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[Continuous nocturnal intragastric feeding in glycogenosis type I and III].

Three patients with type I glycogen storage disease (GT I) and four patients with type III glycogen storage disease (GT III) have been treated with nocturnal intragastric feeding and frequent daytime meals for 2 to 8.5 years. In all of them, hypoglycemia was well controlled. Patients with GT. I showed a normal growth rate and an improvement of serum lactate, triglycerides, cholesterol and urate. Patients with GT. III showed no consistent changes in serum triglycerides cholesterol and transaminases and no improvement of myocardiopathy.

Blood Glucose↗
PubMed1988

[Present-day status of glycogenosis Ib. Report of a new case].

A four-month-old boy affected by glycogen storage disease type I is presented. The child suffered from hepatomegaly, lactic acidosis, fasting hypoglycemia and failure to thrive. He had repeated infectious and cyclic neutropenia. Immunoglobulin and chemotactic neutrophil motility was impaired. Liver biopsy showed increased amounts of glycogen in hepatic cells as assessed by morphological and biochemical grounds. The activity of glucose-6-phosphatase as well as other glycogenolytic enzymes was normal in the frozen liver. The aforementioned characteristics suggested the diagnosis of glycogen storage disease type Ib. The child was first treated by enteral continuous feeding and later on by frequent meals during the daytime and enteral continuous feeding during the night time, improving the hypoglycemia as well as the other biochemical and metabolic abnormalities.

Biopsy↗