| Comparison of the Effects of Portacaval Shunt and Dietary therapy in Type I Gyocogen Storagy Diseases(GSD). |
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Dong Hyun Ju, Sei Won Yang, Hyung Ro Moon |
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Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea |
| Ⅰ형 당원병에 시행한 문맥하공정맥 문합술과
식이요법의 비교 |
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주동현, 양세원, 문형로 |
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서울대학교 의과대학 소아과학교실 |
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Received: 18 November 1987 • Accepted: 27 January 1988 |
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| 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.
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| Key Words:
Type I Glycogen Storage Disease (GSD), Glucose-6-phosphatase, End-to-side Portacaval shunt |
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