Carbohydrate metabolism Glycogen storage disease

Chen, Y.-T., Chapter 350 Glycogen storage diseases and other inherited disorders of carbohydrate metabolism. Harrison s Text online. 2002, accessed 4/2/02. E. Braunwald, A.S. Eauci, K. J. Isselbacher, D.L. Kasper, S.L. Hauser, D.L. Longo, and JL. Jameson, eds. 

Inborn Errors of Carbohydrate Metabolism 163 Glycogen Storage Disease Glycogen Metabolism Von Gierke s Disease... 

The liver s role is to maintain chemical homeostasis and it can, in certain circumstances, be stressed by means of a metabolic load. One example has been to administer an oral fructose load which causes minimal spectral changes in normal subjects but, at very low dosage, results in an increase in the fructose-1-phosphate peak in patients with fructose intolerance. Other metabolic stresses have been administered to investigate galactose intolerance, glycogen storage diseases and cirrhosis. The rat liver (in situ or perfused) has been studied to elucidate details of carbohydrate and fatty acid metabolism, sometimes by a combination of and P NMR. The control of pH in the liver has also been studied. 

Ryman, B.E. (1980), Recent advances and problems in the glycogen storage diseases. In Inherited Disorders of Carbohydrate Metabolism (eds D. Burman, J.B. Holton and C. A. Pennock), MTP Press Ltd., Lancaster, pp. 289-295. 

Because the liver is a major processor of dietary and endogenous carbohydrates, liver disease affects carbohydrate metabolism in a variety of ways (see Chapter 25). However, none of the conventional modes of evaluating carbohydrate metabolism have value in the diagnosis of liver disease. Because the liver is the major site of both glycogen storage and gluconeogenesis, hypoglycemia is a common complication in certain liver diseases, particularly Reye s syndrome, fulminant hepatic failure, advanced cirrhosis, and hepatocellular carcinoma. 

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