Tønnes Pedersen Anette, Cleemann Line, Main Katharina M, Juul Anders
Endocr Dev. 2018;33:113-127. doi: 10.1159/000487529. Epub 2018 Jun 12.
Hypogonadism may be suspected if puberty is delayed. Pubertal delay may be caused by a normal physiological variant, by primary ovarian insufficiency (Turner syndrome), or reflect congenital hypogonadotropic hypogonadism (HH; genetic) or acquired HH (brain lesions). Any underlying chronic disease like inflammatory bowel disease, celiac disease, malnutrition (anorexia or orthorexia), or excessive physical activity may also result in functional HH. Thus, girls with delayed puberty should be evaluated for an underlying pathology before any treatment, including oral contraception, is initiated. Estrogen replacement is important and natural 17β-estradiol, preferably transdermally, is the preferred choice, whereas the oral route can be used as an alternative depending on patient preference and compliance. Sexual activity is often delayed in the hypogonadal adolescent girl. In the adolescent hypogonadal girl, hormone replacement therapy (HRT) most likely has been initiated at the time she becomes sexually active. If a risk of unwanted pregnancy cannot be ruled out, there is a need to consider contraception. This consideration does not contradict the principles of HRT but can be included as a part of the substitution, e.g. oral contraceptives containing 17β-estradiol or a progestogen intrauterine device combined with continuous 17β-estradiol (transdermal or oral).
如果青春期延迟,可能会怀疑性腺功能减退。青春期延迟可能由正常生理变异、原发性卵巢功能不全(特纳综合征)引起,或反映先天性低促性腺激素性腺功能减退(HH;遗传性)或获得性HH(脑部病变)。任何潜在的慢性疾病,如炎症性肠病、乳糜泻、营养不良(厌食或健康食品癖)或过度体育活动,也可能导致功能性HH。因此,在开始任何治疗(包括口服避孕药)之前,应对青春期延迟的女孩进行潜在病理评估。雌激素替代很重要,天然17β-雌二醇,最好是经皮给药,是首选,而口服途径可根据患者偏好和依从性作为替代选择。性腺功能减退的青春期女孩的性活动通常会延迟。在青春期性腺功能减退的女孩中,激素替代疗法(HRT)很可能在她开始性活动时就已开始。如果不能排除意外怀孕的风险,则需要考虑避孕。这种考虑与HRT的原则并不矛盾,但可以作为替代治疗的一部分,例如含有17β-雌二醇的口服避孕药或与持续17β-雌二醇(经皮或口服)联合使用的孕激素宫内节育器。