Birnbaum Wiebke, Bertelloni Silvano
Hormonzentrum für Kinder und Jugendliche, Division of Experimental Paediatric Endocrinology and Diabetes, Department of Paediatrics and Adolescent Medicine, University of Lübeck, Lübeck, Germany.
Endocr Dev. 2014;27:149-59. doi: 10.1159/000363640. Epub 2014 Sep 9.
People with disorders of sex development (DSD) may have impaired sex steroid production or their gonads removed before, during or after adolescence, thus requiring hormone replacement therapy (HRT) to induce puberty and/or maintain secondary sexual characteristics, to optimize bone health, and to promote physical and social well-being. Oestrogens are usually used for this purpose in persons reared as females (eventually combined with progestins if a uterus is present) and androgens in those reared as males. An alternative therapy for women with ascertained complete androgen insensitivity syndrome could be testosterone, because this is the main sex steroid hormone secreted by their gonads, but this approach remains to be better explored. Few sound evidence-based data are available to guide HRT administration at puberty and in adulthood in individuals with DSD, but recent data and new formulations may give better perspectives for the future.
性发育障碍（DSD）患者可能存在性类固醇生成受损的情况，或者在青春期前、青春期期间或青春期后性腺被切除，因此需要激素替代疗法（HRT）来诱导青春期发育和/或维持第二性征，优化骨骼健康，并促进身体和社会幸福感。对于被抚养为女性的人，雌激素通常用于此目的（如果有子宫，最终会与孕激素联合使用），而对于被抚养为男性的人，则使用雄激素。对于已确诊完全雄激素不敏感综合征的女性，一种替代疗法可能是睾酮，因为这是她们性腺分泌的主要性类固醇激素，但这种方法仍有待进一步探索。目前几乎没有可靠的循证数据可用于指导DSD患者在青春期和成年期的HRT给药，但最近的数据和新制剂可能为未来提供更好的前景。
