Prognosis
With appropriate nutritional therapy, recurrent hypoglycaemia can be avoided, ameliorating metabolic sequelae and allowing patients to survive into adulthood. Nevertheless, complications remain a major issue.[31] Hepatic adenomas develop in most patients by the age of 30 years. These lesions may haemorrhage or degenerate into hepatocellular carcinoma (HCC). Metabolic control may be related to HCC formation in patients with GSD Ia. Optimising metabolic control is therefore critical.[32][33] Changes in the management of GSD Ia have resulted in improved metabolic control, and there is increasing evidence that complications can be delayed or prevented with optimal metabolic control.[34][35]
Use of this content is subject to our disclaimer