Abstract
Beta ketothiolase deficiency is a rare inborn error of L isoleucine catabolism and ketone body metabolism We present a 13 month old girl with beta ketothiolase deficiency who was admitted with rapidly progressive coma Severe ketoacidosis and mild hyperammonemia was documented This rare case was treated successfully during the acute episode Turk Arch Ped 2007; 42: 77 9 Key words: Beta ketothiolase deficiency ketoacidosis coma
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