Generalized proximal tubular dysfunction of the kidneys, commonly referred to as Fanconi syndrome, has been found in such diverse clinical entities as cystinosis, Wilson disease, tyrosinosis, hereditary fructose intolerance, multiple myeloma, amyloidosis, and others.1 In 1967, the first case of proximal tubular dysfunction (glycosuria, amino aciduria, phosphaturia and the proximal form of renal tubular acidosis) was described2 in a patient with a renal homotransplant. Since this initial report, only one other conclusive report describing the development of Fanconi syndrome in a renal homotransplant has appeared.3 Our finding of a third patient with Fanconi syndrome in a homotransplanted kidney strongly suggests that the association is more than a fortuitous one.
A 41-year-old white man developed hematuria and hypertension in 1947 at the age of 17 years. In 1959, findings from a renal biopsy were consistent with a diagnosis of chronic glomerulonephritis and cortical cystic disease. Because