Urinary Excretion of Hydroxylysine and Its Glycosides in Alport’s Syndrome and Several Other Glomerulopathies

Abstract

Alport’s syndrome probably is a molecular disorder of basement membrane composition. Investigation of urine on basement membrane components such as hydroxylysine and its glycosides, glucosylgalactosylhydroxylysine and galactosylhydroxylysine, may be helpful for diagnosis of the disease. Urinary specimens of 33 patients and 12 siblings were investigated, and the results were compared with those of 14 healthy adults and of 29 healthy children. The urine of patients with glomerulopathies, occurring during childhood (IgA nephropathy, benign recurrent hematuria, poststreptococcal glomerulonephritis, Henoch-Schönlein nephropathy, membranoproliferative glomerulonephritis, and nephrotic syndrome due to minimal lesions), was also investigated. No marked differences between normal and diseased subjects could be demonstrated, with respect to excretion of hydroxylysine and its glycosides, in contrast to data reported in the literature.

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

“…In a former study, no marked differences in excretion rates of the free compounds between normals and pa tients suffering from Alport's syndrome could be estab lished [5], in contrast to data from the literature [2,3]. It was suggested in that study, that investigation of the peptide-bound fraction of urinary glycosides might be a better diagnostic approach of Alport's syndrome.…”

“…Increased excretion rates of GGH and GH have been reported in this syndrome [2,3]. In a former study, however, we were unable to distinguish clearly Alport patients and patients with other glomeru lopathies from normals [4,5]. The investigation of peptide-bound hydroxylysylglycosides might be a more promising approach from a diagnostic point of view.…”