Nutr Hosp. 1998 Sep-Oct; 13(5): 228-32.

[Corn starch in the treatment of patients with glycogenosis type I and III]

Galiano Segovia MJ, Moreno Villares JM, Medina BenÃtez E, Manzanares LÃ³pez-Manzanares J, Urruzuno TellerÃa P, LeÃ³n Sanz M.

SecciÃ³n de GastroenterologÃa Infantil, Hospital Universitario Doce de Octubre, Madrid, EspaÃ±a.

RATIONALE: Administering raw corn starch can maintain normoglycemia for long periods after being ingested, thus facilitating control in patients with type I and III glycogenosis. METHODS: The metabolic effects and the effects on the nutritional status of a treatment with fractionated administrations of raw starch are assessed in two patients with type I glycogenosis (ages 18 and 12 years) and one patient with type III glycogenosis (aged 13 years). In the first two cases the response was previously studied after administering a load of raw corn starch in a water suspension, in an amount similar to the estimated rate of endogenous glucose production during the fasting period (5 mg/kg/minute). RESULTS: The results of the overload of starch showed a normoglycemia and an absence of lactoacidosis between 4 and 6 hours after its ingestion. The three patients were given two doses of raw corn starch (2 g/kg/dose) at 1.00 and 5.00 hours during the night. After one year of treatment, all patients showed glycemia levels at 9.00 AM that were greater than 90 mg/dl and lactic acid levels that were lower than 2.4 mmol/l. Moreover, in two of the cases there was an increase in the growth rate. In all cases the amount of the hepatomegaly decreased as did the size of the hepatic adenomas that were present in two of the cases. CONCLUSIONS: In patients with type I and III glycogenosis, raw corn starch can balance the results of the nightly gastric glucose infusion, both with regard to the metabolic control and with regard to the growth.