The urinary excretion of total hydroxylysine and its glycosides in normal persons, and in patients suffering from Alport's syndrome--contribution of the peptide-bound fraction.

As Alport's syndrome is probably a molecular disorder of basement membrane composition, investigation of the urine on basement membrane components might be a diagnostic aid. It was shown previously that measurement of the excretion of free hydroxylysine and its glycosides, glucosylgalactosylhydroxylysine and galactosylhydroxylysine, was not useful for this purpose. Because the urinary peptide-bound fraction may be more basement membrane specific, a subsequent study was performed with respect to the total (= free and peptide-bound) concentrations and fractions. To establish reference values, urinary specimens of 38 normal subjects of different ages were investigated. These values were used for comparison with data on 30 Alport patients, and 10 Alport siblings. 10 patients with benign recurrent hematuria were also investigated. No marked differences were observed in the excretory rates between normals and patients.