Banerjee S, Raghavan S, Wasserman E J, Linder B L, Saenger P, DiMartino-Nardi J
Department of Pediatrics, Division of Pediatric Endocrinology, Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, New York, USA.
Pediatrics. 1998 Sep;102(3):E36. doi: 10.1542/peds.102.3.e36.
Premature adrenarche refers to the early maturation of the adrenal zona reticularis such that the resultant modest hyperandrogenism causes the early appearance of pubic hair before the age of 8 years in girls and 9 years in boys. The precise etiology of premature adrenarche is not known. However, recent studies indicate that certain girls with premature adrenarche are at risk of developing functional ovarian hyperandrogenism, polycystic ovarian syndrome, and hyperinsulinism. Caribbean Hispanic women in general are at increased risk of developing polycystic ovarian syndrome, and African-Americans are at increased risk of developing the complications of hyperinsulinism. Previously, girls with premature adrenarche were reported to have androgens in the range found in normal children in the early stages of puberty. We noted that many of our African-American and Caribbean Hispanic patients with premature adrenarche had androgens that were much higher than what has been reported previously.
This retrospective study was performed to characterize the adrenocorticotropin-stimulated androgen response in an African-American and Caribbean Hispanic population of girls with premature adrenarche.
The androgen response to adrenocorticotropin stimulation in 72 African-American and Caribbean Hispanic girls with premature adrenarche was compared with those reported for normal girls in early puberty (Tanner stages II and III). The mean age was 6.8 +/- 0.8 years, bone age was 8 +/- 1.5 years, pubic hair was Tanner stages II and III, and body mass index was 18.6 +/- 4.
Of the girls, 28% were found to have elevated stimulated 17OHPregnenolone (17OHPreg) levels that were >2 SD units above the mean for normal early pubertal children. The stimulated ratio of 17OHPreg/17OHProgesterone also was elevated in 18% of the girls and showed a modest correlation with body mass index.
In contrast to previous studies of girls of mixed ethnic backgrounds with premature adrenarche, 28% of the 72 African-American and Caribbean Hispanic girls with premature adrenarche had adrenocorticotropin-stimulated 17OHPreg levels that were significantly higher than those seen in early pubertal girls. Because 17OHPreg hyperresponsiveness has been described previously in women with hirsutism or polycystic ovarian syndrome, the similar finding in many African-American and Caribbean Hispanic girls with premature adrenarche suggests that the two conditions may share a common mechanism for their hyperandrogenism. Therefore, the hyperandrogenism in certain African-American and Caribbean Hispanic girls with premature adrenarche may not be benign and may be the first presentation of polycystic ovarian syndrome.
肾上腺功能初现过早是指肾上腺网状带过早成熟,导致适度的雄激素过多,致使女孩在8岁前、男孩在9岁前出现阴毛早现。肾上腺功能初现过早的确切病因尚不清楚。然而,最近的研究表明,某些肾上腺功能初现过早的女孩有发生功能性卵巢雄激素过多、多囊卵巢综合征和高胰岛素血症的风险。一般来说,加勒比西班牙裔女性患多囊卵巢综合征的风险增加,非裔美国人患高胰岛素血症并发症的风险增加。此前,据报道肾上腺功能初现过早的女孩在青春期早期的雄激素水平与正常儿童的水平相当。我们注意到,我们的许多患有肾上腺功能初现过早的非裔美国人和加勒比西班牙裔患者的雄激素水平比之前报道的要高得多。
本回顾性研究旨在描述肾上腺皮质激素刺激下,非裔美国人和加勒比西班牙裔肾上腺功能初现过早女孩的雄激素反应特征。
将72名患有肾上腺功能初现过早的非裔美国人和加勒比西班牙裔女孩对促肾上腺皮质激素刺激的雄激素反应,与青春期早期(坦纳Ⅱ期和Ⅲ期)正常女孩的反应进行比较。平均年龄为6.8±0.8岁,骨龄为8±1.5岁,阴毛处于坦纳Ⅱ期和Ⅲ期,体重指数为18.6±4。
发现28%的女孩促肾上腺皮质激素刺激后的17α-羟孕烯醇酮(17OHPreg)水平升高,高于正常青春期早期儿童平均值2个标准差以上。18%的女孩促肾上腺皮质激素刺激后的17OHPreg/17α-羟孕酮比值也升高,且与体重指数呈适度相关。
与之前对肾上腺功能初现过早的混合种族背景女孩的研究不同,72名患有肾上腺功能初现过早的非裔美国人和加勒比西班牙裔女孩中有28%的促肾上腺皮质激素刺激后的17OHPreg水平显著高于青春期早期女孩。由于之前在多毛症或多囊卵巢综合征女性中已描述过17OHPreg反应过度,在许多患有肾上腺功能初现过早的非裔美国人和加勒比西班牙裔女孩中发现的类似情况表明,这两种情况可能有共同的雄激素过多机制。因此,某些患有肾上腺功能初现过早的非裔美国人和加勒比西班牙裔女孩的雄激素过多可能并非良性,可能是多囊卵巢综合征的首发表现。