POLYCYSTIC OVARIES AND GLUCOSETOLERANCE IN HEPATIC GLYCOGEN STORAGE DISEASE

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The dietary management of hepatic glycogen storage disease.

The dietary management of hepatic glycogen storage disease (GSD) is based on an understanding of the primary biochemical abnormalities. Plasma glucose levels are maintained by the release of glucose from the liver. The glucose is formed either by the breakdown of glycogen or it is synthesized from substrates such as fructose, galactose, glycerol or amino acids. The final common step in all thes...

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The clinical spectrum is continuous and broad and three major forms are recognised: infantile, juvenile and adult-onset. [1] In the infantile form, accumulation of glycogen in cardiac muscle leads to cardiac failure. Accumulation may also occur in the liver, which results in hepatomegaly and elevation of hepatic enzymes. Glycogen accumulation in muscle and peripheral nerves causes hypotonia and...

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Pompe's Glycogen Storage Disease

The clinical spectrum is continuous and broad and three major forms are recognised: infantile, juvenile and adult-onset. [1] In the infantile form, accumulation of glycogen in cardiac muscle leads to cardiac failure. Accumulation may also occur in the liver, which results in hepatomegaly and elevation of hepatic enzymes. Glycogen accumulation in muscle and peripheral nerves causes hypotonia and...

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ژورنال

عنوان ژورنال: Pediatric Research

سال: 1993

ISSN: 0031-3998,1530-0447

DOI: 10.1203/00006450-199305001-00063