PEDIATRICS Vol. 67 No. 1 January 1981, pp. 113-118
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Metabolic Abnormalities in the Idiopathic Fanconi Syndrome: Studies of Carbohydrate Metabolism in Two Patients

Russell W. Chesney MD1, Bernard S. Kaplan MB, BCh1, David Teitel MD1, Eleanor Colle MD1, Roderick R. McInnes MD, PhD1, Hy Goldman MD1, and Charles R. Scriver MD1

1 Biochemical Genetics, Renal, and Endocrine Laboratories, McGill University-Montréal Children's Hospital Research Institute and The Department of Pediatrics, McGill University, Montréal, Québec, Canada

Two patients with idiopathic Fanconi syndrome and glucose intolerance were studied from a metabolic perspective. They had fasting hyperglycemia, massive glucosuria, insulinopenia, ketosis, and elevated serum free fatty acids. There was a markedly blunted insulin secretory response to glucagon, tolbutamide, glucose, and arginine. One patient had the findings of diabetic retinopathy and a sensory neuropathy. Neither patient could convert galactose to glucose, but they did not have galactosemia. As a result of these studies, and previous reports in which similar changes were noted, we conclude that diabetes mellitus may occur in patients who have had idiopathic Fanconi syndrome for many years.

Submitted on April 4, 1980
Accepted on June 30, 1980