Glycogen storage disease type 1 : acute decompensation

British Inherited Metabolic Disease Group
British Inherited Metabolic Disease Group (BIMDG)
Publication date:
01 January 2008


Patients with glycogen storage disease type 1 often become hypoglycaemic if they fast for longer than about 2 hours: sometimes it is after a much shorter time. During illness, patients have an even greater tendency to become hypoglycaemic. The treatment aims to maintain normoglycaemia all the time either with frequent oral drinks, a continuous infusion via nasogastric tube or gastrostomy or an intravenous infusion of glucose. This management should be started as soon as patients become unwell. 

Sections of this guideline include: background, admission, initial and ongoing management, oral and intravenous feeding, progress monitoring and discharge.