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USMLE Brs Pathology Vocab

Subjects : health-sciences, usmle
Instructions:
  • Answer 50 questions in 15 minutes.
  • If you are not ready to take this test, you can study here.
  • Match each statement with the correct term.
  • Don't refresh. All questions and answers are randomly picked and ordered every time you load a test.

This is a study tool. The 3 wrong answers for each question are randomly chosen from answers to other questions. So, you might find at times the answers obvious, but you will see it re-enforces your understanding as you take the test each time.
1. Aspirin toxicity seen in kids after acute febrile viral dx - microvesicular fatty change in liver and encephalopathy






2. Catastrophic adrenal insuff and vascular collapse - hemorrhagic necrosis of adrenal cortex






3. 2/neu :: oncogene (aka c - erbB2) - in breast cancer = poor prognosis






4. Tumor suppressor mutated in Von Recklinhousen






5. Seen in Down synd - small white spots on periphery of iris






6. Seen in granuloma inguinale - multiple organisms filling large histiocytes






7. Aka chronic progressive histiocytosis - not as bad as Letterer Siwe






8. Deficient hypoxanthine::guanine phosphoribosyltransferase (HGPRT) - low purine metabolism - high uric acid - thus gout - mental retardation - choreoathetosis - spasticity - self mutilation - aggressiveness






9. Hemihypertrophy - macroglossia - organomegaly - neonatal hypoglycemia - various embryonal tumors - deleted WT 2 gene






10. Lots of tumors - breast CA - soft tissue sarc - brain tumors - leukemias - has loss of p53 tumor suppressor gene






11. 5p chromosome deletion - severe mental retardation - microcephaly - catlike cry - low birth weight - round face - hypertelorism (wide eyes)






12. Severe unconj hyperbilirubinemia due to deficient glucuronyl transferase






13. Pituitary insufficiency post - partum - low TSH ACTH






14. Metastatic stomach CA to ovary






15. Small round eosinophilic masses found in dieing hepatocytes often containing chromatin remnants seen in viral hepatitis






16. X linked - aka immunodeficiency with thrombocytopenia and eczema - total immunoglobulins often normal - recurrent infections






17. Autosomal dominant - fatal - progressive degeneration of striatum and frontal cortex with neuronal depletion and gliosis






18. Retinoblastoma on chromosome 13






19. Autosomal recessive - neutropenia - albinism - cranial and peripheral neuropathy - tendency to develop repeated infections - abnormal WBCs (abnormal mictotubul formation and large cytoplasmic granules = lysosomes)






20. Aka thromboangiitis obliterans - acute inflame of small and med arteries of extremities - extending to adjacent veins and nerves - young jewish men - painful ischemic dx - worse with smoking






21. Aka angiokeratoma corporis diffusum universal - deficient a::galactosidase A - accumulate ceramide trihexoside - skin lesions angiokeratomas - fever - burning pain in extremities - cardiovascular and cerebrovascular involvement - death as adult by re






22. Dysplastic cell seen in HPV






23. Chromosome 19 - allele common in alzheimers






24. Gene mutated in achondroplasia (most common form of dwarfism) - located at 4p16.3






25. B Thalassemia major






26. Manifestation of lymphplasmocytic lymphoma - B cell neoplasm






27. Hereditary nonpolyposis colon cancer - DNA repair genes messed up






28. Intra::alveolar hemosiderin - laden macrophages caused by RBCs from capillary rupture 2* to pulmonary HTN






29. Aka neurofibromatosis - neurofibromas in skin - schwannomas of CN VIII - caf






30. Deficient fibrillin (constituent of microfibrils) - arachnodactyly - ectopia lentis - aortic aneurysm - mitral valve prolapsed






31. Similar to Hurler - deficient L iduronosulfate sulfatase - accumulations of heparin sulfate and dermatan sulfate - hepatospenomegaly - micrognathia - retinal degeneration - joint stiffness - mental retardation - cardiac lesions






32. Deficient glucocerebrosidase - accumulation of glucocerebroside in cells of mononuclear phagocyte system 3 types ::






33. Deficient a1 -4 glucosidase - accumulation of glycogen in liver - heart - skeletal muscle - cardiomegaly - muscle hypotonia - spelnomegaly - intractable hypoglycemia - death from cardiorespiratory failure before age 3






34. Deficient sphingomyelinase - accumulation of sphingomyelin in phagocytes - foamy histiocytes in liver - spleen - lymph nodes - skin - hepatosplenomegaly - anemia fever - occasional neuro degeneration - half have cherry red spot macula






35. Autosomal dominant - numerous adenomatous polyps along with osteomas and soft tissue tumors






36. Mutation here causes pseudohypoparathyroidism






37. Renal tubular dysfunction - impaired reabsorption of glucose - amino acids - phosphate - and bicarb thus glycosuria - hyperphosphaturia - hypophosphatemia - aminoaciduria - systemic acidosis






38. Oncogenes that code for p21 proteins which are membrane signalers






39. Also seen in urine of MM - stacks of RBCs - will have high ESR






40. = MEN1






41. Intracytoplasmic inclusions seen in leydig cell tumors p283






42. Seen in insulinoma - episodic hyperinsulinemia and hypoglycemia - CNS dysfunction - reversal of CNS probs with administration of glucose






43. Modest elevated serum unconj bilirubin - due to decreased uptake by liver cells and reduced activity of glucuronyl transferase






44. At least 2 X and one Y - hypogonadism - tall - gynecomastia - low testosterone - high pituitary gonadotropins - infertility -






45. Seen in CLL - leukemic B- cells that are fragile






46. Deficient hexosaminidase A - GM2 ganglioside accumulation - especially in neurons - CNS degeneration - mental/motor deterioration - blindness - cherry red spot on macula - death by 4 years of age






47. Mesangial matrix material acculmulations seen in nodular glomerulosclerosis seen in diabetic nephropathy






48. Transloc seen in ewing sarcoma






49. Nuclei arranged in horseshoe - shaped pattern about periphery of cell - characteristic of but not specific for granulomatous TB (as opposed to a foreign body giant cell that has scattered nuclei)






50. Aka acute disseminated langerhans cell histiocytosis - aggressive - usually fatal - kids hepatosplenomegaly - LAD - pancytopenia - pulmonary involvement - recurrent infections