Helv Paediatr Acta. 1977 Jun;32(1):71-5.

The dietary treatment of hepatic glycogen synthetase deficiency.

Aynsley-Green A, Williamson DH, Gitzelmann R.

Abstract

A child with glycogen synthetase deficiency has been treated for one year by more frequent feeding with a diet lower in carbohydrate and higher in protein than her previous diet. This treatment virtually abolished the overnight hyperketonaemia and daytime hyperglycaemia which occurred before treatment. On the new dietary regime the child has had no clinical symptoms of hypoglycaemia and her growth velocity has increased dramatically.

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Hepatic glycogen synthetase deficiency. Definition of syndrome from metabolic and enzyme studies on a 9-year-old girl. [Arch Dis Child. 1977]
Hepatic glycogen synthetase deficiency. Definition of syndrome from metabolic and enzyme studies on a 9-year-old girl.
Aynsley-Green A, Williamson DH, Gitzelmann R. Arch Dis Child. 1977 Jul; 52(7):573-9.
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Goldberg T, Slonim AE. J Am Diet Assoc. 1993 Dec; 93(12):1423-30.
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[Hepatic glycogen synthetase deficiency or glycogen storage disease-zero. Mild phenotype with partial enzymatic defect].
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Cited by 2 PubMed Central articles

Review Hepatic glycogen synthase deficiency: an infrequently recognized cause of ketotic hypoglycemia. [Mol Genet Metab. 2006]
Review Hepatic glycogen synthase deficiency: an infrequently recognized cause of ketotic hypoglycemia.
Weinstein DA, Correia CE, Saunders AC, Wolfsdorf JI. Mol Genet Metab. 2006 Apr; 87(4):284-8. Epub 2005 Dec 6.
Mutations in the liver glycogen synthase gene in children with hypoglycemia due to glycogen storage disease type 0. [J Clin Invest. 1998]
Mutations in the liver glycogen synthase gene in children with hypoglycemia due to glycogen storage disease type 0.
Orho M, Bosshard NU, Buist NR, Gitzelmann R, Aynsley-Green A, Blümel P, Gannon MC, Nuttall FQ, Groop LC. J Clin Invest. 1998 Aug 1; 102(3):507-15.

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