Abstract
The hydroxyproline (HP) urinary excretion by Bergman method(1963) in modification of Djatchcova(1971) and glycosaminoglycans (GAG) excretion by Bitter & Muir method(1966- with chromatographic analysis by Shiller(I961) were studied in 35 children with hereditary nephritis and in 28 relatives of them. In control studies of 28children and 6 adultes the 24-hour-excetion was: HP - 69,7±2,5mg, GAG - 4,9±0,59 mg. Children with hereditary nephritis without deafness showed tendency to increase of HP excretion and HP/GAG in urine was more then in controls. HP excretoion in children with Alport's syndrome was low(16,5±1,8mg/24h) but GAG excretion was high(8,2±0,8mg/24h). Some of the children with hereditary nephritis have mainly excretion of chondroitin-4,-6-sulphates, others - GAG's with low carbazol/orcinol. There were identical GAG patterns and similar stigmata of connective tissue disembriogenesis both in proband and some of his relatives. Onr may suppose that the changes of HP and GAG excretion depend not only on pathologic process but also on the genotype.
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Ignatova, M., Djatchcova, A. & Seriogina, V. Glycosaminoglycans and hydroxiproline urinary excretion in children with hereditary nephritis. Pediatr Res 8, 914 (1974). https://doi.org/10.1203/00006450-197411000-00091
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DOI: https://doi.org/10.1203/00006450-197411000-00091