Wilson's Disease - High Yield Topic Series

13 Questions | Total Attempts: 353

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Wilson

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Questions and Answers
  • 1. 
    What is the inheritance pattern of Wilson's disease? 
    • A. 

      AD

    • B. 

      AR

    • C. 

      X-linked

    • D. 

      None

  • 2. 
     Which is the Copper-transporting ATPase gene defective in Wilson's disease ?
    • A. 

      ATP 7A

    • B. 

      ATP 7B

    • C. 

      ATP13A

    • D. 

      ATP13B

  • 3. 
    Gene defective in Menkes Kinky Hair Disease is 
    • A. 

      ATP 7A

    • B. 

      ATP7B

    • C. 

      ATP13A

    • D. 

      ATP13B

  • 4. 
    Which is the locus of ATP 7B gene ?  
    • A. 

      13q14.3

    • B. 

      14q14.3

    • C. 

      8q13.3

    • D. 

      7q14.3

  • 5. 
     What is the inheritance pattern of Menkes disease ?
    • A. 

      AD

    • B. 

      AR

    • C. 

      X-linked

    • D. 

      None

  • 6. 
      Gold Standard test for wilson disease is  
    • A. 

      Serum Ceruloplasmin level

    • B. 

      24 hr urinary copper excretion measurement

    • C. 

      Serum copper level

    • D. 

      Liver Biopsy

  • 7. 
    Wilson's disease patient has more risk of developing Hepatocellular carcinoma.
    • A. 

      True

    • B. 

      False

  • 8. 
    Basic defect in wilson's disease is 
    • A. 

      Reduced ceruloplasmin

    • B. 

      Increased copper absorption

    • C. 

      Defective biliary excretion of copper

    • D. 

      None

  • 9. 
    Which is the treatment of Menkes disease ?
    • A. 

      Zinc

    • B. 

      Penicillamine

    • C. 

      Trientine hydrochloride

    • D. 

      Early parenteral copper administration and supportive measures

  • 10. 
    False about KF ring is
    • A. 

      Copper deposition at the descemets membrane of retina

    • B. 

      First appear in the superior and inferior limbal margins

    • C. 

      Causes no visual problems

    • D. 

      Can be seen with naked eye in advanced cases

  • 11. 
    Face of Giant panda sign is seen in  wilson's disease. In which cut, it is best seen?
    • A. 

      T2-weighted MRI of the midbrain

    • B. 

      T1-weighted MRI of the midbrain

    • C. 

      Both

    • D. 

      Noone

  • 12. 
    Prognostic index of Nazer of wilson disease does not depend upon
    • A. 

      Serum bilirubin

    • B. 

      Serum AST

    • C. 

      Serum albumin

    • D. 

      prolongation of Prothrombin time

  • 13. 
    Which is NOT a feature  of wilson's disease ?
    • A. 

      Testicular atrophy

    • B. 

      Chorea

    • C. 

      Hemolytic Anemia

    • D. 

      Chronic active hepatitis

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