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适合胎龄女孩的早发性肾上腺功能亢进症的严重程度与临床结局的相互关系。

Interrelationship of extent of precocious adrenarche in appropriate for gestational age girls with clinical outcome.

机构信息

Jesse Z and Sara Lea Shafer Institute for Endocrinology and Diabetes, National Center for Childhood Diabetes, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

出版信息

J Pediatr. 2012 Feb;160(2):308-13. doi: 10.1016/j.jpeds.2011.08.009. Epub 2011 Sep 9.

Abstract

OBJECTIVE

To assess the interrelationship between extent of adrenarche at presentation in girls with precocious adrenarche (PA) born appropriate for gestational age and their growth pattern, pubertal course and adult height.

STUDY DESIGN

We reviewed clinical and laboratory data from medical charts of 85 girls with PA aged 5.0 to 8.8 years at referral, stratified in 3 subgroups according to bone age (BA) minus chronological age (CA) ≤0 years; 0 <BA minus CA <1 year; BA minus CA >1 year.

RESULTS

Extent of pubarche and dehydroepiandrosterone-sulfate levels were greatest in the BA-CA >1 subgroup (P=.02, P=.008, respectively), who also were taller at diagnosis (P=.002) and during childhood (P=.01). In all subgroups, pubertal onset was within normal range; menarche occurred earlier than in control subjects (P<.02); all attained adult height within their mid-parental height range. Predicted adult height was overestimated in girls with BA ≤CA (P=.04) and underestimated with BA-CA >1 (P<.001).

CONCLUSIONS

Although a more pronounced adrenarche and BA advancement at diagnosis in girls with PA born appropriate for gestational age had an impact on their pre-pubertal growth pattern, it was not associated with early and rapid progression of puberty or reduced adult height. This reassuring clinical course indicates that PA is a benign condition, irrespective of the extent of adrenarche at presentation. Adult height prediction is unreliable in PA.

摘要

目的

评估在适当胎龄出生的性早熟性肾上腺皮质增生症(PA)女孩中,就诊时肾上腺皮质功能初现的程度与生长模式、青春期进程和成人身高之间的相互关系。

研究设计

我们回顾了 85 名就诊时年龄为 5.0 至 8.8 岁的 PA 女孩的病历中的临床和实验室数据,根据骨龄(BA)减去实际年龄(CA)≤0 岁、0<BA 减去 CA<1 岁、BA 减去 CA>1 岁将其分为 3 个亚组。

结果

BA-CA>1 岁亚组的阴毛发育程度和硫酸脱氢表雄酮(DHEA-S)水平最高(P=.02,P=.008),该亚组在诊断时(P=.002)和儿童期(P=.01)身高更高。在所有亚组中,青春期开始均处于正常范围内;初潮发生时间早于对照组(P<.02);所有女孩的成人身高均在其父母身高的中间范围内。BA≤CA 的女孩预测成人身高偏高(P=.04),BA-CA>1 的女孩预测成人身高偏低(P<.001)。

结论

尽管在适当胎龄出生的 PA 女孩中,就诊时肾上腺皮质功能初现程度更明显、BA 提前,对其青春期前的生长模式有影响,但与青春期提前和快速进展或成人身高降低无关。这种令人安心的临床病程表明,PA 是一种良性疾病,与就诊时肾上腺皮质功能初现的程度无关。PA 患者的成人身高预测不可靠。

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