Cirrhosis glycogen storage diseases

Siciliano, M., de Candia, E., Ballarin, S., Vecchlo, FJM., Senidei, S., Annese, R., Landolfi, R., Rossi, L. Hepatocellular carcinoma complicating liver cirrhosis in type Ilia glycogen storage disease. X Chn. Gastroenterol. 2000 31 80-82... 

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. 

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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