Glycogen storage disease type VI

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Glycogen storage disease type VI Classification and external resources
Glycogen
ICD-10	E74.0
ICD-9	271.0
OMIM	232700
DiseasesDB	5311
eMedicine	med/912  ped/2564
MeSH	D006013

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Glycogen storage disease type VI is a type of glycogen storage disease caused by a deficiency in liver glycogen phosphorylase.

It is also known as "Hers' disease", after H. G. Hers, who characterized it in 1959.^[1]

Presentation

Patients generally have a benign course, and typically present with hepatomegaly and growth retardation early in childhood. Mild hypoglycemia, hyperlipidemia, and hyperketosis may occur. Lactic acid and uric acid levels are normal.

References

1. ↑ synd/2506 at Who Named It

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