Abstract
Collagen metabolism was studied in 9 children with hereditary nephritis and 10 family members with microscopic hematuria. The diagnosis was confirmed in all 9 children by percutaneous renal biopsy. No previous studies exist defining the collagen turnover via the utilization of the urinary OHLG's, hydroxylysylgalactosyl-glucose (HGG) and hydroxylysyl-galactose (HG), in hereditary nephritis. Mean urinary OHLG's values in the biopsy group was 113 uM/g creatinine as compared to 15 control children of 74 uM/g creatinine. The mean urinary values of 128 u M/g creatinine for the siblings were comparable to the nephritis group.
Two additional patients with the Nail-Patella Syndrome also demonstrated increased urinary excretion of OHLG's. The significantly increased urinary excretion of these metabolites, HGG and HG, support the thesis of an altered collagen disorder.
This study suggests that urinary hydroxylysine glycosides may be used as a useful marker in the recognition of the early stage of hereditary nephritis.
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Tina, L., Lou, M., Dizio, P. et al. A USEFUL BIOCHEMICAL MARKER IN THE IDENTIFICATION OF HEREDITARY NEPHRITIS: HYDROXYLYSINE GLYCOSIDES (OHLG's). Pediatr Res 11, 559 (1977). https://doi.org/10.1203/00006450-197704000-01133
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DOI: https://doi.org/10.1203/00006450-197704000-01133
