Congential Steatosis of the Liver: Biochemical Approach to Its Pathogenesis

Abstract
Congenital steatosis of the liver has in common a defect of glucose-6-phosphatase with von Gierke''s disease (Type 1 of glycogen storage disease).-For the development of fatty liver in congenital steatosis, an enhanced lipogenesis from carbohydrate, probably due to an increase in the activity of pentose phosphate pathway, will, we believe, play an important role.