[Type I glycogenosis: extending therapy with uncooked cornstarch]

Abstract

Normal or slightly elevated blood lactate levels and normal to slightly elevated lactate-/creatinine-ratios in 24-h-urine were found in 12 patients (0.9 to 16 years) with glycogenosis type I under conventional treatment with nocturnal gastric drip feeding with maltodextrine combined with frequent daytime feedings. Replacing the nocturnal gastric drip feeding by two doses of uncooked cornstarch suspended in water (single dose 1.4-2.0 g/kg body weight) 4 patients at the ages of 10 to 16 years obtained similar metabolic control. A 7-year old patient with glycogenosis type Ib showing an extremely low fasting tolerance attained stable blood glucose levels by eating two doses of uncooked cornstarch in the morning, so that she was able to attend school. A 2-year old patient received 2-3 g cornstarch/kg body weight every 6 h resulting in constant blood glucose levels, so that she was able to emigrate to Turkey. The therapy with uncooked cornstarch is suitable to augment the therapy of some patients with glycogenosis type I.