Gierke's disease

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Gierke's disease

[gēr´kez]

glycogen storage disease (type I), a condition in which deficiency of the hepatic enzyme glucose-6-phosphatase results in liver and kidney involvement, with hepatomegaly, hypoglycemia, hyperuricemia, and gout. Called also von Gierke's disease and hepatorenal glycogenosis.

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Glycogenosis type I or Von Gierke's disease, was discovered by German physician Gierke, who studied an 8-year-old girl with chronic increase in liver size.

Patients with type I glycogenosis may be classified into various subtypes, with the most common belonging to those who lack the glucose 6-phosphatase enzyme per se, or Von Gierke's disease (Type I a) (5).

Enfermedades de deposito de glucogeno: informe de dos casos en la ciudad de Cartagena

According to the researchers, this finding has implications for a genetic disease called Von Gierke's disease and potentially adult-onset diabetes.