Lazar L, Kauli R, Bruchis C, Nordenberg J, Galatzer A, Pertzelan A
Institute of Pediatric and Adolescent Endocrinology, Children's Medical Center of Israel, Petah Tiqva, Israel.
Eur J Endocrinol. 1995 Oct;133(4):407-11. doi: 10.1530/eje.0.1330407.
Abnormal adrenal response is often observed in girls with precocious adrenarche (1). We studied the adrenal response in 112 girls with idiopathic true central precocious puberty (CPP) at early stages of puberty compared to that in 21 girls with normal puberty (controls). The aims of this study were to determine the prevalence of abnormal adrenal response at early stages of puberty, the possible correlation of abnormal adrenal response with pubertal signs at onset of puberty and with plasma androgen levels, and a possible association with the activity of the hypothalamic-pituitary-gonadal (HPG) axis. All participants underwent a combined i.v. adrenocorticotropic hormone (ACTH)-gonadotropin-releasing hormone (GnRH) test at Tanner stage 2-3: 62 of the CPP girls before and 50 during treatment with GnRH analog. The stimulated levels of 17-hydroxypregnenolone (17OHPreg) and the stimulated 17OHPreg/17-hydroxyprogesterone ratio were analyzed and compared to previously reported norms. The result revealed three patterns of adrenal response: normal (17OHPreg < or = 24 nmol/l and 17OHPreg/17OHP ratio < or = 7) in 50/112 (44.6%) CPP patients and 17/21 (80.9%) controls; exaggerated (17OHPreg > 24 nmol/l, 17OHPreg/17OHP ratio < or = 7) in 50/112 (44.6%) CPP patients and 3/21 (14.3%) controls; and non-classical 3 beta-hydroxysteroid dehydrogenase deficiency (17OHPreg > 24 nmol/l and 17OHPreg/17OHP ratio > 7) in 12/112 (10.8%) CPP patients and 1/21 (4.8%) controls. The clinical features at onset of puberty were comparable in all girls with the CPP in spite of the different adrenal response patterns.(ABSTRACT TRUNCATED AT 250 WORDS)
性早熟的女孩常观察到肾上腺反应异常（1）。我们研究了112名特发性真性中枢性性早熟（CPP）女孩在青春期早期的肾上腺反应，并与21名青春期正常女孩（对照组）进行比较。本研究的目的是确定青春期早期肾上腺反应异常的患病率、肾上腺反应异常与青春期开始时的青春期体征及血浆雄激素水平之间的可能相关性，以及与下丘脑 - 垂体 - 性腺（HPG）轴活性的可能关联。所有参与者在坦纳2 - 3期接受了静脉注射促肾上腺皮质激素（ACTH） - 促性腺激素释放激素（GnRH）联合试验：62名CPP女孩在GnRH类似物治疗前，50名在治疗期间。分析了刺激后的17 - 羟孕烯醇酮（17OHPreg）水平和刺激后的17OHPreg/17 - 羟孕酮比值，并与先前报道的标准进行比较。结果显示肾上腺反应有三种模式：正常（17OHPreg≤24 nmol/l且17OHPreg/17OHP比值≤7），50/112（44.6%）的CPP患者和17/21（80.9%）的对照组；过度反应（17OHPreg＞24 nmol/l，17OHPreg/17OHP比值≤7），50/112（44.6%）的CPP患者和3/21（14.3%）的对照组；以及非经典3β - 羟类固醇脱氢酶缺乏（17OHPreg＞24 nmol/l且17OHPreg/17OHP比值＞7），12/112（10.8%）的CPP患者和1/21（4.8%）的对照组。尽管肾上腺反应模式不同，但所有CPP女孩青春期开始时的临床特征具有可比性。（摘要截短至250字）
