Premature adrenarche in Prader-Willi syndrome is associated with accelerated pre-pubertal growth and advanced bone age.
Premature adrenarche in Prader-Willi syndrome is associated with accelerated pre-pubertal growth and advanced bone age.
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Prader-Willi综合征的过早肾上腺素与加速前的前骨生长和晚期骨时代有关。
DOI：
10.1515/jpem-2022-0468
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发表时间：
2023-02-23
期刊：
影响因子：
--
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Prader-Willi综合征（PWS）的特征是肥胖、生长激素缺乏、性腺功能减退和尽管报告了下丘脑-垂体-肾上腺轴功能障碍但仍有高发病率的过早肾上腺初显。虽然特发性过早肾上腺初显与青春期前生长加速和骨龄提前有关，但肾上腺雄激素升高对PWS生长和骨成熟的影响仍不清楚。因此，本研究旨在描述与年龄相关的硫酸脱氢表雄酮（DHEAS）的变化及其对PWS生长和骨成熟的影响。这项回顾性观察性研究包括62名PWS儿童。建立了年龄、BMI-SDS与DHEAS水平的简单和多元回归模型。身高增长速度与基于年龄和性别的标准进行比较，采用t检验和双因素方差分析。骨龄Z值的模式采用双因素方差分析，年龄、BMI SDS和DHEAS对骨龄Z值的贡献采用多元回归分析。与健康对照组（R2 =0.89和0.88）相比，PWS男性和女性（R2=0.12和0.30）的DHEAS水平升高较早，与年龄的相关性较低，与BMI-SDS无关（校正R2=0.076，年龄p=0.10，BMI-SDS为0.29）。在预期青春期年龄下降之前，PWS男性儿童中期身高增长速度增加，女性保持不变（p<0.0001）。肾上腺皮质周围骨龄提前的方式与DHEAS相关，但与BMI-SDS无关（p<0.0001;调整后的R2=0.48，DHEAS p=0.0014，BMI-SDS p = 0.78）。非肥胖性肾上腺雄激素增加与PWS儿童中期生长和骨成熟加速有关。
Prader–Willi syndrome (PWS) is characterized by obesity, growth hormone deficiency, hypogonadism, and a high prevalence of premature adrenarche despite reported hypothalamic–pituitary–adrenal axis dysfunction. While idiopathic premature adrenarche is associated with accelerated pre-pubertal growth and advanced bone age, the consequences of elevated adrenal androgens on growth and bone maturation in PWS remain unknown. This study therefore sought to describe age-related changes in dehydroepiandrosterone sulfate (DHEAS) and their effects on growth and bone maturation in PWS. This retrospective observational study included 62 children with PWS. Simple and multiple regression models were constructed to relate age and BMI-SDS with DHEAS levels. Height velocity was compared to age and sex-based norms with t-tests and two-way ANOVA. Patterns in bone age Z-score were examined with two-way ANOVA, and the contributions of age, BMI-SDS, and DHEAS to bone age Z-score were analyzed with multiple regression. DHEAS levels rose earlier and were less strongly correlated with age in males and females with PWS (R2=0.12 and 0.30) compared to healthy controls (R2=0.89 and 0.88) in a pattern unrelated to BMI-SDS (adjusted R2=0.076, p=0.10 for age, and 0.29 for BMI-SDS). Mid-childhood height velocity was increased in males and preserved in females with PWS before declining at the age of expected puberty (p<0.0001). Peri-adrenarchal bone age was advanced in a manner associated with DHEAS but not BMI-SDS (p<0.0001; adjusted R2=0.48, p=0.0014 for DHEAS, and 0.78 for BMI-SDS). An obesity-independent increase in adrenal androgens is associated with accelerated mid-childhood growth and bone maturation in PWS.
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DOI：
10.1056/nejmoa1302160
发表时间：
2013-06-27
期刊：
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影响因子：
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影响因子：
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VICENSCALVET, E