Metabolic liver disease of childhood

The broad topic of metabolic liver disease may be divided into three pathophysiologic groups -- storage diseases, cholestatic syndromes, and metabolic defects contributing to hepatocellular necrosis. The important disease entities in each group have been discussed in terms of incidence, clinical course, inheritance, diagnostic criteria, pathogenesis, hepatic pathology, and therapy. An approach to the evaluation of metabolic liver disease has been proposed. It is hoped that this review will be helpful to the clinician who is engaged in the primary care of children.