von Oettingen Julia, Sola Pou Joaquim, Levitsky Lynne L, Misra Madhusmita
Massachusetts General Hospital for Children and Harvard Medical School, Boston, MA 02114, USA.
Clin Pediatr (Phila). 2012 Dec;51(12):1140-9. doi: 10.1177/0009922812456238. Epub 2012 Aug 14.
To describe the phenotype of premature adrenarche (PA) patients, elucidate historical and physical correlates, and distinguish PA from late-onset congenital adrenal hyperplasia (LOCAH).
Retrospective chart review of 122 patients (91 female and 31 male) with PA and 11 with LOCAH.
In PA patients, birth weight was <2 standard deviation scores (SDSs) in 6%, and 14% were premature. Body mass index SDSs were high (2.00 ± 1.84). Bone age (BA) SDS was >2 in 31% and was greater in boys than in girls; PA subjects were taller, and the predicted height was at or above genetic potential. Dehydroepiandrosterone sulfate (DHEAS) was higher in boys. LOCAH patients had earlier pubic hair, more advanced BA, lower height SDSs, lower adult height prediction, and higher adrenal androgen levels.
Patients with PA tend to be overweight with BA acceleration but normal height prediction. LOCAH is distinguished by earlier presentation and higher 17-hydrocyprogesterone and adrenal androgens.
描述性早熟（PA）患者的表型，阐明其病史和体格检查相关因素，并将PA与迟发型先天性肾上腺皮质增生症（LOCAH）相鉴别。
对122例PA患者（91例女性和31例男性）以及11例LOCAH患者进行回顾性病历审查。
在PA患者中，6%的患者出生体重低于2个标准差评分（SDSs），14%为早产儿。体重指数SDSs较高（2.00±1.84）。31%的患者骨龄（BA）SDS大于2，且男孩高于女孩；PA患者更高，预测身高达到或高于遗传潜能。男孩的硫酸脱氢表雄酮（DHEAS）水平更高。LOCAH患者阴毛出现更早，骨龄更超前，身高SDSs更低，成人身高预测值更低，肾上腺雄激素水平更高。
PA患者往往超重且骨龄加速，但身高预测正常。LOCAH的特点是发病更早，17-羟孕酮和肾上腺雄激素水平更高。
