Renal Transplantation in Type 1 Glycogenosis

doi:10.1001/jama.1978.03280430058017

Renal Transplantation in Type 1 Glycogenosis

Failure to Improve Glucose Metabolism

Michael Emmett, MD;
Robert G. Narins, MD

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).

Abstract

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)

Footnotes

Reprint requests to Department of Medicine, University of California at Los Angeles School of Medicine, 10833 Le Conte, Los Angeles, CA 90024 (Dr Narins).