de Muinck Keizer-Schrama Sabine M P F
Erasmus Medical Centre, Sophia Children's Hospital, Endocrinology Subdivision, Rotterdam, The Netherlands.
Horm Res. 2007;68 Suppl 5:80-3. doi: 10.1159/000110584. Epub 2007 Dec 10.
Management of puberty in the hypogonadal girl remains controversial. Estrogens have profound effects on growth and development. Oral estrogens, the most widely used form, undergo first-pass metabolism in the liver and alter many aspects of hepatic function. Transdermal estrogens are not subject to first-pass metabolism and are effective at mimicking spontaneous pubertal estrogen levels resulting in normal pubertal development. Age-appropriate induction of puberty should be initiated at 12 to 13 years of age; delaying puberty any longer may compromise quality of life during adolescence. Estrogens should be started at a low dose, approximately one-tenth to one-eighth of the adult replacement dose, and then increased gradually over a period of 2 to 4 years. Generally, progestins are added after 2 years of estrogen therapy. With these treatment regimens, breast development typically proceeds at a normal pace; however, the effects on uterine dimensions are less clear. Concomitant treatment with oral dehydroepiandrosterone (or dehydroepiandrosterone sulfate) advances pubic hair development in the adolescent girl with panhypopituitarism.
In the hypogonadal girl, puberty should be induced with very low doses of transdermal estradiol at 12 to 13 years of age with gradual dose escalations over 2 to 4 years. Combination estrogen/androgen therapy in girls with hypogonadism should be explored in future research.
性腺功能减退女孩的青春期管理仍存在争议。雌激素对生长发育有深远影响。口服雌激素是最广泛使用的形式，在肝脏中会经历首过代谢，并改变肝功能的许多方面。经皮雌激素不受首过代谢影响，能有效模拟青春期自然雌激素水平，从而实现正常的青春期发育。青春期的适当诱导应在12至13岁开始；青春期延迟过久可能会影响青少年时期的生活质量。雌激素应从低剂量开始，约为成人替代剂量的十分之一至八分之一，然后在2至4年内逐渐增加。一般来说，在雌激素治疗2年后添加孕激素。采用这些治疗方案，乳房发育通常会以正常速度进行；然而，对子宫大小的影响尚不清楚。对于全垂体功能减退的青春期女孩，口服脱氢表雄酮（或硫酸脱氢表雄酮）进行联合治疗可促进阴毛发育。
对于性腺功能减退的女孩，应在12至13岁时用极低剂量的经皮雌二醇诱导青春期，并在2至4年内逐渐增加剂量。未来研究应探索性腺功能减退女孩的雌激素/雄激素联合治疗。
