Uncooked Cornstarch Therapy in Type I Glycogen-Storage Disease (GSD-I)

ABSTRACT

OBJECTIVE: A few years ago it was shown that uncooked cornstarch feeding(UCS) could correct the biochemical abnormalities resulting from a deficiency of glucose-6-phosphatase in children with type ment. So far, little experience is available with UCS in Korea. We studied to compare the effects on biochemical control and growth of therapy with portacaval shunt and therapy with UCS. METHODS: Enghteen patients with GSD-I, ranging in age from 2 to 17 years, were included in these studies. They were thirteen male and five female children, and were diagnosed as GSD-I in Seoul National University from 1982 to 1994. Six patients (age 6.75+/-3.06, range 4.33~12.75 years) received portacaval shunt after preoperative intravenous hyperalimentation, and fifteen patients(age 7.08+/-4.09, rnage 2.00~17.00years) received UCS(1.75~2g/kg, four times daily). They included three patients who have had hepatic adenoma during follow-up after portacaval shunt. Height standard deviation score(SDS), liver size, blood glucose, serum cholesterol, serum uric acid, and serum transaminase were measured. RESULTS: After UCS, the changes of height SDS, liver size, blood glucose, and serum uric acid were significant but the changes of cholesterol, SGOT, and SGPT were not significant. The SDS of height increased significantly form -2.61+/-1.01 at onset of UCS to -1.93+/-0.86 after UCS. The liver size decreased significantly form 10.1+/-2.7cm at onset of UCS to 7.9+/-3.0cm after UCS. Blood glucose levels increased significantly form 50.6+/-14.3mg/dl at onset of UCS to 90.1+/-17.0mg/dl after UCS. Serum uric acid levels decreased significantly form 8.03+/-2.12mg/dl at onset of UCS to 5.67+/-2.34mg/dl after UCS. Statistically, significant difference were not found in those values after portacaval shunt. CONCLUSIONS: UCS was effective to maintain blood glucose, to minimize biochemical abnormalities and to optimize clinical outcome in patients with GSD. Individuals with GSD-I commonly developed hepatic adenoma and malignant transformation of these ademonas has occurred. So in any case, it is advisable to screen all GSD-I patients periodically by imaging techniques for tumor formation, and once tumors have been detected, to adopt strict dietary measures and observe them closely for evidence of suggestive malignant transformation.