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[脉冲式促性腺激素释放激素刺激诱导卡尔曼综合征患者青春期发育]

[Induction of puberty in Kallmann syndrome by pulsatile gonadotropin-releasing hormone stimulation].

作者信息

Rosen-Uzelac G, Lominska-Lasota K, Hengesbach B, Bauer R

机构信息

Universitäts-Hautklinik und Poliklinik, Rheinischen Friedrich-Wilhelms-Universität Bonn.

出版信息

Hautarzt. 1988 Feb;39(2):102-6.

PMID:3129379
Abstract

A 23-year-old male, diagnosed as having hypogonadotropic hypogonadism associated with hyposmia (Kallmann syndrome), was given a trial of pulsatile Gn-RH therapy for 100 days. The therapy led to rhythmic secretion of LH and FSH by the anterior pituitary gland. Both the mean and the peak gonadotropin levels rose steadily. Following the 9th day of treatment the previously subnormal gonadotropin levels were found to be within the normal range. After 23 days a pulsatile response of the Leydig cells parallel to the rhythmic LH secretion was noticed. The testosterone level increased significantly and was 2.6 ng/ml 3 months after the initiation of the therapy. At the same time an increasing activity of androgen-dependent glands became apparent and secondary male sex characteristics were noted. The seminal volume rose from 0.1 ml to 2.0 ml, but after 3 months of treatment spermatozoa could still not be detected. In summary, pulsatile Gn-RH stimulation represents a good option in the treatment of hypothalamic hypogonadotropic hypogonadism as the therapy resembles physiological stimulation and is well tolerated by the patient.

摘要

一名23岁男性,被诊断为伴有嗅觉减退的低促性腺激素性性腺功能减退(卡尔曼综合征),接受了100天的脉冲式Gn-RH治疗试验。该治疗导致垂体前叶有节律地分泌LH和FSH。促性腺激素的平均水平和峰值均稳步上升。治疗第9天后,发现先前低于正常水平的促性腺激素水平已在正常范围内。23天后,观察到睾丸间质细胞的脉冲反应与LH的节律性分泌平行。睾酮水平显著升高,治疗开始3个月后为2.6 ng/ml。与此同时,雄激素依赖腺体的活性增加明显,出现了继发性男性性征。精液量从0.1 ml增加到2.0 ml,但治疗3个月后仍未检测到精子。总之,脉冲式Gn-RH刺激是治疗下丘脑性低促性腺激素性性腺功能减退的一个好选择,因为该疗法类似于生理性刺激,且患者耐受性良好。

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