Abstract

Adrenarche is the maturational increase in adrenal androgen production that normally begins in early childhood. It results from changes in the secretory response to adrenocorticotropin (ACTH) that are best indexed by dehydroepiandrosterone sulfate (DHEAS) rise. These changes are related to the development of the zona reticularis (ZR) and its unique gene/enzyme expression pattern of low 3ß-hydroxysteroid dehydrogenase type 2 with high cytochrome b5A, sulfotransferase 2A1, and 17ß-hydroxysteroid dehydrogenase type 5. Recently 11-ketotestosterone was identified as an important bioactive adrenarchal androgen. Birth weight, body growth, obesity, and prolactin are related to ZR development. Adrenarchal androgens normally contribute to the onset of sexual pubic hair (pubarche) and sebaceous and apocrine gland development. Premature adrenarche causes ≥90% of premature pubarche (PP). Its cause is unknown. Affected children have a significantly increased growth rate with proportionate bone age advancement that typically does not compromise growth potential. Serum DHEAS and testosterone levels increase to levels normal for early female puberty. It is associated with mildly increased risks for obesity, insulin resistance, and possibly mood disorder and polycystic ovary syndrome. Between 5% and 10% of PP is due to virilizing disorders, which are usually characterized by more rapid advancement of pubarche and compromise of adult height potential than premature adrenarche. Most cases are due to nonclassic congenital adrenal hyperplasia. Algorithms are presented for the differential diagnosis of PP. This review highlights recent advances in molecular genetic and developmental biologic understanding of ZR development and insights into adrenarche emanating from mass spectrometric steroid assays.

中文翻译：

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正常和早发性肾上腺皮质激素

摘要

肾上腺皮质激素是通常在儿童早期开始的肾上腺雄激素产生的成熟增加。它是由对促肾上腺皮质激素 (ACTH) 的分泌反应的变化引起的，而脱氢表雄酮硫酸盐 (DHEAS) 的升高最能反映这一变化。这些变化与网状带 (ZR) 的发展及其独特的基因/酶表达模式有关：低 3ß-羟基类固醇脱氢酶 2 型与高细胞色素 b5A、磺基转移酶 2A1 和 17ß-羟基类固醇脱氢酶 5 型。最近 11-酮睾酮被确定为一种重要的生物活性肾上腺雄激素。出生体重、身体发育、肥胖和催乳素与 ZR 发育有关。肾上腺雄激素通常有助于性阴毛 (pubarche) 和皮脂腺和大汗腺的发育。早发性肾上腺皮质机能导致≥90% 的过早阴毛 (PP)。其原因尚不清楚。受影响儿童的生长速度显着增加，骨龄按比例增长，通常不会损害生长潜力。血清 DHEAS 和睾酮水平增加到女性青春期早期的正常水平。它与肥胖、胰岛素抵抗以及可能的情绪障碍和多囊卵巢综合征的风险轻度增加有关。5% 到 10% 的 PP 是由于男性化障碍引起的，其特征通常是阴毛发育较快，成人身高潜力比肾上腺发育早。大多数病例是由于非典型的先天性肾上腺增生。提出了用于鉴别诊断 PP 的算法。