von Gierke's disease


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von Gierke's disease

[fȯn ′gir·kēz di‚zēz]
(medicine)
A form of glycogenosis characterized by marked diminution in or absence of hepatic glucose-6-phosphatase, resulting in hepatic glycogenosis, hypoglycemia, and acidosis. Also known as glycogen storage disease; hepatic glycogenosis; type I of Cori; van Crevald-von Gierke's disease.
References in periodicals archive ?
Absence of the SRC-2 coactivator results in a glycogenopathy resembling Von Gierke's disease.
The object of this presentation is to report the cases of two siblings with different clinical and biochemical manifestations of glycogenosis, which because of its characteristics corresponds to the type I or Von Gierke's disease.
According to the researchers, this finding has implications for a genetic disease called Von Gierke's disease and potentially adult-onset diabetes.