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Metabolic abnormalities in the idiopathic fanconi syndrome studies of carbohydrate metabolism in 2 patients

, : Metabolic abnormalities in the idiopathic fanconi syndrome studies of carbohydrate metabolism in 2 patients. Pediatrics 67(1): 113-118

Two patients with idiopathic Fanconi syndrome and glucose intolerance was 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. These studies and previous reports suggest that diabetes mellitus may occur in patients who have had idiopathic Fanconi syndrome for many years.


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