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非经典型先天性肾上腺皮质增生症女性的妊娠:受孕时机和结局。

Pregnancy in women with nonclassic congenital adrenal hyperplasia: Time to conceive and outcome.

机构信息

Pediatric Endocrinology and Diabetes Unit, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

出版信息

Clin Endocrinol (Oxf). 2017 Nov;87(5):552-556. doi: 10.1111/cen.13429. Epub 2017 Aug 16.

DOI:10.1111/cen.13429
PMID:28731586
Abstract

OBJECTIVE

Nonclassic congenital adrenal hyperplasia (NCAH) is common among Ashkenazi Jews (1:400). It is associated with various degrees of postnatal virilization, irregular menses and infertility. Therapy of symptomatic subjects consists of physiologic doses of glucocorticoids. The objective of this study was to evaluate the effect of glucocorticoid treatment on fertility and on pregnancy outcome in women with NCAH.

DESIGN, SETTING AND PATIENTS: This retrospective study included 75 women diagnosed with NCAH who were followed in our clinic and sought fertility between 2008 and 2015.

RESULTS

Seventy-two women succeeded in conceiving (187 pregnancies). Time to conception was 4.0 ± 7 months without and 3.3 ± 3 months with glucocorticoid therapy (P = .43). Seventeen pregnancies were achieved by glucocorticoid therapy after failure to conceive spontaneously. Time to conception before therapy initiation was 10.2 ± 11.4 months compared to 3.3 ± 3.4 months after therapy initiation (P = .02). Of 187 pregnancies, 135 (72%) resulted in live births, 38 (20.3%) ended in spontaneous miscarriages during the first trimester, seven (3.7%) were electively terminated, three (1.6%) were ectopic and four (2.1%) were ongoing during the study with similar rate in glucocorticoid treated and untreated pregnancies.

CONCLUSIONS

The 96% pregnancy rate among our cohort of NCAH females was similar to the 95% rate reported for the normal population. Glucocorticoid therapy may shorten the time to conceive in a subgroup of women with NCAH. Glucocorticoid therapy did not affect the rate of first trimester miscarriage. Our 77% live birth rate was similar to the 72% live birth rate in the current healthy US population.

摘要

目的

非经典型先天性肾上腺皮质增生症(NCAH)在阿什肯纳兹犹太人中较为常见(1:400)。它与出生后不同程度的男性化、月经不规律和不孕有关。有症状的患者的治疗方法是使用生理剂量的糖皮质激素。本研究的目的是评估糖皮质激素治疗对 NCAH 女性生育能力和妊娠结局的影响。

设计、设置和患者:这项回顾性研究包括了 75 名在我们的诊所被诊断为 NCAH 的女性,她们在 2008 年至 2015 年间寻求生育。

结果

72 名女性成功怀孕(187 次妊娠)。未接受糖皮质激素治疗的受孕时间为 4.0±7 个月,接受治疗的受孕时间为 3.3±3 个月(P=0.43)。17 次妊娠是在未自发受孕后通过糖皮质激素治疗实现的。治疗前开始治疗的受孕时间为 10.2±11.4 个月,而治疗后为 3.3±3.4 个月(P=0.02)。在 187 次妊娠中,135 次(72%)为活产,38 次(20.3%)在孕早期自然流产,7 次(3.7%)为选择性终止妊娠,3 次(1.6%)为异位妊娠,4 次(2.1%)在研究期间仍在继续,糖皮质激素治疗组和未治疗组的妊娠结局相似。

结论

我们的 NCAH 女性队列的 96%妊娠率与正常人群的 95%妊娠率相似。糖皮质激素治疗可能缩短 NCAH 女性受孕的时间。糖皮质激素治疗并未影响孕早期流产率。我们的 77%活产率与当前美国健康人群的 72%活产率相似。

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