Fukami Maki
Department of Molecular Endocrinology, National Research Institute for Child Health and Development, Tokyo, Japan.
Clin Pediatr Endocrinol. 2022;31(3):110-115. doi: 10.1297/cpe.2022-0029. Epub 2022 Jun 11.
11-Oxyandrogens, such as 11-ketotestosterone (11-KT), 11-ketodihydrotestosterone (11-KDHT), 11β-hydroxytestosterone (11-OHT), 11β-hydroxyandrostenedione (11-OHA4), and 11-KA4, are newly specified human androgens. These 11-oxyandrogens are present in the cord blood and placenta, as well as in the blood of men and women of various ages, and are produced primarily in the adrenal gland. Accumulating evidence suggests that these steroids contribute to androgen excess in patients with 21-hydroxylase deficiency or polycystic ovary syndrome. More importantly, unlike classic androgens, 11-oxyandrogens produced in maternal tumors can pass through the placenta without being converted into estrogens, and cause severe virilization of female fetuses. Thus, overproduction of 11-oxyandrogens represents a new mechanism of 46,XX disorders of sex development. On the other hand, the physiological roles of 11-oxyandrogens remain to be clarified. This mini-review introduces the current understanding of 11-oxyandrogens, from the perspective of pediatric endocrinology.
11-氧代雄激素，如11-酮睾酮（11-KT）、11-酮二氢睾酮（11-KDHT）、11β-羟基睾酮（11-OHT）、11β-羟基雄烯二酮（11-OHA4）和11-KA4，是新确定的人体雄激素。这些11-氧代雄激素存在于脐带血和胎盘中，也存在于各年龄段男性和女性的血液中，主要在肾上腺中产生。越来越多的证据表明，这些类固醇会导致21-羟化酶缺乏症或多囊卵巢综合征患者雄激素过多。更重要的是，与经典雄激素不同，母体肿瘤产生的11-氧代雄激素可以穿过胎盘而不转化为雌激素，并导致女性胎儿严重男性化。因此，11-氧代雄激素的过度产生代表了46,XX性发育障碍的一种新机制。另一方面，11-氧代雄激素的生理作用仍有待阐明。本综述从儿科内分泌学的角度介绍了目前对11-氧代雄激素的认识。
