LIPOAMIDE DEHYDROGENASE-DEFICIENCY - A NEWLY DISCOVERED CAUSE OF ACUTE HEPATITIS IN ADULTS

Lipoamide dehydrogenase deficiency is a rare disease, manifested in ea rly childhood by lactic acidemia, progressive neurological damage and death in most cases. We report a case of lipoamide dehydrogenase defic iency in a 34-year-old Ashkenazi-Jewish woman, The deficiency manifest ed as acute hepatitis without cognitive impairment or acidosis, The pa tient's brother also had lipoamide dehydrogenase deficiency diagnosed at the age of 20, and manifested as hepatocellular damage, lactic acid emia and myoglobinuria, We assume that the trigger for this hepatocell ular damage was prolonged fasting, and that otherwise the patient migh t have gone undiagnosed. Other cases in Ashkenazi Jews of mild lipoami de dehydrogenase deficiency with hepatocellular injury but without cen tral nervous system involvement are reviewed.