Glycogen storage disease type IV

Glycogen storage disease type IV
Glycogen
ICD-10	E74.0
ICD-9	271.0
OMIM	232500
DiseasesDB	5303
MeSH	C16.320.565.202.449.540

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Glycogenosis type IV , Andersen's disease , glycogen branching enzyme deficiency (GBED , polyglucosan body disease, amylopectinosis

Overview

Glycogen storage disease type IV is a very rare hereditary metabolic disorder.

Pathophysiology

It is a result of the absence of the glycogen branching enzyme amylo-1,4-1,6 transglucosidase, which is critical in the production of glycogen. This leads to very long unbranched glucose chains being stored in glycogen. The long unbranched molecules (known as amylopectin) have a low solubility which leads to glycogen precipitation in the liver. These deposits subsequently build up in the body tissue, especially the heart and liver.

Natural History , Complications and Prognosis

The end result is liver failure and eventual death occurring in the first year of life.