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21-羟化酶缺乏所致先天性肾上腺皮质增生症新生儿的出生体重

Birth Size in Neonates with Congenital Adrenal Hyperplasia due to 21-hydroxylase Deficiency.

作者信息

Dörr Helmuth G., Penger Theresa, Albrecht Andrea, Marx Michaela, Völkl Thomas M. K.

机构信息

University Hospital of Erlangen, Department of Paediatrics, Division of Paediatrics Endocrinology, Erlangen, Germany

出版信息

J Clin Res Pediatr Endocrinol. 2019 Feb 20;11(1):41-45. doi: 10.4274/jcrpe.galenos.2018.2018.0149. Epub 2018 Sep 4.

DOI:10.4274/jcrpe.galenos.2018.2018.0149
PMID:30178749
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6398197/
Abstract

OBJECTIVE

Classic congenital adrenal hyperplasia (CAH) secondary to 21-hydroxylase deficiency is characterized by increased prenatal adrenal androgen secretion. There are a small number of reports in the literature showing higher birth weight and length in CAH newborns.

METHODS

We analyzed birth weight and length data of 116 German newborns (48 boys, 68 girls) with classic CAH who were born during the period from 1990 to 2017. All children have been followed or are currently treated as outpatients in our clinic. All children were born at term. The mothers were healthy and their pregnancies were uneventful. The diagnosis of CAH was confirmed by molecular analyses of the gene. Birth data were calculated as standard deviation (SD) scores according to German reference values.

RESULTS

Weight and length in male CAH newborns (mean ± SD) (3601±576 g; 52.4±2.85 cm) were significantly higher than in female CAH newborns (3347±442 g; 51.2±2.55 cm), but male-female differences in the CAH cohort were lost when the data were converted into SD scores. The birth sizes of the CAH newborns did not differ from the reference group. The birth sizes also did not differ between the different CAH genotypes. Maternal age, mode of delivery and maternal parity had no influence on birth size.

CONCLUSION

Our data show that prenatal hyperandrogenism does not affect fetal growth.

摘要

目的

21-羟化酶缺乏所致经典型先天性肾上腺皮质增生症(CAH)的特征是产前肾上腺雄激素分泌增加。文献中有少数报道显示CAH新生儿出生体重和身长较高。

方法

我们分析了1990年至2017年期间出生的116例患有经典型CAH的德国新生儿(48例男孩,68例女孩)的出生体重和身长数据。所有儿童均在我们诊所接受随访或目前作为门诊患者接受治疗。所有儿童均为足月出生。母亲健康,孕期无异常。CAH的诊断通过对相关基因的分子分析得以证实。出生数据根据德国参考值计算为标准差(SD)评分。

结果

男性CAH新生儿的体重和身长(均值±标准差)(3601±576 g;52.4±2.85 cm)显著高于女性CAH新生儿(3347±442 g;51.2±2.55 cm),但当数据转换为SD评分时,CAH队列中的男女差异消失。CAH新生儿的出生大小与参考组无差异。不同CAH基因型之间的出生大小也无差异。母亲年龄、分娩方式和母亲产次对出生大小均无影响。

结论

我们的数据表明产前雄激素过多并不影响胎儿生长。

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