Glycogen storage disease: long-term follow-up of nocturnal intragastric feeding

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Abstract
Nocturnal intragastric feeding of patients with certain hepatic forms of glycogen storage disease was advocated as an effective treatment, resulting in improved blood chemical values and linear growth. The long-term follow-up of 5 patients with glycogen storage disease; 3 with type Ia, 1 with type Ib; and 1 with type III disease, are reported. All had improvement in 1 or more of the following: linear growth, serum glutamic oxaloacetate transaminase, total lipids, cholesterol, phospholipids, or triglycerides. None had significant improvement in venous CO2, serum lactate or urate. One of the patients in this study died after 1.1 yr of treatment, and another patient developed hepatocellular carcinoma after 4.4 yr of treatment. Nocturnal intragastric feeding, in conjunction with appropriate daytime feeding, is helpful in the management of patients with glycogen storage disease but response to treatment is variable, and it remains to be determined whether the ultimate prognosis of the diseases can be improved.