What is Glycogen Storage Disease in Children
Another group of disorders of carbohydrate metabolism are those of glycogen storage in Children. These include glycogen storage disease (GSD) types I, II, and III and the phosphorylase system deficiencies. In all forms of GSD careful monitoring of growth and nutritional requirements is necessary and these children should be followed in a specialist centre.
Glycogen storage disease type I (GSDI)
In this form of GSD there is either a deficiency of glucose-6-phosphatase or a defect in one of its transport proteins. Clinical manifestations include growth retardation and hepatomegaly. The main goal of dietary treatment is to promote normal growth by maintaining a normal blood glucose level and this requires frequent administration of dietary glucose throughout the 24-hour period. This generally necessitates frequent carbohydrate-containing meals and snacks during the day, often including drinks of a glucose polymer or uncooked cornstarch preparations (for slower glucose release). Overnight nasogastric feeding is used to provide glucose while the child is asleep.
Glycogen storage disease type II
This form of GSD is caused by a deficiency of acid maltase, leading to an accumulation of glycogen. The infantile form (Pompe’s disease) is associated with a poor prognosis and early death. The childhood form is less severe presenting with generalized muscle weakness which may lead to cardio-respiratory insufficiency and death. Dietary treatment includes a high protein diet with decreased intakes of carbohydrate and fat to provide normal energy intake.
Glycogen storage disease type III
GSDI II involves a deficiency of the glycogen debranching enzyme and the production of glucose from glycogen is limited. Hypoglycaemia and poor growth are common in childhood but symptoms become less severe as the child gets older. Dietary management varies according to the severity of the disease but includes a high protein diet. Some children also require frequent feeding similar to that used for GSD type I and overnight feedings may be used.
Disorders of the phosphorylase system
These have similar symptoms to GSDIII, but are much milder with minimal hypoglycaemia. Adults are usually asymptomatic and there is a normal life expectancy.
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