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uworld 2
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| Question | Answer |
|---|---|
| paranasal sinuses, nasopharynx, layrnx, trachiobroncial tree epithelium | ciliated, psedustratitified, columnar, mucus-secreting epithelium |
| oropharynx, ant. epiglotis, upper post epiglottis, true vocal cords | stratified squamous epithelium, can grow HPV warts) |
| MR, long thin face w/prominent forehead and jaw. post-pubertal macroorchidism, large protruding ears, tooth crowding, arched palat | Fragile X syndrom |
| Pathogenesis of fragile X | FMR1 gene mutation on long arm of X chromosome. Premutation CGG repeats <200, phenotypically nml. Full mutation >200 repeats that causes hypermethylation of FMR1 leading to inactivation. |
| DNA repair defects | chromosomal instability disorders :xeroderma pigmentosa, ataxia-telangiectasia, fanconi's anemia, and bloom syndrome |
| eczema, recurrent infections, thrombocytopenia. x-linked disorder | Wiskott-alrich syndrome (capsular organism infx due to humoral deficiency and PCP and herpes infxn due to T cell dysfxn) |
| oculocutaneous albinishm, peripheral neuropathy, and immunodeficiency | chediak0higashi syndrome (phagosome-lysosome fusion dysfxn) |
| Ataxtia-telangietasia | progressice ataxia with telangiectasas. and immunodeficiency due to combined B and T cell defect |
| immunity to M. Tb | cell mediated immune |
| leukotriene receptor | monteleukast, safleucast |
| TCAs toxocity | HAM, Cardiotoxicyty, coma, convulsions. tx cardiotax with NaHCO3 |
| MHC class I loading | HLA A, B, C, antigen peptides loaded onto MHCI in RER after delivery via TAP peptide transporter. B2 |
| failure to thrive, developmental delay, . microcephaly, tachypniea, and hypotonia. lab: hypoglycemia, hyperalanemia, lactic acidosis | gluconeogenesis |
| inherited lysosoal storage disorder. coarse facial feastures, clouded corneas, restricted joint movement, high lysosomal enzymes | I cell disease |
| osteopetrosis | carbonic anhydrase II deficiency-->defecive osteoclast, thick dense bones prone to fracture. pancytopenia, extramedullary hematopoiesis. CN impingemnet.. tx with transplant |
| peripheral neuropathy of hands and feet, aniokeratomas, die of renal diease | fabry |
| inspiration and murmurs | increased intensity of right heart murmurs |
| hand grip | increase intensity of MR, AR, VSD. decreases AS, hypertrophic cardiomyopathy |
| valsalva (phase II), standing (dec. VR) | decrease intensity of most murmurs (AS), increased hypertrophic murmurs |
| rapid squating | dec. hypertrophic cardiomyopathy, increase AS |
| holosystolic, high pitched blowing murmur, loudest at apex and radiates toward axilla. | MR, enhanced with TPR. due to ischemic heart disease, MVP, LV dilations |
| crescendo-decrescendo systolic ejection murmur. LV>>aortic pressure during systole. loudest at heart base | mitral regurge |
| watershed infarcts b/w anterior and middle cerebral a. from global interruption of the cerebral blood supply | hypoxic encephalopathy |
| aneurysms <1mm occue in pt with long standing HTN. found in arterioles. can rupture and cause intracerebral hemmorrhage. foun in the arteroils of basal ganglia, internal capsule, and deep white matter | charcot-bouchard aneurysms |
| components of cryoprecipitate | only cold soluble protiens (factor VII, fibrinogen, von willebrand factor, and vitronectin) |
| tx of von willebrand | desmopressin |
| hemoglobin M disease | due to a mutation in the heme binding pocket of the alpha or beta chain. replacement of histidine in the heme pocket with tryosine. result in formation of an iron phenolate complex that resists the reduction of iron to ferorus state. |
| five things needed to perform PCR | DNA template: the region of DNA to be amplified. (10kb). 2. two primers sequences complementatory to regions flanking DNA. 3. DNA pol 4. dNTP |
| PCR steps | 1. denature at 98C 2. annealing: primers combine with flankin DNA at 60C 3. elongation: DNA pol |
| what can cause bruise to turn blue | heme oxygenase converts heme to biliverdin (green pigment). lysis of erythocytes causes release of hemoglobin into soft tissues. |
| mental retardation, eczema, mousy, musty body odor | PKU: due to mut in phenylalanine hydrozylase. AR. |
| inactivation of HAV | water chlorination, bleach, formalin, uv irradiation, boiling (85C) for 1 min |
| tumor lysis syndrome and tx | chemotherapy causes destruction of cells leading to release of intracellular ions (K+ and PO4 leading to arrhytmias) along with nucleic acids-->catabolized to uric acid (obstructive uropathy). tx with allopurinol or rasburicase. |
| Rasburicase | recombinant version of urate oxidase, catalyzes the converstion of uric acid to allantoin which is more soluble. effective in preventing and treating hyperuricemia and tumor lysis syndrome |
| Denosumab | humanized monoclonal ab that prevents osteoclast activation by binding to RANKL and preventing its interaction with RANK. decrease the risk of bone loss and fracture in bone metastasis pt |
| Probenecid | increased the exretion of uric acid in urine. tx of gout and hyperuricemia. C/I in pt at risk for nephrolithiasis or uric acid nephropathy |
| CCl4 | causes free radial injury, oxidized by p450 and reacts with lipids of cell membranes. causes promation of H202 via lipid peroxidation. can lead to swelling of RER, destruction of mitrocondria and permeability of cell membranes leading to hepatic necrosis |
| Ecoli virulence factors and disease | LPS: bateremia and spetic shock. K1 capsular polysaccharide-prevents phagocytosis (neonatal meningitis. Verotoxin (60s inactivation): bloody gasteroentritis. Heat stable/labile toxins (promote fluid loss): Watery gasteroentritis. P-fimbriae for UTI |
| severe HA, bitempolar hemianopsia, ophthalmoplegia, panhypopituitarism. +/- development of cardiovascular collapse | pituitary apoplexy (acute hemorrhage into the pit gland) common in pt with preexisting adenoma. CV collapse due to ACTH deficiency . tx w/ glucocoricoids. |
| pigmented dark gall stones in immigrant from central Asia | brown pigment due to secondary infxn (E. coli, ascaris lumbricoides, opisthorchis sinesis) of biliary tract, release of Beta glucuronidase by injured hepatocytes and bacteria. presence of enzyme contributes to the hydrolysis of bilirubin glucuronides |
| cardio abnml in down syndrome | endocardial cushion defect (ostium primum atrial septal defects, regurgitant AV valves) |
| DiGeorge cardio abnml | tetrology of fellot, interrupted aortic arch |
| fredric ataxia cardiac abnml | hypertrophic cardiomyopathy |
| marfan cardiac abml | cystic medial necrosis (aortic dissection and aneurysm), MVP |
| tuberous sclerosis cardiac abnml | valvular obstruction due to cardiac rhabomyomas. also has cutaneous angiofibromas, seizures, mental retardation, nervous system harmatomas, renal cysts. |
| tuner syndrom cardiac abnml | aortic coarctation and bicuspid aortic valve |
| location of femoral hernias | below the inguinal ligament, lateral to the pubic tubercle and lacunar ligament. medial to the fermoral vessels. prne to incareration (cant be reduced with bowel in the sac) impaired blood flow after a few hrs leads to strangulation. |
| direct inguinal hernias | lie medial to inf. epigastric vessels, middle border: rectus abdominis muscle, lower border: inguinal ligament, protrude through hasselbach triangle |
| colchicine | inhibits microtubular polymerization, disrupts membrane dependent functions such as chemotaxis and phagocytosis. reduces formation of LTB4. A/e: nausea/abd pain/ diarrhea. c/i in elderly and renal dysfxn |
| Glucocorticoids | inhibit phospholipase A1, responsible for the conversion of membrane phospholipids to arachidonic acid. decreased PG and LT. preferred for elderly and renal dysfxn in gout tx. |
| cerebellar atroophy in 1st year of life =ataxia. oculocutaneous telangiectasia. severe immunodeficiency with repeated sinopulmonary infections. increased risk of cancer due ineffective dna repair | Ataxia-telangiectasia AR. due to DNA hypersensitivity to ionizing radiation |
| inherited disorders cause by deficent DNA repair enzymes | ataxia telangiectasia, xeroderma pigmentosum, fanconi anemia (hypersenstive DNA to cross linking agents) Bloom syndrome (chr instability). HNPCC (mismatch repair) |
| 47 xxy, cause of hypogonadism, reduced spermatogenesis, male infertility. small atophied testicle replaced by hylalinized tissue. long legs, small penis, no deep voice, beard, or pubic hair. Gynecomastia. increased FSH and estradiol, and low testosterone | klinefelter syndrome |
| cystinuria | AR, due to defective transporter of disbasic aa (cystine, ornitiing, lysine, arginine) lifel ong recurrent urolithiasis. colicky flank pain and hematuria followed by passage of a stone |
| hypercalcemia, hypercalciuria, confusion, polyuria, polydipsia, anorexia, vomiting, muscle weakenss and painful bone demineralization. | vit d intoxication. pt with granulomatous disorders (saracoidsosis, tuberculosis, hodgkins disease, and non hodgin) develop hypercalcemia and hypercalciuria. |
| microcytic anemia, increased HbF. hypochromia and target cells. asymtomatic mediteranean heritage | beta thalassemia minor. (alpha thalasemia is more common in asians). due to trascription, prcessing, and translation of beta mRNA. most commonly caused by aberrant splicing of precursor mRNA or premature mRNA termination during translation |
| PTH fxn | PTH receptors present on bone and kidney tubules. stimulates formation of cyclic AMP. increased bone resorption by acting on osteoblast to make RankL and M-CSF which stimulate osteoclast. pth also decr. osteoprotegrin (decoy receptor for RANKL) |
| cause of squamous cell bladder cancer | schistomsoma haemotobium |
| causes of necrotizing fascitis | step pygenes, stabylococcus aueus, clostridium perfringence (and step agalactiae and aeomonas hydrophila) |
| G+ coci, coagulase negative, catalase, negative, pyrrolidonyl arylamidase (PYR) +. forms small colonis with a wide zone of beta hemolysis. senstive to bacitratcin | s. pygenes. transmited rapidly to deep layers of skin to produce hyaluronidase. M protein to evade phagocytosis. hemolysin O and S are cytotoxins that cuase hemolysis. pyrogenic exotoxin (superantigens) |
| coagulase and catalase negative, grow in chains. produce narrow zone of beta hemolysis that enchances when plated perpendicular to S. aureaus (CAMP test) | strep agalactiae |
| etanercept | TNFalpha inhibitor. tx of rheumatoid arthritis. a/e reactivation of TB/ |
| b antracis pathogenesis | spores inhaled and enter alveoli, ingested by macs, speard to lymph nodes cause heorrhagic mediastinitis. produces D glutamate in capsul. |
| cause of squamous cell bladder cancer | schistomsoma haemotobium |
| causes of necrotizing fascitis | step pygenes, stabylococcus aueus, clostridium perfringence (and step agalactiae and aeomonas hydrophila) |
| G+ coci, coagulase negative, catalase, negative, pyrrolidonyl arylamidase (PYR) +. forms small colonis with a wide zone of beta hemolysis. senstive to bacitratcin | s. pygenes. transmited rapidly to deep layers of skin to produce hyaluronidase. M protein to evade phagocytosis. hemolysin O and S are cytotoxins that cuase hemolysis. pyrogenic exotoxin (superantigens) |
| coagulase and catalase negative, grow in chains. produce narrow zone of beta hemolysis that enchances when plated perpendicular to S. aureaus (CAMP test) | strep agalactiae |
| etanercept | TNFalpha inhibitor. tx of rheumatoid arthritis. a/e reactivation of TB/ |
| b antracis pathogenesis | spores inhaled and enter alveoli, ingested by macs, speard to lymph nodes cause heorrhagic mediastinitis. produces D glutamate in capsul. |
| Statin | inhibit HMG CoA reductase, decrease cholesterol synthesis leads to upregulation of LDL receptors, causes increased uptake of LDL from circulation. |
| bile acid-binding resins (chlestyramine) | bind bilein the GI tract, interfere with recirulation of bile. result in increased hepatic synthesis of bile which consumes liver cholesterol stores. Hepatic uptake of LDL from circulation is increased. increased hmg-coa reductase (block w/ statins) |
| glucocoricoid effects in different tissues | -stimulated liver gluconeogenesis, increase glycogen syhtetase. -antagonise actions of insulin in muscle and adipose tiusse (catabolism: proteolysis and lipolysis). inhibit RNA and protein synthesis in many tissues, but stimulated in liver |
| dry mucalar degenelation | subretinal drusen deposites or pigment changes |
| wet AMD | abnml blood vessels with subretinal fluild hemorrhage, gray subretinal membrane or neovascularization. |
| tx of AMD | zinc, for more advanced wet AMD use VEGF inhibitors, laser therapy, or phototherapy |
| chronic upper extremitiy pain, and temp sensation, UE weakness and hyporeflexia, lower extremity weakness and hyperreflexia, kyphoscoliosis | syringomyelia causes damage to ventral white commisure and anterior horns. , further expansion can lead to the extremity weakness and hyperreflexia (UMN defect0 |
| pseudogout | calcium pyrophosphate crystals in the synovial fluid. usually in the knee. increased WBC with neutrophils. rhomboid shaped calcium phyophosphat cyrstals. positively birefringent under polarized light (blue and yello) |
| gout | monosodium urate deposits in the joints and soft tissues |
| filtration fraction | GFR/RPF |
| RPF | (1-hematocrit) (RBF) |
| thickned bronchial walls, neutrophil infiltrates, mucous gland enlargment and patchy squamous metaplasia of broncial mucosa | chronic bronchitis |
| drugs implicated in serotonin syndrome | antidepressants, tramadol, ondanstron, linozelid, and triptans |
| hyperrelexa, clonus, myoclonus, and rigidity, hyperthermia, tachycardia, diaphoresis, and tremor. AMS + SSRI | seratonin syndrome |
| acid fast staining | analine dye (carbofushin) penetrates bacterial cell wall and bind mycolic acids. tx with hydrochloric acid and alcohol to dessulve outer cell membrane of non tb bacteria. counter stain with methylene blue taken up by non acid fast bacteria |
| housemaids knee. chronic trauma from repeated kneeling. | prepatellar bursitis, located b/w patella and overlying skin and prepatellar tendone. |
| anserine busitis (pes anserinus bursitis) | pain along the medial aspect of the knee. results from overuse in athletes or from chonic trauma in pt with heavy body habitus. |
| drinking infected milk or direct contact with infected sheep and goat. fever, malaise, lymphadenopathy, hepatosplenomegaly | brucella melitensis |
| tetrodotoxin (puffer fish) or saxitoxin (dinoflagellates in red tide) | bind to voltage gated Na channels, inhibitor Na influx and prevent the action potential conduction (paresthesias weakness, dizziness, and n/v/d) |
| ciguatoxin (exotic fish, moray eel) or batrachotoxin (frogs) | bind voltage gaed Na chennlel and keep it open causing persistent depolaritation. |
| receptors on pancrease that increase insulin | M3, Glucogon, B2, and GLP-1 |
| receopts on pancrease that decrease insulin release | alpha 2, and stomatostatin 2. both are Gi coupled |
| mucosal and axillay dryness, fever, cutaneous flushing, mydriasis, cycoplegia, and delerium | anticholinergic toxicity. seen with amytriptyline and other TCAs |
| benzodiazepine overdose sx | sedation, anterograde amnesia, and respiratory depression. |
| a/e of nonselective Beta blockers | bronchial constriction, masked sx of hypoglycemia in diabetes, bradyarrhythmias, worsening of raynaud phenomenon and CNS depression |
| pathogenesis of analgesic nephropathy | nsaids concentrate in renal medulla, allow higher levels in the papillae than renal cortex. decreased PG synsthesis = constiction of vasa recta =ischemia. nsaids uncouple oxidative phosphorylation in renal mitochondria casuing direct cell damage. |
| chronic interstitial nephritis | patchy interstitial inflammation with fibrosis, necrosis and scarring of papillae. tubular atrophy on LM. incased cr. polyuria or nocuria. fanconi syndrome. papillary necrosis=colicky flank pain, gross hematuria, and passage of tissue fragments in urine |
| fanconi syndrome | aminoacidura, glycosuria, hypohphatemia, hypouricemia |
| kozak consensus sequence | gccRccAUGG (R is either adenine or guanine). kozak sequence serves as initiator for translation (mRNA binding to rbosomes). mutation in G replace with C in B-globin gene is associated with thalassemia intermedia |
| predisposing risk factors for first dose hypotension | hyponatremia, hypovolemia secondary to diuretic therapy, low baseline blood pressure, high renin or aldosterone levels, renal impairment, and heart failure. |
| ACE inhibitors + nonselective beta blockers | increased risk of hyperkalemia |
| MOA of ACE inhibitos (enalapril) | decrease circulating ag II (usually causes systemic casoconstriction, preferential constriction of eefect arteriole, and enhance adrenal aldosterone secretion). sever efferent arteriolar dilation and decreased systemic vascular resitance both reduce GFR. |
| CMV EBV HIV Rabies rhinovirus and associated cell receptor | CMV-cellular integrins EBV-CR2 (CD21) HIV-CD8 Rabies- N-ach rhinovirus-ICAM (CD54) |
| fxn of clara cell | non-ciliated, secretory constituents of the terminal resp, epithelium. secret clara cell secretory protein (CCSP) and may source of surfactant. detoxify inhaled toxins by a p450 mechanism. |
| NRTI that require viral TK phosphorylation | acyclovir, valacyclovir, famciclovir, Ganciclovir |
| NRTI that require host cell kinase phosphorylation only | Zidovudine, cytarabine, vidarabine |
| Moa of AZT | bind to reverse trancriptase and is incorported into the viral genome as a thymidine analog. inhibits 3-5' phosphodiesterase bond formation and prevents DNA chain elongation |
| life threatening arrhythmias, anorexia, n/v/abd pain, fatigue, confusion, wakness and color vision alteration. drug tox | digoxin toxicity occurs with hypokalemia (increased dig binding), hypovolema, and renal failure. |
| femoral triangle | inguinal ligament superiorly, sartorius muscle laterally and adductor longus muslce medially. |
| intractable diarrhea, metabolic acidosis, hypokalemia, due to tumor | VIPoma |
| jejunal ulcers and other upper GI ulcers, abdominal pain, and diarrhea | gastrinoma |
| drug induced acute interstitial nephritis | b-lactam abx, nsaids, sulfanomids, rifampin, and diuretics. invovles interstitium, glomeruli intact. mononulcear infiltrates. +/- plasma and eosinophils. may cause tubulorrhexis w/ foreign body granuloma. increased IgE. |
| polymyositis | immune mediated myopathy. due to increased expression of MHC class 1 protiens by muscle cells. infiltration of endomysium by macrophages and CD8 lymphocytes. symetrical porx muscle wakenss. incr. cr. and anti jo-1 ab. |
| polymyalgia rheumatical | bilateral stiffness of sholder and pelvic girdle muscle muscle, fever, weight loss, increased ESR. associated with tempral arteritis |
| homonymous hemianopia with macular sparing | occulusion of PCA: supplies the occiital lobe which contains the striate or primary visual cortex. cuneus gyrus recieves from upper retina (lower visual field) and lingal gyrus receives info from the lower retina (upper visual field). |
| homonymous hemianopia with macular involvement | MCA w/ contralateral motor and sensory deficits |
| diffuse skin thickening, cutaenous hypopigmentation in plaques (w/ hair loss), leonine facies, paresis and regional anesthesia of motor and sensory nerves. testicular destruction and blindness | lepromatous leprosy.occurs in pt with a weak cell mediated (Th1) response, w/o t response macs can't kill. grows primary in skin, superficial nerves, eyes and testes. |
| cyclic breathing in which apnea is follwed by gradully increasing tidal vlumes and then gradualy decreasing tidal volum until the next apneic period | cheyn-stokes respiration. seen in cardiac disease (advanced CHF) and neuro disease (stroke, tumors, trauma). poor prognosis |