[Carbohydrates in the treatment of glycogenoses]

Glycogen storage diseases (GSD) include inborn errors in glycogen synthesis and degradation which, like most metabolic diseases, evades any therapeutical concept up to now. Nevertheless, in a subgroup of glycogenoses, characterized by functional absence of the key glycogenolytic enzyme glucose-6-phosphatase (GSD-1), dietary treatment is able to ensure almost normal development of affected patients. The principle of treatment consists of oral application of raw cornstarch, which is hydrolyzed slowly in the intestinum resulting in normoglycemia over a period of several hours. Thus, nighttime nasogastric tube-feeding, the standard dietary regimen in GSD-1, could be avoided. Based on our experiences, this therapeutic approach, which has proven successful up to now only in elder children and adults, can be recommended even in children under 2 years of age, if the individual response to an oral starchload has been examined prior to the starch diet.