UWORLD 4

X linked recessive: Affected father: all daughters are carriers. All sons are fine. Carrier mother: 50% of daughters will be carrier. 50% of sons will have disease. X linked dominant: Affected father: all daughters are affected. All sons are fine. Affected mother: 50% of daughters will have disease. 50% of sons will have disease.

1727 X linked recessive Vs. X linked dominant?

Henoch-Schonlein purpura (HSP) -2-10 y.o -often follows a few weeks after viral or step upper respiratory airway infections. -IgA containing immune complex deposits causing inflammation 1. GIT: can occur in upper and lower GI. Hematemesis and bloody diarrhea. Bowel wall edema. Complications: intussusception 2. Kidneys: Berger disease aka IgA nephropathy mesangial proliferation and crescent formation. 3. Skin: palpable purpura on buttocks and lower extremities. Begin as either urticarial papule or plaques and then into purpura. 4. Joints: self limited migratory arthralgia and arthritis c/d: swollen lymph nodes and red eyes = Kawasaki. Other symptoms high fevers, strawberry tongue, perioral erythema and fissuring, and periungual desquamation.

1850 A 7 y.o boy has 2 day history of colicky abdominal pain and now developing bloody stools. His urine is red. PE shows palpable skin lesion on his buttocks. Which of the following additional findings will you see? a. impetigo b. joint pain c. swollen lymph nodes d. red eyes e. oral ulcers

Stone bodies are generated in the liver from fatty acids and yield energy when converted to acetyl CoA n the mitochondria of target cells. Erythrocytes cannot use ketone bodies for energy because they lack mitochondria. Liver cannot use ketone bodies for energy because it lacks the enzyme succinyl CoA-acetoacetate CoA transferase (thiophorase), which is required to convert acetoacetate to acetoacetyl CoA.

1887 Which of the following tissues cannot use ketone as energy source? a. brain b. erythrocytes c. hear muscle d. renal cortex e. skeletal muscle

Tetralogy of Fallot: pulmonary stenosis, overriding aorta, VSD and right ventricular hypertrophy. Pulmonary stenosis and overriding aorta = low SVR:PVR allowing deoxygenated right ventricular output to take the low resistance route to the systemic circulation = acute hypoxemia ("Tet spell"). To relieve this, patient squat to increase SVR:PVR ratio that forces a higher proportion of the right ventricular output to enter the pulmonary circulation and oxygenate in the pulmonary capillary beds, increasing arterial oxygen concentration and relieving the Tet spell. a/d: squatting restricts chest wall expansion and slight increases work of breathing. Lung compliance doesn't change in this position. b/c: squatting doesn't significantly impact PVR as the compressed vessels are primarily in the abdomen and lower extremities. Pulmonary blood flow increases, not decreases, during the decreased right to left shunt with increased SVR.

205 An adopted kid from asia has chronic fatigue, shortness of breath and failure to gain weight. PE reveals a loud systolic murmur. ECG shows pulmonary stenosis and sub aortic ventricular septal defect with deviation of the origin of the aorta to the right. Occasional episodes of severe dyspnea and cyanosis were relieved by child quickly assuming a squatting position. Which of the following mechanisms during squatting relieves this child's symptoms? a. decreasing left ventricular workload b. decreasing pulmonary blood flow c. decreasing pulmonary vascular resistance d. improving lung compliance e. increasing systemic vascular resistance.

Pure motor hemiparesis and small cavitary lesion in the internal capsule likely has a lacunar infarct. Lacunar infarcts are form of ischemic stroke involving the small penetrating vessels that supply the deep brain structures (eg, basal ganglia, pons) and subcortical white matter (eg, internal capsule, corona radiate). They are primary caused by chronic hypertension, which predisposes arterioles to undergo lipohyalinosis, microatheroma formation, and occlusion (hypertensive arteriolar sclerosis). Diabetes and smoking are additional risk factors. Depending on the portion of the brain that is affected, characteristic clinical syndromes develop, such as the following: -Pure motor hemiparesis = posterior limb of the internal capsule or basal pons -Pure sensory stroke = VPL or VPM thalamus -Ataxia-hemiplegia syndrome = posterior limb of the internal capsule or basal pons -Dysarthria-clumsy hand syndrome = gene of the internal capsule or basal pons. In the acute setting, CT imaging may not show the expected hypo density of ischemic stroke d/t the small infarct size. After several weeks, these necrotic lesions turn into cavitary spaces filled with cerebrospinal fluid and surrounded by scar tissue called lacunas. a. brain arteriovenous malformations typically present with intracranial hemorrhage in children. b/c: cardiac embolism nd carotid atherosclerosis usually result in occlusion in medium to large size cerebral vessels (eg, middle cerebral artery), causing large territory ischemic infarctions of the cerebral cortex. d. cerebral amyloid antipathy is the most common cause of spontaneous lobar/corticla hemorrhage (eg, occipital, parietal) in the elderly. e. charcot bouchard aneurysm rupture is typically caused y chronic hypertension and result in in hemorrhagic stroke involving deep brain structures (eg, basal ganglia, cerebellar nuclei, thalamus, pons). Hemorrhage would appear as a focus intraparencyhmal hyper density on initial CT scan g. hypertensive encephalopathy is characterized by progressive headache and nausea/vomiting followed by non localizing neurologic symptoms h. Hypoxic encephalopathy occurs from global interruption of cerebral blood supply. It commonly presents with decreased consciousness (eg, coma, vegetative state) as opposed to focal neurologic deficits. Neuroimaging may show watershed infarcts at the border of perfusion zones between the major cerebral arteries. I. giant cell arteritis (temporal arteririts) is a medium to large vessel arteritis that typically presents in elderly patients with subacute onset of headache, aww claudication, monocular vision loss and polymyalgia rheumatic j. saccular aneurysm rupture si associated with subarachnoid hemorrhage. patient usually presents with abrupt onset of severe headache,and head CT shows hyperlattenuation of the sulk and basal cisterns. Focal neurologic deficits are uncommon.

22 A 58 y.o man has sudden right-sided weakness. PE shows intact cranial nerves and sensory function. No sensory loss, problems speaking/swallowing, or difficulty with balance. the patient has decreased strength (3/5) on hi right side. Initial CT scan without contrast shows no abnormalities. Four weeks later, repeat brain imagine shows a 9 mm, lake like cavitary lesion in the left internal capsule. This patient has what? a. arteriovenous malformation b. cardiac embolism c. carotid artery atherosclerosis d. cerebral amyloid antipathy e. Charcot-Bouchard aneurysm f. Hypertensive arteriolar sclerosis g. hypertensive encephalopathy h. hypoxic encephalopathy i. large vessel arteritis j. saccular aneurysm

Acute rheumatic fever is an immune-mediated sequela of an untreated group A strep pharyngeal infection. Most serious manifestation of ARF is myocarditis. Valvular dysfunction, specifically acute mitral valve regurgitation, is the most likely cause of this patient's holosystolic murmur. This patient's myocardial biopsy shows interstitial fibrosis with central lymphocytes and macrophages as well as scattered multinucleate giant cells. this interstitial myocardial granuloma, or Aschoff body is pathognomonic for ARf-related myocarditis. Plump macrophages with abundant cytoplasm and central, slender chromatin ribbons called Anitschkow (or caterpillar) cells are also often present. Over subsequent years, Aschoff bodies are replaced by fibrous scar tissue, leading to chronic mitral valve stenosis and regurgitation. a. hypersensitivity myocarditis, which results from an autoimmune reaction to a med, is characterized by an interstitial infiltrate of eosinophils. Many classes of drugs, including diuretics (eg, furosemide, hydrochlorothiazide) and antibiotics (eg, ampicillin, azithromycin), can cause this hypersensitivity c. anthracyclines (eg, doxorubicin, daunorubimcin) are cardiotoxic chemotherapeutic agents. These agents can cause dose related acute and chronic cardiac damage (eg, dilated cardiomyopathy). On biopsy, patchy fibrosis with vacuolization and lysis of myocytes are evident. d. a genetic mutation involving sarcomere genes can lead to the development of hypertrophic cardiomyopathy. HCM leads to left ventricular hypertrophy and, in turn, both systolic and diastolic dysfunction. The pathology involves disorganized, hypertrophied myocytes. e. recent travel to South America is associated with Chagas disease, which is caused by the intracellular protozoan parasite Trypanosome cruz. Chugs disease can result in myocarditis characterized by distention of individual myofibers with intracellular trypanosomes. f viral myocarditis produces a predominantly lymphocytec interstitial infiltrate with focal necrosis of myocytes. Viral infection (eg, adenovirus, Coxsackie B virus, parvovirus B19) is the most common cause of myocarditis in the general population.

240 A 10 y.o boy has shortness of breath and palpitations for a day. He also has had fiver, poor appetite, and fatigue for 3 days. Vital signs indicate tachypnea, tachycardia and hypotension. On cardiac auscultation, the patient has a new holosystolic murmur. He is admitted to the hospital for further workup and management. Biopsy shows interstitial fibrosis with central lymphocytes and macrophages as well as scattered multinucleate giant cells. Which of the following most likely preceded and led this patient's current condition? a. antibiotic exposure b. bacterial infection c. chemotherapy d. genetic mutation e. genetic mutation e. travel to S.A f. viral infection

Hep A: RNA picornavirus -30 day incubation period -Transmisssion: fecal oral. Outbreak from contaminated water or food (shellfish are the classical foods). -malaise, fatigue, anorexia, nausea and aversion to smoking. -Anti-HAV IgM is marker for active disease. -Self limiting no complication of progression to chronic hep, cirrhosis and hepatocellular carcinoma. a/b/e: are important risk factors for Hep B and C. -This cannot be hepB because she is immune to it -This cannot be hepC because she is neg for anti=HbC. c. boiled eggs = salmonella.

372 A 34 y.o woman has malaise. She feels "run down and under the weather." She developed aversion to smoking. PE shows hepatomegaly. Labs shows: positive anti-HAV IgM and anti-HbS. Which of the following is most likely to be elicited on further history taking? a. had a tattoo recently at a local tattoo parlor. b. had accidental needle stick somewhere c. had a boiled eggs from hospital cafeteria d. had a steamed oysters at a neighborhood restaurant e. had unprotected sex with a new partner.

Protein and carb yield 4 calories per gram Fat yields 9 calories per gram Ethanol yields 7 calories per gram

755 How much calories are there in gram of protein, carb, fat and ethanol?

Furosemide is a loop diuretic that works by inhibit Na/K-2Cl symporters in the ascending limb of the loop of henle. Loop diuretic also stimulate prostaglandin relate. Prostangalndins have vasodilatory effects. Thus by stimulating renal prostaglandin relate, loop diuretics also increase renal blog flow leading to increase GFR and enhanced drug delivery. Both factors ultimately enhance diuretic response. NSAID (eg, ibuprofen, naproxen, and indomethacin) inhibit prostaglandin synthesis. Thus concurrent use of NSAIDs with loop diuretics can result in a decreased diuretic response.

A 43 y.o male has hepatic cirrhosis d/t prolonged heavy alcohol consumption. He develops massive ascites and lower extremity edema that responds well to furosemide therapy. However, a week later the patient receives high dose ibuprofen for joint pains and soon develops worsening abdominal distention. Blunting of the diuretic response in this patient is d/t interruption of which of the following substances? a. prostaglandins b. endothelia c. angiotensin II d. aldosterone e. natriuretic peptides f. bradykinin

Concept of gender at age 3-4 Sense of permanency of gender around 5-6 Gender dysphoria is the prolonged and intense feeling that a person' gender doesn't match one's assigned birth sex. When this feeling is strongly expressed, it is more likely to continue into puberty. People with gender dysphoria have increased rates of psychiatric illness, including depression and anxiety, and need monitoring and support.

11875 When do children began to understand the concept of gender? What is gender dysphoria?

Verapamil is a nondihydropyridine calcium channel blocker that acts by blocking L-type calcium channel. In cardiac and smooth muscle, L-type calcium channel is not directly connected to RyR channels. Thus you can have calcium induced calcium release. In skeletal muscle, L-tyep calcium channel is directly connected to RyR. Thus when depolarization open L-typed calcium channel, the RyR will open also. Therefore verapamil wouldn't work on skeletal muscle because it has little dependency on extracellular calcium influx.

144 How come verapamil doesn't work on skeletal muscle? a. calmodulin-independent excitation-contraction coupling b. decreased troponin affinity to intracellular calcium c. elaborate T-tubular system d. lack of internal automaticity e. little dependence on extracellular calcium influx

Adenomyosis is the presence of endometrial glandular tissue within the myometrium. -heavy menstrual bleeding (d/t increase endometrial surface) and dysmenorrhea (d/t endometrial growth confined in myometrial space). a. Leiomyomas (aka fibroids) are proliferation of myometrial smooth muscle cells. It ay cause heavy menstrual bleeding, but the uterus is usually irregularly enlarged. b. Ectopic pregnancy usually presents with amenorrhea with or without acute lower abdominal pain and a positive urine pregnancy test e. Endometrial hyperplasia is characterized by a greater increase in endometrial gland proliferation as compared to storm. It often presents with irregular, but not painful, menstrual bleeding. Endometrial polyps can cause abnormal uterine bleeding. Doesn't cause uterine enlargement.

1957 A 42 y.o woman, gravid 4, para 4, has heavy and painful menstrual bleeding over the past 3 months. She is sexually active with her husband and doesn't have pain with intercourse. She had a bilateral tubal ligation 3 years ago after her last child. On bimanual examination, the uterus is uniformly enlarged. Urine beta-hCG is negative. Biopsy shows security endometrium. Which of the following is most likely the cause of this patient's symptoms? a. benign myometrial smooth muscle cell proliferation b. blastocyst implantation in fallopian tube c. def of von Willebrand factor d. endometrial tissue in myometrium e. greater increase in enomdetrial gland proliferation compared to storm f. hyper plastic growth of tissue from endometrial surface

Pancreatic pseudocyst is a potential complication of pancreatitis. Pancreatic juices duces inflammatory reaction in walls of surrounding organs. Granulation tissue forms, encapsulating the fluid collection and foreign a pseudocyst. True cysts are lined by epithelial cells. Pseudocyst are lined with granulation, tissue and no epithelial lining. Fibrosis, thickening and organization of the walls occur with time. Most common location is the lesser peritoneal sac, bordered by the stomach, duodenum and transverse colon. a. glycogen rich cuboidal epithelium is seen in serous pancreatic neoplasms b. columnar mutinous epithelium is seen in mutinous cystic neoplasms of the pancreas c. atypical cells forming papillary projections are seen in the papillary variant of pancreatic adenocarcinoma.

435 A 48 y.o man has severe epigastric abdominal pain and vomiting after an episode of binge drinking. Four weeks later, he has a palpable upper abdominal mass and cystic lesion visualized. The inner walls of the lesions are meekly lined with? a. glycogen rich cuboidal epithelium b. columnar mutinous epithelium c. atypical cells forming papillary projections d. fibrous and granulation tissue e. endothelial cells

Giant cell (temporal)arteritis (GCA) -Jaw claudication (most specific symptom of GCA), headache, facial pain and vision loss. -Granulomatous inflammation of media. -Takayasu often affects aortic arch, though there is involvement of the remainder of the aorta. -If younger = takayasu. If older = GCA. a. leukocytoclastic vasculitis (microscopic polyangiitis, microscopic polyarterittis, hypersensitivity vasculitis) is characterized by segmental fibrinoid necrosis of small vessels (i.e.. arterioles, capillaries and venues). Histology similar to PAN, segmental transmural necrotizing inflammation of medium to small arteries. Necrotic tissue and deposits of immune complexes, complement and plasma proteins produce a smudgy eosinophilic deposit (fibrinoid necrosis) to give a histologic pattern consistent with immune complex vasculitis. d. Thromboaniitis obliterates (Buerger's disease) is a thrombosing vasculitis of medium and small sized arteritis, principally the tibial and radial arteries. Commonly seen with heavy smokers and onset usually before 35.

452 A 68 y.o male has difficulty chewing and persistent headaches. PE shows tenderness over patient's temples. What is this condition most similar to? a. leukocytoclastic vasculitis b. polyarteritis nodes c. takayasu d. thromboangiitis obliterates e. rheumatoid vasculitis

First generation antipsychotics are split into lower potency (non-neurological eg, chlorpromazine and thioridazine) and high potency (neurological eg, haloperidol and fluphenazine). Lower potency causes non-neurological side effects by having anti-cholinergic, antihistaminergic and anti-alpha1 adrenergic. High potency causes neurological side effects such as akathisia, acute dystonia and parkinsonism. a/c/g: constipation, dry mouth and urinary hesitancy are d/t cholinergic receptor blockade. b: dizziness and orthostatic hypotension is alpha-1 blockade d. hyperglycemia is a metabolic effect seen more in second-generation antipsychotics, aka atypical. f. sedation is d/t anti histaminergic.

515 Dude has schizophrenia. He was given chlorpromazine. He was changed to fluphenzaine. Compared to patient's initial treatment, fluphenzaine is more likely to cause which of the following things? a. constipation b. dizziness c. dry mouth d. hyperglycemia e. muscular rigidity f. sedation g. urinary hesitancy

Malignant otitis extern (MOE), severe infection most commonly seen in elderly diabetic patients. It is most frequently caused by Pseudomonas aeruginosa, a non lactose fermenting, oxidase positive, motile Gram neg rod. Typical manifestations are exquisite ear pain and drainage. The granulation tissue seen within the ear canal is an important characteristic finding of MOE, and the tympanic membrane is usually intact. Progression of this infection can lead to osteomyelitis of the skull base and cranial nerve damage. Treatment: ciprofloxacin A. vibrio cholera is an oxidase positive, Gram negative, comma shaped rod that grow on an alkaline medium that kills most organisms. V cholera causes a toxin mediated (stimulates adenylyl cyclase) watery diarrhea. b. gram neg rods that are fast lactose fermenters include Klebsiella and E.coli. They commonly cause urinary tract infections. d. shigella is a gram neg, nonlactose-fermenting, nonmotile, rods shaped bacterium. It causes shigellosis, a hemorrhagic diarrheal disease e. H influenza is gram neg, rod shaped bacterium that requires factor X (hematin) and factor V (NAD) for growth. Acute otitis media is associated with an inflamed, erythematous building, immobile tympanic membrane d/t the presence of infected fluid in the middle ear.

8342 A 73 y.o has progressive, severe pain and discharge from her left ear for the past 2 days. She has type 2 DM and has been noncompliant with her meds. PE moving or touching the pinna produces extreme pain. Otoscopic examination shows granulation tissue in the left ear canal with a scant amount of discharge. The tympanic membrane is clear, and there tis no middle ear effusion Initial cultures from the ear show a Gram-negative rod. Which of the following microbiological characteristics best describes the infecting organism? a. comma-shaped and grows well in high pH b. fast lactose fermenter c. motile and oxidase positive d. nonmotile and a lactose nonfermenter e. requires factor V and X for growth

Most cases of hereditary beast and ovarian cancer are associated with mutations in the tumor suppressor genes BRCA1 and BRCA2. tumor suppressor genes are involved in: 1. DNA repair 2. cellular differentiation 3. check point control of the cell cycle 4. transcription factor regulation BRCA1/2 are particularly involved in repair of double-stranded DNA breaks. -Inherited autosomal dominant with 70-80% life time risk for developing cancer. -BRCA1 also increase risk of having ovarian cancer by 40% (this is not associated with BRCA2) a. angiogenesis is an essential factor in the growth of tumors d/t their rapid rate of division and high metabolic demands. they stimulate angiogenesis by increasing production of substances such as vascular endothelial growth factor. c. intercellular adhesion is impaired in majority of malignant tumors, which allows portions of the tumor to break of and metastasize. Adhesion molecules such as E-cadherin are also responsible for transduction of growth signals important for normal tissue growth and regeneration; disruption of intercellular adhesion signaling can promote uncheck cellular proliferation. d. protein degradation is essential for regulation of cellular functions and is performed by the proteasome as well as cytosolic proteinases. Increased degradation of protective proteins such as p53 or decreased breakdown of proteins that favor cell division can lead to malignancy e. a large number of malignancies are associated with a aberrant signal transduction. For example, over expression of HHER2 ( a receptor tyrosine kinase involved in growth signal transduction) causes the development of certain types of aggressive breast cancer. Unlike BRCA1/BRCA2, HER2 mutations are acquired, not inherited.

1083 A 28 y.o woman has breast cancer. Her mother died of ovarian cancer. Her aunt died of breast cancer. This patient most likely inherited a mutation in a gene normally responsible for which of the following processes? a. angiogenesis b. DNA repair c. intercellular adhesion d. protein degradation e. signal transduction

Spasticity is defined as an increase in resistance to passive muscle movement or a velocity dependent increase in tonic stretch reflexes. The loss of deciding inhibitory control from the UMN results in LMN overactivity, leading to increased tone and hyperreflexia. Baclofen, an agonist at the GABA-B receptor, It is an effective as mono therapy for the treatment of spasticity secondary to both brain and spinal cord disease, including MS. Tizanidine (centrally located alpha-2 adrenergic) is also effective and commonly used. b. duloxetine is a NE reuptake inhibitor used to treat anxiety/depression and as off labile treatment for migranes and neuropathic pain. c. Levetiracetam is an anti-epileptic drug d. nortriptyline is a TCA used to treat neuropathic pain and migraine headaches, especially in patients who have comorbid depression/anxiety. e. propranolol is a beta blocker used to treat essential tremor.

11458 Patient has spastic paraparesis from MS. Which of the following would provide the most effective monotherapy for this patient's spasms? a. baclofen b. duloxetine c. levetiracetam d. nortriptyline e. propranolol

CMV typically presents as mono like illness with fever and mild hepatitis. -Vertical transmission with highest risk during the first trimester: -Chorioretinitis -sensorineureal deafness, seizures, jaundice, hepatosplenomegaly and microcephaly b. choroidal nevus (eye freckle) benign pigmented area c. congenital cataract = rubella d. glaucoma = tumor e. conjunctivitis = chlamydia f. retinitis pigments = inherited degenerative eye disease g. retinoblastoma = most common malignant eye cancer in children

11551 Patient is pregnant and has CMV infection 2 months ago. Th eunborn infant is at greatest risk of which of the following eye conditions? a. chorioretinitis b. choroidal nevus c. congenital cataract d. glaucoma e. inclusion conjunctivitis f. retinitis pigments g. retinoblastoma

The second-generation antipsychotic clozapine is the only med that has consistently shown superior efficacy in treatment resistant schizophrenia. mOA: multiple dopamine and serotonin receptors, but the precise pharmacological mech of why it is superior is unknown. Side effects: agranulocytosis, weight gain nd metabolic effects. a/g: aripiprazole and ziprasidone are second-generation antipsychotics used in schizophrenia, but clozapine is preferred d/t patient's history of treatment resistance b. chlorpromazine is a low-potency, first-generation antipsychotic that is unlikely to be effective int treatment-resistant schizo c. clonazepamn is a bento that can be used adjectively to target agitation in dividiuals with psychosis, but it doesn't specifically treat psychotic symptoms e. Haloperidol long acting injectable would be a good strategy for a patient who is non-adherent and has a history of responding well to oral haloperidol. However, this patient is adherent and has responded poorly to haloperidol f. valproate is an anticonvulsant mood stabilizer used in treatment of bipolar disorder.

11743 Dude has schizoprhenia. Previous trails of haloperidol and risperidone resulted in minimal improvement. Which of the following meds would be most effective for this patient's illness? a. aripiprazole b. chlorpromazine c. clonazepam d. clozapine e. haloperidol long acting injectable f. valproate g. ziprasidone

Iliohypogastirc nerve arises form the L1 nerve root, emerges from the lateral border of the upper posts major, and passes behind the kidney anterior to the quadrates lumborum. The nerve provides motor function to the anterolateral abdominal wall muscles. Its anterior branch emerges above the superficial inguinal ring to innervate the skin above the pubic region. The lateral branch descends over the iliac crest to innervate the gluteal region. Injury to the anterior branch during appendectomy causes decreased sensation at the suprapubic region. a. the femoral nerve arises from L2-L4 nerve roots. It provides sensation to the upper thing and inner leg and innervates muscles that extends the knee. b. the genitofemoral nerve arises from L1-L2 nerve roots and provides sensation to the upper anterior thigh and motor fun to parts of the genitalia (eg, cremasteric reflex in men, mon pubis in women). d. The ilioinguinal nerve originates form the L1 nerve root and accompanies the spermatic cord through the superficial inguinal ring. It provides sensation to the upper and medial thigh and parts of the external genitalia e. The lateral femoral cutaneous nerve arises from the L2-L3 nerve roos and provides sensation to the skin on the lateral thigh. f. the obturator nerve arises form the L2-L4 nerve roots. It innervates the skin of the medial thigh and provides motor function for the adductor muscle of the lower extremity.

11769 A 13 y.o had an acute appendicitis and underwent appendectomy without any apparent immediate complications and was released home. Several days later, he starts having burning pain at the surgical scar radiating to the suprapubic region but otherwise fells well. Examination shows a healed surgical incision centered over McBurney point. There is a loss of sensation over the right suprapubic area. The cremasteric reflex is normal. Which of the following nerves is messed up? a. femoral b. genitofemoral c. iliohypogastric d. ilioinguinal e. lateral femoral cutaneous f. obturator

Cushing triad (HTN, brady cardia and bradypnea) that developed increased intracranial pressure d/t hemorrhage intracranial metastases involving the left temporal lobe. Expanding space occupying lesions within the temporal lobe can cause elevated intracranial pressure with transtentorial herniation of the uncus. Uncle herniation may result in compression of the ipsilateral third cranial nerve as it exits the midbrain. Patients often develop ipsilateral oculomotor nerve palsy with fixed dilated pupil d/t damage to thepreganglionic parasympathetic fibers running on the outer proton of the third cranial nerve. As the herniation progresses, damage to the midbrain results in mid-positioned and fixed pupils bilaterally. Additional findings in advanced transtentorial herniation include loss of vestibular-ocular reflexes and decorticate followed by decerebrate posturing. a. abducens can occur d/t elevated intracranial pressure; however, it is more often associated with downward displacement of the brainstem (central herniation) d/t nerve traction along the clivus. c. Horner syndrome (ptosis, mitosis and anhidrosis) is associated with lesions affecting the lateral hypothalamus or sympathetic tracts in the brainstem (eg, lateral medulla) d. uncle herniation can compress the cereal peduncles, resulting in corticospinal tract injury with hemiplegia. However, patients initially have flaccid paralysis with hyporeflexia (i.e., spinal shock) and develop spasticity/hyperreflexia days to weeks following acute upper motor neuron damage e. Nystagmus is generally seen with lesions to the pons, medulla or cerebellum d/t disruption of vestibular pathways f. pinpoint pupils are usually associated with large bilateral pontine lesions caused by damage to the deciding sympathetic tracts. g. hydrocephalus can cause impaired upward gaze d/t dilation of the third ventricle with compression of the dorsal midbrain (pretectal area).

11804 A 65 y.o man has sudden onset of headache. PMH are HTN and lung adenocarcinoma with abdominal metastasis. CT reveals small temporal lobe hemorrhage. Over the next 24 hrs, he becomes progressively obtunded and develops generalized tonic-clonic seizures. His BP is 190/96, pusle is 38 and RR is 6. A repeated non contrast head CT reveals acute hematoma expansion with brain herniation. Which of the following findings is most likely to be observed on his repeated neurologic examination? a. abducens palsy b. dilated pupil c. horner syndrome d. hyperreflexia e. nystagmus f. pinpoint pupils g. upward gaze

SCD is an AR disorder resulting in hemoglobin molecules that polymerize during times of hypoxic stress. -SCD results in DNA point mutation in the coding region of the hemoglobin beta global chain. This single nucleotide substitution is a missense mutation that causes a glutamic acid to be replaced by valine at the 6th position during translation. Silent: base substitution codes for same a.a Missense: base substitution codes for different a.a. Conservative: base sub codes for different a.a. with similar chemical structure Nonsense: base sub introducing a premature stop codon Nonstop: base sub within stop codon result in continued translation Splice site: mutation at splice site alters intron removal form pre-mRNA Frameshift: delationinsertion of bases causes downstream misreading

12019 What are the differences between silent, missense, conservative, nonsense, nonstop, splice site and frameshift mutations? -In sickle cell disease, what type of mutation happened and what are the amino acids involved?

Valproate is an anticonvulsant and a mood stabilizer. MOA: blocks voltage gated Na channels and enhances GABA synthesis and relate. As an anticonvulsant, it is effective in treating absence, myoclonic and generalized tonic clonic seizures. It is DOC for myoclonic seizure, it is a second line agent for absence seizures (ethosuximide is preferred). As a mood stabilizer, valproate is used in acute mania and maintenance treatment of bipolar disorder, especially in patients with rapid cycling (>4 episodes a year) and episodes with mixed manic depressive features. Other anticonvulsants used as mood stabilizer in bipolar disorder include carbamazepine who is effective in treatment of mania, but it is a CYP 450 inducer that can lower levels of concomitant meds. Lamotrigine is particularly effective in the depressed phase of bipolar disorder and is associated with skin rash, which rarely can progress to life-threatening mucocutaneous reactions (eg, stevens johnson syndrome). a. ehtosuximide is the anticonvulsant of choice for absence seizures. It reduces Calcium currents in T-type calcium channels in the neurons, thereby inhaling the propagation of action potentials. b. lithium is a mood stabilizer that is effective in the acute and maintenance treatment of bipolar disorder. It works via modifying second messenger signal transduction at several levels, resultingg in decreased excitatory neurotransmission. Lithium is not an anticonvulsant. c. lorazepam is abenzo that enhances the affinity of GABA for it receptor (increases frequency of Cl channel openings), thereby increasing its inhibitory potential. Lorazepam is first line med for treatment of active seizures, acute anxiety, and alcohol withdrawal, but it is not use to treat bipolar disorder d. olanzapine is a second generation antipsychotic use to treat psychotic and mood disorders. Side effects include weight gain and increased risk of metabolic syndrome ( dyslipidemia, HTN and hyperglycemia). Antipsychotics are not indicated for seizures and may lower the seizure threshold. e. sertraline, is an SSRI is indicated in major depression, obsessive compulsive disorder, and anxiety disorders. Mono therapy with SSRIs is avoided in bipolar disorder d/t the risk of inducing mania; they have no role in treatment of seizures.

1193 Patient has generalized tonic clonic seizures and bipolar disorder. Which of the following meds would be dost helpful in treating both his acute seizure disorder and bipolar disorder over the long term? a. ethoxsuximide b. lithium c. lorazepam d. olanzapine e. sertraline f. valproate

Collagen is the most abundant protein in the human body and is synthesized by fibroblasts, osteoblasts and chondroblasts. It consists of 3 polypeptide alpha chains held together by hydrogen bonds, forming a rope-like triple helix (collagen molecule). Collagen molecules self-assemble into fibrils, which subsequently cross link to form collagen fibers. The triple helical conformation of collagen molecules occurs d/t the simple and repetitive amino acid sequence within each alpha chain, in which GLYCINE occupies every third amino acid position (Gly-X-Y). Glycine is the most abundant amino acid in collagen and , d/t its small size is the only amino acid that can fit into the confined. X often represents proline and Y is often hydroxyproline or hydroxylysine. Proline residue are essential for alpha helix formation because their ring configuration introduces a kind in the polypeptide chain, enhancing the rigidity of the helical structure. Hydroxylysine is necessary for cross-linking, which greatly increases the tensile strength of assembled collagen fibers.

1248 After several days, the fibroblasts begin synthesizing polypeptide chains that assemble into triple helical structures, followed by fibrils. The fibrillar proteins are hydrolyzed an separated into their constituent amino acids. Which of the following amino acids is the most likely to be found in highest quantity in these proteins? a. alanine c. glycine d. leucine e lysine f. proline

Lactotroph (prolactin): hypogonadism, infertility in male. Galactorrhea, amenorrhea in women. Corticotrophin (ACTH): cushing disease Somatotroph ( GH): acromegaly and giantism Thyrotroph (TSH): thyrotoxicosis Gonadotroph (LH, FSH): nonfunctioning (hypopituitarism) and mass effect. a/b/d: gliomas constitute a family of CNS tumorr that arises from precursors to astrocytes (astrocytomas, glioblastomas), oligodendrocytes (oligodendrogliomas), or ependymal cells (ependymomas). -diffuse astrocytomas and oligodendrogliomas usually originate in the cerebral hemispheres and commonly present with signs of increased intracranial pressure (eg, nausea, vomitng, headache), seizures, and focal neurologic deficits. Ependymomas are most common in children and usually arise in the walls of the ventricles, hampering the flow of cerebrospinal fluid and causing hydrocephalus. e. the axons from retinal ganglion cells join to form the optic nerve. Optic neuropathies are characterized by monocular vision symptoms, often with pain. Common etiologies include ischemic optic neuropathy, MS and meningitis/encephalitis. f. Vestibular schwannoma (acoustic neuroma) is associated with symptoms of CN VIII compression, including sensorineural hearing loss and tinnitus.

1262 A 27 y.o has bitemporal hemianopia. An abnormality originating in which of the following cells is the most likely cause of this patient's symptoms? a. astrocytes b. ependymal cells c. lactrotrophs d. oligodendrocytes e. retinal ganglion cell axons f. schwann cells

alpha-1: located at the peripheral vasculature, bladder and eye. Causes: Increase SBP, contraction of the internal urethral sphincter, mydriasis (contraction of pupillary dilator muscle). Examples: epi, NE, phenylephrine, methoxamine beta-1: heart Causes: increase HR, contractility and conductance examples: epi, Dopa, dobutamine and isoproterenol beta-2: peripheral vasculature (skeletal muscle), bronchi, uterus Causes: vasodilatation, decrease DBP, bronchi dilatation and relaxation. Examples: isoproterenol, terbutaline and ritodrine Inhibits uterine contraction = beta-2 agonist. Mydriasis = alpha 1 agonist. a beta-adrenergic agonist would decrease uterine contraction, however it would cause dilatation of the eyes. c. alpha-adrenergic agonist would cause dilation of the eyes, however it doesn't cause uterine relaxation. d. beta-adrenergic antagonist would increase uterine contraction. e. alpha adrenergic aciagnosit would cause eyes to miosed.

1368 A drug when given to a pregnant dog. It inhibits uterine contractions and dilates the pupils. Which of the following best characterized drug A. a. beta-adrenergic agonist b. alpha and beta-adrenergic agonist c. alpha-adrenergic agonist d. beta-adrenergic antagonist e alpha-adrenergic antagonist and beta adrenergic agonist

Neurologic symptoms (eg, tingling and difficulty concentrating), recurrent episodes of nonspecific abdominal pain and urine changed to dark purple when left standing for an hour= AIP (acute intermittent porphyria), an AD d/t def of prophobilinogen deaminase (AKA hydroxymethylbilane synthase), an enzyme in heme synthesis. The rate limiting step is aminolevulenate synthase (ALAS), which comes in 2 isoforms. -ALAS1 is found in the liver and is used for CYP450 which is regulated largely by heme, glucose and CYP450 inducers. -ALAS2 is found in only fetal liver and adult BM and is used to make RBCs, which is regulated by iron and erythropoietin. Besides def of the enzymes, lead can inhibit ALA dehydrates and ferrocheltase = accumulation of ALA and some protoporphyrin IX. Def of Vitamin B6 (pyridoxal phosphate (PLP) is need for ALA synthase. AIP attacks are d/t accumulation of ALA and PBG, generally resulting from a combination of 2 factors: -PBG deaminase def -ALA synthase induction, typically precipitated by certain meds (eg, phenobarbital, griseofulvin, phenytoin), alcohol use, smoking, progesterone (eg, puberty), or a low calorie diet. Glucose or heme inhibit ALA synthase and are used in AIP management. PBG deaminase def by itself is generally not sufficient for development of AIP attacks (as many with def are asymptomatic), and PBG deaminase enzyme acitivyt is not directly affected by hemming infusion. a/d: aminolevulinate dehydrates and ferrochelatase (inhibited by lead) are seen with lead poisoning. Ferrochelatase def is aka erythropoietic protoporphyria, characterized by cutaneous photosensitivity beginning in early childhood. c. Bilirubin glucuronyl transverse is a hepatic enzyme that is responsible for the conjugation of bilirubin (byproduct of heme catabolism) with glucuronide, a polar molecule that improve bilirubin solubility for subsequent biliary excretion. A decrease is glucuronyl transferase activity results in unconuugated hyperbilirubinemia.

1338 A 22 y.o woman has nausea, constipation and severe poorly localized abdominal pain. She also has anxiety, difficulty concentrating, poor sleep quality and tingling of the limbs. Sample of her urine turned dark purple after standing for an hour. She has no photosensitivity. Patient receives an IV infusion of a heme preparation that leads to rapid resolution of her symptoms. The improvement in symptoms is most likely d/t to treatment induced downregualtion of which of the following enzymes? a. aminolevulinate dehydrates b. aminovulinate synthase c. bilirubin glucoronyl transferase d. ferrochelatase e. prophobilinogen deaminase

Middle ear has 3 auditory ossicles (malleus, incus and stapes) and 2 muscles (tensor tympani and stapedius) Stapedius inserts at the neck of the stapes function to stabilize the bone. It is innervated by CN VII (Facial nerve). Lesion to this CN can cause stapes to oscillate widely inducing hyperacusis. Ipsilateral hyperacusis is common associated with Bell's palsy. Tensor tympani inserts into malleus. It is controlled by CN V3 (trigeminal nerve) to contract tympanic membrane medially to increase tension and dampen sound transmission.

1452 Dude has 3 week history of difficulty hearing. He has become less intolerant to everyday sounds. He complains of ear pain and often avoids public places. Injury to which of the following CNs is most likely responsible for his condition a. accessory b. facial c. glossopharyngeal d. hypoglossal e. vagus

This patient with increased HR, CO and unchanged PaO2 and PaCO2 is likely undergoing an exercise test. Changes in partial pressures of oxygen and CO2 during exercise occur predominantly in the venous blood as muscle tissue extracts a higher portion of oxygen and produces greater amount of CO2. The PaO2 and PaCO2 remain relatively close to resting values at most levels of exercise d/t a number of adaptions in the lungs and pulmonary circulation, such as hyperventilation and improved ventilation-perfusion (V/Q) matching. a. carotid sinus massage causes reflex parasympathetic stimulation of the sinoatrial node, atrial myocytes, and atrioventricular node, resulting in a transient decrease in HR and CO. c. High altitude simulation is associated with lower ambient oxygen pressures; consequently, HR and CO increase in order to improve oxygen delivery to tissues. The lower oxygen pressure cause hypoxemia and result in hypoxic stimulation of respiration, causing hyperventilation and decreased PaCO2. d. Panic attack can increase HR and CO via sympathetic stimulation of the heart; however, there is usually associated hyperventilation without a significant increase in muscle activity and therefore a fan in the PaCO2 e. Pulmonary artery occlusion as seen in PE results in increased PVR, right ventricular strain, and frequently decreased left ventricular stroke volume. Reflex tachycardia can result to main (not increase) CO. Additionally, PE often causes hypoxemia (low PaO2) d/t acute V/Q mismatching in the affected lung.

1589 A 32 y.o woman is undergoing evaluation that causes her heart rate and cardiac output to increase. Her PaO2 and PaCO2 are normal. These changes are most likely part of an integrated response to which of the following? a. carotid sinus message b. exercise c. high altitude exposure d. panic attack e. pulmonary artery occlusion

EBV is the etiologic agent most strongly associated with Burkitt lymphoma. Crucial step in the transformation of infected cells is the balanced t*8;14) translocation, which causes over expression of c-MYC, a transcriptional regulator that controls cell proliferation. Histology of Burkitt lymphoma: diffuse medium-sized lymphocytes and a high proliferation index represented by the high Ki-67 fraction. The classic "starry sky" appearance is usually seen, and is d/t to the presence of benign macrophages. B. HPV = invasive squamous cancer. (16, 18, 31, 33, 35 and 51). c. H. pylori plays a role in development of lymphomas d. HSV 8 = Kapok's sarcoma, most commonly in HIV infected patient. Histology of primary effusion lymphoma is distinct from burrito lymphoma, with large cells, big nuclei and prominent nucleoli e. Hep B causes hepatocellular carcinoma.

1630 A 30 y.o IV drug user with history of HIV infection has abdominal distention and anorexia. A CT scan of the abdomen shows ascites and a large mass surrounding the small intestine. Biopsy of the mass reveals uniform, round, medium sized tumor cells with basophilic cytoplasm and proliferation fraction (Ki-67 fraction) of over 99%. Which of the following infectious agents is most closely associated with the development of his current condition? a. EBV b. HPV serotype 31 and 33 c. H. pylori d. HHV 8 e. Hep B

Patient paresthesias d/t hypocalcemia. Prior to storage, whole blood (for transfusions) is generally mixed with solutions containing citrate anticoagulant. Packed cells derived from these whole blood collections also contain citrate. Infused citrate can chelate serum calcium and magnesium, causing hypocalcemia. c. stored RBCs gradually lose intracellular K to the surrounding solution. this mech has the potential to cause hyperkalemia, but not hypocalcemia.

1654 Patient was in a motor vehicle accident. His BP is 80/40 and pulse is 110. He receives several units of packed RBCs. Once stabilized, the patient begins complaining of a tingling sensation in his toes and fingers. His serum calcium level is 7.2 (normal is 8-10). Which of the following is the most likely cause of this patient's current symptoms? a. increased renal tubular secretion of calcium b. calcium chelation by a substance in the transfused blood c. electrolyte leakage from RBCs during pre-transfusion storage d antibody-mediated RBC membrane damage e. PTH suppression d/t fluid overload

CYP 450 enzymes are group of heme-containing proteins that are responsible for majority of drug metabolism. these enzymes generally function to deactivate drugs and facilitate excretion from the body by improving water solubility. However, they also metabolize certain compounds to their active forms. Polymorphisms may occur in the genes coding for these enzymes, altering their expression or activity. Three important phenotypes exist: poor, intermediate and rapid metabolizer. Tamoxifen is a selective estrogen receptor modulator used in treatment of estrogen receptor positive breast cancer, is a prodrug metabolized by CYP2D to its active metabolite, endoxifen. Patients with genetic polymorphisms resulting poor CYP2D activity are exposed to decreased levels of the active metabolite and have a higher risk of disease relapse. a. activation of downstream signal transducer proteins, such as KRAS, leads to activation of transcription factors that promote cell growth. Mutation is associated with development of colorectal and lung cancers b. decreased activity of hepatic N-acetyltransferase result in diminished ability o metabolized drug such as isoniazid and sulfonamides, leading to an increased likelihood of toxicity. c. thiopurine methyltransferase is responsible for the metabolism of thipurine compounds such as immunosuppressive drug 6-mercaptopurine d. P-glycoprotein is a cell membrane protein the drives efflux of a number of substances out of the cell. Over expression of p-glycoprotein in tumor cells has been identified as a cause of multi drug resistance.

1711 Researchers are studying tamoxifen as an adjuvant therapy for breast cancer. A comparison of patients who had disease recurrence with those who remained cancer free showed that some of the relapsed patients had lower serum concentrations of endoxifen and 4-hydroxytamoxifen, the active metabolites of tamoxifen. Why? a. activating mutation affecting a downstream signal transducer b. decreased hepatic N-acetyltransferase activity c. def of thiopurine methyltransferase enzyme d. over expression of P-glycoprotein in the tumor cells e. polymorphism of cytochrome eP450 enzyme

Potter sequence: Urinary tract anomaly > anuria/oliguria in utero > oligohydramnios > pulmonary hypoplasia, flat facies and limb deformities. Bilateral renal genesis is the classic finding, but other lesions such as posterior urethral valves or ARPCKD can also be the cause. Since amniotic fluid depends on fetal urine production, affected infants have severely reduced or absent amniotic fluid. Lack of amniotic fluid causes external compression of the face (Potter facies) and lower extremities (club feet). In addition, the umbilical cord is often compressed and fetal HR anomalies are common during labor. Pulmonary hypoplasia results d/t the lack of normal alveolar distention by aspirated amniotic fluid. Thus respiratory failure d/t severe pulmonary hypoplasia is the most common cause of death among infants with Potter sequence. a. Congenital diaphragmatic hernia causes severe respiratory disease, pulmonary hypertension and absent breath sounds unilaterally but would not cause this infant's facial and lower limb findings. b/e: infants with GI obstruction proximal to small bowel (eg, esophageal or duodenal atresia) cannot absorb swallowed amniotic fluid, resulting in polyhydramnios d. although surfactant def (respiratory distress syndrome) can cause hypoxia and respiratory distress, it is most commonly associated with prematurity and would not cause facial or lower limb deformities.

1752 A 1 hour old boy has tachypnea and hypoxia. Pregnancy was complicated by lack of prenatal care. PE shows flattened nose and bilateral club feet. Great sounds are markedly diminished bilaterally. The infant is intubated and mechanically ventilated, but his oxygen levels do not improve. He dies. Which of the following is most likely to be found during autopsy? a. congenital diaphragmatic hernia b. duodenal atresia c. renal genesis d. surfactant def e. tracheoesophageal fistula

The metabolism of ethanol by alcohol DH and aldehyde DH reduces NAD to NADH and increases the NADH/NAD ratio. This inhibits all other pathways required NAD, including reaction required for gluconeogensis. In particular, lactate cannot be converted to pyruvate and instead reaction is driven from pyruvate toward lactate. Inaddition, excess NADH inhibits the conversion of malate to oxaloacetate. Pyruvate and oxaloacetate are intermediates of glucnoenogensis; therefore, conversion of these molecules to lactate and malate inhibits gluconoegnesis.

1866 A 27 y.o man has hypoglycemia and urine strongly positive for ketones following a couple days of binge drinking. Suppression of which of the flowing is the primary cause of this patient's hypoglycemia? a. gluconeogeneiss b. glycogenolysis c. insulin clearance d. insulin sensitivity e. lipolysis

Malignant ovarian neoplasms: 1. epithelial a. serous cystadenocarcinoma: Most common. Bilateral. Psammoma bodies b. mutinous cystuadenocarcinoma: pseudomyxoma peritonea. Mucin producing epithelial cells. 2. Germ cell a. Dysgerminoma: adolescent. Increase beta-hCG and LDH. Fried egg cells b. Endodermal sinus (yolk sac). Increase AFP, aggressive and has Schiller Duval bodies resembling glomeruli 3. Stroma (sex cord) a. granuloma cell: increase estrogen (eg, endometrial hyperplasia, postmenopausal bleeding). Increase inhibin. Call-Exner bodies, coffee bean nuclei. b. sertoli-leydig: androgen (eg, hirsutism, clitoromegaly)

1928 A 26 y.o woman has facial hair and her voice is getting deeper. Mother died of invasive lobular breast carcinoma. PE shows clitoromegaly with large adnexal mass. Urine pregnancy is negative. Ultrasound shows large ovarian cyst. Which of the following is most likely diagnosis? a. dysgerminoma b. endodermal sinus tumor c. granuloma cell tumor d. mature cystic teratoma e. serous cystadenocarcinoma f. sertoli=leydig tumor

Manifestations of sickle cell disease: 1. hemolysis: repeated sickling of RBCs leads to permanent deformation and premature erythrocyte destruction by macrophages and mechanical stress. This causes intra-and extravascular hemolysis, resulting inincreafed indirect bilirubin and lactate DH and decreased haptoglobin. Haptoglobin binds circulating hemoglobin and reduces renal excretion of free hemoglobin, preventing tubular injury. 2. vasoocclusive symptoms: patients with SCD may experience pain from hypoxic tissue injury and infarctions d/t obstruction of small vessels by sickled cells. This patient is presenting with dactylics (hand-foot syndrome), resulting from small infarctions in the bones of the extremities. 3. infections: patients with SCD are predisposed to infections with encapsulated organisms (eg, S. pneumoniae) because repeated splenic infarcts cause functional asplenia. a/e: chronic anemia in SCD can result in cardiomegaly d/t chronically increased CO (systolic function is largely preserved d/t an increase in stroke volume). However, this is typically don't occur until after infancy. Older patients can develop congestive heart failure d/t ischemic cardiomyopathy and present with decreased CO and increased systemic venous pressure. b/f: serum albumin and urine cortisol excretion are typically normal in SCD c. hereditary angioedema is aware AD disorder that is associated with painless episodes of swelling involving the face, lips, larynx and extremities. It is caused by C1-inhibitor def, no vasoocclusion.

1855 A 1 y.o African American boy has 3 hour history of severe swelling and tenderness of the hands and feet. The patient has no history of recent illness and is up to date on all immunizations. The boy attends day care, but his parents are unsure about any sick contacts. His FH is significant for an older brother who died of pneumococcal sepsis at age 6. PE shows bilateral, severe swelling of the hands and feet. Which of the following is most likely abnormal in this patient? a left ventricular systolic function b. serum albumin c. serum C4 complement complement fraction d. serum haptoglobin e. systemic venous pressure f. urine cortisol excretion

Femoral neck factor can lead to an increase in risk of osteonecrosis if the blood supply is disrupted. It is supplied by mostly the medial circumflex artery. b/c: branches the lateral circumflex and superior and inferior gluteal arteries join with the medial circumflex artery to form the trochanteric anastomosis. e. the obturator artery gives rise to the artery of the ligament trees, which supplies a minor portion of the femoral head. proximal to the epiphyseal growth plate, but it is of minimal clinical significance in adults.

1956 Patient has a hip fracture at the femoral neck. Injury involving which of the following arteries is most likely to lead to osteonecrosis in this patient? a. deep femoral b. inferior gluteal c. lateral circumflex d. medial circumflex e. obturator

Seizures: 1. Focal (involve 1 cerebral hemisphere at onset) a. simple: no loss of consciousness or postictal state. Involves motor, sensory, autonomic or psychic symptoms. b. complex: loss of consciousness and postictal state. May have automatisms (eg, lip smacking) Treat with narrow spectrum: -carbamazepine, gabapentin, phenobarbital and phenytoin. 2. generalized (involves both hemispheres at onset) a. tonic clonic: loss of consouciness and postictal state. Diffuse muscle contraction of limb (tonic) followed by rhythmic jerking (clonic) b. myoclonic: no loss of consciousness or postitical state. brief jerking movements c. absence: brief loss of consciousness (eg, blank stare). May have automatisms. Usually no postitictal state. Treat with broad spectrum: -lamotrigine, levetiracetam, topiramate, valproic acid. Ethosuxamide (only in absence) a. baclofen is a GABA-B agonist use to treat muscle spasticity. b/f: carbamazepine and phenobarbital are used in focal seizures (simple/complex) not generalized seen in this patient. c. clonidine is an alpha-2 agonist use to treat ADHD kids who are refractory to stimulants (eg, methylphenidate). d. diazepam is a bento that is use to acutely treat status epilepticcus. e. fluphenazine is a D2 high potency neuroleptic that can be use to treat tourette. g. propranolol is a non-selective beta blocker can use to treat benign essential tremor. Usually present in middle age adults that worsens during fine motor activity.

355 A 15 y.o has 3 month history of periodic, sudden-onset, jerking movement involving both arms. Usually happen in early morning and are aggravated by sleep deprivation. He has never lost consciousness. Uncle had similar shit. Which of the following is the best initial treatment for this patient? a. baclofen b. carbamazepine c. clonidine d. diazepam e. fluphenazine f phenobarbital g. propranolol h. valproic acid

Neurons contain large, round, centrally located nuclei with large nucleoli. The cell bodies of the neurons contain basophilic granule called Nissl substance. It represents rough endoplasmic reticulum and is responsible for protein synthesis. During disease or injury, the neurons display a number of characteristic changes. The types of neuronal reaction to injury are as follows. 1. acute neuronal injury (red neuron): transient severe insult that leads to cell death. Histology shows shrinkage of the cell body, pyknosis of the nucleus, loss of Nissl substance and eosinophilic cytoplasm 2. Axonal reaction: loss of axon. Enlargement of the cell body, eccentric nucleus, enlargement of the nucleolus, dispersion of the Nissl substance. 3. Neuronal atrophy: caused by progressive degenerative disease. Loss of neurons and functional groups of neurons. Reactive gloss. A neuron that is reacting to acute irreversible damage is called a "red neuron." This can occur d/t transient ischemia, hypoxia, toxicity or an episode of hypoglycemia. Remnants of the messed up neuron will be phagocytize by microglia. Astrocytes proliferation on the site of injury and form a glial scar. a. hypertrophy is an enlargement of a cell in response to increased stimulation. b. axonal reaction occurs when neurons axon is severed. This reaction entails swelling of the cell body, moment of the nucleus to the periphery and the dispersion of Nissl substance c. atrophy is decrease is cells size d/t decrease blood supply, loss of innervation or decreased function. Compression atrophy may occur in the CNS d/t increased intracranial pressure or mass lesion that compresses the surrounding tissue e. the common causes of metabolic encephalopathy are hypoglycemia, hyperglycemia and hepatic encephalopathy. f. normal again is associated with progressive atrophy and neuronal loss. however, "red neuron" changes are not characteristic of normal aging.

492 LM of brain tissue taken from experimental animal shows neurons with shrunken nuclei, no detectable Nissl substance and intensely eosinophilic cytoplasm. The findings described most likely indicate: a. compensatory hypertrophy b. axonal regeneration c. compression atrophy d. irreversible cell injury e. metabolic encephalopathy f. normal aging

Ways to stop testosterone: 1. Gn-RH agonist (leuprolide, goserelin, nafarelin and histrelin) inhibits anterior pit from releasing LH, thus decreasing Leydig cell stimulation by LH. 2. Ketoconazole and spironolactone inhibits testosterone production in the Leydig cells 3. Finasteride is a 5-alpha-reductase inhibitor that stop peripheral conversion of testosterone to DHT 4. Spironolactone, Flutamide and cyproterone are competitor of androgen hormone receptor on target cells. DHT: much higher affinity for testosterone receptor and mediates majority of testosterone effects, including development of male external genitalia and *prostate enlargement.* Finasteride: is used for treatment of BPH and balding (because patients usually have high amount of 5-alpha-reductase) c. Anastrozole is an aromatase inhibitor that selectively blocks estrogen production.

658 A 64 y.o man has 6 month history of difficulty urinating with frequent straining and dribbling. He was given drug therapy, which gives moderate symptomatic relief. However, he also notices an increase in hair growth over his scalp. Which of the following is best description of this drug's MOA? a. decreased Leydig cell stimulation by LH b. decreased Leydig cell androgen synthesis c. Decreased peripheral androgen aromatization d. Decreased peripheral androgen 5-alpha-reduction e. impaired androgen receptor interaction f. impaired second messenger action.

Allergic bronchopulmonary aspergillosis (ABPA) History: asthma, CF Chest imagine: recurrent fleeting infiltrates, bronchiectasis Diagnosis: eosinophilia, positive skin test and Ig for Aspergillus, and elevated IgE Aspergillum fumigates is a low virulence fungus the generally doesn't cause significant infections except in immunocompromised or debilitated patients. It may, however, colonize the bronchial mucosa. Patients with asthma or CF in particular may develop an allergic hypersensitivity reaction to the fungus. The result is ABPA, which occurs i 5-10% of corticosteroid dependent asthmatics. patients with ABPA have a very high serum IgE levels, eosinophilia, and IgE plus IgG serum antibodies to Aspergillus. Repeated exacerbating may produce transient pulmonary infiltrates and proximal bronchiectasis. a. although viral respiratory infections can cause asthma exacerbations and adenovirus colonization of the lung may be associated with asthma chronicity, pure viral pneumonias do not progress to bronchiectasis c. asthmatics do not have a predisposition to lung colonization with Legionella d. pseudomonas aeruginosa is an opportunistic, aerobic gram neg rod that is a frequent, and sometimes deadly, pulmonary pathogen in patients with CF and neutropenia e. although asthma may be a risk factor for pneumonocccal infections. It is generally result in resolution with preservation of lung lobular architecture. Potential complications of penuumococcal bronchopneumonia or lobar pneumonia include lung abscess, empyema and lung fibrosis. Bronchiectasis is not a common outcome. f. strongyloidiasis is typically asymptomatic but can sometimes cause pulmonary symptoms or transient pneumonia as larvae migrate through the lungs on their way to the laryngopharynx.

665 A 34 y.o woman has recurrent transient pulmonary infiltrates. She has a history of bronchial asthma and has had several exacerbation over the past few years, particularly during the winter months. She has no other medical problems and has never traveled outside the US. Her meds include albuterol. CBC shows eosinophilia. CT scan reveals proximal bronchiectasis. a. adenovirus b. aspergillum fumigates c. legionella pneumophila d. pseudomonas aeruginosa e. strep pneumonia f. strongyloides stercoralis

Polymyositis: -symmetrical proximal muscle weakness. -Path features: elevated muscle enzymes (CK, aldolase). Autoantibodies (ANA, anti-Jo-1). -Biopsy endomysial mononuclear infiltrate, patchy necrosis, regeneration and fibrosis of muscle fibers. -Associated complications: interstitial lung disease and myocarditis. Most likely triggered by viral antigens d/t MHC I antigens and CD8 cytotoxic cells. d. polyarteritis nodosa is a systemic vasculitis with intermitted episodes of abdominal pain, peripheral neuropathy, renal insufficiency, and severe HTN. Biopsy is characterized by transmural inflammation of the arterial wall with fibrinoid necrosis e. polymyalgia rheumatic would cause myalgia of the shoulder and pelvic girlde muscles, of with systemic symptoms (eg, fever, weight loss). However, weakness is not typically seen. Occurring exclusively in people over 50 y.o

748 A 43 y.o man has muscle weakness. Worst in hips and shoulders. Started 6 weeks ago. Has difficulty rising form chairs and combing his hair. Muscle biopsy reveals major histocompatibility complex class I molecule over expression on the sarcolemma with Cd8 lymphocyte infiltration. Which of the following is the most likely cause of this patient's condition? a diffuse systemic sclerosis b. eaton-Lambert syndrome c. Myasthenia gravid d. polyarteririts nodosa e. polymyalgia rheumatic f. polymyositis

Patient has a lingual thyroid. Thyroid gland si formed by an out pouching (evagination) of the pharyngeal epithelium and subsequently descends to the lower neck anterior to the upper trachea and larynx. The lowest part of the evagination forms the thyroid gland and the remaining portion forms the thyroglossal duct. D/t failure of migration, the thyroid can reside anwyereh along the thyroglossal duct's usual path, including tongue. Enlargement (of lingual thyroid can lead to obstructive symptoms (eg, dysphagia, dysphonia and dyspnea), typically during times of heightened thyroid stimulation (eg, puberty and pregnancy). a. apoptosis is a process of programmed cell death and is important in regulation the turnover of cells. Inappropriate apoptosis is responsible for number endocrine disease, including type 1 DM. b. differentiation if the formation of mature cells from undifferentiated precursor cells (eg, formation of RBCs, WBCs and palette form BM stem cells). Although thyroid cells do differentiate after their descent from the pharynx, defective differentiation is not responsible for lingual thyroid. c. Fusion is the joining of cell lines form different origins to form a single organ system. The mature thyroid formed by fusion of thyroid follicular cells (derived from the pharyngeal epithelium) and parafocllicular C cells (origination in ultmiobranchial bodies) as the thyroid descends through th neck. e. Proliferation denotes an increase in number of cells by mitosis. Some organs (such as liver, GI epithelium, BM) require high proliferative rates, wherascardiac and neuronal cells have a low priferative capacity. Failure of proliferation in BM leads to aplastic anemia.

763 A 13 y.o is having progressive difficulty breathing, hoarseness and dysphagia. Symptoms began 6 months ago but have rapidly become worse in the last 4 weeks. Vital signs are normal. he is at 70th percentile for heigh and weight. Oropharyngeal examination reveal a large, red lingual mass. Biopsy shows thyroid epithelium. Which of the following embryologic processes is responsible for this patient's lesion? a. apoptosis b. differentiation c. fusion d. migration e. proliferation

Myxomas is the most common primary cardiac neoplasm arising in the left atrium 80% of the time. His: mucopolysaccharide storm, abnormal blood vessels, and hemorrhaging. Myxomas produce large amounts of vascular endothelial growth factors, which contribute to the angiogenesis, hemorrhaging and friability seen in these tumors. Myxomas also produce large amount son interleukin 6, thus patients frequently present with constitutional symptoms. Myxomas are often pedunculate and gelatinous and can become quite large. They tend to present with emboli and/or cardiovascular symptoms. The CVS symptoms are secondary to valve obstruction by the myxoma, which accounts for why symptoms are position dependent. a/b: coarse filamented, branching septet hyphae are found in fungal endocarditis, while Grampositive cocci in clusters are consistent with bacterial endocarditis Both are unlikely in a young woman with no history of prosthetic heart valves, valvular disease, IV drug use, immunosuppression, indwelling catheters or total parenteral nutrition. In addition, endocarditis typically causes a regurgitant murmur, with valvular vegetations seen on ECGs. c. AML can metastasize to the heart. However, patients with AML generally present with symptoms related to pancytopenia (eg, anemia, neutropenia, and thrombocytopenia), including weakness, easy fatgability, infections, ecchymoses, and epistaxis. e. the murmur is this patient is a mitral stenosis, which is commonly caused by rheumatic fever. Mitral stenosis result in afib and left atrial thrombus formation. However, this patient has positional dyspnea, fever, weight loss, and penduculated mass seenon ECG.

8296 A 22 y.o woman has worsening dyspnea. She has low grade fevers, a 15 pound weight loss and syncopal episodes over the last 3 months. Her SOB worsens when sitting and improves when lying down. Her lungs are clear. Cardiac auscultation reveals a low-pitched, mid-diastolic rumble at the cardiac apex. The remainder of her PE is normal. ECG reveals left atrialenlargement, and ECG shows large pedunculate mass attached to the left atrium. Histology would show what? a. coarse filamented, branching septet hyphae b. gram positive cocci in clusters c. malignant myeloid cells d. scattered cells with mucopolysaccharide storm e solid mass of plateles and fibrin

Pus consists of a thin, protein rich fluid, known as liquor puris, and dead leukocytes, primarily neutrophils. During infection, macrophages and surrounding endothelial cells release cytokines such as IL-8 that trigger neutrophils to enter the site of infection via chemotaxis. IL-8 also induces phagocytosis in neutrophils once they have arrived. a. bradykinin vasodilators, increases vascular permeability, stimulates nonvascular smooth muscle contraction, and mediates pain. b. C3a, C4a and C5a are are inflammatory anaphylotoxins that trigger histamine release from mast cells, resulting in vasodilation and enhanced vascular permeability. -C5a also recruits and activates neutrophils, monocytes, eosinophils and basophils. -C3a recruited and activates eosinophils and basophils, but no neutrophils. c. IL-3 is produced by activated T cells. Stimulates growth and differentiation of stem cells in bone marrow (hematopoiesis). e. IL-10 is anti-inflammatory cytokine produced by macrophages and Th2 cells. IL-10 limits production of pro-inflammatory cytokines (eg, INF-y, IL-2, IL-3 and TNF-alpha). f. Leukotriene C4 (and its relatives, leukotriene D4 and E) triggers intense vasoconstriction, increased vascular permeability and bronchospasm. Leukotriene B4 and the leukotriene precursor 5-HETE stimulate neutrophil migration to the site of inflammation.

8539 A 37 y.o mantas increasing pain and tenderness in his right forearm. During a bar crawl he suffered from several deep laceration through the skin and subcutaneous. It is infected. PE shows erythema surround the wound site and expression of yellow pus when pressure is applied adjacent to the wound. Which of the following molecules is most likely involved in mediating the accumulation of pus? a. bradykinin b. C3a c IL-3 d. IL-8 e. IL-10 f. Leukotriene C

Patient has secondary amenorrhea = functional hypothalamic amenorrhea (FHA). Pathophysiology: low adipose tissue > low leptin levels > inhibition of pulsatile gonadotropin-releasing hormone please from the hypothalamus. b. hyperthyroidism can cause irregular menses or amenorrhea, along with weight loss. However, this patient has no other features to suggest a thyroid disorder (eg, goiter, temp intolerance, diarrhea, hair loss).

925 A 16 y.o girl who is in her high school soccer team and competes in local beauty pageants is experiencing amenorrhea. Her last menstrual period was 8 months ago. Monarch was at 13. Her BMI is 16.6 kg/m2. PE shows a pale and thin girl with fine hair around her trunk. Which of the following is most likely mech of her amenorrhea? a. hyperprolactinemia b. hyperthyroidism c. hypothalamic suppression d. primary ovarian failure e. primary pituitary dysfunction.

Type 1 (alpha) error: falsely conclude there is a difference, meaning you incorrectly rejected the null hypothesis. Type 2 (beta) error: you falsely conclude there is NO difference, meaning that you incorrectly accepted the null hypothesis. The probability of a type II (beta) error si related to how much power a study has to detect a difference when a difference actually exists (power = 1 - beta). Sample size and power are related in that studies with larger sample size has a greater power to detect differences if these exists. a. Bergson's bias refers to selection bias that can be created by selecting hospitalized patients as the control group b. placebo effect refers to patient's expectations affecting an outcome. However, in this case, the control group is taking neither HRT nor a placebo med c/d: given the RR obtained in the first study that was similar to the one obtained in metaanalysis, it is unlikely that design flaws (eg, poor blinding, researcher expectancy) were present in the study.

1303 Researchers studying the effects of hormone replacement therapy (HRT) on risk of MI. IN a study where alpha cutoff value is 0.05, they were unable to find statistical significant increase. However, in a meta-analysis they were able to find statistical significant. What type of error did they have the first time. And what is the problem? a. Bergson's bias b. placebo effect c. poor blinding d. researcher expectancy e. sample size

Type 1 interferon (alpha and beta) are synthesized by most human cells in response to viral infections. These binds to infected and neighboring cells (autocrine/paracrine signaling), resulting in halting of protein synthesis via transcription of antiviral enzymes such as RNase L (endonuclease that degrades all RNA in the cell) and protein kinase R (inactivate eIF-2, inhibiting translation initiation) However, these enzymes become active only in the presence of double-stranded RNA, which forms in infected cells as a result of viral replication. As a result, normal metabolism and protein synthesis can continue in uninfected cells but is selectively inhibited in virally infected cells. a. interferons alpha and beta induce MHC class I expression on all cells and stimulates the activity of NK and cytotoxic T cells. These processes act to increase the proportion of virally infected cells that undergo apoptosis c/d: interferon y is a type II interferon produced mainly by T cells and NK cells. It promotes Th1 differentiation, increases expression of class II MHC molecules, and improves the intracellular killing ability of macrophages. However, virally infected respiratory epithelial cells would secrete interferons alpha and beta, not interferon gamma e. neutrophils primarily phagocytize and destroy bacterial and final pathogens. They do not play a significant role in fighting viral infection, and alpha and beta interferon release by virally infected cells doesn't promote neutrophil recruitment.

1468 A 45 y.o woman has fever, headache, severe muscle aches, and sore throat for the last 4 days. PE shows mild pharyngeal erythema and nasal congestion. A rapid influenza antigen test is positive. The patient's condition improves over the next several days despite receive only symptomatic treatment. In response to the influenza virus, infected respiratory epithelial cells begin secreting increased quantities of interferons. the specific interferons secreted by these cells will most likely cause which of the following changes? a. decreased apoptosis of infected cells b. decreased protein synthesis by infected cells c. increased class II MHC expression d. increased intracellular killing by macrophages e. increased neutorphil recruitment.

Alkaptonuria is AR def of homogentisic acid deoxygenase. Tyrosine is made from phenylalanine (via phenylalanine hydroxylase/BH4). It can then be used to make DOPA (turns into melanin and catecholamines) or turn into homogentisate then maleylacetoacetate, then fumarylacetoacetate and ultimately fumarate to enter TCA cycle. Without homogentisic acid deoxygenase, homogentisate cannot be converted into maleylacetoacetate. Accumulation of hemogentisic acid deposits in connective tissues of the body causing joint pain, blue/black deposits in sclerae and ear cartilage. Urine will turn black when exposed to air d/t oxidization of homogentisic acid. c. osteoarthritis is d/t combined genetic, metabolic and mechanical factors causing defects in articular cartilage. Joint pain peaks in afternoon or evening after activity. d. reactive arthritis is usually from enteric or UTIs such as Slamonella, shigella, campylobacter and chlamydia. Pattern is often -assymetric -enthesitis (inflammation at insertion of tendons)

1503 A 3.8 y.o man has pain in multiple joints. He has 5 year history of lumbar pain and a 2 year history of bilateral knee pain. PE shows blue-black spots on his sclerae and diffuse darkening of the auricular helices. Which of the following is most likely cause of this patient's arthritis? a. homogentisic acid deoxygenate def b. hyperuricemia c. multifactorial articular cartilage failure d. recent infection with Salmonella e. tyrosinase def

Phase 0 (rapid depolarization) -Na channels opening. -Class I anti arrhythmic (eg, procainamide, flecainide, lidocaine). Phase 1 (early depolarization) -K efflux w/rapid inward Na current. -Not modulated by antiarrhythmics Phase 2 (plateau phase) -Balanced by influx of Calcium and efflux of K -Class IV antiarrythmics (CCB, verapamil, diltiazem). Blocks L-type channel. They slow sinus rate and prolong conduction through AV node. Also has negative inotropic effect Phase 3 (late repol) -opening of K channels -Class III antiarrythmics (eg, amiodarone, sotalol, dofelitide). Causes prolongation of depolarization, AP duration and QT interval ECG. Phase 4 (recovery) -antiarrhythmic drugs do not affect phase 4 of myocyte AP, but beta blockers and calcium channel blockers can slow diastolic depolarization in pacemakers.

1507 A 45 y.o man has recurrent palpitations accompanied by chest discomfort and shortness of breath. He was diagnosed a year ago with paroxysmal atrial fibrillation treated with rate control using beta blockers. 24 hr Holter monitoring shows he has afib. He was given dofelitide to maintain normal sinus rhythm. Mediation exerts it main value on which of the following phases? a. phase 0 b. phase 1 c. phase 2 d. phase 3 e. phase 4

CF is AR affecting CFTR gene that codes for ATP-binding cassette transmembrane ion transporter that pumps chloride out of epithelial cells against a concentration gradient using ATP. deltaF508 mutation, a deletion of 3 nucleotides coding for phenylalanine at position 508 located on chromosome 7. By pumping chloride, the CFTR establishes a membrane potential that draws sodium and water across the membrane as well. This hydrates the mucosal surfaces like the airways and bowels. Through this mech, the CFTR also hydrates and promotes secretion from the cuts of the exocrine pancreas. IN addition, CFTR plays a role in formation of hypotonic sweat. In the eccrine gland, sweat is initially isotonic with plasma. During transport through the eccrine ducts, salt is normally removed form the ductal lumen by action of CFTR. patients with CF, however, have an elevated sweat chloride level d/t CFTR defect. Sweat chloride test is frequently used to screen for CF.

1514 A 3 y.o caucasian male has history of recurrent pneumonia and weight loss has a high sweat chloride content. The dysfunctional transmembrane protein in this patient is of which of the following types? a. Voltage-gated b. ATP-gated c. G-protein activated d. Ca+2 gated e. cGMP gated

First part of the duodenum emerges from the pylorus of the stomach and is horizontally oriented over the first lumbar vertebra. It is the only part of the duodenum that is not retroperitoneal. Second part of the duodenum coursed inferiorly from the lvl of L1 to L3. this part of the duodenum is close relation to the head of the pancreas and contains the ampulla of Vater, the site where pancreatic and common bile duct secretion are released. Third part of the duodenum coursed horizontally over L3, the abdominal aorta and the inferior vena cava. It is in close association with the uncinate process of the pancras and the SMA and vein. Small bowel malignancies are aware; if they occur in the third part of the duodenum, anterior tumor invasion could compromise the superior mesenteric vessels. Fourth part of the duodenum courses superiorly and to the left of L2 and L3 vertebrae and becomes the jejunum past the pigment of Treitz. a. The common bile duct is formed when the common hepatic duct and cystic duct join in the aorta hepatic region of the hepatoduodenal ligament. The common bile duct courses inferiorly, posterior to the first part of the duodenum and within the head of the pancreas, to drain into the second part of the duodenum. b. The gastroduodenal artery arises from the common hepatic artery and courses inferiorly, posterior to the first part of the duodenum, where it splits into the anterior superior pancraticoduodenal artery and the right gastroepiploic artery Peptic ulcers involving the posterior duodenal bulb can erode into the gastroduodenal artery and cause bleeding c. The portal vein is formed by the union of the superior mesenteric and splenic veins in the retorperitoenum. It is located posterior to the pancreas at the level of the first lumbar vertebra and makes no contact with the duodenum e. the left ureter has no relation to the duodenum. the right ureter courses retroperitoneally, posterior to the second part of the duodenum, for a short length.

1832 A 65 y.o man has several months history of abdominal pain and vomiting. The pain starts 2 to 3 hours after meals, is characterized as crampy, and is often associated with billows vomiting. He lost 12 lbs in the past 4 months. On PE, he has mild epigastric tenderness on deep palpation. Contrast-enhanced CT scan of the abdomen shows an irregular mass in the third portion of the duodenum that is infiltrating beyond the gut wall. If this mass continues to enlarge, which of the following structures is most likely to be compromised in this patient? a. common bile duct b. gastroduodenal artery c. portal vein d. SMA e. ureter

Apoptosis can be triggered by a number of things such as deprivation of growth factors, DNa damage, intracellular accumulation of misfiled proteins, mediation by cytotoxic T lymph, and activation of receptors in the TNF receptor family (such as Fas). Fas receptors initiation the extrinsic pathway of apoptosis through a cytoplasmic component known as the death domain. Upon biding Fas ligand, the receptors trimerize, allowing the death domains to form a binding site for an adapter protein called Fas-associated death domain (FADD). Receptor bound FADD then stimulates the activation of initiation caspases (8 & 10) that begin an activation cascade culminating the activation of executioner caspases (3 & 6). These initiate the terminal processes of apoptosis, including cleavage of DNA, fragmentation of the nucleus, organelle auto digestion and plasma membrane blebbing. The Fas receptor is expressed on T-lymphocytes and play an important role in pathogenesis of numerous diseases, including cancer and autoimmune. Once activated, T lymphocytes begin to express FasL, which can bind to Fas on the same cell or adjacent lymphocytes. During intimal clonal expansion, activated T lymphocytes are resistant to Fas-induced apoptosis. However, they become more sensitive with progressive stimulation. In the contrast presence of stimulating self-antigens, activated T lymph eventually undergo apoptosis in a process known as activation-induced cell death. Mutations involving Fas or FasL impair this process, resulting in excessive accumulation of auto reactive Tcell and the development of autoimmune disease such as SLE. b. As B lymph undergo affinity maturation, cell that exhibit a stronger affinity for the antigen (which acts a limited growth resource) are able to proliferate more than cells with lower affinity. This results in cells that are more efficient and accurate in binding to pathogen. Affinity maturation does' tinovvle the Fas pathway c. Anergy is state of prolonged unresponsiveness that occurs in T lymph as a form of immune tolerance. It occurs when self-reactive T cells bind MHC molecule without receiving the necessary costimulatory signal (i.e., binding of CD28 on T cells with the B7 on antigen presenting cells). d. isotope switching is the process through which activated B lyme switch production from IgM immunoglobulins to IgG and IgA. This process requires the interaction of CD40 on activated B cells with CD40 ligand expressed by activated T cells and is modulated by cytokines secreted by T cells. e. on exposure to an antigen, naive T helper (Th0) cells dedifferentiate into Th1 (cell-mediating) and Th2 (antibody mediating) subtypes based on the local cytokine milieu (environment) IFN-y and IL-12 induce Th1 formation; IL-4 stimulate Th2 development

298 A 23 y.o woman has migratory joint pains involving her hands and knees. PE shows bilateral tenderness in her wrists and proximal interphalangeal joints. There is also a molar skin rash and generazlied lymphadenopathy. A urinalysis reveals proteinuria. Further evaluation shows that the patients lymphocytes contain a mutated and functionally defective Fas gene product. Which of the following immunologic mech is most likely impaired in this patients as a result of this molecular defect a. activation induced T lymphocyte death b. affinity anergy of T lymphocytes c. clonal anergy of T lymphocytes d. isotope switching of B lymphocytes e. Th1 and Th2 lyme differentiation

Inhaled anesthetic hepatoxociity most commonly associated with halothane, which remains one of the world's most commonly used inhaled anesthetics worldwide. In US it has been largely replaced with other halogenated anesthetics such as enflurane, isoflurane, desflurane and sevoflurane. Damage liver will cause rapid atrophy. Histo would show widespread centrilobular necrosis and inflammation of portal tracts and parenchyma. Labs: elevated serum aminotransferase, prolonged PT, leukocytosis and eosinophilia. prolonged PT is from def of factor VII (has the shortest half life). a. albumin level is affected in chronic liver failure because they have long half life. b/d: distended abdominal veins and ascites along with palma erythema is seen in chronic hepatitis and fibrosis. f. splenomegaly develops secondary to portal hypertension and can be seen in cirrhotic patients. Not acute hepatitis

369 A 55 y.o woman has nausea, fever, fatigue and anorexia. She had a cholecystectomy two weeks ago in Mexico. She died 2 days later. Postmortem viral serologies are negative. Liver is atrophic. Which of the following additional findings would have most likely been found in this patient? a. decreased serum albumin level b. distended abdominal veins and ascites c. normal ALT d. palmar erythema e. prolonged PT f. splenomegaly

Tamoxifen and raloxifene are selective estrogen receptor modulators (SERMs). They interact with estrogen receptor and have agonist or antagonist activity depending on the tissue. In breast tissue, tamoxifen has an anti estrogenic effect and it is sued for adjutant treatment of estrogen receptor positive breast cancer. Tamoxifen reduces the risk of recurrentt cancer as well as estrogen dependent benign breast lesions (eg, fibroadenoma, cystic changes). In endometrial tissue, however, tamoxifen has a stimulatory effect can lead to development of endometrial hyperplasia and endometrial cancer. This risk is not seen with raloxifene. Other adverse effects: hot flashes, venous thromboembolism a. tamoxifen and raloxifene act as partial estrogen receptor agonists in bone and can cause an increase in bone mineral density after menopause. b/e: tamoxifen has a favorable effect on serum lipids, with a decrease in total and LDL cholesterol and no significant change in HDL. Serum TAGs may increase in some patients.

581 Patient is taking tamoxifen. Which of the following conditions is the patient also likely to experience as a result of this therapy? a. decrease in bone mass b. decrease in HDL level c. endometrial hypeplaisa d. fibroadenoma of the breast e. increase in LDL

Age-related Macular Degeneration (AMD) -genetically predispose with smoking and advanced age as risk factors. -Dry AMD is d/t chronic oxidative damage. Gradual vision loss. Drusen deposits. -Wet AMD d/t progressive ECM accumulation that leads to hypoxia, that stimulates VEGF production and causes sub retinal neovascularization with formation of leaky vessels. Acute vision loss w/ metamorphosis (distortion of straight lines). Funduscopy shows grayish-green sub retinal discoloration with adjacent fluid/hemorrhage. Treatment: Wet and Dry: antioxidant vitamins and zinc. counsel for smoking cessation. Wet AMD: VEGF inhibitor (eg, ranibizumab and bevecizumab). a. Therapy against CD20 glycoprotein on B cell (eg, ritixumab) use to treat lymphoma and RA. b. Epidermal growth factor receptor inhibitors (eg, erlotinib, gefitinib) are used to treat advanced non-small cell lung caner. c. anti IL-2 is for immunosuppression in organ transplant patients and in conditions such as graft versus host disease d. TNF-alpha inhibitors: RA, inflammatory bowel disease and seronegative spondyloarthropathies. Basically autoimmune conditions.

7721 A 67 y.o smoker has 2 week history of blurry and distorted vision in his right eye. PMH is unremarkable for DM and HTN. Ophthalmologic examination shows discoloration of the macula with areas of adjacent hemorrhage. Treatment should be targeted at? a. cd20 lymphocyte glycoprotein e. epidermal growth factor receptor c. IL-2 d. TNF-alpha e. vascular endothelial growth factor

Hypercalciuria is the most common risk factor for calcium stones. However, these patins rain normocalcemic d/t intact regulation by vitamin D and PTH. a. hypercalciuria as a result of hypercalcemia can be caused by primary hyperPTH, sarcoidosis, malignancy and chronic academia. b/e: Hyperuricouria with hyperuricemia can happen in myeloproliferative disorder, tumor lysis syndrome, gout and Lesch-Nyhand syndrome. -High-protein diets typically cause hyperuricouria with normouricemia. -Hyperuricouria can cause uric acid stone formation and also predisposes to calcium nephrolithiasis (uric acid precipitation acts as a nidus for calcium deposition). d. hyperoxaluria can result from a diet high in oxalate (chocolate, nuts and spinach). Low calcium edits and intestinal malasporiton syndromes such as Croh disease can also cause hyperoxaluria as they both cause less calcium to be available to bind and trap oxalate in gut.

813 A 32 y.o man has severe right flank pain that radiates toward the groin. He has gross hematuria but not fever or pyuria. The right flank is tender to palpation. There is no costovertebral angle tenderness. Imaging shows stone in the middle of the right ureter. Which of the following is most likely to be seen on lab evaluation of this patient? a. hypercalcemia, hypercalciuria b. hyperuricemia, hyperuricosuria c. normoclacemia, hypercalciuria d. normocalcemia, hyperoxaluria e. normouricemia, hyperuricouria

Inflammatory leukocyte accumulation: 1. Margination: hemoconcentration & decreased wall shear stress. 2. Rolling: Sialylated glycoprotein on neutrophils binds to P-selectin and E-selectin on endothelium. 3. Activation: Chemokines leads to activation of integrins. 4. Tight adhesion/crawling: neutrophils beomce firmly attached to the endothelium via the binding of CD18 beta 2 interns (Mac-1 and LFA-1) to intercellular adhesion molecule -1 (ICAM-1) on endothelial cells. 5. Transmigration: after crawling, neutrophils eventually migrate out of the vasculature by squeezing in the between the cells via intern attachments and adherence to platelet endothelial cell adhesion molecules (PECAM-1). This protein is found primarily at the peripheral intercellular junctions of endothelial cells. 3 leukocyte adhesion def (LAD) syndromes have been identified whereby leukocytes cannot leave the vasculature to migrate into tissues under conditions of inflammation. All are rare, ARs. LAD type 1: absence of CD18 > inability to synthesize beta-2 integers MAC-1 and LFA-1, affecting tight adhesion/crawling and transmigration. Clinical manifestations include recurrent skin infections without pus formation, delayed detachment of the umbilical cord and poor wound healing. LAD type 2: is a milder condition, with no delay in the separation of the umbilical cord and less severe and fewer infections. Caused by impaired fucosylation of sialylated carb ligands prevents selection binding. LAD type 3: Impaired cytokine signaling prevents intern activation. Clinical similar to LAD type 1.

8480 There was a def in platelet endothelial cell adhesion molecule 1 (PECAM-1) gene. The protein product of this gene is mainly localized to specific areas on the endothelial cells. Absent expression of this gene will most likely affect which of the following neutrophil function. a. crawling b. margination c. rolling d. tight adhesion e. transmigration

Mitral regurgitation causes abnormal retrograde blood flow through mitral valve into the left atrium during systole. This cause increase in left atrial pressure (reflected as increase "v wave"). a. aortic regurg have retrograde blood flow from aorta into left ventricle during diastole. Thus left ventricular pressure would be high. Aortic systolic pressure would be low. b. aortic stenosis. Since the left ventricle has to push blood through a tinier hole, LV systolic pressure is often much higher than that of aortic systolic pressure c. mitral stenosis. left atrial pressure during diastole would be super high. e tricuspid regard would show increased right atrial and jugular venous pressure. Left heart pressures are not affected. Right heart failure signs are (eg, ascites, increased jugular venous pressure and peripheral edema).

945 Patient has exertion dyspnea and fatigue. She needs to sleep using 2 to 3 pillows a night. Has an elevated v-wave. what is your diagnosis? a. aortic regurg b. aortic stenosis c. mitral regard d. mitral stenosis e. tricuspid regurg

Aromatase is a key enzyme involved in steroidogenesis converting androstenedione into estrone and testosterone to estradiol. Aromatase def is an AR disorder the manifests early in embryonal life with: -High androgen -Low estrogen Levels in the female fetus Maternal virilization (eg, hirsutism) commonly occurs during pregnancy d/t the transfer of excess androgens into the maternal circulation. Affected newborn girls will have normal internal genitalia and ambiguous or malts-type eternal genitalia (eg. clittoromegaly, female pseudohermaphrodism). At puberty, impaired ovarian estrogen synthesis causes primary amenorrhea, osteoporosis, and tall stature (low estrogen delays fusion of the epiphyses) Men with aromatase def have tall stature and osteoporosis but no genital abnormalities. a. 5alpha-reducatse def results in decreased dihydrotestosterone (DHT), which is responsible for development of external male genitaliea (eg, scrotum, penis), affected boys are born with underdeveloped genitalia. b. 17alpha-hyroxylase def is a form of CAH (congenital adrenal hyperplasia). Symptoms result from decreased production of glucocorticoids and androgens (ambiguous genitalia in males) and increased production of mineral corticoid (sodium retention and hypertension). c. 21-hydroxlase defies the most common type of CAH. Symptoms is from increased androgen production (ambiguous genitalia in females) and decreased aldosterone synthesis (salt-wasting and hypotension). Maternal virilizaiton will not be seen d/t intact placental aromatase activity. e. HMG-COA reductase is the rate-limiting enzyme in cholesterol synthesis; it is not involved in sexual differential. Statins inhibit this enzyme and reduce endogenous cholesterol synthesis.

955 A 23 y.o woman has a full term neonate. PE shows ambiguous genitalia and clitoromegaly. Lab shows elevated testosterone and androstenedione. Karyotype testing shows 46,XX genotype. The mother experienced facial hair growth an voice deepening during the pregnancy. Which of the following enzymes is most likely to be deficient in the newborn? a. 5alpha-reductase b. 17alpha-hydroxylase c. 21-hydroxylase d. Aromatase e. HMG-CoA reductase

This patient has H. influenza. Introduction of Hib vaccine has led to a dramatic decrease in the incidence of invasive disease caused by H. influenza type b including epiglottis, meningitis, sepsis and other diseases commonly caused by this bacterium. a. recent travel can alert the physician to suspect disease that are endemic to the area that the patient traveled. Epiglottitis is not associated with travel. Malaria is associated with travel in Africa, HIV is rampant in Africa and Haiti, Coccidioides immitis is common in the southwest US, blastomycosis in the Mississippi river valley and Histoplasmosis common in the Ohio River valley. c. Bee sting can induce clinical disease ranging from local allergic reactions around the site of the sting to anaphylaxis and death. Bee venom contains hyaluronidase, phospholipase, and other proteins which initiate an IgE-mediated response d. penicillin allergy is not associated with epiglottis. Adverse reactions to penicillin can range from rash and hives to angioedema, bronchospasm, and anaphylaxis. e. Family history is not a factor in epiglottis except for cases of heritable immune system defects that predispose to recurrent bacterial infections, but these disorders would not predispose purely to epiglottis.

967 A 5 y.o boy is brought to the ER with breathing difficulty, dysphagia, drooling and fever. Temp is 103. WBC count is 23,000 with many band forms. Laryngoscopy in the operating room shows a swollen and cherry red epiglottis. This patient most likely: a. traveled recently b. missed vaccination c. was stung by a bee d. has penicillin allergy e. has characteristic family history

Lymphatics of extremities are divided into superficial lymphatic vessels that flows w/ venous system and deep lymphatic vessels that flow w/ arterial system. Lower limbs superficial lymphatics are divided into the medial and lateral system. Medial follows the great saphenous veins to the superficial inguinal lymph nodes, bypassing the popliteal. Lateral communicate with the popliteal and inguinal nodes. a. prostate = internal iliac b. lymph from scrotum = superficial inguinal lymph nodes. Testes = paraaortic (mirror testicular arteries) c. glans = deep inguinal nodes.

A 48 y.o man has right inguinal discomfort. He noticed the bumps in his groin when he was taking a shower. Had gonorrhea several years ago. Mother died of metastatic melanoma. PE enlarged, tender right inguinal lymph nodes and several nodes in the right popliteal area. What will you see in this patient? a. asymmetric, hard prostate nodules b. firm, nontender right testicular mass c. large irregular mole on the right great toe d. purulent abrasion on right lateral foot e. ulcerative lesion at the glans penis