Aminoaciduria in an Elderly Man with the Nephrotic Syndrome and in a Young Man with a Variant of the Fanconi Syndrome

Abstract

The plasma and urinary amino acids and alpha-keto acids were studied in 2 patients; 1 because of the nephrotic syndrome, the other for a persistent proteinuria. Both were found to have generalized aminoaciduria. In the elderly men with the nephrotic syndrome this was accompanied by glycosuria, impairment in the mechanism for acidification of the urine, and an apparent alpha-keto-aciduria. In the young man with proteinuria, the aminoaciduria was accompanied by urinouria, creatinuria, and a low serum phosphorus. This patient''s brother was found to have aminoaciduria and glycosuria. Neither the patient nor his brother had overt evidence of bone disease. The occurrence of aminoaciduria in association with the nephrotic syndrome is reemphasized. The younger man is considered to have a variant of the Fanconi syndrome.