Type IA and IB Glycogen Storage Diseases

Beside severe hypoglycemia, growth failure, and acidosis, they also carry a risk of liver adenoma, possibly complicated by intratumoral hemorrhage or liver cell carcinoma.

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Liver transplantation may be recommended in patients with adenomas or failure of medical therapy; it allows restoration of a normal glucose metabolism and significant improvement in growth. In type IB, polymorphonuclear dysfunction is not corrected by liver transplantation and requires permanent treatment with granulocyte colony-stimulating factor (GCSF).

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