Endocrinology and Metabolism Division, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Endocr J. 2010;57(10):909-13. doi: 10.1507/endocrj.k10e-092. Epub 2010 Aug 24.
The gonadal function of patients with Turner syndrome (TS) is variable. Individuals with mosaicism characterized by 45,X/46,XX or 45,X/47,XXX are more likely to experience spontaneous menarche compared with other karyotypes. Prepubertal gonadotropins of TS patients with spontaneous menarche are reportedly normal or significantly lower than those of patients with induced menarche. The present study investigated an index of spontaneous and cyclical menstruation at 10-12 years old in TS. Subjects comprised 50 patients with TS, divided into three groups: Group A (n=7), with spontaneous menarche before 16 years old and regular menstruation for at least 1 year and 6 months; Group B (n=6), with irregular menstruation since menarche leading to secondary amenorrhea despite spontaneous menarche before 16 years old; and Group C (n=37), without spontaneous breast budding before 14 years old or without spontaneous menarche before 16 years old. Karyotype, LH and FSH concentrations at 10 and 12 years old were analyzed retrospectively. Spontaneous and cyclical menstruation was more frequently observed in TS with mosaicism characterized by 45,X/46,XX or 45,X/47,XXX than in TS with other karyotypes, as previously described. Spontaneous and cyclical menstruation in TS was observed when serum FSH level was <10 mIU/mL at 12 years old, suggesting this FSH level as an index of spontaneous and cyclical menstruation in TS.
特纳综合征（TS）患者的性腺功能具有可变性。具有 45,X/46,XX 或 45,X/47,XXX 嵌合体特征的个体比其他核型更有可能经历自发性月经初潮。据报道，具有自发性月经初潮的 TS 患者的青春期前促性腺激素正常或明显低于诱导月经初潮的患者。本研究调查了 10-12 岁 TS 患者自发性和周期性月经的指标。研究对象包括 50 名 TS 患者，分为三组：A 组（n=7），16 岁前自发性月经初潮，且至少 1 年 6 个月月经规律；B 组（n=6），16 岁前自发性月经初潮后月经不规则，导致继发性闭经；C 组（n=37），14 岁前无自发性乳房发育或 16 岁前无自发性月经初潮。回顾性分析了 10 岁和 12 岁时的核型、LH 和 FSH 浓度。如前所述，具有 45,X/46,XX 或 45,X/47,XXX 嵌合体特征的 TS 比其他核型的 TS 更常出现自发性和周期性月经。12 岁时血清 FSH 水平<10mIU/mL 时观察到 TS 的自发性和周期性月经，表明该 FSH 水平可作为 TS 自发性和周期性月经的指标。
