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雌激素/孕激素诱导特纳综合征患者生长及内分泌变化的新方面。

New aspects of oestrogen/gestagen-induced growth and endocrine changes in individuals with Turner syndrome.

作者信息

Bohnet H G

出版信息

Eur J Pediatr. 1986 Sep;145(4):275-9. doi: 10.1007/BF00439400.

Abstract

Patients with Turner syndrome (n = 33) were subjected to a sequential oestrogen/gestagen replacement therapy (21 days 2 mg oestradiol valerate plus 0.5 mg norgestrel for 10 days) for 1-4 years. In individuals aged 12-17 years, growth velocities before treatment ranged between 2.9 and 1.9 cm/year and increased to 6.8-3.8 cm/year following sex steroid substitution for 1 year. Thereafter a decrease was observed; depending on age, pre-treatment velocities were observed within 2-4 years of replacement therapy. On average, bone age advanced 1 year following 1 year of sex steroid administration and adult bone age was reached following 2-4 years of treatment. Approximately 70% of the individuals noted breast development to stage IV according to Tanner and experienced regular withdrawal bleedings. Oral administration of oestradiol valerate resulted in serum oestradiol levels normally seen during the early follicular phase; hypergonadotropic LH and FSH levels decreased slightly following 1 year of therapy. Growth ranged between 4 and 13 cm; in the oldest individuals the final height ranged between 149 and 156 cm. In summary, the oestrogen/gestagen replacement therapy chosen for this study achieved development of secondary sex characteristics, endocrine changes, growth velocities, bone age advancement and to a certain extent growth, which was almost comparable to that observed during puberty in healthy girls.

摘要

33例特纳综合征患者接受了为期1至4年的序贯雌激素/孕激素替代疗法(21天,戊酸雌二醇2毫克加炔诺孕酮0.5毫克,共10天)。12至17岁的个体,治疗前生长速度在每年2.9至1.9厘米之间,在进行1年性类固醇替代治疗后增加至每年6.8至3.8厘米。此后观察到生长速度下降;根据年龄,在替代治疗2至4年内观察到治疗前的生长速度。平均而言,给予1年性类固醇后骨龄提前1岁,治疗2至4年后达到成人骨龄。约70%的个体根据坦纳分期法乳房发育达到IV期,并出现规律的撤退性出血。口服戊酸雌二醇导致血清雌二醇水平处于卵泡早期的正常水平;治疗1年后,高促性腺激素性促黄体生成素(LH)和促卵泡生成素(FSH)水平略有下降。生长幅度在4至13厘米之间;年龄最大的个体最终身高在149至156厘米之间。总之,本研究选择的雌激素/孕激素替代疗法实现了第二性征发育、内分泌变化、生长速度、骨龄进展,并在一定程度上实现了生长,这与健康女孩青春期观察到的情况几乎相当。

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