Klujber L, Turi S, Haszon I, Baranyai Z, Sulyok E
Department of Paediatrics, University Medical School of Pécs, Hungary.
Pediatr Nephrol. 1989 Apr;3(2):179-85. doi: 10.1007/BF00852905.
To assess the characteristics of connective tissue metabolism in chronic renal failure (CRF), urinary excretion of glycosaminoglycan (GAG) fractions and hydroxyproline (HYP) was determined in ten patients with CRF and in ten age-matched healthy children. CRF was found to be associated with elevated free HYP (19.9 +/- 6.1 vs 9.8 +/- 3.6 mumol/day, P less than 0.05) and depressed peptide HYP excretion (33.1 +/- 13.5 vs 225.2 +/- 17.7 mumol/day, P less than 0.01), a low rate of total GAG excretion (7.0 +/- 2.4 vs 16.1 +/- 1.9 mumol uronic acid/day, P less than 0.05) with low chondroitin 4 -sulphate + chondroitin 6 -sulphate (Ch-Ss) (14.0 +/- 9.9 vs 65.0 +/- 22.1%) and a high proportion of non-sulphated or under-sulphated fractions, i.e. hyaluronic acid + chondroitin + heparan sulphate (HA + Ch + HS) (75.3 +/- 11.4 vs 31.5 +/- 5.7%). Urinary 3-methyl-histidine (3-met-HIS) excretion and plasma essential free amino acids did not differ in the two groups. In response to haemodialysis no consistent change occurred in urinary excretion of 3-met-HIS, peptide-bound HYP, total GAG or percentage distribution of individual GAG fractions. After haemodialysis all plasma amino acids decreased significantly, and there was a significant increase in urinary excretion of free HYP (P less than 0.05). We conclude that the alterations in urinary excretion of total and individual GAGs observed in CRF may reflect disturbed connective tissue metabolism which does not appear to be accounted for by protein malnutrition or enhanced protein breakdown and remains uninfluenced by haemodialysis therapy.
为评估慢性肾衰竭（CRF）患者结缔组织代谢的特征，对10例CRF患者及10例年龄匹配的健康儿童测定了尿中糖胺聚糖（GAG）组分及羟脯氨酸（HYP）的排泄情况。结果发现，CRF患者游离HYP排泄量升高（19.9±6.1对9.8±3.6μmol/天，P<0.05），肽结合型HYP排泄量降低（33.1±13.5对225.2±17.7μmol/天，P<0.01），总GAG排泄率降低（7.0±2.4对16.1±1.9μmol糖醛酸/天；P<0.05），其中硫酸软骨素4-硫酸酯+硫酸软骨素6-硫酸酯（Ch-Ss）含量低（14.0±9.9对65.0±22.1%），非硫酸化或低硫酸化组分（即透明质酸+软骨素+硫酸乙酰肝素，HA+Ch+HS）比例高（75.3±11.4对31.5±5.7%）。两组患者尿中3-甲基组氨酸（Met-HIS）排泄量及血浆必需游离氨基酸水平无差异。血液透析后，尿中Met-HIS、肽结合型HYP、总GAG或各GAG组分的百分比分布无一致变化。血液透析后，所有血浆氨基酸均显著降低，游离HYP尿排泄量显著增加（P < 0.05）。我们得出结论，CRF患者尿中总GAG及各GAG排泄量的改变可能反映结缔组织代谢紊乱，这似乎并非由蛋白质营养不良或蛋白质分解增强所致，且不受血液透析治疗的影响。
