All are metabolic causes of liver disease except: (PGI Nov 2010)
High-Yield Explanation
Ans:A (Histiocytosis) [Ref O.P.Ghai 7th/280)Histiocytosis is a rare childhood malignancyO.P.Ghai 7th/595Table :Etiology of metabolic liver diseases in children (AIIMS 1991-2004; n - 159)Etiology'%Wilson's disease47.7Glycogen storage disease14.4Hereditary fructose intolerance8.8Lipid storage disorder8.1Gaucher's disease3.7Bile acid metabolic defect3.7Tyrosinemia1.2Hemochromatosis"2.5Organic acidemia2.5Galactosemia1.2Indian Chilhood cirrhosis1.2Byler's disease1.2Niemann Pick disease1.2Unknown1.8Features of Metabolic Liver Disease O.RGnai 7th/280 H Hypoglycemia, lactic acidemia, hyperammonemiaRecurrent vomiting, failure to thrive, dysmorphic faciesRickets, unusual odors, cataracts, cardiac dysfunctionDevelopmental delay, hypotonia, neuromuscular deterioration, seizuresApproach to bronchiectasis