Treatment of Glycogen Storage Disease Type IIIa in Adolescents and Adults

Adolescents and adults with glycogen storage disease type IIIa (GSD IIIa) have distinct nutritional needs that diverge from those of younger patients. Clinical management in this age group centres on a targeted dietary approach, with specific considerations for patients who develop myopathy.

Clinical Scenario

This protocol is designed for adolescents and adults with glycogen storage disease type IIIa. In this population, protein adequacy takes on particular importance, and the composition of dietary carbohydrate is a key management consideration.

Approach Overview

Management involves a structured dietary strategy that prioritises protein intake and restricts certain types of carbohydrate, while also addressing the risk of fasting. For patients who develop myopathy, additional nutritional support measures are part of the approach.

Specific targets, full dietary composition, and overnight nutrition guidance are detailed in the complete protocol.

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References

DOI: 10.1097/GIM.0b013e3181e655b6

The emphasis of the diet for the adult with GSD IIIa is on a higher percentage of protein.
Carbohydrates may be limited, but preference should be given to complex carbohydrates, as opposed to simple sugars.
Simple sugars should be avoided as they result in sudden rise and fall of BG.
Bedtime snack (low-fat milk with protein powder) or a high protein formula for overnight enteral feeding may be of benefit to those with myopathy.

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