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Liver & GIT

4 out of 9

All of the following are features of Wilson's disease, except- (AIIMS MAY 08)

A Hemolytic anemia

B Testicular atrophy

C Chorea

D Chronic active hepatitis

Ans. B Testicular atrophy

Clinical Findings

1. It presents as liver disease and neuropsychiatric disease in young adults.

2. The diagnosis should always be considered in any child or young adult with hepatitis, splenomegaly with hypersplenism, Coombs negative hemolytic anemia, portal hypertension, and neurologic or psychiatric abnormalities.

3. Hepatic involvement may range from elevated liver tests (although the alkaline phosphatase may be low) to cirrhosis and portal hypertension. (Acute hepatitis, fulminant hepatitis, CAH cirrhosis)

4. The neurologic manifestations are related to basal ganglia dysfunction and include an akinetic-rigid syndrome similar to parkinsonism, pseudosclerosis with tremor, ataxia, and a dystonic syndrome,

Three main feature : a. Dystonia, b. incoordination, c. Tremor (H 18th Pg-3188),

5. Autonomic disturbance may occur which include orthostatic hypotension and sweating abnormalities as well as bowel, bladder, and sexual dysfunction.

6. Pathognomonic sign of the condition is the brownish or gray-green Kayser Fleischer ring, (Fine pigmented granular deposits in Descemet membrane in the cornea).

7. The ring is most marked at the superior and inferior poles of the cornea.

8. Renal calculi, aminoaciduria, renal tubular acidosis, hyperparathyroidism, infertility, and hemolytic cholelithiasis anemia may occur in patients with Wilson disease,

9. It does not lead to hepatoma(PGI Dec 08)

10. Testicular Atrophy is not a feature.

(Extra Edge: Sensory neuropathy & Myopathy are not the features) (H 18th Pg-3188)

Liver & GIT Flashcard List

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