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Glycogen storage disease type VI



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

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


 
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Glycogen_storage_disease_type_VI". A list of authors is available in Wikipedia.
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