[PDF] Glycogen storage disease type III : acute decompensation

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

Abstract

Patients with glycogen storage disease type III may become hypoglycaemic during a fast, the length of which varies widely between patients. During illness, patients have a greater tendency to become hypoglycaemic. The treatment aims to maintain normoglycaemia all the time either with frequent oral drinks, a continuous infusion  via naso-gastric 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.