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Annals of Clinical and Laboratory Science, Vol 12, Issue 5, 424-430
Copyright © 1982 by Association of Clinical Scientists

Articles

Secondary metabolic changes in von Gierke's disease (Type I glycogen storage disease)

PR Blackett

Deficiency of glucose-6-phosphatase in Type I glycogen storage disease (GSD) results in hypoglycemia and excessive accumulation of glucose-6-phosphate. As a result, lactic acid, uric acid, and lipids are formed as end-products. The formation of these metabolites are discussed with an emphasis on monitoring therapeutic progress. In addition, hyperlipidemia and associated changes in apolipoproteins are considered as indices of the clinical course.




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Copyright © 1982 by the Association of Clinical Scientists.