患有乌尔里希-特纳综合征的女孩尿中胶原蛋白交联物排泄增加。

Increased urinary excretion of collagen crosslinks in girls with Ullrich-Turner syndrome.

作者信息

Rauch F, Seibel M, Woitge H, Kruse K, Schönau E

机构信息

Klinik und Poliklinik für Pädiatrie der Universität zu Köln, Germany.

出版信息

Acta Paediatr. 1995 Jan;84(1):66-9. doi: 10.1111/j.1651-2227.1995.tb13486.x.

DOI:10.1111/j.1651-2227.1995.tb13486.x

PMID:7734903

Abstract

Skeletal abnormalities and "osteoporosis" are frequent features of Ullrich-Turner syndrome (UTS), but their cause remains largely unknown. In this study, we compared the urinary excretion of hydroxyproline (OHP), pyridinoline (PYD) and deoxypyridinoline (DPD) in 28 girls (bone age 3.5-11.0 years, mean 7.4 years) with UTS and 30 healthy prepubertal children (chronological age 3.9-10.9 years, mean 7.6 years). Expressed relative to the square of the height, the excretion of both collagen crosslinks was significantly higher in UTS than in controls (23.4% for PYD, 33.6% for DPD, p < 0.05). In contrast, no significant difference was found for OHP. The molar PYD/DPD ratio was significantly lower in UTS children than in controls (mean (+/- SD) 3.4 (+/- 0.41) versus 3.8 (+/- 0.55); p = 0.004). While the higher excretion of collagen crosslinks reflects enhanced bone resorptive activity in UTS, the lower PYD/DPD ratio might be due to structural alterations in collagen.

摘要

骨骼异常和“骨质疏松症”是乌尔里希-特纳综合征（UTS）的常见特征，但其病因仍 largely 不明。在本研究中，我们比较了 28 名UTS 女孩（骨龄 3.5 - 11.0 岁，平均 7.4 岁）和 30 名健康青春期前儿童（实际年龄 3.9 - 10.9 岁，平均 7.6 岁）的尿羟脯氨酸（OHP）、吡啶啉（PYD）和脱氧吡啶啉（DPD）排泄情况。相对于身高的平方，UTS 患者中两种胶原蛋白交联物的排泄量均显著高于对照组（PYD 为 23.4%，DPD 为 33.6%，p < 0.05）。相比之下，OHP 未发现显著差异。UTS 儿童的摩尔 PYD/DPD 比值显著低于对照组（均值（±标准差）3.4（±0.41）对 3.8（±0.55）；p = 0.004）。虽然胶原蛋白交联物排泄量增加反映了 UTS 中骨吸收活性增强，但较低的 PYD/DPD 比值可能是由于胶原蛋白的结构改变。