Impaired Sertoli Cell Function in Males Diagnosed with Noonan Syndrome
Impaired Sertoli Cell Function in Males Diagnosed with Noonan Syndrome
复制标题
诊断为努南综合征的男性支持细胞功能受损
DOI：
10.1515/jpem.2008.21.11.1079
复制
发表时间：
2008
影响因子：
1.4
通讯作者：
C. Noordam
中科院分区：
文献类型：
--
作者：
K. A. Marcus;C. Sweep;I. Burgt;C. Noordam
为了研究努南综合征男性性腺功能，收集了9例确诊为努南综合征的青春期男性的临床和实验室资料，包括抑制素B。双侧睾丸下降4例，单侧睾丸隐睾2例。3例患者的青春期被推迟。本研究中所有患者的促黄体生成素（LH）水平均正常，而7例促卵泡激素（FSH）水平升高。抑制素B在6名男性中较低，在2名男性中略高于正常下限。重要的是，所有三名睾丸下降正常的男性都表现出支持细胞功能障碍的迹象，这表明，与早期的报道相反，双侧隐睾似乎不是Noonan综合征睾丸功能障碍的主要因素。这些发现提示了男性性腺功能紊乱的不同机制，这通常与支持功能障碍有关。
In order to study male gonadal function in Noonan syndrome, clinical and laboratory data, including inhibin B, were gathered in nine pubertal males diagnosed with Noonan syndrome. Bilateral testicular maldescent was observed in four, and unilateral cryptorchidism occurred in two. Puberty was delayed in three patients. Luteinising hormone (LH) levels were normal in all patients in our series, while follicle stimulating hormone (FSH) levels were raised in seven. Inhibin B was low in six males and just above the lower limit of normal in two. Importantly, all three men with normal testicular descent displayed signs of Sertoli cell dysfunction, indicating, in contrast to earlier reports, that bilateral cryptorchidism does not seem to be the main contributing factor to impairment of testicular function in Noonan syndrome. These findings suggest different mechanisms of disturbance in male gonadal function, which is frequently associated with Sertoli dysfunction.
抑制素 B：以前隐睾男性和对照男性的生育指数比较。
DOI：
10.1210/jcem.86.6.7583
发表时间：
2001
期刊：
The Journal of clinical endocrinology and metabolism.
影响因子：
--
作者：
Lee,PA;Coughlin,MT;Bellinger,MF
通讯作者：
Bellinger,MF