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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. Defective cilia - sinusitis - bronchiectasis - situs inversus - sometimes hearing loss and male sterility






2. = MEN1






3. Transloc seen in ewing sarcoma






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






5. Hemangioblastoma or cavernous hemangioma of cerebellum - brainstem or retina - adenomas - cysts in liver - kidney - pancreas - and other organs - increased renal cell CA - gene = short arm of chromosome 3






6. Impaired tubular reabsorption of tryptophan - pellagra::like manifestations






7. Aka allergic granulomatous angiitis - necrotizing vasculitis - variant of polyarteritis nodosa - involves pulmonary vasculature - peripheral eosinophilia - and asthma






8. 5p chromosome deletion - severe mental retardation - microcephaly - catlike cry - low birth weight - round face - hypertelorism (wide eyes)






9. Iron deficient anemia associated with upper::esophageal web






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






11. 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






12. Chromosome 19 - allele common in alzheimers






13. Pituitary insufficiency post - partum - low TSH ACTH






14. Metastatic stomach CA to ovary






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






16. Appears as multiple wart - like lesions resembling condyloma accuminatum






17. Malignant lymphoma with features resembling inflammatory disorder - young men - Reed Sternburg cells






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






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






20. Philadelphia chromosome - CML bcr abl fusion c - abl on 9 - bcr on 22






21. Defective in x- linked brutons agammaglobulinemia - B- cell tyrosine kinase






22. Manifestation of lymphplasmocytic lymphoma - B cell neoplasm






23. Acute inflammatory demyelinating dx primarily involving peripheral nerves






24. Variant of rheumatoid arthritis - aka juvenile rheumatoid arthritis






25. Triad of ovarian fibroma - ascites - hydrothorax






26. Variant of rheumatoid arthritis - splenmegally - neutropenia - RA






27. Aka osteopetrosis - marble bone dx - dense skeleton - failure of osteoclastic activity






28. Associated with breast alone (isn't one of these associated with prostate? Check)






29. Pigmented iris hamartoma seen in neurofibromatosis






30. Adenomatous polyps with tumors of CNS






31. Tennis racket shaped cytoplasmic inclusion seen in langerhans cells (so also seen in langerhan cell histiocytosis)






32. Primary adrenal failure






33. Parkinsonism with autonomic dysfunction and orthostatic hypotension






34. Autosomal recessive - hepatitis - accumulation of copper due to transport problem - low ceruloplasmin seen - kayser fleischer rings in eyes






35. Scheinker syndrome :: prion dx - fatal familial insomnia






36. X linked - absence of plasma cells and serum immunoglobulins - cell mediated immunity still ok - no germinal centers - recurrent bacterial infections - resistances to fungal and viral still - btk gene defect






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






38. Autosomal recessive - unusually large platelets - lack of 'GPIb IX V'






39. Infectious disorder - caused postencephalitic parkinsonism






40. Aka antiglomerular basement membrane dx - antibodies against alveolar and glomerular basement membranes - linear immunoflorescence






41. Seen in hyperacute rejection - = acute inflammation - fibrinoid necrosis of small vessels - and extensive thrombosis






42. DsDNA antibodies and Sm Smith antigen = highly specific for SLE






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






44. Aka infantile progressive spinal muscular atrophy - autosomal recessive - LMN disease - infants






45. Urethritis - conjunctivitis - arthritis - associated with venereal or intestinal infection






46. Mutation here causes pseudohypoparathyroidism






47. Tumor suppressor mutated in wilms tumor






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






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






50. Deficient glucose -6- phosphatase - accumulation of glycogen in liver and kidney - hepatomegaly - hypoglycemia







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