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苏格兰儿童中促肾上腺皮质激素分泌亢进症的夸大表现:临床特征和生物化学。

Exaggerated adrenarche in a cohort of Scottish children: clinical features and biochemistry.

机构信息

Royal Hospital for Sick Children, Glasgow, UK.

出版信息

Clin Endocrinol (Oxf). 2010 Apr;72(4):496-501. doi: 10.1111/j.1365-2265.2009.03739.x. Epub 2009 Oct 26.

Abstract

OBJECTIVE

To investigate the reported association between exaggerated adrenarche (EA) and reduced foetal growth and to identify possible risk factors for future morbidity in Scottish children with clinical features of EA.

DESIGN

Three-year prospective study.

MEASUREMENTS

Auxology, blood pressure (BP), biochemical analysis of blood and urine, pelvic ultrasound in girls.

RESULTS

Fifty-two patients were recruited of whom one girl had nonclassical congenital adrenal hyperplasia (17-OHP 17 nmol/l) and one had insufficient blood for analysis. The final cohort comprised 42 girls of mean (SD) age 7.7 (0.99) and eight boys of 8.8 (0.67) years. Mean (SD) birth weight was 3.27 (0.49) and 3.10 (0.76) kg in girls and boys respectively. Height/weight SDS were 1.13/1.69 in girls and 1.69/1.88 in boys. Mean systolic/diastolic BP was 107.8/60.4 (50th-75th centile) in girls and 115.5/63.9 (75th-91st centile) in boys. Uterine and ovarian development was prepubertal. Median serum dehydroepiandrosterone sulphate (DHEAS) was 2.1 and 4.1 mumol/l, androstenedione 3.1 and 3.8 nmol/l in girls and boys respectively, with DHEAS within the reference range/undetectable in 18/2 and androstenedione in 12/6 patients. Fasting insulin was 9.0 and 15.0 mU/l in girls and boys respectively, with concomitant low normal SHBG. Anti-Mullerian hormone (AMH) was 15.7 pmol/l in 27 girls, compared with 5.0 pmol/l in normal girls aged 5-8 years.

CONCLUSIONS

Our Scottish EA cohort showed female predominance, no evidence of reduced foetal growth, a tendency to overweight with commensurate mild hyperinsulinaemia and modest elevation of serum androgens in some patients. We have found raised AMH levels in the girls, indicating advanced ovarian follicular development.

摘要

目的

探讨肾上腺功能亢进(EA)与胎儿生长受限之间的关联,并确定苏格兰具有 EA 临床特征的儿童未来发病的可能风险因素。

设计

前瞻性三年研究。

测量

人体测量学、血压(BP)、血液和尿液的生化分析、女孩盆腔超声。

结果

共招募了 52 名患者,其中 1 名女孩患有非经典先天性肾上腺皮质增生症(17-羟孕酮 17nmol/L),1 名女孩血样不足无法进行分析。最终的队列包括 42 名年龄均为 7.7(0.99)岁的女孩和 8 名年龄为 8.8(0.67)岁的男孩。女孩和男孩的平均(SD)出生体重分别为 3.27(0.49)和 3.10(0.76)kg。身高/体重 SDS 分别为 1.13/1.69 的女孩和 1.69/1.88 的男孩。女孩的平均收缩压/舒张压为 107.8/60.4(50-75 百分位),男孩为 115.5/63.9(75-91 百分位)。子宫和卵巢发育处于青春期前阶段。女孩血清脱氢表雄酮硫酸酯(DHEAS)中位数为 2.1 和 4.1umol/L,雄烯二酮中位数为 3.1 和 3.8nmol/L,18/2 名女孩的 DHEAS 值处于参考范围内/无法检测,6/6 名女孩的雄烯二酮值处于参考范围内/无法检测。女孩的空腹胰岛素为 9.0 和 15.0mU/L,同时伴有低正常的性激素结合球蛋白(SHBG)。27 名女孩的抗苗勒管激素(AMH)为 15.7pmol/L,而 5-8 岁正常女孩的 AMH 为 5.0pmol/L。

结论

我们的苏格兰 EA 队列显示女性占优势,没有证据表明胎儿生长受限,超重的趋势伴有相应的轻度高胰岛素血症,一些患者的血清雄激素适度升高。我们发现女孩的 AMH 水平升高,表明卵巢卵泡发育成熟。

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