Wilson disease hepatolenticular degeneration

autosomal-recessive inheritance with mutations on chromosome 13. Most patients present in second decade of life with hepatic involvement. Neuropsychiatric disease is apparent by third and fourth decades of life. Associated abnormalities include hematologic, endocrine, ocular (Kayser-Fleischer ring), and renal anomalies, with copper accumulation in these tissues.

5. Progressive familial intrahepatic cholestasis (Byler syndrome).

6. Benign recurrent cholestasis.

7. Familial cholestasis of North American Indians.

E. Toxic Causes. Drugs (acetaminophen, amiodarone, estrogens, isoniazid, ketoconazole, phenytoin, valproic acid, halothane,

L-asparaginase, among others), toxins (eg, mushroom poisoning, some herbs), or parenteral nutrition.

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