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早熟肾上腺初现——一种表现多样的常见病症。

Premature adrenarche--a common condition with variable presentation.

作者信息

Utriainen Pauliina, Laakso Saila, Liimatta Jani, Jääskeläinen Jarmo, Voutilainen Raimo

机构信息

Department of Pediatrics, Kuopio University Hospital and University of Eastern Finland, Kuopio, Finland.

出版信息

Horm Res Paediatr. 2015;83(4):221-31. doi: 10.1159/000369458. Epub 2015 Feb 7.

DOI:10.1159/000369458
PMID:25676474
Abstract

Adrenarche refers to a maturational increase in the secretion of adrenal androgen precursors, mainly dehydroepiandrosterone (DHEA) and its sulfate (DHEAS). In premature adrenarche (PA), clinical signs of androgen action appear before the age of 8/9 years in girls/boys, concurrently with the circulating DHEA(S) concentrations above the usually low prepubertal level. The most pronounced sign of PA is the appearance of pubic/axillary hair, but also other signs of androgen effect (adult type body odor, acne/comedones, greasy hair, accelerated statural growth) are important to recognize. PA children are often overweight and taller than their peers, and the higher prevalence of PA in girls than in boys is probably explained by higher female adiposity and peripheral DHEA(S) conversion to active androgens. PA diagnosis requires exclusion of other causes of androgen excess: congenital adrenal hyperplasia, androgen-producing tumors, precocious puberty, and exogenous source of androgens. PA has been linked with unfavorable metabolic features including hyperinsulinism, dyslipidemia, and later-appearing ovarian hyperandrogenism. Although this common condition is usually benign, PA children with additional risk factors including obesity should be followed up, with the focus on weight and lifestyle. Long-term follow-up studies are warranted to clarify if the metabolic changes detected in PA children persist until adulthood.

摘要

肾上腺初现是指肾上腺雄激素前体分泌的成熟性增加,主要是脱氢表雄酮(DHEA)及其硫酸盐(DHEAS)。在性早熟型肾上腺初现(PA)中,女孩/男孩在8/9岁之前出现雄激素作用的临床体征,同时循环中的DHEA(S)浓度高于通常较低的青春期前水平。PA最明显的体征是阴毛/腋毛出现,但其他雄激素效应体征(成人型体味、痤疮/粉刺、油性头发、身高加速增长)也很重要,需加以识别。PA儿童通常超重且比同龄人高,女孩中PA患病率高于男孩,这可能是由于女性肥胖率较高以及外周DHEA(S)转化为活性雄激素所致。PA的诊断需要排除其他雄激素过多的原因:先天性肾上腺增生、雄激素分泌肿瘤、性早熟和外源性雄激素来源。PA与不良代谢特征有关,包括高胰岛素血症、血脂异常和后期出现的卵巢高雄激素血症。尽管这种常见情况通常是良性的,但有肥胖等额外危险因素的PA儿童应进行随访,重点关注体重和生活方式。有必要进行长期随访研究,以明确PA儿童中检测到的代谢变化是否会持续到成年期。

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