Usmle Step 1 First Aid Questions & Answers(RATED A)

polyostotic fibrous dysplasia, precocious puberty, cafe-au-lait spots, short stature, young girls - diagnosis? -ANSWER Albright's syndrome blue-domed cysts - diagnosis? -ANSWER fibrocystic change of the breast Wermer's syndrome - also known as? -ANSWER MEN type I "wire loop" appearance on Light microscopy- diagnosis? -ANSWER lupus nephropathy what is the defect in Job's syndrome? -ANSWER neutrophil chemotaxis abnormality - due to failure of IFN-gamma production by helper T cells xanthochromia of CSF - diagnosis? -ANSWER subarachnoid hemorrhage 4 most common brain tumors in adults -ANSWER mets > astrocytoma (including glioblastoma multiforme) > meningioma > schwannoma most common supratentorial brain tumor in kids -ANSWER craniopharyngioma most common bug in debilitated, hospitalized pneumonia patient -ANSWER Klebsiella pseudopalisade tumor cell arrangement - diagnosis? -ANSWER glioblastoma multiforme pseudorosettes - diagnosis? -ANSWER Tumour(Ewing's sarcoma) nutmeg liver - diagnosis? -ANSWER CHF "Orphan Annie" nuclei - diagnosis? -ANSWER papillary carcinoma of the thyroid painless jaundice - diagnosis? -ANSWER pancreatic cancer (of head) most common liver disease -ANSWER alcoholic liver disease "brown tumor" of bone - possible diagnoses? - what causes the brown color? -ANSWER hyperparathyroidism or osteitis fibrosa cystica (von Recklinghausen's disease) - color is due to hemorrhage in cysts renal epithelial casts in urine - diagnosis? -ANSWER acute toxic/viral nephrosis most common location of brain tumors in kids -ANSWER infratentorial cardiomegaly with apical atrophy - diagnosis? -ANSWER Chagas' disease most common mets to bone -ANSWER breast, lung, thyroid, testes, prostate, kidney most common mets to brain -ANSWER lung, breast, skin (melanoma), kidney (renal cell carcinoma), GI most common mets to liver -ANSWER colon, gastric, pancreatic, breast, lung carcinomas most common motor neuron disease -ANSWER ALS Charcot's triad of cholangitis -ANSWER jaundice, RUQ pain, fever Charcot-Leyden crystals - diagnosis? - what are they? -ANSWER bronchial asthma - eosinophil membranes what is the defect in Chediak-Higashi disease? -ANSWER phagocyte deficiency S3 - possible diagnoses? -ANSWER left-to-right shunt (VSD, PDA, ASD), mitral regurgitation, CHF (LV failure) what are Cheyne-Stokes respirations, and in what circumstance do they occur? -ANSWER central apnea in CHF and increased intracranial pressure Schiller-Duval bodies - diagnosis? -ANSWER yolk sac tumor what does chronic atrophic gastritis predispose to? -ANSWER gastric carcinoma what is Sezary syndrome? -ANSWER cutaneous T-cell lymphoma DES (diethylstilbesterol- prescribed to pregnant women in 60s) exposure in utero predisposes to what? -ANSWER clear cell adenocarcinoma of the vagina cold agglutinins - possible diagnoses? -ANSWER Mycoplasma pneumonia or infectious mononucleosis Schwartzman reaction - associated with? -ANSWER Neisseria meningitidis signet-ring cells - diagnosis? -ANSWER gastric carcinoma Councilman bodies - diagnosis? -ANSWER toxic or viral hepatitis Cowdry type A bodies - caused by what? -ANSWER herpesvirus hypochromic microcytosis - possible diagnoses? -ANSWER iron deficiency anemia, lead poisoning periosteal elevation on x-ray - diagnosis? -ANSWER pyogenic osteomyelitis benign polyposis - diagnosis? -ANSWER Peutz-Jeghers syndrome penile fibrosis - diagnosis? -ANSWER Peyronie's disease: an inflammatory, benign condition that may cause a curvature, deformity or shortening of the erect penis most common organs sending mets -ANSWER lung > breast, stomach ferruginous bodies - diagnosis? -ANSWER asbestosis string sign on x-ray - diagnosis? -ANSWER Crohn's disease idiopathic peripheral polyneuritis - diagnosis? -ANSWER Guillain-Barre syndrome most common ethnic group affected by cystic fibrosis - what dietary deficiency is associated with CF? - what lung problems occur? -ANSWER Caucasians - fat-soluble vitamin deficiencies - mucous plugs and lung infections most common heart murmur -ANSWER mitral valve prolapse what is Hand-Schuller-Christian disease? -ANSWER chronic progressive histiocytosis (Langerhans cell histiocytosis) Reinke crystals - diagnosis? -ANSWER Leydig cell tumor most common helminth infection in the U.S. - 2nd most common? -ANSWER Enterobius vermicularis - Ascaris lumbricoides is 2nd most common renal cell carcinoma, cavernous hemangiomas, adenomas - diagnosis? -ANSWER von Hippel-Lindau disease hypersensitivity vasculitis associated with hemorrhagic urticaria and upper respiratory tract infections - diagnosis? -ANSWER Henoch-Schonlein purpura most common tumor of infancy -ANSWER hemangioma Charcot's triad of multiple sclerosis -ANSWER nystagmus, intention tremor, scanning speech (where the same emphasis is put on each syllable like scanning a poem) most common causes of DIC -ANSWER Gram-negative sepsis, obstetric complications, cancer, burn trauma most common cause of myocarditis -ANSWER coxsackie B Kussmaul hyperpnea - diagnosis? -ANSWER diabetic ketoacidosis Lisch nodules - diagnosis? -ANSWER neurofibromatosis (von Recklinghausen's disease) polyneuropathy, cardiac pathology, and edema - diagnosis? - what causes it? -ANSWER wet beriberi (vitamin B1 deficiency) most common malignancy associated with noninfectious fever -ANSWER Hodgkin's disease port-wine stain - diagnosis? -ANSWER hemangioma what is Trousseau's sign of malignancy? - possible diagnoses? -ANSWER migratory thrombophlebitis - visceral cancer or pancreatic adenocarcinoma Homer Wright rosettes - diagnosis? -ANSWER neuroblastoma what is Trousseau's sign of latent tetany? - diagnosis? -ANSWER carpal spasm - hypocalcemia what is Virchow's triad? - predilection for what? -ANSWER blood stasis, endothelial damage, hypercoagulation - pulmonary embolism what is von Recklinghausen's disease of bone? -ANSWER osteitis fibrosa cystica ("brown tumor") adrenal hemorrhage associated with meningococcemia - diagnosis? -ANSWER Waterhouse-Friderichsen syndrome most common cause of hemochromatosis - causes risk of? -ANSWER multiple blood transfusions - can cause CHF and increased risk of hepatocellular carcinoma mechanism of action of macrolides and clindamycin? -ANSWER bind 50S subunit and block translocation most common cause of congenital adrenal hyperplasia -ANSWER 21-hydroxylase deficiency most common tumor of the adrenal medulla in kids - benign or malignant? -ANSWER neuroblastoma - malignant most common cause of SIADH -ANSWER small cell carcinoma of the lung most common cause of Cushing's syndrome - 2nd most common? -ANSWER corticosteroid therapy - excess ACTH secretion by pituitary is 2nd most common cause of hypopituitarism -ANSWER adenoma suboccipital lymphadenopathy - diagnosis? -ANSWER rubella most common vasculitis - poses risk for? - what causes this? -ANSWER temporal arteritis - risk of ipsilateral blindness - due to thrombosis of opthalmic artery most common cause of hepatocellular carcinoma -ANSWER cirrhotic liver, often due to hepatitis B and C most common cause of viral encephalitis -ANSWER HSV what is the defect in Chediak-Higashi syndrome? - symptoms? -ANSWER defect in microtubule polymerization, causing decreased phagocytosis - symptoms: recurrent pyogenic infections, partial albinism, peripheral neuropathy most common cause of Addison's - 2nd most common? -ANSWER autoimmune - infection is 2nd "smudge cell" - diagnosis? -ANSWER CLL most common cause of constrictive pericarditis -ANSWER tuberculosis soap bubble on x-ray - diagnosis? -ANSWER giant cell tumor of bone spike and dome on EM - diagnosis? -ANSWER membranous glomerulonephritis what is a Spitz nevus? -ANSWER benign juvenile melanoma splinter hemorrhages in fingernails - diagnosis? -ANSWER endocarditis most common pituitary tumor - 2nd most common? how does it stain? -ANSWER prolactinoma - somatotropic adenoma is 2nd - acidophilic what gene is mutated in cystic fibrosis, and how? - treatment? how does it work? -ANSWER CFTR - deletion of Phe 508 - N-acetylcysteine cleaves disulfide bonds in mucus substrates in urea cycle -ANSWER Ordinarily, Careless Crappers Are Also Frivolous About Urination: Ornithine - Carbamoyl phosphate - Citrulline - Aspartate - Argininosuccinate - Fumarate - Arginine - Urea treatment for malaria -ANSWER chloroquine + primaquine to prevent relapse by dormant species in liver name 10 X-linked recessive disorders -ANSWER Be Wise, Fool's GOLD Heeds False Hope: Bruton's agammaglobulinemia Wiskott-Aldrich syndrome Fragile X G6PD defiency Ocular albinism Lesch-Nyhan syndrome Duchenne's/Becker's muscular dystrophy Hemophilia A and B Fabry's disease Hunter's syndrome name 8 facultative intracellular bacteria -ANSWER Some Nasty Bugs May Live FacultativeLY: Salmonella Neisseria Brucella Mycobacterium Listeria Francisella Legionella Yersinia most common cause of primary hyperparathyroidism -ANSWER adenoma what is the defect in Fabry's disease? - what substrate accumulates? - what is fabry's -ANSWER deficient alpha-galactosidase A - ceramide trihexoside accumulates - lysosomal storage disease involving dysfunction metabolism of sphingolipids most common cause of glomerulonephritis in adults -ANSWER IgA nephropathy (Berger's disease) functions of ATP and GTP in protein synthesis -ANSWER ATP - for tRNA charging (Activation) GTP - for tRNA binding to ribosome and translocation (Gripping and Going places) chloramphenicol - mechanism of action? -ANSWER inhibits 50S peptidyltransferase (which catalyzes peptide bond formation) function of Vibrio cholerae toxin -ANSWER activates Gs, stimulating adenylyl cyclase - increases Cl- in gut and decreases Na+ absorption what is the defect in Hurler's syndrome? - what substrates accumulate? -ANSWER deficient alpha-L-iduronidase - heparan sulfate and dermatan sulfate accumulates NB: also called "gargolysm" dwarfism, unique facial features, hepatosplenomegally most common preventable cancer -ANSWER lung cancer most common esophageal cancer -ANSWER squamous cell carcinoma what is the defect in Hunter's syndrome? - what substrates accumulate? - what is the inheritance pattern? -ANSWER deficient iduronate sulfatase - heparan sulfate and dermatan sulfate accumulate - X-linked recessive (hunters aim for the X) structures that perform fetal erythropoiesis during development, in order -ANSWER Young Liver Synthesizes Blood: Yolk sac (3-8 wk) Liver (6-30 wk) Spleen (9-28 wk) Bone marrow (28+ wk) what is the only Gram-positive bacteria with endotoxin? -ANSWER Listeria monocytogenes (Endotoxins= toxin released with breakdown of bacteria) most common cause of primary hyperparathyroidism - other causes? -ANSWER adenomas - also hyperplasia and carcinoma most common primary liver tumor -ANSWER hepatoma what is the defect in hypophosphatemic rickets (vitamin D-resistant rickets)? - what is the inheritance pattern? -ANSWER phosphate wasting at proximal tubule - X-linked dominant most common renal tumor - what diseases is it associated with? - what paraneoplastic syndromes is it associated with? -ANSWER renal cell carcinoma - von Hippel-Lindau and acquired polycystic kidney disease - erythropoietin, renin, PTH, ACTH what are the treatments for Actinomyces israelii and Nocardia asteroides (types of bacteria) -ANSWER SNAP: Sulfamethoxazole for Nocardia Actinomyces gets Penicillin thyroidization of kidney - diagnosis? -ANSWER chronic bacterial pyelonephritis most common cardiac primary tumor in kids -ANSWER rhabdomyoma most common cardiac tumor in adults -ANSWER mets what type of bacteria grow pink colonies on MacConkey's agar? - name 5 examples -ANSWER lactose fermenters (lactose is KEE) - MacConKEE's agar: Citrobacter, Klebsiella, E. coli, Enterobacter, Serratia functions of apolipoproteins A-I, B-100, C-II, B-48, and E -ANSWER A-I - activates LCAT B-100 - Binds LDL receptor and mediates VLDL secretion C-II - Cofactor for lipoprotein lipase B-48 - mediates chylomicron secretion E - mediates remnant (Extra) uptake most common chromosomal disorder - what other disorders is it associated with? -ANSWER Down syndrome - associated with ALL, Alzheimer's dementia, endocardial cushion defects most common age of patient with CML -ANSWER adult 35-50 direction of transport of LDL and HDL -ANSWER LDL - from liver to tissues (LDL is Lousy) HDL - from periphery to liver (HDL is Healthy) most common chronic arrhythmia - what is the patient at high risk for? -ANSWER atrial fibrillation - high risk of emboli how do you distinguish between Neisseria gonorrhoeae and meningitidis? -ANSWER MeninGococci ferment Maltose and Glucose Gonococci ferment only Glucose what is the defect in hyperchylomicronemia? -ANSWER lipoprotein lipase deficiency or altered apolipoprotein C-II most common patient with Hodgkin's - exception: nodular sclerosis type - what patient? -ANSWER young male - nodular sclerosis type - female most common patient with minimal change disease -ANSWER young child what diseases does Haemophilus influenzae cause? -ANSWER HaEMOPhilus causes Epiglottitis, Meningitis, Otitis media, Pneumonia most common congenital cardiac anomaly -ANSWER VSD what is the defect in familial hypercholesterolemia? -ANSWER absent or decreased LDL receptors what special culture is needed to grow Haemophilus influenzae? -ANSWER chocolate agar with factors V (NAD) and X (hematin) (when a child has the "flu", mom goes to the five (V) and dime (X) to buy some chocolate) how do you distinguish between Staphylococci and Streptococci? -ANSWER catalase (Staph make catalase because they have more staff) what is the primary symptom in Leber's hereditary optic neuropathy? - what causes this? - what is the inheritance pattern? -ANSWER loss of central vision due to degeneration of retinal ganglion cells and axons - mitochondrial inheritance how do you stain Legionella pneumophila? - what special culture is needed to grow it? -ANSWER silver stain - charcoal yeast extract with iron and cysteine (French legionnaire with silver helmet sitting around charcoal fire with an iron dagger - he is no sissy/cysteine) what is the defect in orotic aciduria? - findings? -ANSWER inability to convert orotic acid to UMP (in de novo pyrimidine synthesis pathway) - orotic acid in urine, megaloblastic anemia most common cause of peau d'orange -ANSWER carcinoma of the breast what is the adult derivative of the umbilical arteries? -ANSWER umbiLical arteries become mediaL umbilical ligaments treatment for Lyme disease -ANSWER doxycycline trimethoprim - mechanism of action? - side effect? how can it be alleviated? -ANSWER inhibits dihydrofolate reductase (folate synthesis) - bone marrow suppression (TMP = Treats Marrow Poorly) - alleviate with folinic acid what is the adult derivative of the allantois/urachus? -ANSWER allaNtois becomes mediaN umbilical ligaments most common cause of death in CML -ANSWER blast crisis what infections cause a palm and sole rash? -ANSWER drive CARS using palms and soles: Coxsackievirus A Rocky Mountain spotted fever Syphilis most common sexually transmitted disease -ANSWER Chlamydia fluoroquinolones - mechanism of action? - side effects? -ANSWER inhibit DNA gyrase - damage to cartilage, tendonitis, tendon rupture (fluoroquinoLONES hurt attachments to your BONES) function of insulin with regard to glucose -ANSWER Insulin moves glucose Into cells name 2 obligate intracellular bacteria -ANSWER stay inside cells when it's Really Cold: Rickettsia Chlamydia what are some symptoms of von Hippel-Lindau disease? - what gene is mutated? - what chromosome is it on? -ANSWER hemangioblastomas, bilateral renal cell carcinomas - VHL gene (tumor suppressor) on chromosome 3 (von Hippel-Lindau = 3 words) what gene is mutated in achondroplasia? -ANSWER fibroblast growth factor receptor 3 most common cause of PID -ANSWER Neisseria gonorrhoeae what is Dandy-Walker? -ANSWER large posterior fossa, absent cerebellum, enlarged 4th ventricle symptoms of rheumatic fever -ANSWER no rheum for SPECCulation: Subcutaneous nodules Polyarthritis Erythema marginatum Chorea Carditis most common site of diverticula -ANSWER sigmoid colon Chlamydia elementary body vs. reticulate body - what is the function of each? -ANSWER Elementary body Enters the cell Reticulate body Replicates in the cell most common cause of death in SLE -ANSWER lupus nephropathy what diseases does Streptococcus pneumoniae cause? -ANSWER MOPS (Most OPtochin Sensitive): Meningitis Otitis media Pneumonia Sinusitis name some derivatives of branchial arch 3 -ANSWER greater horn of hyoid, stylopharyngeus (innervated by glossopharyngeal nerve) what is the adult derivative of the 1st aortic arch? -ANSWER part of the MAXillary artery (1st arch is MAXimal) treatment for syphilis -ANSWER penicillin most common skin cancer -ANSWER basal cell carcinoma most common cause of hospital-acquired pneumonia -ANSWER Klebsiella tissues that don't need insulin for glucose transport -ANSWER BRICK L: Brain, RBCs, Intestine, Cornea, Kidney, Liver what bacteria are treated with metronidazole? - side effects? -ANSWER GET GAP on the metro: Giardia, Entamoeba, Trichomonas, Gardnerella vaginalis, Anaerobes, H. Pylori - disulfiram-like reaction with alcohol, metallic taste what gene is mutated in familial adenomatous polyposis? - what chromosome is it located on? -ANSWER APC gene - chromosome 5 (5 letters in "polyp") most common cause of pneumonia in cystic fibrosis or burn infection -ANSWER Pseudomonas aeruginosa what chromosome is the mutated gene in neurofibromatosis type 1 (von Recklinghausen's disease) located on? - symptoms? -ANSWER chromosome 17 (17 letters in von Recklinghausen) - symptoms: cafe-au-lait spots, neural tumors, Lisch nodules (iris hamartomas) most common cause of dementia - 2nd most common? -ANSWER Alzheimer's - multi-infarct is 2nd what are the derivatives of the 3rd branchial pouch? -ANSWER 3rd pouch contributes to 3 structures: thymus and inferior parathyroids most common stomach cancer -ANSWER adenocarcinoma what gene is mutated in neurofibromatosis type 2? - what chromosome is it on? - symptoms? -ANSWER NF2 gene on chromosome 22 (type 2 = 22) - symptoms: bilateral acoustic neuromas, juvenile cataracts what is the defect in hereditary nonpolyposis colorectal cancer? -ANSWER mutated mismatch repair genes what species of viridans group Strep causes subacute bacterial endocarditis? -ANSWER S. sanguis (blood - there is lots of blood in the heart) what are the 3 types of RNA, and what are their distinguishing characteristics? -ANSWER rRNA - most abundant (Rampant) mRNA - longest (Massive) tRNA - smallest (Tiny) what are the adult derivatives of the 2nd aortic arch? -ANSWER Stapedial artery (Second arch) and hyoid artery mRNA start codon -ANSWER AUG (inAUGurates protein synthesis) mRNA stop codons -ANSWER UGA (U Go Away) UAA (U Are Away) UAG (U Are Gone) what is the defect in familial hypercholesterolemia? -ANSWER defective or absent LDL receptor kwashiorkor vs. marasmus - what are the causes and symptoms of each? -ANSWER kwashiorkor - protein malnutrition - results from a protein-deficient MEAL: malnutrition, edema, anemia, liver fatty change marasmus - energy malnutrition - Marasmus results in Muscle wasting sequelae of Strep pharyngitis -ANSWER rheumatic fever (PHever), glomerulonePHritis (due to PHaryngitis) vitamin B6 - other name? - what types of reactions does it participate in? -ANSWER pyridoxine - transamination and decarboxylation reactions cell processes that take place in both mitochondria and cytoplasm -ANSWER HUGs take two: Heme synthesis Urea cycle Gluconeogenesis most common testicular tumor -ANSWER seminoma vitamin B12 - other name? -ANSWER cobalamin hexokinase vs. glucokinase - what do they do? - where are they each located? - high or low affinity? - high or low Vmax? -ANSWER both convert glucose to glucose-6-phosphate - hexokinase is everywhere and has high affinity and low Vmax - glucokinase is in liver and beta cells of pancreas - has low affinity and high Vmax source of folate -ANSWER green leaves (FOLate from FOLiage) what is the defect in holoprosencephaly? -ANSWER cerebral hemispheres do not separate across midline cofactors involved in pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase reactions -ANSWER TPP (B1), FAD (B2), NAD (B3), CoA (B5), lipoic acid mechanism of action of methotrexate and trimethoprim -ANSWER inhibit dihydrofolate reductase (makes THF, which is needed for dTMP synthesis) purely ketogenic amino acids -ANSWER Lysine and Leucine what are the adult derivatives of the 3rd aortic arch? -ANSWER common Carotid artery and internal Carotid artery (C is 3rd letter of alphabet) what are some symptoms of tuberous sclerosis? -ANSWER facial lesions, hypopigmented "ash leaf spots" on skin, cortical/retinal hamartomas, renal cysts, cardiac rhabdomyomas substrates in TCA cycle -ANSWER Citrate Is Krebs' Starting Substrate For Making Oxaloacetate: Citrate - Isocitrate - alpha-Ketoglutarate - Succinyl-CoA - Succinate - Fumarate - Malate - Oxaloacetate vitamin K - function? -ANSWER needed for proper function of clotting factors II, VII, IX, and X and proteins C and S (K is for Koagulation) enzymes unique to gluconeogenesis (all irreversible) -ANSWER Pathway Produces Fresh Glucose: Pyruvate carboxylase PEP carboxykinase Fructose-1,6-bisphosphatase Glucose-6-phosphatase