Häusler G, Frisch H, Schober E, Waldhauser F
Universitäts-Kinderklinik, Wien.
Monatsschr Kinderheilkd. 1988 Mar;136(3):152-5.
In 22 girls with Ullrich-Turner-Syndrome (UTS) aged 1.3 to 17.6 years basal and LHRH-stimulated gonadotropin (Gn)-concentrations as well as spontaneous variations of Gn-secretions were measured in order to recognize age dependent changes. Basal Gn-concentrations were elevated in the 1-year old girl, showed normal values in patients whose bone age was less than 11 years and were elevated again in the group with a bone age greater than 11 years. Stimulated LH and FSH concentrations were significantly elevated in all age-groups when compared with the values of age-matched controls. Minimal and maximal Gn-concentrations during spontaneous secretion were low in patients with a bone age less than 11 years, whereas a distinct elevation could be noted in the 1-year old child and in the elder group when compared with age-matched controls. According to these findings the typical age-dependent change of Gn-secretory dynamics could also be demonstrated in children with UTS albeit on an elevated level. A bone age of 11 to 11.5 years, when serum concentrations of Gn rise in healthy girls followed by onset of puberty seems to be the biological indicator for changes of the hypothalamo-hypopituitary level also in girls with Ullrich-Turner-Syndrome.
对22名年龄在1.3至17.6岁之间的乌尔里希 - 特纳综合征（UTS）女孩，测量基础促性腺激素（Gn）水平、促性腺激素释放激素（LHRH）刺激后的Gn浓度以及Gn分泌的自发变化，以识别年龄依赖性变化。1岁女孩的基础Gn浓度升高，骨龄小于11岁的患者Gn浓度值正常，而骨龄大于11岁的组中Gn浓度再次升高。与年龄匹配的对照组相比，所有年龄组中刺激后的促黄体生成素（LH）和促卵泡生成素（FSH）浓度均显著升高。骨龄小于11岁的患者在自发分泌期间的最低和最高Gn浓度较低，而与年龄匹配的对照组相比，1岁儿童和年龄较大的组中Gn浓度有明显升高。根据这些发现，虽然处于升高水平，但在UTS儿童中也可证明Gn分泌动力学存在典型的年龄依赖性变化。在健康女孩中，血清Gn浓度升高并随后进入青春期时的骨龄为11至11.5岁，这似乎也是乌尔里希 - 特纳综合征女孩下丘脑 - 垂体水平变化的生物学指标。
