifestations—— -Erythematous/Morbiliform Rash: MC skin eruption. Generalized distribution of "bright-red" macules and papules that coalesce and form plaques. Rash typically begins 2-14 days after med initiation (ex: abx, NSAIDs, allopurinol, thiazides) -Urticarial= 2nd MC type: Occurs mins=hours after drug admin. MC triggers= NSAIDs, aBX, opiates, radiocontrast media -Erythema Multiforme: 3rd MC. Target lesions may not always be present in drug-induced EM. MC drug= sulfonamides, PCN's, phenobarbital, dilantin -Fever, ab, or joint pain may accompnay cutaneous drug rxn -Less common: Aceniform, eczematous, exfoliative, photosensitivity, and vasculitis —-Tx—- -Discontinue offending med -Exanthematous/Morbiliform: PO Antihistamines -Drug induced urticaria/angioededma: PO CS, antihistamines -Erythema minor: Symptomatic therapy -Anaphylaxis: IM Epi

Umbilical Hernia

-More common in full-term African American babies. -Small bowel may incarcerate in small diameter umbilical hernias ---Signs/sx---- -Increasing in size: usually contain omentum, but small and/or large bowel may be present -Sharp pain on coughing or straing -Large hernias produce dragging or aching sensation ----Tx---- -Most regress spontaneously if defect has diameter less than >1 cm -If 4 years or older: surgery -Note: reducing the hernia and strapping the skin over the abdominal wall does NOT accelerate healing.

Reflexes

-Moro: disappears by 4-5 months -Rooting reflex: disappears around 2-3 months -Tonic neck: appears after 2-3 weeks, disappears around 6-7 months of age. Prominent during 2nd month of life -Parachute reflex: begins at 6-7 months and is well developed by 1 year.

Tympanic membrane perforation

-Occasionally, an episode of AOM may cause this. Can lead to choleastoma -Discharge from ear and relief of pain seen -Usually heal iwthin a couple of weeks on its own. -Ototopical abx recommended for 10-14 day course and pts should be refered to ENT 2-3 weeks after rupture for exam -When perforations fail to heal after 3-6 months, surgical repair may be needed. -Avoid water activities, topical aminoglycosides -Typically need surgery if >25% perforation

Osgood-Schlatter disease

-Osteochondritis of the patellar tendon at tibial tuberosity from *overuse* (repeated stress) or small avulsions (d/t quad contraction on the patellar tendon insertion on tibia) -*MCC of chronic knee pain in young, active adolescents* -MC males, boys commonly 12-15, girls 11-13, with *growth spurts* (bone growth faster than soft tissue growth, so quads contraction transmitted thru patellar tendon to tuberosity ——-Manifestations———- -Activity related knee pain/swelling (running, jumping, kneeling) -Painful lump below knee, *tenderness to anterior tibial tubercle* -Prominent tibial tuberosity ————Dx——— -Radiographs (not usually used) show prominence or heterotropic ossification at tibial tuberosity. Demonstrate fragmentation or irregular ossification of the tibial tubercle ———tx——- -RICE -NSAIDs -Quad stretching, PT -Surgery only in refractory cases (if done, usually performed after growth plate has closed)

Congenital Hip Dysplasia

-Pathophys: neonate hip unstable 2ry to undeveloped muscle, easily deformed cartilaginous surfaces, lax ligaments. Exaggerated positoing may occur in utero= excess stretching of posterior hip capsule. This instability may lead to subluxation/dislocation, causing abnormal development of femoral head and acetabular structures. Usually unilateral, *L>R* -RF's: Female, breech positon >/= 34 weeks gestation, fam hx, tight swaddling. -Assocaited conditions: birthweight >4kg, torticollis, plagiocephaly, metatarsus adductus, clubfoot, oligohydraminos, multiple gestation pregnancy, 1st born ——————-Sx—————— -Newborn= instability on PE -Infant= limited abduciton -Toddler= assymetric gait -Adolescent= activity-related pain -Adult= OA ————PE———- -Eval begins as newborn. Continue eval at well checks until 9 mos of age or walking independtly. 2-week and 4-week old exams particularly impt ———clinical tests—— -Asymmetric skin folds: dislocated hip displaces proximally= leg shorter and creates accordian phenomen of the thigh skin folds. Most signifcant fold is b/t genitals and glut max (not reliable alone, occurs in 25% of all infants). -<3 mos: *Ortalani, Barlow*: Ortalani- confirms dislocation, trying to relocate the hip. Palpable "clunk" represents reduction of femoral head back into acetabulum. Technique: supine, hip, knee flexed to 90. Index and middle finger along greater trochanter, thumb on inner thigh. From adducted position, gently abduct while pushing trochanter anteriorly. Barlow- provactive test that picks up unstable but located hip, *trying to dislocate the hip*. *CI'd* if child has dislocated hip. Technique: supine, hip and knee flexed to 90. Thumb on inner thigh, middle and index finger on greater trochanter. Gently adduct and apply downward pressure. Palpate for posterior movement of femoral head. ->3 mos, not yet ambulating: *Galeazzi Sign, passive hip abduction* Galeazzi: detect dislocated hip/shortened femur. Technique: supine on table w/ flexed knees and feet flat on table w/ femurs aligned. Observe height of knees: position self at end of table near pts feet. Passive hip abduction: Normal ROM= >75 degrees abduciton, at least 30 degrees adduction. Abnormal: *<45 degrees= most reliable sign* unilateral limited hip adduction after 8 weeks is 78% sensitive, 93% specific -Walking age= trendelenburg—> inability to maintain pelvis horizontally while standing on ipsilateral leg resulting in *swaying gait* ———Imaging———- -<4-6 mos: US (if breech delivery, FH of DDH, or clinical findings) ->4-6 mos: Radiographs (ossified nucleus in femoral head deveops, single AP view w/ hips held in 20-30 degrees flexion) -Do US of hips at 4-6 weeks of age if risk factors present ———TX——— -Goals= obtain concentric reduction of hip into acetabulum, maintain reduction, and allow normal growth stimulation to correct acetabular dysplasia. 0-1 Month old: -Laxity: reassess at 6 wks -Dislocation: Referral and *Pavlik Harness* 0-6 months old -Pavlik harness used -If reduction can not be maintained, closed reduction and spica casting x 6 wks 6-18 mos (before walking) OR failed Pavlik -Closed reduction and spica cast 18 mos to 2 yrs+ -If reduciton/casting has failed, open reduction w/ pelvic and/or femoral osteotomy and post-op casting required, followed by prolonged bracing to resolve residual dysplasia q

Meckel Diverticulum

-Persistent poriton of embryonic vitteline duct (yolk sac) -MC form of omphalosmesenteric remnant ---Essentials---- -*Rule of 2's: 2 % of population, 2 feet from ileocecal valve, 2% symptomatic, 2 inches in length, 2 types of ectopic tissue (gastric or pancreatic), 2 years mc age at presentation, 2x more common in boys.* ---Signs/sx--- -Painless episodes of maroon or melanotic rectal bleeding (seen in 40-60%) -If pain is present, often periumbilical but radiates to RLQ -Bleeding often due to deep ileal ulcers adjacent to diverticulum caused by acid secreted by heterotopic gastric tissue and may be voluminous enough to cause shock and anemia -Occult bleeding less commonly -Intestinal obstruction (25%) that can occur d/t ileocolonic intussception; volvulus, or obstruction. Can cause diverticulitis in adults ---Diagnosis---- -Meckel scan (technetium-99 taken up by hterotopic gastric mucosa in diverticulum and outlines the diverticium on nuclear scan)- nuclear scan that looks for ectopic gastric tissue in the ileal area ----Tx---- -Surgical; good prognosis

Nursemaid's elbow

-Radial head subluxation from annular ligament -*MC elbow injury in children*.. Agest 1-4 MC. By age 5, annular ligament thick and strong. F>M. Left>Right -MOI: axial traction on pronated forearm w/ extended elbow. *Child stumbles while holding adult's hand. Or swung around by arms* ————-Sx———- -Clinical presentation: Child stops using affected arm. Holds elbow close to side, slightly flexed (take pressure off/reduce pain), pronated. Complains of elbow pain and *avoids motion at elbow*. Point tenderness over radial head ———-Imaging————- -X-rays: usually normal. Not needed if consistent MOI. Use it to r/o fx if swelling, bruising, or other MOI ———-Tx——— REDUCTION (techniques below) -Hyperpronation technique: support elbow, finger applying pressure to radial head. Other arm pronates forearm until palpable click -Supination/flexion technique: Support elbow, finger applying pressure over radial head. Other arm pulls traction, while flexing and supinating elbow ————-Referral/ Other info——— -27-39% recurrence. No long term issues -Refer if: unable to reduce OR child won't use arm despite normal x-ray

Small for Gestation Age/Intrauterine growth Restriction

-SGA infants have birth weights <10th percentile -Several different causes. Can be d/t poor maternal environment, intrinsic fetal abnormalities (Russ-silver, noonan, bloom, progeria). -Many children with wild SGA/IUGR and no intrinsic fetal abnormalities exhibit catch-up growth during first 3 yrs, however 15-20% remain short throughout life -Children who do not show catch-up growth may have normal growth velocity, but follow lower heigh percentile -In contrast to children with constitutional growth delay, those with SGA/IUGR have skeletal maturation that corresponds to chronological age or only mildly delayed -Tx: GH therapy is FDA approved and appears to increase growth velocity and final adult height.

SJS/TENs

-SJS= <10% BSA, TENS= >30% BSA, SJS/TENS overlap= 10-30% BSA - >90% have mucous membrane involvement ——Etiology/Pathophys—— -Pathophys not completely understood -Severe mucocutaneous rxn—> extensive necrosis and detachment of the epidermis - *MC trigger= Meds (75-80% of cases)* -At risk within 8 weeks of starting meds. -MC causative meds in adults= allopurinol, aromatic anticonvulsants, antibacterial sulfonamides, lamotrigine, nevirapine, NSAIDs -MC causative meds in kids= phenobarbitol, carbamazepine, antibacterial sulfonamides, laomtrigene -*2nd MC trigger= Myoplasma pneumoniae infection (especially in peds)* ——-Signs/sx—— -Prodrome of influenza-like sx 1-3 days prior to onset of skin changes (fever often > 102.2, malaise, myalgias, arthralgias, photophobia, painful swallowing) -Cutaneous lesions: ill defined, coalescing erythematous macules with purpuric centers or diffuse erythema. Skin very tender to touch -Cutaenous lesions often start on face and thorax before spreading to other areas. *Scalp, palms, soles typically SPARED* -Vesicles and bullae form -Targe tlesions may be seen ——Mucosal lesion—— -Occur in 90% of cases -Oral= mucosa nad vermillion border nearly always involved. Mucositis and stomatitis lead to impaired PO intake= malnutrition and dehydration -Ocular= in 80% of cases. MC presentation= severe conjunctivitis with purulent discharge. Can see corneal ulceration, uveitis -Urethritis in 2/3 of patients. Genital erosions frequent -Other sites less commonly involved= tracheal, bronchial, esophageal, intestinal (rarely) ——Dx—- -Clinical -Hx of drug exposure (avg is 2 weeks prior) or recent febrile illness -Prodrome, painful rash, erythematous macules, target lesions, or diffuse erythema progressing to vesicles and bullae -(+) Nikolsky sign -Mucous membrane involvement -Epidermal sloughing —-Labs—- -CBC (anemia, lymphopenia), CMP (hypoalbuminemia, electrolyte imbalance,^ BUN and Glucose, ^ LFT's in 50%) ——Tx—- -Admit all patients with this. -SCORETEN >/=2 means they go to ICU -REMOVE CAUSATIVE DRUG FOR LIFE -Supportive care: IV crystalloids like LR -Use 2/3 parkland formula for fluid resusciation (Parkland formula= 4mL/KgBW for each % BSA affected; 1/2 given in 1st 8 hours, rest given over 16 hrs)

Bacterial Tracheitis

-Severe, life-threatening form of laryngotracehobronchitis -MCC= Staph Aureus -Other causes= H. Flu, group A strep pyognes, Neisseria species, Moraxella catarrhalis -The disease probably represents localized mucosal invasion of bacteria in pts with primary viral croup, resulting in inflammatory edema, purulent secretion, and pseudomembranes -Although cultures of tracheal secretions are frequently positive, blood cultures are almost always negative —-Signs/Sx—— -Early picture similar to that of croup -However, instead of gradual improvement, pts develop higher fever, toxicity, and progressive or intermittent severe upper airway obstruction and is unresponsive to standard croup therapy. -Findings of toxic shock and acute respiratory distress syndrome may also be seen ——Labs/Imaging—— -CBC: WBC usually elevated with left shift -Cultures of tracheal secretions usually demonstrate one of causative organisms -Lateral neck radiographs: normal epiglottis but severe subglottic and tracheal narrowing. -Bronchoscopy: normal epiglottis and presence of purulent tracheal secretions and membranes confirms diagnosis ——Tx—— -Requires direct visualization of airway in a controlled environment and debridement of the airway -Most pts will be intubated because incidence of respiratory distress/failure/arrest is high -Pts may also require further debridement, humidification, frequent suctioning, and intensive care monitoring -IV abx to cover staph aureus, H flu, and other organisms indicated ——Prognosis——- -Mortality rate is very low if recognized and treated properly

Syncope

-Sx defined as a transinet, self-limited LOC, usually leading to a fall. -Specific cause found in 50% of cases during initial eval. -In many w/ recurrent or near syncope, arrhtymias are not the cause. -*The hx is most important component of the eval to ID the cause of syncope* -Abrupt onset, transient (lasting secs to a few mins), and followed by prompt recovery to full consciounsness (no post-ictal state) ———-*Vasodepressor Syncope*———— -May be d/t excessive vagal tone or impaired reflex control of peripheral circulation. -MC type= vasovagal hypotension= COMMON faint. Usually initiated by a stressful, painful, or claustrophobic experience, esp in young women - Premonitory sx= nausea, diaphoresis, tachy, palor (usual in common faint). Episodes can be aborted by lying down or removing the inticing stimulus ———-*Orthostatic (postural) Hypotension*———- -Another common cause of vasodepressor syncope -Seen esp in elderly, diabetics, or others with autonomic neuropathy, in pts with hypovolemia or vasodilators, diuretics, adrenergic blockers -*Sx*: A greater than normal decline (*>20 mmHg*) in SBP or >*10mmHg diastolic* immediatley upon arising from supine to standing. ————*Cardiogenic Syncope*———— -Can occur on a mechanic or arrhthmic basis -Usually no prodrome, thus injury 2ry to falling is common -Mechanical examples: aortic steonsis, pulmonary stenosis, HCM, congeital lesions associated with pulmonary HTN or right-to-left shunting, LA myxoma. -Ischemia -Episodes commonly exertional or post-exertional -More commonly, d/t disorders of automaticity (sick sinus syndrome), conduction disorders (like AV block), or tachyarrhythmias (esp Vtach and SVT with rapid ventricular rate) ————-Other Non cardiac or Non Neuro Causes——- Hypoxia Hypoglycemia Hyperventilation Vertigo CNS Seizures ————-Dx———————— -Resting EKG recommended for all patients undergoing syncope evaluation.High risk findings= non-sinus rhythm and complete or partial left BBB. Holter monitoring or continuous cardiac telemetry to r/o transient or intermittent arrhythmia -Autonomic Testing- Orthostatic hypotension from autonomic function can be diagnosed with more certainty by observing BP and HR responses to Valsalva maneuver and by Tilt testing. Do *Tilt Testing* before invasive procedures unless convincing clinical or EKG findings say otherwise. Procedure for tilt test: tilt passively to atleast 70 degrees for 10-40 mins in conjuncition with either isoproterenol or nitroglycerin sublingual. -Electrophysiologic studies (assess sinus node function, AV conduction and to include SVT or Vtach). Indications: recurrent syncopal episodes, nondiagnostic ambulatory EKG's, negative autonomic testing (if vasomotor syncope is a consideration) ———-Tx——————— -Vasodepressor syncope- counseling pts to avoid predisposing situations and maintaining adequate hydration. -BB's have been used in pts with altered autonomic function uncovered by head-up tilt testing but generally provide minimal benefit -Volume expanders like fludrocortisone, or vasoconstrictors like midorine, may also be tried. -If symptomatic bradyarrhythmias or SVT detected, therapy can be initiated w/o additional dx studies. -Permanent pacing indicated in pts with syncope and documented severe pauses (>3 secs), bradycardia, or high-degree AV block (2nd degree Mobitz type II or complete heart block) ————Recommendations for resumption of Driving—————— -Pts with syncope or aborted sudden death thought to have been d/t temporary factors (acute MI, bradyarrhtymias subsequently txed with permanent pacing, med SE, electrolyte imblance) should not drive for at least 1 week. -Other pts with symptomatic SVT or aborted sudden death, whether txed pharmacoligically, with antitachycardia devices or ablation therapy, should not drive for at least 6 months.

Neonatal Graves Disease

-Transient congenital hyperthyroidism; occurs in 1% of infants born to mothers with Graves —-Pathophys—- -Occurs when maternal TSH receptor antibodies (TRAB's) cross placenta and stimulate excess thyroid hormone in the newborn -Can be associated with irritability, IUGR (intrauterine growth restriction), poor weight gain, flushing, jaundice, HSM, thrombocytonepenia. In severe cases: cardiac failure and death -Thyroid studies should be obtained at brith and repeated within the 1st week —-Tx/Prognosis—- -Temporary tx may be necessary with iodide, antithyroid agents, BB's, or CS (so same drugs seen in thyroid storm tx) - Gradually resolves over 1-3 months as maternal antibodies declien -AS TSH receptor antibodies may still be present in the serum of previously hyperthyroid mothers s/p thyroidectomy or RAIA, neonatal Graves disease should be considered in all mothers with hx of hyperthyroidism.

Pinworm

-Typical clinical picture: kid plays in sandbox, get itchy butt later, esp at night. -Multiple tiny pearly-white worms seen at anus -Dx: Tape TEST EARLY MORNING: before shower, eggs laid at night. -Tx: Mebednazole 100 mg. repeat in 1 week. Cure rate of 96% -MOA: work by inhibiting the microtubule function in the pinworm adults, causing glycogen depletion, thereby effectively starving the parasite cure rate of 96%

Familial Short Stature and Constitutional growth delay

-Typically have normal birth weight and hiegh -In 1st 2 yrs of life, their linear growth velocity decelerates as they near their genetically determined %ile. -Once target %ile is reached, the child resumes their normal linear growth parallel to growth curve, usually b/t 2-3 yo. Skeletal maturation and timing of puberty consistent with chronological age. -Example of familal short stature.: an infant boy a mother who is 5 ft 0 in and a 5 ft 5 father (calculated midpaternal height= 5 ft 5 in) may have a birth length in 50th percentile. However, this childs length percentile will drift downard during the 1st 2 yrs of lie and will settle in at the 5th percentile where it will stay; curve is very smooth. Final Heigh appropriate for family heigh -Children with constitutional growth delay do not necessarily have short parents, but have a growth pattern similar to those with familial short stature. The difference is that kids with constitutional growth delay have a delay in skeletal matturation and delay in puberty onset. In these children, growth continues beyond the time average child stops growing, and final heigh is appropriate for target height. *Often a history of other family members being "late bloomers". * -Constitutional delay of growth curve often shows them in the lower percentiles and shooting up into average percentiles during puberty. You see a huge acceleration. Final height is appropriate for target heigh. -Tx: can use GH for those with idiopathic short stature whose current heigh is more than 2.25 standard deviations below normal height for age.

Idiopathic hypercalcemia of Infancy (Williams Syndrome)

-Uncommon -Characterized by elfin-appearing facies and hypercalcemia in infancy -Other features= failure to thrive, mental/motor retardation, CV abnormalities (primarily supravalvular aortic stenosis), irritability, purposeless movements, constipation, hypotonia, polyuria, polydipsia, HTN -A gregarious and affectionate personality is the rule in children with this syndrome -Hypercalcemia may not appear until several months —-Tx—— -Restriction of calcium and Vit D (Calcilo formula) -In severe cases, moderate doses of glucocorticoids or even bisphosphonates

Traveler's Diarrhea

-Usually benign, self-limited occuring about 1 week into travel -Significant risk factor for developing IBS -Prophylaxis not recommended unless there is a comorbid condition (IBS, HIV, immunosuppressive) -Whenever one travels from one country to another, diarrhea can develop 2-10 days. -Bacteria cause 80% of cases, with E coli, shigella, and campylobacter being MC. -Contributory causes= unusual food and drink, change in living habits, occasional viral infections (adeno and rotaviruses) , changes in bowel flora. Chronic water diarrhea may be d/t amebiasis or giardiasis. -Sx: May be up to 10 or more loose stools a day accompanied by ab cramps and nausea, occasional vomitting, +/- fever -Watery stools usually not associated with fever when cause to entertoxigenic E coli. -Invasive bacterial pathogens like shigella, campylobactera, samonella= stools can be bloody and fever be present.. -Illness usually stops in 1-5 days. -Labs: In pts with fever and bloody diarrhea, stool culture indicated, but in most cases, cultures are reserved for those who don't respond to abx -Prevention: Avoid fresh food and water source likely to be contaminated. -Current approach is to give traveler a supply of antimicrobials to take IF significant diarrhea occurs during the trip. -In areas where toxin-producing bacteria are major diarrhea (latin america and africa) cause, a loperamide and a single dose of a flouroquinolone should be taken. -In areas where invasive bacteria (like india, asia, thailand) and in pregnant women= azithromycin (or rifaxamin)

Phases of growth

1)Prenatal -2.5cm/wk @ 20 wks gestation -Environment is most significant impact (maternal nutrition, drugs, smoking, etc -Uterine size -Genetic influence is limited 2)Infancy -Rapid, but rapidly decelerating linear growth -Initially nutrition dependent -Hormonal effects start at 6-8 mos -Genetic track and correlation b/t child/parent hiegh increases around 2-3 yo. 3) Childhood -Slow deceleration from 3/4 yo- early teen years -* GH and thyroid hormones are what influences growth* 4)Puberty -"pubertal growth spurt"= rapid acceleration then deceleration -*Sex steroids and ^ GH secretion are major hormone influences* GH and IGF-1 together are responsible for about 30% of adult stature. Liver converts GH to IGF-1, IGF-1 is primary factor involved in bone growth at epiphysis.

Types of Syncope

1. Orthostatic 2. Neuro (vasovagal) 3. Cardiogenic (Cardiomyopathy, electrical disturbances, valvular disease, etc) 4.Other (drugs, seizures, etc)

Familial hypocalciuric hypercalcemia (FHH)

1. autosomal dominant, 2. decreased urinary Ca2+ excretion and increased serum Ca2+ 3. In most cases, the genetic defect is a mutation in the membrane-bound calcium sensing receptor exrpessed in parathyroid and renal tubule cells 4. PTH high normal, 24 hr urine calcium low (versus primary hyperparathyroidism where urine Ca ^^) 5. Most pats asymptomatic, and treatment is unnecessary

If boys aren't showing signs of puberty by this age, they should be evaluated for pubertal delay

14

Maternal hormones or colonization of Malassezia Neonatal Acne No tx. Usually presents within 3 mos of birth, resolves 1-3 months

2 mo old pt presents like this. Potential causes? Dx? Tx?

Constipation in kids definition

2 or more of the following events for 2 months: 1)<3 bowel movements per week 2) > 1 episode of encorpresis per week 3) impaction of rectum with stool 4) passage of stool so large it obstructives toilet 5) retentive posturing and fecal witholding 6) Pain with defication.

Ewing sarcoma

2nd MC primary bone malignancy in children and adolescents. MC in males 5-25. Femur and pelvis= MC sites -Accounts for only 10% of primary malignant boen tumors, can also affect soft tissue -Disease of white males, almost never affects blacks, MC in 2nd decade of life -Considered a "*small, round, blue cell*" malignancy -sx: Pain at site of primary tumor MC sign +/- swelling or erythema. Associated sx: fever and wt loss can be seen -Labs: Elevated LDH may be seen, is prognostically significant -Imaging:Usually involves diaphyses of long ones. Should have MRI done.. Radiographjs show lytic lesion layered periostal reaction ONION SKIN apperance on radiographs. +/- Codman's triangle -MC METs sites= lung, bone, BM -CT of chest, bone scan, and bilateral BM aspirates and biopsies essential for workoup -Biopsy essential for establishing dx: Sheets of undifferentiated cells w/ hyperchromatic nuclei, well-defined cell borders and scanty cytoplasm. Necrosis common. -Electron microscopy, immunocytochemistry, and cytogenetics may be needed to confirm dx: t(11;22) Id'd, PNET seen very frequently. C-myc also expressed, helping ddx it from neuroblastoma -TX: Commences with admin of chemo after biopsy and followed by local control measures: either surgery, radiation or a combo. Following local control, chemo for 1 yr.

Turner Syndrome

45 XO, female hypogonadism, hypothyroid, short, webbed neck, 1* amenorrhea p52 —-Essentials——- -Webbed neck, trinagular facies, short stature, wide-set nipples, amenorrhea, and absence of 2ry sex characteristics -Assocaited with coarctation of aorta and GU malformations -IQ usually normal but learning disabilities are common -Mosaic individuals may manifest only short stature and amenorrhea ——Hallmarks—— -Hypogonadism—>primary amnenorrhea or early ovarian failure, delayed 2ry sex characteristics (absence of breasts), infertility, short stature (with normal GH levels), webbed neck, edema, low hairline, low set ears, widely spaced nipples. Renal and CV abnormalities ——Pathophys—— -Mosaicism (67-90%): some cells have a combo of X monosomy (45, XO), some cells that are normal (46, XX) cells with partial monosomies (X/abnormal X) or cells that have a Y chromosome (46, XY) -45, XO= absence/nonfxn of 1 of the X chromosome= gonadal dysgenesis ——Clinical Manifestations—— -Hypogonadism:: primary amenorrhea, early ovarian failure -Newborns may have: webbed neck, edema of hands/feet, coarctation of aorta, characteristic triangular facies -Later sx include: wide spaced nipples with shield chest, streak ovaries, short stature, absence of 2ry sex characteristics, infertility -Renal: may see horsehoe kidney, hydronephrosis -Endocrine: osteoporosis, hypothyroidism, DM, -GI: telangectasias, IBD, colon cancer, liver disease —-Dx—- -Karyotyping= definitive dx (45, XO), mosaicism, or X chormosome abnormalities -High serum FSH and LH levels —-Tx—— -GH replacement (may increase final heigh) -Estrogen/Progesterone replacment to cause pubertal development

Possible Precocious puberty Infantile Acne Topical Abx or BZP +/- oral ABX. Seen in infants 6 mos-3 yrs. Does have scarring potential

7 mo old pt presents like this Possible linpos Tx?

Buckle Fracture treatment

Ace wrap or anterior splinting

Hyaline Membrane Disease

Acute lung disease in premature infants; respiratory distress disease syndrome of the newborn. *Essentials* - Tachypnea, Cyanosis, and Expiratory grunting - Poor air movement despite increased work of breathing - Chest radiograph showing hypoexpansion and air bronchograms MC cause of resp distress in preterm infants Surfactant production starts 24-28 weeks, enough surfactant produced by 35 weeks Increased incidence from 5% at 35-36wks to >50% of infants 26-28 wks Caused by *def of surfactant production* *Sx* - Diminished air mvmt despite vigorous effort - CXR shows diffuse bilateral atelectasis *ground glass appearance* - Major airways are highlighted by the atelectatic air sacs, creating bronchograms - Doming of the diaphragm and hypoinflation *Tx* - Supp O2, Nasal CPAP, early intubation, and placement of umbilical artery/vein lines - Ventilator may provide resp assistance - Surfactant replacement *Prevention* Corticosteroids given if expected prematurity (b/t 24-36 weeks

alopecia areata

Autoimmune disorder that causes the affected hair follicles to be mistakenly attacked by a person's own immune system; usually begins with one or more small, round, smooth bald patches on the scalp. -Non scarring, commonly associated with autoimmune disorders ——Signs/sx—— -Exclamation point hairs- short hairsa broken off a few mm from teh scalp at margins of the patches with tapering near the proximal hair shaft -Smooth discrete circular patches of complete hair loss that develops over a period of weeks -Nail abnormalities: pitting, fissuring, trachyonychia -May spontaneously resolve or porgress to allpecia totalis (scalp loss) or universalis (total body loss) ——Tx—- -Local: intralesional steroids -Extensive: topical CS -Systemic immunosuppressives, irritatns (anthralin) + minoxidil, PUVA

Juvenile RA

Autoimmune mono or polyarthritis in kids *<16yo* Often resolves by puberty 3 types: 1) Pauci-articular - 50% (MC) - < 5 joint invovlement in 1st 6 months - MC large joints; usually assymetric -BC this arthritis is assymetrical, can develop leg length descrepancy - Can develop synovitis. Usually mild/painless - Type I: associated w/ anterior uveitis; monitor q 3 months if ANA positive and 6 months if ANA negative - Type II: assocaited w/ ankylosing spondylitis 2) Systemic/Acute Febrile (Stills Disease) - 20% - Daily arthritis, *Diurnal high fever* in 1st 6 months - Small and large joints - Salmon colored/pink migratory rash - No iridocyclitis, but has hepatosplenomegaly, hepatitis, lymphadenopathy and serositis 3) Polyarticular - 30% - Arthritis >5 joints in 1st 6 months, small or large. Typically in symetrical pattern - Most similar to adult RA - Hip involvement in common; can cause limp -Rheumatoid nodules and anemia may be present. - ^ risk of iridocyclitis - Rheumm factor negative disease associated w/ better prognosis 4) Enthesitis-Associated -MC in males >10 -Associated with lower extremity, large joint arthritis -Hallmark= inflammation of tendinous insertions such as tibial tubercle or the heel. -LBP and sacroilitis common as well. *Lab* -No diagnostic test - ^^ESR/CRP (but normal ESR does not exclude dx) - (+) ANA in oligoarticular (connection with iridocyclitis) - (+) Rheum factor in 5% - HLA B27 (+) associated with ^ risk of enesthesitis associated -Anti-CCP *DDx* -THis dx is associated with morning stiffness. -Growing pains, characterized by poorly localized pain at night, which frequently wakes child from sleep; no objective signs of inflammation and no daytime sx; these kids often asked to be massaged, not typical of those with arthritis. *Tx* -1st line therapy is NSAID's: Naproxen, Ibuprofen, Meloxicam -For pts who fail to respond to NSAIDs, weekly MTX is 2nd line med of choice -Leflunomide can also be tried -Local steroid injections helpful in pts who have arthritis in 1 or a few joints. Oral or parenteral steroids reserved for kids with severe involvement, primarily in systemic disease -Iridiocystitis should be closely monitored by ophthalmologist; typical tx is CS eye drops and dilating agents. -PT/OT *Prognosis* -Variable -Children with persistent oliogoarticular form have highest rate of remission; while those with RF positive least likely to remiss and highest risk for chronic -Systemic features associated with systemic arthritis tend to remit in months to years; prognosis worse if remains after 6 months. - Methotrexate or Leflunomide - NSAIDs, Steroids - Frequent eye exams

Diabetes and gestational weight/size

Baby will be large for gestational age is mother is diabetic. It is because the hyperglycemia from mother will pass to the baby. The baby responds to the hypergylcemia by producing insulin, resulting in hyperinsulemia. This causes the kid to get big

Enchondroma

Benign cartilaginous neoplasm found in intramedullary bone. Usually distal extremities -Present at any age, but > in 2nd decade. M=F ——Imaging—— -Usually a silent lesion unless it produces a pathologic fracture -On radiograph it is radiolucent, usually in a long bone. Speckled Calcification can be seen -Oval, well-circumscribed central lucent lesion -Occur in long bones of hand>humerus>femur -*The classic lesion looks as though someone dragged their fingernails through clay, making streaks in the bone ——Tx—— -Surgical curretage and bone grafting —-Prognosis—- -Excellent -Malignant transformation may occur but is very rare in childhood.

Giant Cell tumor

Benign, locally aggressive tumor 2-3% metastasize to lung Benign Pulmonary Implants 15-20% of all benign bone tumors Occurs after skeletal maturity Usually in 20's and 30's Females slightly > males -Clinical Presentation Pain, swelling, decreased ROM at site Usually occur in epiphysis of long bones: Distal Femur Proximal Tibia -Radiographic Imaging Expansive, eccentrically placed lytic area May extend to subchondral plate or joint

Rashes that affect palms and soles

CARS: Coxsackievirus A, RMSF, Syphilis Others: Janeway lesions (endocarditis), measles, kawasaki, toxic shock syndrome, reactive arthritis, meningococcemia

Chondrosarcoma

Cancer of the cartilage. MC seen in adults 40-75 3rd MC primary malignancy of bone after myeloma nad osteosarcoma —-Features—— -Low to intermediate grade malignancy -Part of chondroid production -Slow growing ——Dx—— -Mineralized chondroid matrix with punctate or ring and arc appearance pattern of calcification

Pertussis

Causative Agent= Bordotella Pertussis (gram negative coccobaccilus) -High contagious infection -Mc in children <2 yo -Incubation= 7-17 days —-Signs/Sx—— Catarrhal phase—> paroxysmal phase—> convalescent phase 1) Catarrhal phase: URI sx lasting 1-2 weeks, most contagious during this phase. insidious onset, lacrimation, sneezing, coryza, malaise, hacking, and diurnal night cough. Slight fever may be present 2) Paroxysmal: Severe paroxysmal coughing fits with inspiratory whooping sound after cough fits (10-30 forceful coughs) (deep high pitched respiratory whoop). Coughing fits may occur spontaneously or can be provoked by laughing, yawning, etc. Infants and adults with otherwise typical severe condition often lack whooping. Vomitting commonly follows a paroxysm. May develop subconjunctival hemorrhage from increased pressure due to the coughing fits. Lasts 2-4 weeks 3) Convalescent phase: Resolution of the cough (coughing staage can last up to 6 weeks) ——Dx—— -Lymphocytosis (60-80% on differential). WBC count can be as high as 50k -*PCR of nasopharngeal swabs= gold standard* sensitivity decreased if > 3 weeks duration —-Tx—— -Azithromycin tx of choice especially if infants <1 mo (erythromycin has been associated with pyloric stenosis in infants <1 mo)===> only shortens duration within 1st 7 days of sx onset. -Ampicillin or bactrim good 2nd line choices -CS reduce severity of disease but can mask signs of backterial superinfection -Supportive tx: oxygenation, nebulizers, mechanical vent as needed. —-Complications—— -Pneumonia, encephalopathy, otitis media, sinusitis, seizures. ^ mortality in infants d/t apnea/cerebral hypoxia associated with coughing fits

Mother with hypertension

Causes Intrauterine growth reduction. Decrease blood supply to placenta can cause placental insufficiency and result in low birth weight child.

Prader-Willi Syndrome

Characterized by prenatal hypotonia, growth delay, developmental disabilities, hypogonadotropic hypogonadism, and obesity after infancy ——Pathophys—— -75% occurs d/t small deletion/inexpression of genes on the paternal chromosome 15 from loss of paternal copy of a region of chromosome 15 seen in up to 75%. The maternal copy of gene is silence thru imprinting -25% occurs d/t maternal uinpaternal disomy (a person receives 2 copies of a chromsome or part of a chromosome from one parent and no copies from the other parent) ——Clinical Manifestations—— -Prenatal: Breech positioning, polyhydraminos, and reduced fetal movement -Neonates: Severe hypotonia, floppy baby, weak cry, feeding difficulties, genital hypoplasia, *cryptorchidism*, depigmentation of skin/eyes -Early childhood: Hyperphagia, obesity, short stature, learning difficulties, skin picking -Later childhood: premature development of pubes and axillary hair with delay of other 2ry sex characteristics ——PE—— -*almond shaped eyes* -High/narrow forehead, thin upper lip with small, downturned mouth -Prominent nasal brdiges. SMall hands/feet, soft skin that easily brusies. -Excess fat (truncal obseity) ——Dx—— -DNA testing (DNA based methylation studies) —-Tx—— -GH replacement -Obesity control by monitoring food intake.

Psoriasis

Chronic, multisystemic inflammatory immune disorder with genetic predisposition ——Pathophysiology—— -Keratin hyperplasia (proliferating cells in the stratum basale + stratum spinosum d/t T cell activation and cytokine release)==> greater epidermal thickness and increased epidermal turnover -Obesity worsens condition, significant weight loss improves —-Signs/sx—- -Itching may occur and be severe -Favored sites= scalp, elbow, knees, palms, soles, nails -Lesions= red, sharply defined plaques covered with silvery scale -Occasionally only flexures are involved (inverse psoriasis) —Types—— 1) Plaque (MC type): raised dark red plaques/papules with thick silver/white scale. MC on extensor surfaces of elbows/knees, scalp, nape of neck. Usually pruritic. *Nail pitting* may be seen. Yellow-brown discoloration under nail (oil spot) is pathognomonic. -Auspitz sign -Koebner's phenomenon (new skin lesion at site of trauma) 2) Pustular: deep, yellow non-infected pustules that evolve into red macules on palms/soles 3) Guttate: Small, erythematous papules with fine scales, discrete lesions, and confluent plaque 4) inverse: erythematous, lacking scale. MC in body folds 5) Erythrodermic: Generalized erythematous rash involving most of skin (worst type) 6) Psoriatic arthritis: Sausage digits, pencil in cup deformity ——Ddx—— -Combo of red plaques with silvery scale on elbow and knees, with scaliness in scalp or nail findings, is diagnostic -Well demarcated plaques and affect extensor surfaces, whereas atopic dermatitis tends to be poorly demarcated plaques in flexural distribution —-Tx—— -Systemic CS should never be used to treat flares. -Topical Calcineurin inhibitors (like Tacrolimus) good in intertrignous areas -Limited disease (<10%BSA): High-Ultra high potency CS are mainstay of tx.If numerous small plaques, phototherapy is best. Can use vit D analogs, topical tar, retinaoids/Vit A analogs. Tacrolimus if other agents not working -Moderate disease (10-30% BSA): UV phototherapy, systemic agents (which will be mentioend next) - Generalized disease (>30% BSA): Tx of choice is narrowband UVB (NB-UVB) 3x/wk. Psoralen plus UVA (PUVA) photochemotherapy may be effective in those nonresponsive to NBUVB. MTX is very effective in severe cases. Others: cyclosprine, retinoids (Acitretin), biologics.

Tetralogy of Fallot

Congenital malformation involving four (tetra-) distinct heart defects ——Defects——- 1) RV outflow obstruction= Pulmonic stenosis 2) RVH 3) Overriding aorta (between ventricles) 4)VSD (large unrestrictive) ——Essentials—— -MC Cyanotic heart disease overall -Associated with deletion in long arm of chromosome 22 (DiGeorge syndrome) -Cyanosis in infants, Tet Spells in older children (periodic episodes of cyanosis relieved with squatting or putting an infants knee to chest) -CXR= Boot-Shaped heart -Tx= Surgical Correction. Prostaglandin E1 prior to surgery to maintain patency of ductus arteriosus ———Signs/sx——— -Blue baby syndrome (cyanosis) -Older kids: exertional dyspnea, cyanosis worsens with age -Tet spells: paroxysms of cyanosis- older children relieve spells by squatting or putting knees to chest. These spells can occur spontaneously and at any time, but in infants occur commonly with crying or feeding; while in older children they can occur with exercise. -Harsh holosysolic murmur at left upper sternal border (similar to pulmonic stenosis). Grade II-IV rough systolic ejction murmur that radiates well to back. -RV heave -Digital clubbing -Those with severe obstruction cyanotic at birth. Nearly all have cyanosis by age 4. usually progressive cyanosis -Growth not delayed. ———Labs——- -Hgb, HCT, RBC usually elevated in older infants or children 2ry to chronic arterial desaturation ——-Imaging——— -CXR= boot shaped heart (RV hypertrophy); aortic knob right of trachea, decreased pulm vasc markings -EKG: RAD, p waves normal, RVH usually present -echo: diagnostic -Cath: not necessary but is done if hypoplastic pulm arteries. Major indications are to establish coronary artery and distal pulmonary artery anatomy if not able to be clearly defined by echo. ——-Tx——- -Tet spells: place in knee to chest position or squatting. Use IV morphine cautiously, but does have good sedative effect -Propanolol produces B blockade. and may reduce obstruction across RV outflow tract through its negative inotropic action. Although onset of tet spells usually prompts surgical intervention -Elective surgical intervention occurs around the age of 3 months so as to avoid development of Tet Seplls -Surgical repair usually done though by first year or 2. -PGE 1 infusion prevents ductal closure if patient is cyanotic prior to surgery.

Duodenal Atresia

Congenital malformation of the duodenum resulting from failed recanalization of the intestinal lumen during gestation (complete absence or complete closure of a portion of lumen of duodenum)= gastric outlet obstruction -Assocaited with Trisomy 21 (Down Syndrome) -Polyhydraminos (increased amnitoic fluid) is commonly seen during pregnancy ---Signs/Sx--- -Hallmark is bilious vomiting without abdominal distension usually within 1st day of life -Lethargy -Upper ab distension may be seen, respiratory difficulty may also be seen) ---Dx---- -Ab XR: *Double buble sign* caused by distended and gas filled stomach and proximal duodenum. Distended duodenum and distended stomach separted by pyloric valve -----Tx---- -Nasogastric or orogastric decompression and IV fluid therapy followed by surgical repair of the defect (duodenoduodenostomy) ---Prognosis---- - good in absence of other congenital anomalies

Crigler-Najjar syndrome

Congenital unconjugated hyperbilirubinemia - Autosomal Recessive in type 1, autosomal dominant in type 2 ----Pathophys----- -Glucuronosyltransferase (UGT) is the enzyme needed to convert indirect bili to direct bili -Type 1= no UGT activity -Type 2= Very little UGT activity (<10% of normal) -If untreated, both can cause severe unconjugated hyperbilirubinemia, bilirubin encephalopathy, and death if untreated ---Clinical Manifestations----- -Type 1: Neonatal jaundice with severe progression in the 2nd week, leading to kernicterus- ^ bilirubin in CNS and basal ganglia==> hypotonia, deafness, lethargy, oculomotor palsy and death (often by 15 mos of age) -Type 2: Usually asymptomatic. Often an incidental finding on routine lab testing ----Dx---- -Isolated indirect (unconjugated) hyperbilirubinemia with normal LFT's -Type 1: serum indirect bili often between 20-50 -type 2: serum indirect bili often between 7-10. May increase during illness/fasting -Liver looks normal on biopsy ----Tx---- 1) Type 1 -Phototherapy= mainstay of tx -Plasmapharessis may be used in acute elvations of bilirubin (ex: crisis) -Liver transplant= definitive 2) Type 2 -Tx usually isn't necessary -*Phenobarbital* has been shown to increase UGT activity. -Type 1 is NOT responsive to phenobarbital (since there is no UGT activity)

3 main categories of pubertal delay

Constituional delay in growth Hypogonadotropic hypogonadism Hypergonadotropic hypogonadism

Primary amenorrhea

Defined as having no periods or secondary sex characteristics by age 13 OR no menses in presence of 2ry sex characteristics by age 15. ——Etiologies—— -MCC= constitutional delay of growth and puberty (strong genetic basis) -Mullerian agenesis: Mayer-Rokitansky-Kuster Hauser syndrome (my one pt on endo rotation had this): no uterus, intact ovaries -PCOS (hyperandrogenism) -Turner Syndrome (45,XO) -Pituitary causes: tumor, empty sella, Sheehan syndrome -CNS and hypothalamus: Kallman's syndrome (Hypogonadotropic hypogonadism), anorexia, hormone therapy ——Initial Labs—— - B-HCG (most important), FSH, LH, PRL, T, TSH, FT4 -May need MRI if pituitary cause suspected -Can go on to look at other testing such as Progesterone challenge test ——Tx—— -Tx underlying causes, may need exogenous Estrogen and/or progresterone -If constituional delay of puberty: obesrvation and reassurance.

Enuresis

Defined as repeated urination into clothing during the day and at night in bed by a child who is chronologically and developmentally older than 5 years in absence of sx of infection; this must occur at least twice a week x 3 months ——Classifications—— 1)Monosymptomatic uncomplicated nocturnal enuresis- must never have been dry at night over 6 months; no daytime accidents. Reflects a delay in achieving nightime continence and delay in maturation of urological and neurological sx. -Most children are continent at night within 2 yrs of achieving daytime control; however 15.5% of 7.5 yr old children wet the bet but only 2.5% meet critieria for enuresis. -Causes are varied and probably interact with one another. Genetics strongly implicated, as it runs in families. Many kids with nocturnal enuresis have a higher threshold for arousal and don't wake up to sensation of full bladder.. Can also occur as overproduction of urine from decreased ADH or resistance to ADH (SIADH type sx) 2)Complicated (nonmonosymptomatic): involves nocturnal enuresis and daytime incontinence and often reflects an underlying disorder —-Evaluation: Noncomplicated—— —Complete H&P to r/o any anatomical abnormalities, underlying pathology, or presence of constipation -Every child with nocturnal enuresis should undergo a UA including a specific gravity -Urine culture should be obtained, esp in girls ——-Tx: Noncomplicated—— 1)Behavioral: education and avoidance of judging or shaming child. Use of washable products, room deoderizers. Regular voiding scheuule, deliberate voiding prior to sleep. Waking up child periodically to urinate. Avoid caffeine based drinks and sugary drinks. Night time fluid restriction 2) Enuresis Alarm: Usually used if kid fails to respond to behavioral therapy. Often attempted before medical therapy. A sensor is placed on a bed pad or in underpants and goes off when wet. Most effective long term therapy. The most common cause of failure is when child doesn't awaken or parents don't wake child. Therapy needs to be continued for at least 3 months and used every night. Parents must participate 3) Desmopression (DDAVP): 1st line tx when behavioral therapy (and alarm) fails. -Imipramine (TCA) can be tried, but high SE profile ——Complicated Enuresis Notes—— -Daytime continence achieved by 70% of kids by age 3 and 90% of kids by age 6. -When this is not the case, one needs to consider other pathology like: cystitis, diabetes insipidus, DM, seizure disorders, neurogenic bladder, or other anatomic abnormalities like urethral obstruction, constipation, psychological stress, child abuse. -Tx= tx underlying pathology, pediatric subspecialty referral.

DRESS

Drug Reaction with Eosinophilia and Systemic Symptoms -Potentially life threatening, drug induced hypersensitivity rxn -Induces skin eruption, heme abnormalities (eosinophilia, atypical lymphocytosis), lymphadenopathy, internal organ involvement (liver, kidney, lung) —-Characteristics—- 2-8 weeks between drug exposure and onset -Prolonged course with frequent relapses despite d/c'ing drug -Frequent association w/ reactiviation of Human herpesvirus infeciton ——Epidemiology and pathogenesis—- -primarily in adults -1/100 children exposed to lamotrigene -1-5/1000 exposured to carbamazepine or phenytoin -Han Chinese, European, Portugese may be at ^ risk -80% of cases d/t drug exposure, mc drugs= allopurinol, carbamazepine, lamotrigene, phenytoin, sulfasalzine, vanco, minocycline, dapsone, sulfamethoxazole —-Presentation—— -Common initial sx= fever, malaise, lymphadenopathy -Inflmmation and mucous membrane pain, typically at single site, but no ulceration (occurs in 50%) -Skin eruption: morbiliform rapidly progressing to a diffuse, confluent and infiltrated erythema with follicular atteunation -Face, upper part of trunk, extremities involved first -*Dx suggested when eruption is >/= 50% of BSA and/or includes 2 or more of: 1) facial edema; 2) infiltrated lesions; 3) Scaling; 4) Purpura* -Often w/ coalescing erythematous papules and possible desquamation of palms and soles -Involvment of atleast one internal organ in ~90%, 2+ organns in 50-60%: Liver (MC), kidney, lungs. ——Labs—— -CBC w/ diff (leukocytosis, ^ Eosinophils, atypical lymphocytes) -CMP (^ ALT) -UA -EKG and troponin -May see (+) HHV 6 —-Tx—- -Mainstay= Stop offending drug -If no severe organ involement (no renal/pulm involvment and <3x increase of LFT's)= High or super high potency topical CS -Severe organ involvment: PO Steroids tapering over 8-12 weeks

Causative agent of pinworms

Enterobius vermicularis

Meniere's disease

Episodic vertigo + Hearing loss Idiopathic cause —Tx—— -symptomatic (antiemetics, meclizine, diuretics, sodium decrease intake, no smoking)

Guttate psoriasis

Erythematous plaques with silvery scale on trunk, strep infection, nail pitting, geographic tongue Tx= moisturizers, oatmeal baths, sunlight, topical CS- UpToDate likes NBUVB as 1st line

Seborrheic dermatitis

Erythematous scaly dermatitis accompanied by overproduction of sebum occuring in areas rich in sebaceous glands (face, scalp, perineum) -Occurs predominantly in newborns and at puberty -Can appear clinically similar to atopic dermatitis, and distinction may become clear only after other areas are involved -*Psoriasis* also occurs in seborrheic areas in older children and should be considered in dx. —ddx: unlike tinea capitis, this DOES NOT result in hair loss ——-Tx—— -Low potency topical CS -Tar based shampoos -Topical antifungals

Paget's disease of bone

Essentials - Often Asymptomatic - Bone pain is 1st symptom - Kyphosis, bowed tibias, large head, deafness, and frequent fractures - Serum Ca and PO NORMAL; AlkPO ^^^; Urinary hydroxyproline ^ - Dense, expanded bones on radiographs High bone turnover followed by disorganized osteoid formation - Increased osteoclast activity lead to lytic bone lesion - Increased osteoblast activity produces disorganized bone formation - Results in weak, vascular, deformed bone 2-3% of all people > 55. Yo, Men slightly > F Chance doubles q decade p 40yo ^ risk of Osteosarcoma or Giant Cell tumor Sx - Often aysmptomatic - Deep, achy pain worse at night - Multiple bones (72%) > Single bone (28%) - MC pelvis, vertebrae, femur, humerus, skull - Starts distally on long bones and advances proximally - Weakened bones lead to deformity (bowing, kyphosis, or "chalkstick" fracture) - Bones can become soft, leading to bowed tibias, kyphosis, and frequent "Chalkstick" fractures -Can lead to deafeness if skull involvement (seen in up to 50% due to compression of CN8) Lab - ^^^ AlkPO -Normal Ca and Phosphate Imaging - Lytic lesion - Osteoporosis Cirucmscripta in skull (Focal radiolucency) - Flame-shaped lytic lesions in long bones - Deformity of bone - Lesions may eventually become sclerotic Complications - Skull involvement may cause "vascular steal syndrome" = somnolence or ischmeic neuro event - Vertebral involvement can cause paralysis Tx - Asypmtomatic = surveillance - Biphonphonates (ie. IV Zolendronate is Tx of choice)

tonsillar exudates, cervical LAN, fever, no cough

Group A strep, mono (EBV) in teens

Infant GER

Healthy happy spitty uppy babies. Gets better as diet gets more THICC and as they are able to hold up own posture as well. Without dysplasia or pathology. 1/2 of all infants get this, resolves spontaneously. -TX: Positioning -feeding strategies: don't feed them as much at once, feed them more frequently. Rice cereal may help Nipple size on bottle should be big enough for thicker formula. Wedges in crib. Keep baby upright on sholuder nice and vertical for 20-30 mins after feeding.

ABO incompatibility

Hemolytic disease that occurs when the mother's blood type is O and the newborn's is A, B, or AB -Hemolysis is usually mild, but the severity is unpredictable because of variability in the amount of naturally occuring maternal anti-A or anti-B IgG antibodies -Jaundice occurs in the first 24 hours after birth -*Coombs test positive*(only in 33%) -Only 20% of these develop jaundice that requires therapy -Reticulocyte count= high, H&H: low ----Tx---- -Since maternal antibodies may persist for several months after birth, the newborn may become progressively more anemic over the first few weeks of life, occasionally to the point of requiring transfusion. -Phototherapy

Gilbert Syndrome

Hereditary unconjugated (indirect) hyperbilirubinemia. Relatively common (5-10% of US population) -Autosomal dominant ---Pathophys----- -Reduced UGT activity (10-30% of normal) and decreased bilirubin uptake= ^ indirect bilirubin -Affected individuals tend to develop hyperbilirubinemia in the presence of conditions that increase bilirubin load, such as G6PD deficiency; they are also more likely to have neonatal jaundice and breast-milk jaundice ----Clinical manifestations---- -Most asymptomatic -*May develop transient episodes of jaundice during periods of stress, fasting, ETOH, illness* ----Dx---- -usually incidental finding -Slight ^ isolated indirect bilirubin level with normal LFT's ---Tx--- -Usualy none needed because it is a mild, benign disease

Impetigo

Highly contagious superficial vesiculopustular skin infection -Occurs typically at sites of superifical skin trauma (like an insect bite), primarily on exposed surfaces of face and extremities -RF's: warm, humid conditions, poor personal hygeine —-Types—- -Nonbullous (MC type): vesicles, pustules—> characteristic *honey colored crust* Staph Aureus= MCC, GABHS (2nd MCC) -Bullous: Vesicles form large bullae (rapidly)—> rupture—> thin *"varnish-like crusts"*. Fever, diarrhea. Staph Aureus= MCC. Rare -Ecthyma: ulcerative pyoderma caused by GABHS (heals with scarring) Not common. —-Tx—- -Mupirocin (Bactroban) topical= drug of choice tid x 10 days. Can also try Bacitracin -If extensive disease or systemic sx (such as fever) use SYSTEMIC (PO) abx: Cephalexin, dicloxacillin, clindamycin, macrolides

Erythema Toxicum Neonatorum Happens in half of all full term infants Within first week of life neonate gets small erythematous papules and vesicles Lesions move around neonates body Self limited

Identify Describe

-Scabies (Sarcoptes Scabei) - Small, erythematous nondescript papule, often excoriated and tipped with hemorrhagic crust. Brown linear burrows 2-15 mm= pathognomnoic. Firm erythematous extremely pruritic dome-shaped nodules 5-6 mm= pathognomonic. -Flexor surface of wrists and web spaces of hands very commonly seen in boards. Groin, genitalia, buttocks, and axillary folds common for nodular involvement. -DX: Mineral oil scrape. Microscopic exam -Topical Permethrin 5% cream 1st line in uncomplicated cases. PO Ivermectin 1st line for outbreaks in nursing homes or other facilities, may be more beneficial in nodular scabies as well. Txed individuals can return to school/work the day following tx.

Identify Dx buzzwords? Distribution? Tx?

-Lice -Direct visualizaiton (wet combing preferred, can do woods lamp) -Permethrin shampoo (children should NOT be excluded from going to school). PO Ivermectin 2nd line

Identify Dx? Tx?

-Tinea Corpis -Trichophyton Rubrum -Erythematous Ring shaped lesions that have an advancing scaly border and central clearing. May also see cracking and vesicles. The presence of scales in tinea corpis distinguishes it from erythema migrans -KOH prep: budding yeast and pseudohyphae. -Woods lamp: Green fluorescence if d/t Microsporum. -TX: topical antifungals such as: econazole, miconazole, clotrimazole, butefaine, and terbenafine. Terbinafine and butenafine require shorter coures and lead to rapid respone. -TX should continue for 1-2 weeks after clinical clearing. -TX: PO griseofulvin if topical solutions ineffective

Identify MCC agent Sx Dx Tx

-Lichen Planus -Idiopathic cell-mediated immune response -5 P's (purple, pruritic, polygonal, papules/plauqes). Wickham Striae. Koebners phenomenon may be seen (new lesions at sites of trauma, also seen in psoriasis) - Wrists, forearms, genitals, oral mucosa - Hep C and antihypertensives -Topical corticosteroids 1st line. Antihistamines can be used for symptomatic tx for pruritis -2nd line= PO steroids, UVB therapy, retinoids.

Identify Pathophys? Sx? Common Sites? Increased incidence seen with? Tx?

-Tinea Capitis -Varied presentation: annular scaling lesions and broken hair shafts at surface of scalp (black dot sign). Inflamed plaques with multiple pustules (kerion- boggy fluctuant mass) with scarring and alopecia -Fungal cultures -PO Griseofulvin 1st line -2nd line= Terbenafine, itraconazole, fluconazole

Identify Sx: Dx: Tx:

-Tinea Cruris -Marked itching in intertriginous areas, usually sparing the scrotum. Peripherally spreading, sharply demarcated, centrally clearing erythematous lesions. -KOH prep: hyphae/budding yeast ---Ddx--- -Candida typically involves the scrotum. Typically bright red and marked by satellite papules and pustules outside main border of lesion -Intertrigo tends to be more red, less scaly, and present in obese individuals in moist body folds. ----Tx---- -Drying powder (miconazole nitrate) can be dusted into the involved area in pts with excessive sweating or oclusion of skin d/t obesity as preventative measure. -topical antifungals -PO Griseofulvin if topicals innefective

Identify Sx? Dx? DDX? TX?

Molluscum contagiosum Currettage, liquid nitrogen, cantharidin (blister beetle juice). Most go away on own and don't need tx

Identify Tx?

Scarlet Fever PCN or Amoxicillin

Identify Tx?

Tinea Versicolor Topical Antifungal or selenium sulfide. Can do PO antifungal like ketocanazole.

Identify Tx?

-Ptyriasis Rosea -Unknown etiology, but associated with HHV7. Not contagious. -Trunk and proximal extremities (face usually spared) ---Sx---- -Hearld patch (solitary salmon-colored macule) on the trunk 2-6 cm in diameter--> general exanthem 1-2 weeks later: smaller, VERY PRURITIC 1 cm round/oval *salmon colored papules* with whie circular scaling along cleavage lines in a *christmas tree pattern* ---Tx---- -Usually requires no tx -Tx only indicated if pt is symptomatic: UVB -Mild to moderate cases: topical CS like Triamcinolone and PO antihistamines can be used if pruritis is bothersome. Oatmeal baths ---Prognosis---- -Usually self limiting that disappears in about 6 weeks

Identify: Etiology/Epidemiology? Common Sites? Characteristics? Tx? Prognosis?

-Tinea Pedis ---Sx---- -Asymptomatic scaling that may progress to fssuring or macerattion in toe web spaces. Can be a portal of entry for bacteria causing lower extremity cellulitis. MC predisposing cause of lower extremity cellulitis in healthy ppl -Can appear mocasion in appearane (like in the pic) -Itching, burning, stinging of interdigital web, scaling palms and soles, and vesicles of soles in inflammatory causes ---dx---- -KOH and culture do not always demonstrate pathogenic fungi from macerated areas as bacterial species begin to dominate ---Tx--- 1) Macerated stage- Tx with aluminum subacetate solution soaks for 20 mins twice daily. -Broad spectrum antifungal creams and solutions will help combat diphtheroids and other gram positive organisms present at this stage -If topical imidazoles fail, 1 week of once daily topical allylamine treatment (turbenafine or butenafine) will often result in clearing 2) Dry and scaly stage- topical antifungal 3) Refractory cases- Itraconazole or terbenafine PO

Identify: Sx? Dx? Tx?

When to do surgical intervention for cryptorchidism?

If testicles don't descend on own by 6 mos.

Herpangina

Infection due to coxsackievirus A that is characterized by acute onset of fever, sore throat, and flu-like symptoms associated with small red macules on the soft palate-tonsillar pillar area -Transmission=Fecal Oral route or oral-oral route -MC in late summer/early fall —Signs/sx—- -Sudden onset of high fever (102-104F), sore throat, stiff neck, nasuea, ab pain -Stomatitis: small vesicles on the soft palate, uvula and tonsillar pillars that ulcerate before healing. Sore throat. -Lasts 3-5 days -Mc in children 3-10 —-Dx—- -Clinical, can do Enterovirus PCR but is pretty unnecessary -PE: throat exam revelas <10 hyperemic and yellow gray-white papulovesicle lesions

Acute epiglottitis

Inflammation of the epiglottis; H influenzae type b is the most common cause, especially in nonimmunized children -MC in children 3 mos- 6 yrs. M>F 2x. Occurs any season. ——Signs/Sx—— -3 D's= dysphagia, drooling, distress. -Fever, odynophagia, inspiratory stridor, dyspnea, hoarseness, muffled voice -*Tripoding: sitting leaned forward with elbow on lap* -Suspect in a patient with rapidly developing pharyngitis, muffled voice, and *odynophagia out of proportion to exam* ——Dx—— -Laryngoscopy= definitive dx, but may provoke spasm -Lateral X ray= *Thumb print Sign*: swollen enlarged epiglottis - *If high suspicion, do not attempt to visualize epiglottis with tongue depressor in kids* ——Tx—— -Maintaining airway and supportive management= Key management. Dexamethasone to reduce airway edema -IV ABx: Ceftraixone or Cefotaxime.

Breast feeding associated jaundice

Is caused when the baby does not get enough milk. -It is not related to breast milk jaundice. -Adequate amounts of breast milk increase a baby's bowel movements, which help secrete the buildup of bilirubin. -Breastfeeding jaundice can occur when a newborn does not get a good start on breastfeeding, has an improper latch, or is supplemented with other substitutes which interfere with breastfeeding. -Breastfeeding jaundice often will resolve itself with increased feedings (q 2 hrs) and help from a lactation consultant to make sure the baby is taking in adequate amounts. Add supplemental formula -Phototherapy if levels are >15 mg/dL

Dermatographism

Localized hives over an area that results from the physical trauma of rubbing.

Osteosarcoma

MC bone malignancy, Mc in adolescents. Produces osteoid (immature bone) MC METS= lungs -Cardinal signs of bone tumor= pain at site of involvement, following slight trauma, mass formation, fracture through an area of cortical bone destruction -6th MC malignancy in childhood, 3rd MC in adolescents and young adults -Peak occurrence with growth spurt suggests casual relationship b/t gone growth and malignant transformation -Occurs at earlier age in girls than boys (growth spurt earlier) -Distal femur accounts for more than 40% of cases, with proximal tibia, proximal humerus, and mild and proximal femur following that -Sx: Pain over involved area usual presenting sx w/ or w/o an associated soft tissue mass. Generally have sx for several months prior to dx. Systemic sx rare. -Lab: may reveal ^ serum ALP or ADH levels —-Imaging—— -Radiographs= hair on end appearance or sunray/burst appearnce of soft tissue mass. INdistinct margins, destruction of normal trabeculae bone pattern -Codman's triangle= ossification of raised periosteum. (Can be seen with ewing sarcoma -Staging:MRI has taken over CT. But CT scan of chest and bone still essential to detect METS. Don't need BM biopsy -Tissue sample needed to confirm dx -Tx: Highly radioresistant. Chemo often admin prior to definitive surgery, and often done post surgery as well.

Still's Murmur

MC innocent murmur MC Heard between ages 2-7 -Early to mid-systolic musical, vibratory noisy twanging high pitched murmur loudest in the inferior aspect of the LLSB and apex. -May radiate to carotids -Thought to be d/t vibration of valve leaflets

G6PD deficiency

MC red cell enzyme defect causing hemolysis -X-linked recessive: esp Black men -Deficiency to this enzyme forces hgb to denature and form precipitants= Heinz Bodies--> cause RBC membrane damage--> hemolysis -Episodic hemolysis in response to triggers -Triggers: Illnesses, painkillers/antipyretics, meds (sulfa drugs, antimalarials, dapsone, isoniazid), fava beans, moth balls - Labs: Increased Retic, normal MCV -Smear: Bite Cells, blister cells, Heinz Bodies -Rapid flourescent spot test= diagnostic... decreased G6PD between hemolytic episodes -Tx: avoid known triggers as well as oxidant meds

Bacterial Conjunctivitis

MCC's= Staph aureus, strep pneumo, h flu —-Signs/sx—- -Unilateral, may become bilateral -Purulent discharge, lid crusting -Mattering thick in morning, consistent throughout day -Usually no visual changes -Only mild pain -Absence of ciliary injection -Photophobia NOT typical —-Tx—- -Warm compress -Topical abx: Gentamycin, moxifloxacin, polymixin-bacitracin, erythromycin -Fluroquinolones in contact wearers (consider pseudomonas)

Croup

MCC= Human Parainfluenza Virus -MC in children <5, but any age can happen ——Signs/Sx—— -Barking cough (seal like, harsh) -Stridor- both inspiratory and expiratory (worsened by crying or agitation) -Hoarseness -Dyspnea (worse at night) -+/- URI sx either preceding or concurrent fever -Gradual onset over 1-3 days —-Ddx—- -To differentiate from epiglottits: lack of drooling, presence of cough, acute epiglottitis more acute in presentation ——Dx—— -Clinical: once epiglottitis and foreign body aspiration excluded -Frontal Cervical radiograph= Steepling of subglottic trache (only seen in 50%) ——Tx—— -Mild (no stridor at rest and no resp distress): Cool humidified air mist, hydration. Dexamethasone provides significant relife as early as 6 hours after single dose. Supplemental O2 if sats <92%. Pt can be discharged home. -Moderate (Stridor at rest with mild-moderate retractions): Dexamethasone PO or IM + supportive care +/- nebulized Epi. Should be observed for 3-4 hrs and can be discharged at that time if improvement seen -Severe: (stridor at rest with marked retractions/ severe respiratory distress) Dexamethasone + Nebulized Racemic Epi (used first since it acts faster) and hospitalize pt.

Klinefleter's Sydnrome

Males with an extra X chromsome= 47,XXY (extra sex chromosome d/t failure of separation of sex chromosome or translocation)= males with *hypogaonadism and small testes.* ——Clinical manifestations—— -Normal apperance before puberty onset=> tall stature (thin and long limbed with Eunochid features). In adulthood, they become obese -+/-: scoliosis, ataxia, mild developmental delays, expressive language features, expressive language disorders -^ risk of testicular cancer -Hypogonadism: small testicles and infertility (azospermia), gynecomastia, scarce pubic hair. —-Dx—— -47, XXY Karyotype -Low serum T (testosterone may help with 2ry sex charactersitics).

3 screenings to check on when newborn leaves hospital

Metabolic screening, Congenital cyanotic heart disease screen, hearing

Strabismus

Misalignment of eyes. Stable ocular alignment not present until 2-3 mos of age -Esotropia= deviated inward (cross eyed) -Exotropia= divergent (deviated outward) —-Sx—- -Diplo;lia, scotomas, or amblyopia —Dx—- -Corneal light reflex -Cover uncover test -Convergence testing —-Tx—- -Referral inidicated if persists beyond 6 months of age -Surgery is mainstay of tx for congenital esotropia -Accomodative esotropia= glasses with or without bifocals, amblyopia tx, and in some cases, surgery -Exotropia: surgery, orthoptic exercises, patching, occasionally glasses

RH Alloimmunization quick note

Mom RH- Father RH+ 1st baby is RH+ Mom develops antibodies to RH+ Antibody screen (indirect Coombs test)/Rhogam At 28 wks/PP

Physiologic Jaundice

Normal newborn physiology, no other manifestations, appears after 24 hours -*Appears after 24 hrs; peaks at 3-5 days of age, with a total bilirubin of no more than 15 mg/dL* -Increases by <5 mg/dL per day -Resolves by 1 week in full term infant and by 2 weeks in preemie -usually d/t increased indirect (unconjugated) bilirubin (the immature liver of the newborn is unable to efficiency conjugate bilirubin d/t decreased UGT enzyme activity). -other causes: relatively high red cell mass, absence of intestinal flora, slow gut motility, increased enterohepatic circulation of bili in first days of life. ---Tx--- -Phototherapy if >15 mg/dL and not descending

Tourette Syndrome

Onset usually in childhood (2-5 yo); M>F, +/- associated with obsessions/compulsions ---Signs/Sx---- -Motor tics: of face, head, neck (blinking, shrugging, head thrusting, sniffling -Verbal or phonetic tics: examples: grunts, throat clearing, obsence words (coprolalia), repetitive phrases, repeating phrases of others (echolalia) -Self mutilating tics: hair pulling, nail biting, biting of lips, etc. ----Tx---- -Habit reversal therapy: 50% have sx resolution by age 18 -Dopamine blocking agnets: Ex haloperidol, risperidone, fluphenazine, pimozide, tetrabenazine -Alpha adrengerics: Clonidine, guanafacine -Clonazepam can be used as adjunct

Telogen effluvium

Premature shedding of hair in the resting phase -Reaction pattern to a stress 3-4 months prior ->100-150hairs per day shed, up to 50% total -resolves if inciting stress eliminated in 6-12 months -(+) hair pull test (>6 hairs lost) -Associated with iron deficiency —-Tx—- -Reassurance -Rogaine 5% foam BID -Biotin 5000 mcg daily -Ketoconazole 2% shampoo

Otitis media with effusion (OME) (MEE)

Presence of fluid in middle ear space WITHOUT signs or sx of acute inflammation -May be some discomfort, but acute pain is not characteristic -A retracted or neutral TM with decreased mobility seen on pneumatic otoscopy. -Can progress to AOM or (often) follows after a case of AOM. After AOM, fluid can remain in ear for several weeks, with 60-70% still having MEE 2 weeks after successful tx. -Impt to distinguish OME from AOM as the former does NOT benefit from abx —-Tx—- -Audiology eval should be performed after approximately 3 months of continuous bilateral effusion in kids<3 and those at risk of language delay. -Any hearing loss or speech delay should be referred to ENT -ABX, antihistamines, and steroids have not been useful -May place tympanostomy tubes if >3 months of persistence -Usually resolves on own within 6 weeks.

Chondroblastoma

Presenting complaint is pain around a jpoint -This neoplasm may produce a pathologic fx ——Radiographs—— -Lesion is radiolucent and usually located in epiphysis -With little to no reactive bone, calcification is unusual —-Tx—- -Surgical curretage and bone grafting —-Prognosis—— -Excellent; no known malignant transformation

Atopic Dermatitis

Pruritic, xerotic, exudative or lichenified eruption on face, neck, upper trunk, wrists, hands, and in antecutbital and popliteal folds —-Etiology/General info—— -Distinct presentations in ppl of different ages and races -Diagnostic criteria must include: pruritis, typical morphology and distribution (flexural lichenification, hand eczema, nipple eczema, eyelid eczema in adults), onset in childhoood, and chronicit -Also helpful are: 1) personal fam hx of atopy; 2) xerosisichtyosis, 3) facial pallor with infraorbital darkening; 4) elevated serum IgE; 5) repeated skin infections ——Signs/sx—— -Ill-defined, scaly red plauqes affecting face, neck, upper trunk -Infants tend to have cheeks and extensor surfaces involved, unlike older kids and adults -Flexor surfaces (esp of knee and elbow) often involved -Chronic cases: skin dry and lichenified -During acute flares: widespread redness with weeping, either diffusely or in discrete plaques, is common. -Nummular: Sharply defined discoid/coin shaped lesions especially on dorsum of hands, feet, and extensor surfaces ——Ddx—— -Since virtually all pts with this condition have skin disease before age 5, a new diagnosis in anyone over 30 should be made only after consulting with derm. ——Tx—- -Avoid triggers; try not to bathe more than once daily. Soap confined to armpits, groin, scalp, feet. Avoid washcloths and brushes. Pat skin dry (not rubbed) and immediately aftery drying; cover with thin film of emmolient and CS ——Local tx—— -CS should be applied sparingly to the dermatitis 1-2 times daily and rubbed in well. -It is vital that patients taper off CS and substitiute emolients as dermatitis clears to avoid CS SE's. -Tacrolimus ointment and pimecrolimus cream can be effective substitute (no skin atrophy unlike CS). However there is risk of T-Cell lymphoma, so avoid completely in high risk pts and use sparingly overall -Acute weeping lesions: Use water of aluminum subacetate solution. Colloidal oatmeal soaks. Lesions on extremities should be bandaged. Use high-potency CS after soaking, but spare face and body folds. Tacrolimus not tolerated at this stage. -Subacute or scaly lesions: mid-high potency CS ointment should be continued until elevated lesions are cleared and itching decreased. At that point pts should begin a 2-4 week taper from twice daily to daily dosing with topical CS to reliance on emollients; only occasional use of CS in inflamed areas. Can also switch to low potency CS or use tacrolimus/pimecrolimus if CS can't be stopped -Chronic dry lichenified lesions: Best treated with ultra high potency CS ointments -Maintenance tx: Moisturizers, and occasional topical anti-inflammatories (only on weekends or 3 times per week) -Systemic CS may be needed in severe exacerbations.

-Rose Spots -Typhoid salmonella -FQ or ceftriaxone

Pt has fever, ab pain, chills, bowel issues, +/- brady cardia and this (image): -What does the image show? What is it indicative of? What is tx?

Osteoid osteoma

Radiolucent lesion in cortex of proximal femur, nocturnal pain relieved by aspirin. -2nd decade M>F; Most commonly in proximal femur -Nidus (1-15.cm) that secretes prostaglandings (pain therefore relieved by ASA and NSAIs)) -Benign bone-forming lesions -Clasically produces night pain that can be relieved by NSAIDs -One PE: usually tenderness over the lesion; can cause refrred pain to knee if in upper femur. —-Radiography—— -Radiolucent nidus surrounded by dense osteosclerosis that may obscure the nidus -Central ossification may be present -Bone scan shows intense uptake in the lesion -CT scans are confirmatory and delineate the nidus well ——Tx—— -Surgical excision or radiofrequency ablation of the nidus is curative and may be done using CT imaging and a minimally invasive technique ——Prognosis—— -Excellent -No known cases of malignant transformation, although lesion has tendency to recur if incompletely excised

Febrile Seizures

Seizures that result from sudden high fevers, particularly in children. ---Criteria--- 1) age 3 months to 6 yrs (most occur between 6-18 mos) 2)Fever > 38.8C 3) Non-CNS infection ----Info---- ->90% are generalized -Acute respiratory illnesses MC associated with these; gastroenteritis and UTI less commonly -Roseola infantum rare but classic cause -Increased risk of epilepsy later in life ---Types---- 1) Simple: Meets all 3 criteria: -Duration <15 mins -Generalized tonic-clonic siezure -Occurs once in a 24 h period 2) Complex: These features are suggestive of (at least one): - Duration > 15 mins - More than one seizure in same day -Focal features ---Diagnostic Evaluation--- -Evaluate source of fever, must exclude CNS infection 1) Simple febrile seizure: -No blood studies, neuroimaging, or EEG for most -EEG not helpful -LP in infants <18 mos, if recovery is slow, or if no other cause for fever is found, or if close f/u not possible -If previous febrile seizure, do not r/o meningitis as a cause 2) Complex febrile seizure -CBC w/ culture -U/A with culture -LP: CSF with culture -EEG -MRI ----Tx----- -Tx underlying illness/cause. -Prophylactic anticonvulsant are NOT recommended after a febrile seizure - If the seizures are complicated or prolonged, or if medical reassurance fails to relieve family anxiety, anticonvulsant prophylaxis may be indicated and appropriate. *Only phenobaritol and valproic acid have demonstrated efficacy in preventing febrile seizures.* -Diazepam started at first onset of fever for duration of febrile illness may be effective but will sedate a child and possibly complicate evaluation of the source. -Measures to control fever such as sponging, tepid baths, antipyretics, and administration of abx for proven bacterial illness reasonable but unproven to prevent febrile seizures.

Precocious puberty

Sexual characteristics before 8 in girls, 9 in boys. (F MC than M) —Etiologies—— -Endogenous: gonads, adrenal cortex -Exogenous: estrogen containing creams, OCPs, anabolic steroids, excessive fat tissue —-Endogenous causes—— -Central precocity: often idiopathic,can be d/t CNS abnormalities. The younger the child with precocity, the ^ the chance of CNS abnormality -Peripheral (primary) precocity: Ovarian/testicular abnromalities, adrenal hyperplasia, others Hormonal workup: -In females: GnRH, FSH, LH, E and P -In males: GnRH, FSH, LH, T -Bone Age (xray of left wrist) -Endocrine referal

Breast milk jaundice

Starts around the first week peaks at the second week; persists 6-8 weeks Due to high levels of beta-glucuronidase activity that deconjugates intestinal bilirubin. Normal infant examination (except for the jaundice). Breast feeding adequately ----Book notes---- -Unconjugated hyperbilirubinemia lasting until 2-3 mos of age is common in breastfed infants -*Moderate unconjugated hyperbilirubinemia for 6-12 weeks in a thriving breast-fed infant without evidence of hemolysis, hypothyroidism, or other disease strongly suggests this diagnosis.

Staph Scalded Skin Syndrome

Sudden onset bright red, acutely painful skin, most obvious periorally, periorbitally, and in flexural areas of neck, axillae, popliteal, antecubital and groin. -Slightest pressure on skin results in severe pain and separation of epidermis -Typically begins with skin tenderness, erythema, and fever. This is followed a day or two later by flaccid blisters and sloughing off of the superficial layer of skin to reveal moist, red tissue underneath, giving the area a "scalded"-looking appearance. Mucous membranes are spared -Caused by circulating toxin (exfolatin) which binds to desmoglein-1 resulting in separation of cells in granular layer. -Causative staphylococci may be isolated NOT from skin, but rather from nasopharynx, an abscess, sinus, blood culture, joint fluid, or other focus of infection Acute onset of fever Generalized red rash followed by exudate Skin then develops wrinkles and sloughs off Caused by S. aureus Antibiotics IV ampicillin

RH incompatability

Suspect when mother is RH- and baby is Rh +

ABO incompatibility

Suspect when mother is blood type O and baby is A, B, or AB

Ebstein's anomaly

Tricuspid flaps fused to inside of right ventricle; creates constant opening between atrium & ventricle

Allergic Rhinitis

Type 1 hypersensitivity, IgE -Antigents: Fixed or cyclic ——Signs/Sx—— -Clear rhinorrhea, sneezing -Tearing eyes, irritation -Pruritis, nasal obstruction, head fullness -Temporary hyponosmia or anosmia -Sx tend to be worse in morning. —-PE—— -Mucosa of turbinates usually pale or violaceous d/t venous engorgement. This is in contrast to viral rhinitis which is typically erythematous -Polyps may also be seen -Cobblestone mucosa of conjunctiva. ——Dx—- -Can do skin testing (patch test) -In vitro testing such as RAST or ELISA can be used too —-Tx—— -Avoidance, specific desensitization -Intranasal CS=Mainstay of Tx (Beclomethazone) -Others= Antihistamines, nasal decongestants, antileukotrienes (montelukast), nasal sinus rinse, ipatropium spray

Lead Poisoning (plumbism)

Usually occurs insidiously in children <5. -Most likely sources= flaking lead paint, artists paints, fruit tree sprays, solder, brass alloys, home-glazed pottery, and fumes from burning battery. -Toxic effects likely to occur if >0.5 mg lead/day is absorbed. —-Pathophys—— -Causes cell death, shortening life span of RBC and inhibits multiple enzymes needed for heme synthesis= acquired sideroblastic anemia. ——Sx—— -Causes vague symptoms including weakness, iritability, weight loss, vomitting, personality changes, ataxia, constipation, HA, colicky ab pian -Late manifestatons= retarded development, convulsions, and coma associated with increased intracranial pressure (medical emergency) —-Labs—- -Venous blood levels used to assess exposure -CBC and ferritin: Microcytic anemia, ^Fe, Decreased TIBC, +/- ^ ferritin (looks like anemia of chronic disease except in anemia of chronic disease we see decreased FE) -Basophilic stippling and ringed sideroblasts in bone marrow -X ray= lead lines: linear hyperdensities at metaphyseal plate. Lead lines in gums in adults -CSF protein is elevated and WBC count usually <100 cells/mL; pressure may be elevated in pts with encephalopathy. Do LP cautiously to prevent herniation. —-Tx—- -Chelation therapy: Succimer (PO) approved for use in children and reported to be as efficacious as calcium edetate; Succimer should be initiated at blood lead levels >45 -Courses of dimercaprol and calcium edetate can be used but no longer preferred unless encephalopathy -Tx for blood lead levels b/t 20-45 in children not determined.

"Broad Spectrum" antiepileptics

Valproate, topiramate, lamotrigene, felbamate, onfi

What Vitmain do you need to take with isoniazid

Vit B 6 (pyridoxine)

Down Syndrome

a condition of intellectual disability and associated physical disorders caused by an extra copy of chromosome 21. (Trisomy 21) —-General Info——- -Occurs in 1:700 newborns -Cognitive disabilities in mild/moderater range are characteristic, as is generalized hypotonia -May have prolonged physiologic jaundice. -Leukemia is 12-20 times more common in these pts -Problems that may be seen in childhood: thyroid dysfxn, visual issues, hearing loss, OSA, celiac disease, atlanto-occipital instability, autism. ——-Clinical findings——- -Flattend occiput -Characteristic facies= upslanting palpebral fissures, epicathal folds, midface hypoplasia, small dysplastic pinnae; minor limb abnormalities -1/3-1/2 have congenital heart disease, most often endocardial cushion defects or other septal defects- *AV septal defects*, VSDs, ASD, Tet of Fallot, PDA -Esophageal and duodenal atresia seen in about 15% of cases. -*Brushfield spots*= white/gray/brown spot on iris -Single palmar *(Simian crease)* -Neonates: poor Moro reflex, dysplasia of pelvis, hypotonia. -Extremities: hyperflexibility of joints, short broad hands, increased space b/t 1st and 2nd toes (sandal gap)

Fragile X syndrome

a disorder produced by injury to a gene on the X chromosome, producing mild to moderate mental retardation CCG trinucleotide expansion —-Clinical Manifestations—— -Younger males: MVP, hyperextensivle joints, hypotonia, soft skin, flexible flat feet, macrocephaly -Older males: macrocephaly, long and narrow face, prominent forehead and chin, frontal bossing, large ears, macroorchidism (huge testes) -Behavioral: wide range of manifestations expressive language deficits> receptive. -Attention deficit, hand flapping, hand biting, averting one's gaze

Vestibular neuritis

continuous vertigo, no hearing loss Typically after viral URI Same tx as acute labrynthitis

cholesteatoma

cystlike mass composed of epithelial cells and cholesterol occurring in the middle ear; may be associated with chronic otitis media

Isotretinoin side effects

decreased HDL levels, hypertriglyceridemia, arthralgia, increased creatine kinase levels, musculoskeletal pain, highly teratogenic

Peritonsilar abscess

deep abscess adjacent to infected tonsils (collection of pus/swelling which is usually only visible if drained) —-Etiology——- -MCC's= S. Pyogenes, Strep aninosus, Staph aureus, polymicrobial (commonly) —-Clinical manifestations—— -Severe sore throat (usually unilateral) -Dysphagia/odynophagia -Muffled "hot potato" voice -Drooling -Pooling of secretions -Uvula deviation to contralateral side -Trismus -Tonsillitis -Anterior cervical lymphadenopathy ——Labs—- -Not necessary for dx -Obtain cultures before IV abx and culture of drainage form I and D —-Imaging—- -Not always necessary, but useful to define extent -CT soft tissue neck w/ contrast 1st line ——Tx—— -I and D (or aspiratiopn) + IV Abx -IV abx= Clindamycin or Unasyn (amp sulbactam) IV -Analgesic, glucocorticoids to decrease swelling and pain -Tonsillectomy indications: recurrent strep infections, recurrent peritonsillar infections, chronic tonsillitis

Transient tachypnea of the newborn

due to retained fluid in the lungs (*especially after C-section*), and can result in increased interstitial markings from the hilum on CXR, but usually *resolves within three days*. Seen less in vaginal deliveries as the narrrow birth canal squeezes the lungs and helps push the fluid into the lymphatic system.

Secondary Amenorrhea

no menses for 3 months after having normal menses prior to Pregnancy=MCC

Idiopathic short stature

non-GH-deficient short stature -2.25 SD

black dot sign

tinea capitis

viral vs. bacterial conjunctivitis

viral: watery discharge; bacterial: thicker, yellow d/c (treat viral with warm/cool compress)

Hearing loss

—-Conductive—- -Weber: lateralizes to affected ear -Rinne: BC>AC in affected ear -causes (not all inclusive): otitis media, cerumen impaction, mastoiditis, choleastoma, foriegn body ——Sensorineural—— Weber: Lateralizes to normal ear Rinne: AC>BC (normal) -Causes: acoustic neruoma, loud noise (>85 db), Presbycusis (MC), labrynthitis, meinere's diseas

Encorpresis

—-Definition—— -Repeated passage of stool in inappropriate places (such as underpants) by a child who is chronologically or developmentally older than 4 years old. Occurs once per month x 3 months ——Subtypes——- 1) Retentive encopresis 2) Continuous encopresis 3) Discontinuous Encopresis —-General Info—— ->90% of cases result from constipation; thus in eval of child with encopresis, one must r/o underlying pathology associated with constipation such as: hypothyroidism, neuro disorders like CP or tethered cord, anatomical abnormalities of anus, and stress/child abuse -1-3% of kids 4-11 have this. Highest prevalence between 5-6 -H&P should be done: do rectal eval and spine/neuro eval -Ab radiograph can be helpful in finding degree of constipation and check for signs of obstruction ——-Tx—— -Assuming no GI abnormalities, initial intervention starts with tx of constipation -When medical management indicated, PO meds or an enema for "bowel cleanout" followed by PO meds should be used; such tx can be monitored by ab radiographs to be sure colon is clean -A bowel regimen needs to be established in attempt to achieve continence and being on schedule. -Next, edu, support, and guidance around evacuation are essential -Behavioral strategies like having child sit on toilet after meals to stimulate gastrocolic reflex -It is most important to avoid punishing child and making them feel ashamed; helping child clean themself and clothing in a nonjudgmental, nonpunitive manner is far more productive approach than criticism and reproach -Child should be encouraged to have a daily BM, and the use of fiber, some laxatives, and even mineral oil can be helpful. -Consult with GI should be considered in more refractory cases.

Hypercalcemia

—-Essentials—— -Defined as a serum Ca >11 mg/dL; severe hypercalcemia defined as >13.5 mg/dL -Ab pain, polyuria, polydipsia, HTN, nephrocalcinosis, failure to thrive, renal stopnes, intractable peptic ulcer, constipation, uremia, pancreatitis -Bone pain or pathologic fractures, subperiosteal bone resorption, renal parenchymal calcification or stones, and osteitis fibrosa cystica —-General Considerations—— ->80% of hypercalcemic children or adolescents have either hyperparathyroidism or a malignant tumor. -Hyperparathyroidism is rare in childhood and may be 1ry or 2ry -MCC of hyperparathyroidism is parathyroid adenoma. -Familial hyperparathyroidism may be isolated disease or associated with MEN type 1 or 2A. -Hypercalcemia of malignancy associated with solid and hematologic malignancies and is d/t either local destruction of bone by tumor or ectopic PTH excretion -Chronic renal disease with impaired phosphate excretion is MC 2ry cause of hyperparathyroidism ——Signs/Sx—— -Hypotonicity, weakness, apathy, mood swings, bizzare behavior, N/V, ab pain, constipation, wt loss, hyperextensibility of joints, HTN -Shortened QT interval -Band keratopathy- Ca deposits in cornea or conjunctiva -Intractable peptic ulcer and pancreatitis occur -D.t increased calcium and phosphate excretion: loss of renal concentrating ability leads to polyuria, polydipsia, CaPO4 deposition in renal parenchyma or as urinary claculi with progressive renal damage -Osteitis fibrosa cystica, subperiosteal bone absorption in the distal clavicles and phalnges, absence of lamina dura around the teeth, spontaneous fractures, and moth-eaten appearnce of skull on radiographs -Can see decreased DTR's. Nephrogenic DI: polyuria, nocturia ——-Labs/Imaging—— -^ ionized Ca2+ (most accurate), ^ total Ca2+ -Check PTH-related protein, Vit D levels, 24 hr urinary calcium -Shortened QT interval on EKG, prolonged PR interval, QRS widening —Imaging: bone changes may be subtle in kids. Technetium sestamibi cintigraphy is preferred over conventional procudres (US, CT, MRI) for localizing parathyroid tumors. ——Tx: Symptomatic—— -Initial mangement= vigorous hydration with normal saline and forced calcium diuresis with a loop diruect (furosemide). -If response inadequate, CS or calcitonin can be used -Bisphosphonates, standard agents for tx of acute hypercalcemia in adults, are being used more often in refractory pediatric hypercalcemia ——Tx: Chronic—- -options vary with underlying cause -Resection of parathyroid adenoma or subtotal removal of hyperplastic parathyroid glands is preferred t. Postop, need diet high in calcium and Vit D -Tx of secondary hyperparathyroidism from chronic renal disease is primarily directed at controlling serum phsophorous levels with phosphate binders. -PHarmacologic doses of calcitrol to suppress PTH -Hypercalcemia of malignancy: tx underlying disorder

Orbital Cellulitis

—-Etiology—— -Children> Adults -Usually 2ry to sinus infection (Ethmoid in 90%) -Causative agents: S. Aureus, S. Pneumo, GABHS, H flu -Teeth, middle ear, face infection -Infected mucocele ——Manifestations—— -Decreased vision, pain with ocular movement, proptosis (bulging eye), eyelid edema and erythema. Some ophthalmoplegia may be seen too. —Dx—- -High res CT: infection of fat and ocular muscles -May do MRI too ——DDX—— -Preseptal cellulitis: infection of eyelid and periocular tissue. May have ocular pain and swelling but NO visual changes and NO pain with ocular movements. Usually no proptosis. —-Tx—- -IV abx: Vanco, Pip-Tazo, Clinda, Amp-sulbactam, etc. -Amoxicillin if preseptal -Surgery if abx unsuccessful or in severe cases

Herpes Simplex Virus

—-Type 1—- -Face and upper body -85% of adults have this -Skin to skin transmission -Triggers= Sun, stress, illness, etc. -sx: Grouped vesicles on an erythematous base—> pustules/vesicles—> ulcerations wtih crusts. Sting/burning may precede -TX= oral antivirals (ciclovir/-cylcovir) started within 72 hrs x 7-10 days. If very mild orolabial condition in immunocompetent ppl, does not need to be treated —-Type 2—- -Most cases below the waist -Classically can only get during sexual contact with someone else who has it -Pregnant women can pass to baby during childbirth- active lesions indication for c-section: consider prophylaxis at 36 weeks with acyclovir -Same triggers as above -Tx= Oral antivirals at 1st sign of sx (may prophylax if frequent/severe recurrences— use acyclovir) ——Dx—- -Clinical diagnosis -Can use PCR or Tzanck smear that shows multinucleated giant cells and intranuclear inclusion bodies

Niacin Deficiency (Vit B3)

—-Vit Info—— -Involved in synthesis and metabolism of carbs, fatty acids, proteins -Absorbed in stomach and SI -Dietary sources: Meat, poultry, fish, yeast, grains, legumes -Deficiency often d/t diets high in corn (lacks niacin and tryptophan) or diets which lack tryptophan -Deficiency also seen as complication in alcoholics, after bariatric surgery, anorexia, or other malabsorptive disease. -Can occur in carcinoid syndrome and prolong isoniazid use. —-Manifestations——- -Pellagra -*3 D's: Dermatitis, dementia, diarrhea; Hyper-pigmented rash typically in sun exposed area with gloved hand appearance*

Vitamin C deficiency (Scurvy)

—-Vitamin Info—— -Essential for electron transport rxns and redox rxns -Fatty acid transport, collagen syntehsis, neurotransmitter synthesis, prostaglandin metabolism -Absorbed in Small intestine -Dietary sources: Citrus fruits, tomatoes, strawberries, spinach, broccoli, cabbage -RF's for deficiency: Diets lacking raw citrus fruits and green veggies (excess heat denatures vitamin), smoking, ETOH, malnourished ppl, elderly ppl ——Signs/Sx—— -3 H's: 1) *Hyperkeratosis*: hyperkeratotic follicular papules (often surrounded by hemorrhage) 2) *Hemorrhage*: Vascular fragility (d/t abnormal collagen production) with *recurrent hemorrhages in gums, skin (perifollicular) and joints. Impaired wound healing* 3)*Hematologic*: Anemia, glossitis, malaise, weakness. Increased bleeding time. Petechia -Cardiorespiratory complications can occur —-Tx—— -Replace Vit C

Vit A deficiency

—-Vitamin facts—— -Plays a role in fetal eye development, phototransduction, and other eye functions -Dietary sources: leafy greens, sweet potatoes, carrots, liver, kidney, egg yolk, butter -Rarely seen in resource-rich countries -*Can occur w/ fat malabsorption: CF, celiac disease, Crohn, bariatric surgery, short bowel syndrome* ——Clinical Manifestations—— -Eye disease: Night blindness, xerophthalmia (dry conjunctiva, lack of tears), and complete blidness -Poor bone growth -Improvement of humoral and cell-mediated immunity -Bitot spot: buildup of keratin located superficially in the conjunctiva: appears as white spots ——-Dx—— -Primarily clinical -Serum retinol confirms —-Tx—— -Vit A supplementation

GH deficiency

—Characterized by decreased growth velocity and delayed skeletal maturity in the absence of other explanations. -GH resistance presents similar than GH deficiency but short stature is often severe, with little or no response to GH therapy. -Infants with GHD have normal birth weight with only slightly reduced length, suggesting that GH is a minor contributor to intrauterine growth. -GH deficient infants may present with hypoglycemia, particularly when paired with other pituitary deficiencies -Micropenis may be a feature of newborn males with gonadotropin and GH deficeicny. -Isolated GHD and hypopituarism may be unrecognized until late in infancy or childhood as growth retardation may be delayed untiler later childhood. -*Regardless of onset, the primary manifestation of idiopathic or acquired GHD is subnormal growth velocity. -Because GH promotes lipolysis, many GH deficient children have excess truncal adiposity* ——PPT notes——- Congenital "Idiopathic", isolated (most common diagnosis in childhood) Genetic defects Hypopituitarism/Panhypopituitarism with or without abnormal pituitary anatomy Intracranial malformations and midline defects e.g. Septo-optic dysplasia Cleft lip/palate Single central incisor syndrome Acquired Trauma (e.g. breech delivery, cerebral hypoperfusion, head injury) Tumor (especially craniopharyngioma) Radiation, surgery to sella Some chemotherapeutic agents Infection (e.g. encephalitis, meningitis) Inflammatory/infiltrative (e.g. hypophysitis, histiocytosis) ——Signs/Sx—— Infancy Hypoglycemia Micropenis Prolonged jaundice Childhood Growth deceleration after 2 years of age "Cherubic" facial appearance Delayed dentition Retained "baby fat" Central adiposity; "ripply" abdominal fat Acromicria (small hands and feet) Delayed gross motor development Teen years Delayed puberty Young appearance for age Central adiposity ——Labs—— -Since GH is so pulsatile, IGF-1 typically first step in evaluation of adequately nourished child. -IGFBP-3 much less sensitive marker of GHD, but may be useful in underweight child or child <4 -Provocation tests usually not very helpful -When tests are equivocal and clinical suspicion high, can trial GH tx —-Tx—- -GH replacment- SubQ 7 days per week with dose of 0.15-0.3 mg/kg. -With early dx and TX, children with GHD reach normal or near normal adult height.

Coarctation of the aorta

—Essentials—- -Congenital narrowing of descending thoracic aorta. -M>F 2:1 -Acyanotic -80-85% also have biscuspid aortic valve -Associated with Turner's Syndrome (45, XO) -*suspect in child with 2ry hypertension*, bilateral LE claudication -SBP in UE>LE's (gradient >15) -Blowing systolic murmur in the back or the left axilla ——Signs/Sx——- -Cardinal finding= decreased or absent femoral pulses -Infants with severe defect have equal upper and lower extremity pulses from birth until ductus arteriosus closes -Presents insidiously in the 60% of children with no sx from infancy -Usually dxed by a pulse and BP (>15 mmHg) discrepancy between the arms and legs on PE -Left subclavian artery occasionally involved= left brachial pulse weak -Pathognomonic murmur heard in left axilla and left back. usually systolic but may spill in diasole as forward flow continues across narrowing. ——-Imaging—— -CXR= rib notching: increased collateral circulation via intercostal arteries. 3 sign= narrowed aorta looks like the notch of the number 3 -EKG: LVH -Echo w/ doppler used to visualize directly -Angiogram= gold standard but rarely used. May be done if transcatheter intervention is planned ——Tx—— -If LV dysfunction and low CO: PGE2 infusion to reopen ductus arteriosus; may need inotropic support -Once stablazied: corrective repair via surgery -In pts with poor LV fxn: balloon angioplasty sometimes performed as palliative measure -Main complication of both surgery and balloon angioplasty= recurrent coarctation. Fortunately this is treatable in the cath lab -In older pts, particuarly those of adult size, transcatheter stent placement is effective for recurrent coarctation.

Ventricular Septal Defect

—— Essentials——- -*Holosystolic murmur at LLSB with RV heave* -*MC congenital heart malformation, accounting for about 30% of all congenital heart disease* -Defects in membranous portion more common than muscular - Clinical features: Failure to thrive, tachypnea, diaphoresis with feeds -Left to right shunt with normal pulmonary vascular resistance -Non restrictive= non pressure difference b/t ventricles -Restrictive= normal pressures b/t ventricles maintained. -Large defects may cause Eisenmenger syndrome if not repaired early ——-Presentations——- 1)Small, hemodynamically insignficant (80-85%): <3mm in diamter. Many will close spontaneously.Never requires surgical closure. 50% close by age 2, and 90% by 6 yo. Muscular defects close sooner than membranous 2) Moderate sized (3-5%): 3-5mm in diameter. In general, do not have clear indicators for surgical closures. If asymptomatic w/o evidence of pulmonary HTN, serial monitoring as some close sponatenously over time 3) Large with normal pulmonary vascular resistance: 6-10 mm in diameter. Unless they become markedly smaller within a few months after birth, they often require surgery. Many develop sx by age 3-6 mos, and require correction at that time. Surgery before age 2 yrs essentially eliminates risk of pulmonary vascular disease 4) Large with pulmonary vascular obstructive disease: direction of flow determined by resistance in systemic and pulmonary vasculature, explaining why flow is usually left to right. In large ones, ventricular pressures are equalized, resulting in increased pulmonary artery pressure. In addition, shear stress caused by increased volume in pulm circuit causes increased resistance over time. *Almost all cases of irreversible pulm HTN can be prevented by surgical repair of a large VSD before age 2* ——Sx/Signs—— -Small or moderate Left to right shunts: usually no CV sx -Pts with large left to right shunts usually ill early in infancy. Usually have frequent resp infections and gain weight slowly. Dyspnea, diaphoresis, and fatigue are common -Acyanotic unless Eisenmenger's develops. ——Imaging——- -CXR normal in small shunts. Larger shunts have significant cardiac enlargement involving LV, RV, LA. Main pulm artery segment may be dilated and vascular markings increased. -EKG: Normal in small -LVH in pts with large left to right shunts -Echo w/ doppler: can identify defect. A pressure difference >50 mm Hg in LV compared to RV confirms the absence of severe pulm HTN -Cath: indicated in those pts with increased pulm vasc resistance ——-Tx—— -Pts who develop sx can be managed with HF type treatment. Particularly diuretics and afterload reduction. Can do prior to surgery or if it's expected the defect will close over time -Surgical tx: pts with cardiomegaly, poor growth, poor exercise tolerance, or other clinical abnormalities who have a significant shunt typically undergo repair at 3-6 months= patch closure -Transcatheter closure of muscular dects possible, not as much with perimembranous.

Jaundice

——-Essentials——- -Never normal in the first 24 hours of life, suspect congenital disorder or hemolytic disorder if this is the case -65% of newborns develop visible jaundice with total serum bili >6 during 1st week of life. -Extremely high levels can cause kernicterus, characterized by injury to basal ganglia nad brainstem. -Excess accumulation of bili in blood depends on both the rate of bili production and rate of excretion —-About Bilirubin——- -Produced by breakdown of heme—- unconjugated bilirubin——binds to albumin—-> liver—-> in prezense of enzyme UDPGT—-> Conjugated bili—-> intestine—->gut flora turns conjugated bili to urobilinogen—> stool (or 20% reabsorbed and then excreted to feces) -Asians more likely to have peak neonatal TSB greater than 12. This can be due to racial variations of UDPGT gene polymorphisms and G6PD deficiency -Preemies more likely to have increased TSB because of delayed stooling, poor enteral intake, and increased enterohepatic circulation ——Causes——- 1) Increased production -ABO/Rh incompatibility -RBC defects -Sepsis -Hemolysis 2) Decreased Clearance -Crigler-Najjar -Gilbert Syndrome 3) Increased Enterohepatic Circulation -Breastmilk jaundice -Breastfeeding failure Jaundice -----Types---- 1) Physiologic -usually d/t increased indirect (unconjugated) bilirubin (the immature liver of the newborn is unable to efficiency conjugate bilirubin d/t decreasd UGT enzyme activity) -Indirect bili rises in days 3-5 and falls in about half of the neonates during the 1st week of life. 2) Pathologic -May be suggestive if jaundice occurs in 1st 24hrs of life (usually indicates hereditary spherocytosis) -*Bili >20can lead to kernicterus and neurotoxicity* -Increase indirect causes: MCC are physiologic (after 24 hrs and peaks 3-5 days), prematurity and breast feeding jaundice (2nd-3rd day of life). Others: Crigler-Najjar, Gilbers, Cretinism, hemolytic anemia (1st 24 hrs of life for hemolytic anemia) -Increased direct causes: Dubin-Johnson syndrome, rotor syndrome, infections ----Dx---- -bhutani nomogram -Hyperbilirubinemia (>95% on nomogram) -At a minimum, eval should consist of following: 1) feeding and elimination history 2) birth weight and % change in weight since birth 3) Examination for sources of excessive heme breakdown 4) Assessment of blood type, Coombs testing, CBC with smear, serum albumin, and TSB 5) G6PD test if jaundice otherwise unexplained, and in Black infants with severe jaundice 6)Fractionated bili levels in infants who appear ill, those with prolonged jaundice, acholic stool, hepatosplenomegaly, or dark urine. ---Tx---- -Will see more on other cards -Phototherapy used in all types (started typically at levels ~6 mg/dL). Used in physiologic jaundice or jaundice associated with breastfeeding, when bili >15, or if the levels fail to decrease -Exchange transfusion used in severe cases, ABO incompatibility, RH isoimmunization, and hemolysis

Dehydration

——-Essentials——- -One of the MC encountered problems in clinical peds; children have high incidence of GI diseases like gastroenteritis. Their high ratio of surface area to weight promotes significant evaporative losses -Renal concentrating mechanisms do not maximally conserve water in early life, and fever may ^ fluid needs -Clinical eval should focus on composition and vol of fluid intake, frequency and amount of V/D and UO, meds, recent weight -Estimating degree of dehydration includes: cap refill time, postural BP and HR changes, dryness of lips/mucous membranes, JVP during supine, sunken fontanelles, AMS. Kids to to respond to the decreased volume by increasing pulse rate -A low BP or falling BP in kids is a late sign of shock -Labs: high urine specific gravity, ^BUN (burned BUNs), Low uNA, ^ HCT or albumin 2ry to hemoconcentration ——-Classifications——- 1) Mild: 3-5% decrease in body weight -Skin turgor and color often normal. Mucous membranes dry -Pulse normal, cap refill 2-3 secs, BP and perfusion normal -Fluid loss: mild oliguria. Decreased tears. -Specific gravity >1.020; uNa <20 2) Moderate: 6-10% decrease in body weight -Skin turgor decreased, skin color pale, mucous membranes dry -Slight increase in pulse, cap refill 3-4 secs, normal BP/perfusion -Oliguria -Specific gravity >1.020; uNa <20 3) Severe: 11-15% decrease in body weight -Skin turgor and color markedly decreased. Mucous membranes mottled or gray and parched -Tachycardic, cap refill >4 secs, low BP, circulatory collapse -Anuria, absent tears ——-Tx——- -Emergent IV therapy indicated when there is evidence of compromised perfusion (inadequate cap refill, tachycardia, poor color, oliguria, hypotensive). THe goal is to rapidly ^ plasma vol and prevent circulatory collapse. 20mL/kg isotonic fluid often use. -Colloid useful in hypernatremic pts in shock, malnourished infants, neonates -If no response to 1st bolus, 2nd can be given -If adequate perfusion not restored after 40ml/kg istonic fluids, other pathologic mechanims must be suspected (sepsis, cardiogenic shock, etc) -Isotonic dehydration can betreated by providing half the remaining fluid deficit over 8 hrs and the 2nd half over ensuing 16 hrs via D5W with 1/2NS with 20 mEq of KCL. -PO rehydration can be given to children with mild-moderate dehydration. Contraindicated in children with altered levels of consciousness or respiratory distress or can't drink freely

Atrial septal defect (ASD)

——-Types—— 1) Ostium Secundum (MC) 2) Ostium Primum 3) Sinus Venosus defect ——Signs/Sx—— -Most infants/children have no CV sx -Usually not symptomatic until >30 -Older children and adults can present with exercise intolerance, easy fatigueability, or in rare cases- heart failure -Typically acyanotic (left to right shunt) -Cyanosis rarely occurs unless pulm HTN occurs (Eisenmenger's) -Peripheral pluses normal and equal -S2 widely split and often fixed (doesn't vary with respirations) -May develop stroke d/t paradoxic emboli -A grade I-III systolic ejection crescendo-decrescendomurmur heard best at Left sternal border in 2nd intercostal space. -A middiastolic murmur is often heard in 4th ICS at Left sternal border. The presence of this murmur suggests high flow with a pulmonary-to-systemic blood flow ratio greater than 2:1 ———Imaging——- -CXR: cardiomegaly, +/- dilated pulm arteries -EKG: RAD; *Crochetage sign: notching of the peak of the R wave in inferior leads* -Echo: Dilated right atrium and RV, left to right shunt on color doppler confirms dx and elmiinates need for cardiac cath prior to procedure -Cardiac cath rarely needed for dx purposes, but can use transcath closure of ostium secundum of ASD ——-Tx——- -Surgical or catheterization closure is generally recommended for symptomatic children with large atrial defect and associated right heart dilation -Asymptomatic child with a large hemodynamically significant defect, closure performed electively at ages 1-3 yo -Most defects are amenable to non-operative device closure during cardiac cath, but the defect must have adequate tissue rims on all sides on which to anchor the device.

Otitis Externa

——Causes—— -Cellulitis of soft tissues of external auditory cannal -Excess H20 or local trauma changes the normal acidic pH of the ear, causing bacterial overgrowth -Keeping ear to clean can also cause this -*MCC's = Pseudomonas auerginosa (MC) and Staph Aureus* ——Signs/Sx—— -Pain, aural fullness, decreased hearing, sometimes itching in the ear -Manipulation of the pinna or tragus causes considerable pain -Ear canal typically swollen and narrow -Debris present in canal and usually very difficult to visualize TM due to edema ——Dx—— -Clinical ——Tx—— -Pain control -Remove debris from canal -Topical antimicrobial therapy= Floroquinolone ear drops= 1st line -Oral abx indicated for any signs of invasive infection such as fever, cellulitis of the face or auricle, or tender periauricular or cervical lymphadenopathy. In such cases, culture -If malignant OE (2ry to Pseudomnas MC, MC seen in DM and immuncompromised): Tx= IV antipseudomnal abs's. Ex: Ceftazidime or Piperacillin + Fluoroquinolones or Aminoglycosides ——Complications——- -If untreated, facial celluitis may result -Immuncomprmoised pts can develop malignant OE, which is a spread of the infection to the skull base with resultant osteomyelitis. This is a life threatening condition

Acute Pharyngitis

——Causes—— -Viral= MC overall cause -Bacterial= GABHS is MCC ——-Signs/Sx—— -Sore throat, pain or swallowing with phonation -Others:HA, congestion, cough, hoarseness, sinus pain, ear pain -Bacterial Sx (Sx seen more commonly in bacterial infection than viral): Fever >38C, Tender anterior cervical lymphadenopathy, lack of cough, and tonsillar exudate (Centor Criteria) —-Dx—— -Centor Criteria (listed above): if 0-1= no abx or throat culture needed. If 2-3 get throat culture (or rapid strep) If 4 give ABX -Rapid antigen detection test: 95% specific but only 55-90% sensitive (most useful if positive, *if negative- throat cultures need to be obtained* -Throat cultures= gold standard ——Tx—— -Normal course of illness is 3-5 days. The course is shortened by 48 hrs with treatement, therefore treatment is given mostly to prevent complications (namely rheumatic fever) -Abx= Pencillin G or VK 1st line (can also do amoxicillin or augmentin) -2nd line= Macrolides if PCN allergic ——Complications—— -Rheumatic Fever (preventable with abx) -Glomerulonephritis (not preventable with ABX) -Peritonsillar abscess, cellulitis

PDA

——Essentials—— -*Continuous machinery type murmur* -Bounding peripheral pulses if large defect -Clinical features of large defect= failure to thrive, tachypnea, diaphoresis with feeds -Left to right shunt with normal pulmonary vascular resistance ——General Info—— -Persistence of normal fetal vessel joining pulmonary artery to the aorta -Closes spontaneously in normal term infants at 1-5 days of age -Accounts for 10% of all congenital heart disease -More common in preemies <1500 grams -Patency necessary in some pts with complex forms of congenital heart disease. Prostaglandin E2 (PGE2) is a product of arachidonic acid metabolism and continuous IV infusion maintains patency ——Signs/Sx—— -Findings and course depends on size of shunt and degree of pulm HTN -Mod to large: bounding pulses, widened pulse pressure d/t runoff through ductus -S1 normal and S2 usually narrowly split. In large shunts S2 may have a paradoxical split. Paradoxical splitting caused by vol overload of LV and prolonged ejection of blood from this chamber -*Rough machinery murmur maximal at 2nd left ICS*. Begins shortly after S1, rises to a peak at S2, and passes through the S2 into diastole, where it becomes a decrescendo murmur and fades before S1. -Murmur tends to radiate well to the anterior lung fields but relatively poor to the posterior lung fields. -A diastolic flow murmur is often heard at apex. ——Imaging—— -CXR: if shunt small, heart not enlarged. If large,both LA and LV enlargement may be seen. Aorta and main pulm artery segment may be prominent -EKG: normal or LVH if shunt. In pulm vascular obstructive disease, pure RVH occurs -Echo: provides direct visualization and confirms direction and egree of shunting -Cath: not used for dx, but is routinely used for closure in all but the smallest of neonates and infants. ——Tx—— -IV indomethacin 1st line tx; surgical correction if not successful -Surgical closure indicated when PDA is large and pt is small. -Pts with large left to right shunts require repair by age 1 to prevent development of pulmonary vascular obstructive disease -May need to keep patent if concurrent congenital heart disease going on .

Hypothyroidism (Congenital and Acquired)

——Essentials—— -Growth retardation, decreased PA, wt gain, constipation, dry skin, cold intolerance, delayed puberty -Neonates with congenital: thick tongue, large fontanels, poor muscle tone, hoarseness, umbilical hernia, jaundice, intellectual retardation -T4, FT4, and T3 resin uptake are low; TSH levels are elevated in primary cases ——General Considerations—- -Congenital cases occur in 1:3000-1:4000 infants; untreated it casuses severe neurocognitive development -Cases are sporadic result from hypo/aplasia of thyroid gland or failure of gland to migrate back to its anatomical location -Dyshormogenesis d/t enzyme defects can be another cause -Another cause: Antithyroid drugs taken by mother crossing placenta -decreased TSH secretion d/t prolonged steroid use, dopamine, or somatostatin. -Juvenile cases often due to Hashimoto's thyoridits ——Signs/Sx—- -Most appear normal at birth and gain weight for first few months of life w/o tx. -Jaundice associated with unconjugated hyperbilirubinemia may be seen in newborns with congenital hypothyroidism -THick tongue, hypotonia, large fontanelles, constipation, umbilical hernia, hoarse cry, dry skin -Juvenile hypothyroidism: short stature, abnormal weight gain. Other findings= delayed epiphyseal dvelopment, delayed closure of fontanelles, retarded dental erruptiopn. Skin may be dry, thick, scaly, coarse, mottle. Hair may be coarse or birittle. Lateral thinning of eyebrows. MSK= hypotonia, slow DTRS (esp in ankles). -Other juvenile SX= sluggishness, nonpitting mxyedema, constipation, large tongue, hypotehrmia, bradycardia, hoarse voice/cry, umbilical hernia, transient deafness, pubertal delay, metromenorrhagia in older girls. Pseudopuberty and galactorrhea can be seen. -PE: thyroid usually symmetrical, moderately firm, not nodular. In Hashimotos, however, it frequently has a cobblestone surface. ——Dx—— -*Since this condition should be tx'ed as early as possible, you should treat based on newborn screening test and NOT based on signs/sx* -T4, FT4, and T3 resin uptake are low; TSH levels are elevated in primary cases.; in central cases TSH may be low or inappropriately normal -Circulating autoantibodies to thyroid peroxidase and thyrogloblulin may be present -May see ^ PRL and decreased GH ——Imaging—— -While helpful in establishing cause of congenital hypothyroidism, it does NOT affect tx plan and is not necessary -May see porous bone on plain films; cardiomegaly common ——Screening——- -All newborns screened shortly after birth as part of metabolic panel, typically get both total T4 and TSH level; if abnormal tx ASAP -Initiation of tx in the 1st mo of life and good compliance usually results in normal neurocognitive outcome. —-Tx—— -Levothyroxine

Type 1 Diabetes

——Essentials—— -MC type of diabetes in ppl <20, but can develop at any age. -Classical presentation= polydipsia, polyuria, and weight loss; however the patinet may be overweight or obese. -C peptide levels are low -Autoimmune destruction of pancreatic islet B cells. This destruction occurs over months-yrs and sx dont appear until most islet have been destroyed. ——Prevention—— -Free antibody screening is available for familes having a relative with T1DM. Immunomodulation may help slow down the damage. ——-Signs/sx—— -polyuria, polydispsia, weight loss (often missed) -DKA (ab pain, N/V that can mimic acute abdomen; mild-mod dehydration; kussmaul respiration, somnolent, obtunded) -BG >200 in a child always abnormal -Stress induced/steroid-induced hyperglycemia can occur with illness. In a well-Child, the dx must NOT be based on a single plasma glucose test or borderline result using a glucometer; If HbA1C is normal, home monitor glucose for several days ——Dx—— -Random blood glucose >200 + symptoms -Fasting plasma glucose >126 or plasma glucose >200 2 hrs after oral glucose load confirms dx -HbA1C >6.5% is diagnostic -Impaired fasting glucose= 100-125 -Impaired 2 hour OGTT= 140-200 -HbA1c 5.7-6.4% ——Tx—— -Insulin therapy, exercise, psychosocial support -Goal A1C= <7.5 or the lowest that can be sustained w/o severe hypoglycemia or moderate hypoglycemia -Children presenting in DKA (pH<7.30 or bicarb <15 meq/L) require IV insulin in addition to ffluid replacement. Giving IV insulin bolus before completion of initial fluid bolus has been associated with brain edema. -In children without DKA and have adequate PO intake, initial insulin dose can be subQ 0.2U/Kg short acting regular insulin, or preferentially rapid acting analgo: lispro, aspart, glulisine. At the same time 0.2-0.3U/Kg long acting insulin can be used. -Approx 3-6 weeks after dx, most school children and adolsecents experience honeymoon period where temporary decrease is needed. -Basal/Bolus regimen typically used. -Pumps best way to restore body's physiologic insulin profile, but compliance is bad. —-Preventative Care—— -Yearly eye exams, beginning 3-5 years after diagnosis -Check thyroid levels

Colic

——Essentials—— -Typical features: An otherwise healthy infant aged 2-3 mos seems to be in pain, cries for >3 hours a day, for more than 3 days a week, for more than 3 weeks (*rule of threes*) -Typically subsides or decreases in the 3rd and 4th month of life -Before dx can be made, must rule out disease that may cause crying.j -Treatment is centered around behavioral management. No meds have really been found helpful unless GERD/gas distension is thought to cause this. ——Information—- -Characterized by severe and paroxysmal crying that occurs mainly in late afternoon -Infants knees drawn up, fists clenched, flatus expelled. -Facies typically pained, minimal response to soothing attempts -Studies in US shows that among middle class infants, crying occupies 2 hours per day at 2 weeks of age, 3 hours per day by 6 weeks, and gradually decreases to about 1 hr per day by 3 months -No basis of why the infant cries -Is a behavioral sign/sx that begins in 1st few weeks of life and peaks around ages 2-3 mos. In about 30-40% of cases, it continues into 4th and 5th months. -Little firm evidence of association to allergic disorders other than some infants who respond to elimination from its own diet or mothers diet -GER may be a cause; also rule out undetected corneal abrasion, UTI, child abuse. -Some attempts have been made to elimnate gas with simethicone and slow guy motility with dicoclimine ——-Management——- 1) Parents may need to be educated about developmental characteristic of crying behavior and made aware that crying increases normally into 2nd month and abates by 3rd-4th mo 2) Parents may need reassurance that the infant is not sick (after doing History and physical). Despite being stressful, this is a normal variant and usually self limited. Can help relieve parental stress by keeping a log of crying and weight gain. If weight gain is adequate, less likely to be disease process 3) Find cues and how to effectively soothe/comfort the infant. Keep quiet environment, gentle swinging/rocking, soothing music, drives in car, or walks in stroller. Another approach is changing feeding habits so infant not rushed, has ample time to burp, and may need to be fed more frequently to reduce gastric distension 4) Meds such as phenobarbital elixir and dicyloclimine have been found somewhat helpful, but use discouraged due to SE's. A trial of PPI's or ranitidine HCL may help if GERD is concerned 5) If refractory to behavioral tx, a trial of changing feeds and eliminating cow's milk from formula or mothers diet may be needed. The use of why hydrolysate formulas for formula-fed infants has been suggested.

Mastoiditis

——Etiologies—— -Usually complication of proonged or inadequately treated otitis media. All pts with AOM have some degree of mastoiditis bc the mastoid and middle ear are connected ——Signs/Sx—— -Postauricular pain, fever, outwardly displaced pinna -Mastoi area indurated and red -Earliest finding= severe mastoid tenderness to palpation -Late findings= pinna that's pushed forward by postauricular swelling and an ear cannal that is narrowed. -In infants <1 yr, the swelling occurs superior to ear and pushes pinna downward rather than outward —-Dx—— -CT scan reveals coalescence of mastoid air cells d/t destruction of bony septa -Myringotomy for culture and drainage -Can just do tympanocentesis for culture before doing myringotomy —-tx—- -IV abx + middle ear drainage/mastoid drainage (via myringotomy) with our without tympanostomyk -ABX choice= Cefazolin for IV cases. Concurrently use abx drops as well. Progress to PO. Change abx based on culture and sensitivity results. -Refractory or complicated cases= mastoidectomy

Allergic Conjunctivitis

——Manifestations—— -BILATERAL -Redness, tearing, and itchiness is universal -Conjunctival hyperemia and edema -Cobblestone Mucosa -Clear, mucoserous, sometimes stringy discharge —-Tx—— Stepwise -Cool compress artifical tears (supportice) -Topical antihistamines (Olopatadine, Emedastine)= 1st line in mild to moderate -Topical decongestants -Mast cell stablizers -Topical CS= 1st lin in severe (beware of long term steroid use SE's like glaucoma, catarcts, HSV keratitis)

Acne Vulgaris

——Pathophys—— 4 main pathophysiologic features: 1) Increased sebum production (puberty) 2) Clogged sebaceous glands 3) P. Acne overgrowth (lipase production by p. Acne converts sebum into inflammatory fatty acids that damage healthy cells that leads to inflammatory response) 4)Inflammatory response —-Features—- -Comodones= smal noninflammatory bumps. Open= blackheads (incomplete blockage, is black due to oxidized sebacoues content). Closed= whiteheads (complete blockage, follicular opening is unapparent, keratin accumulates) -Papules or pustules -Cysts or nodules —-Dx—— -Mild= comedones (+/- small amounts of papules and/or pustules) -Moderate= comedones, larger amounts of papules and pustules -Severe= nodular (>5mm) or cystic acne —-Tx—- -Comedone acne: Topical retinoids (unclog pores, reduce inflammatory response) (tretinoin cream). Can also do benzoyl peroxide (sebostatic, comedolytic, inhibits growth of p. acnes) in combo with adaplene(another kind of topical retinoid used in comedonal acne) and topical abx like erythromycin or clindamycin ——Papular or cystic acne (any abx listed below is PO)—— 1)Mild= combo of erythromycin or clinda with benzoyl peroxide gel or wash 2) Moderate= Doxy or minocycline or bactrim or keflex PLUS benzoyl peroxide 3) Severe= Isotretinoin 0.5-1 mg/kg/day for 20 weeks

Vit D deficiency (Rickets)

——Vitamin Info——- -UV light= major natural source -Actions= calcium homeostasis, bone development -Dietary sources: fatty fish, some dairy products, OJ, soy milk, cereal -RDA: Age 1-70; pregnant/lactating: 600 IU, 71+: 800 IU ——Info on deficiency—— -Caused by 1 of 4 mechanisms: 1) Impaired availability of Vit D 2) Impaired hepatic conversion to 25-hydroxyvitamin D 3) Impaired Renal conversion to 1, 25 dihydroxyvitamin D 4) End organ insensitivity to Vit D (hereditary rickets) -Oral glucocorticoids (when used chronically in high doses) inhibit intestinal Vit D dependent calcium absorption ——Clinical manifestations: -Hypophosphatemia and hypocalcemia (hypoPO4 and hypoCa2+) -2ry hyperparathyroidism -Softening of bones leading to bowing deformities (bowed legs), fractures, costochondral thickening (rachitic rosary), dental problems, muscle weakness, developmental delays -Pain in spine, pelvis, legs —-Dx—- -Low serum 25, hdyroxyl vit D (low Vit D levels) —-Tx— -Ergocalciferol (vit D replacement)