All issues > Volume 31(6); 1988

Original Article

J Korean Pediatr Soc. 1988;31(6):762-771. Published online June 30, 1988.

Comparison of the Effects of Portacaval Shunt and Dietary therapy in Type I Gyocogen Storagy Diseases(GSD).

Dong Hyun Ju¹, Sei Won Yang¹, Hyung Ro Moon¹

¹Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea

Received: November 18, 1987;  Accepted: January 27, 1988.

Abstract

Type I GSD is a metabolic disease resulted from defective glucose-6-phosphatase activities in liver, kindey and intestine. They are characterized by growth retardation, enlargement of liver and kidney, hypoglycemia, hyperuricemia, and hyperlipidemia. Various methods were suggested for the management of GSD. They are night-time nasogastric feeding of glucose solution with frequent day-time feeding, intravenous hyperalimentation, cornstarch therapy end-to-side portacaval shunt, etc. In this study, the effects of portacaval shunt were compared with that of dietary therapy, and the efforts were concentrated on the recognition of more effective method for the correction of abnormal- ities of type I GSD. Patients group contained 11 cases of type I GSD, diagnosed in Seoul National University Hospital from 1980 to 1986. Six of them were managed with portacaval shunt. Four cases were treated with dietary therapy. One case was lost after discharge. The changes of height, weight, hepatic size and metabolic abnormalities were observed and the
results
were compared between 2 groups. Portacaval shunt was more effective than dietary therapy for the abolishment of epistaxis and hypoglycemic symptoms, the increase of height and weight, the reduction of hepatic size and the correction of hyperuricemia and hyperlipidemia. Significant difference was not found in the increase of fasting blood sugar level and the decrease of GOT/GPT level between 2 groups. Especially, in dietary therapy group, there was no case who showed significant increase of height and weight or decrease of hepatomegaly. Conclusively, portacaval shunt was more effective than dietary therapy in the management of type I GSD.

Keywords :Type I Glycogen Storage Disease (GSD), Glucose-6-phosphatase, End-to-side Portacaval shunt