Jakubowski L
Horm Metab Res. 1981 Jul;13(7):399-403. doi: 10.1055/s-2007-1019281.
Urinary total hydroxyproline excretion in patients with Turner's syndrome and Klinefelter's syndrome was studied. Among the patients with Turner's syndrome hydroxyproline excretion was relatively low in girls 11-14 years old and somewhat increased in 14-17 age groups. Above 17 years of age urinary total hydroxyproline excretion was significantly higher than in the control. In patients with Klinefelter's syndrome at the age below 11 and above 17 years normal hydroxyproline values were observed. In 3 sixteen-year-old boys with 47, XXY karyotype, excretion of hydroxyproline with urine was significantly lower than in the control. Relatively low values of total hydroxyproline in urine of 11-14 year-old girls with Turner's syndrome and decreased in boys with Klinefelter's syndrome result, most probably, from the absence of hormonal changes typical for the puberty. However, concentration of hydroxyproline in urine does not correlate with serum FSH and LH levels. It is not affected by the enhancement of changes in the bone system either. The presented data encourage further studies on the connective tissue biochemistry in the patients with numerical aberrations sex chromosomes.
对特纳综合征和克兰费尔特综合征患者的尿总羟脯氨酸排泄情况进行了研究。在特纳综合征患者中,11 - 14岁女孩的羟脯氨酸排泄相对较低,在14 - 17岁年龄组中有所增加。17岁以上患者的尿总羟脯氨酸排泄显著高于对照组。在克兰费尔特综合征患者中,11岁以下和17岁以上的患者观察到正常的羟脯氨酸值。在3名核型为47, XXY的16岁男孩中,尿羟脯氨酸排泄显著低于对照组。11 - 14岁特纳综合征女孩尿中总羟脯氨酸值相对较低,克兰费尔特综合征男孩尿中总羟脯氨酸值降低,很可能是由于缺乏青春期典型的激素变化。然而,尿中羟脯氨酸浓度与血清促卵泡激素(FSH)和促黄体生成素(LH)水平无关。它也不受骨骼系统变化增强的影响。所呈现的数据促使对性染色体数目异常患者的结缔组织生物化学进行进一步研究。