<prompt> All are metabolic causes of liver disease except: PGI Nov 2010HistiocytosisHemochromatosisGauchers diseaseWilsons disease  <response>: Ans:A Histiocytosis Ref O.P.Ghai 7th280Histiocytosis is a rare childhood malignancyO.P.Ghai 7th595Table :Etiology of metabolic liver diseases in children AIIMS 1991-2004; n - 159EtiologyWilsons disease47.7Glycogen storage disease14.4Hereditary fructose intolerance8.8Lipid storage disorder8.1Gauchers disease3.7Bile acid metabolic defect3.7Tyrosinemia1.2Hemochromatosis2.5Organic acidemia2.5Galactosemia1.2Indian Chilhood cirrhosis1.2Bylers disease1.2Niemann Pick disease1.2Unknown1.8Features of Metabolic Liver Disease O.RGnai 7th280 H Hypoglycemia, lactic acidemia, hyperammonemiaRecurrent vomiting, failure to thrive, dysmorphic faciesRickets, unusual odors, cataracts, cardiac dysfunctionDevelopmental delay, hypotonia, neuromuscular deterioration, seizuresApproach to bronchiectasis 