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April 21, 1978

Renal Transplantation in Type 1 Glycogenosis: Failure to Improve Glucose Metabolism

Author Affiliations

From the University of Texas, Southwestern Medical School, and Baylor University Hospital, Dallas (Dr Emmett), and the Division of Nephrology, University of California at Los Angeles, and the Center for the Health Sciences, Los Angeles (Dr Narins).

JAMA. 1978;239(16):1642-1644. doi:10.1001/jama.1978.03280430058017

In glycogenosis type 1 (GT1), glucose synthesis is deficient due to absence of glucose-6-phosphatase. Development of renal failure in such a patient provided the opportunity to test whether or not this metabolic defect could be reversed by a renal allograft, which contains the missing enzyme and has potential for glucose synthesis. Despite normalization of renal function and both glucocorticoid therapy and the infusion of amino-acid precursors of glucose, fasting hypoglycemia persisted unabated. We conclude that a functioning renal allograft is incapable of meeting the metabolic demands of a patient with glucose-6-phosphatase deficiency.

(JAMA 239:1642-1644, 1978)