Delemarre Eveline M, Felius Bram, Delemarre-van de Waal Henriette A
Medical School Leiden Department of Pediatrics, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands.
Eur J Endocrinol. 2008 Dec;159 Suppl 1:S9-15. doi: 10.1530/EJE-08-0314. Epub 2008 Sep 16.
Puberty is the result of increasing pulsatile secretion of the hypothalamic gonadotropin releasing hormone (GnRH), which stimulates the release of gonadotropins and in turn gonadal activity. In general in females, development of secondary sex characteristics due to the activity of the gonadal axis, i.e., the growth of breasts, is the result of exposure to estrogens, while in boys testicular growth is dependent on gonadotropins and virilization on androgens. Hypogonadotropic hypogonadism is a rare disease. More common is the clinical picture of delayed puberty, often associated with a delay of growth and more often familial occurring. Especially, boys are referred because of the delay of growth and puberty. A short course (3-6 months) of androgens may help these boys to overcome the psychosocial repercussions, and during this period an increase in the velocity of height growth and some virilization will occur. Hypogonadotropic hypogonadism may present in a congenital form caused by developmental disorders, some of which are related to a genetic disorder, or secondary to hypothalamic-pituitary dysfunction due to, among others, a cerebral tumor. In hypogonadotropic hypogonadism puberty can be initiated by the use of pulsatile GnRH, gonadotropins, and sex steroids. Sex steroids will induce development of the secondary sex characteristics alone, while combined administration of gonadotropins and GnRH may induce gonadal development including fertility.
青春期是下丘脑促性腺激素释放激素（GnRH）脉冲式分泌增加的结果，GnRH刺激促性腺激素释放，进而促进性腺活动。一般来说，在女性中，由于性腺轴的活动导致的第二性征发育，即乳房的生长，是雌激素作用的结果，而在男性中，睾丸的生长依赖于促性腺激素，男性化则依赖于雄激素。低促性腺激素性性腺功能减退是一种罕见疾病。更常见的临床表现是青春期延迟，常伴有生长延迟，且多为家族性发病。尤其是男孩，常因生长和青春期延迟前来就诊。短期（3 - 6个月）使用雄激素可能有助于这些男孩克服心理社会影响，在此期间身高增长速度会加快，还会出现一些男性化表现。低促性腺激素性性腺功能减退可能以先天性形式出现，由发育障碍引起，其中一些与遗传疾病有关，或继发于下丘脑 - 垂体功能障碍，如脑肿瘤等。在低促性腺激素性性腺功能减退中，青春期可通过使用脉冲式GnRH、促性腺激素和性类固醇来启动。性类固醇单独使用可诱导第二性征发育，而促性腺激素和GnRH联合使用可能诱导性腺发育，包括生育能力。
