许多患有先天性肾上腺增生(CAH)的患者避免怀孕,患有不孕症或担心怀孕并发症,主要是因为生殖器异常,无排卵,子宫内膜和代谢紊乱。尽管面临这些挑战,据报道,许多活产。在这次系统审查中,我们重点研究了辅助生殖策略成功的关键和潜在的妊娠并发症.我们对Pubmed进行了系统的文献检索,Medline和Scopus报道了除21-羟化酶缺乏症以外的CAH成功怀孕的文章,发现25项研究报告了39例妊娠,涵盖了类固醇急性调节蛋白的缺乏,17α-羟化酶/17,20-裂解酶,11β-羟化酶,P450氧化还原酶,细胞色素b5和3β-羟基类固醇脱氢酶。我们总结了每种亚型的各种临床表现和量身定制的生殖策略。此外,进行了一项荟萃分析,以评估CAH患者的妊娠并发症.共纳入19项横断面或队列研究,涉及1311例经典和非经典CAH患者的妊娠。令人惊讶的是,高达5.5%(95%CI2.3%-9.7%)的妊娠被选择性流产,与仅有经典CAH的患者相比,经典CAH比例较高的研究风险明显更高(8.43%(4.1%-13.81%)VS3.75%(1.2%-7.49%)),这要求更好的计划生育。与对照组相比,流产的合并发生率为18.2%(13.4%-23.4%),相对风险(RR)为1.86(1.27-2.72)。与未治疗组相比,非经典CAH患者的糖皮质激素治疗显着降低了流产率(RR0.25(0.13-0.47))。CAH患者也更容易患妊娠期糖尿病,患病率为7.3%(2.4%-14.1%),RR为2.57(1.29-5.12)。然而,先兆子痫的风险,早产和小于胎龄没有显著差异.67.8%(50.8%-86.9%)的CAH患者行剖宫产,为对照组的3.86(1.66-8.97)倍。这些结果表明,对于CAH患者来说,生育是可能的,但在计划时需要特别护理,寻找和怀孕期间。
PROSPEROhttps://www.crd.约克。AC.uk/PROSPERO/display_record。php?RecordID=342642,CRD42022342642。
Many patients with congenital adrenal hyperplasia (CAH) refrain from seeking pregnancy, suffer from infertility or worry about pregnancy complications, mainly due to genitalia abnormalities, anovulation, unreceptive endometrium and metabolic disturbances. Despite those challenges, many live births have been reported. In this systematic
review, we focused on the key to successful assisted reproduction strategies and the potential pregnancy complications. We did a systematic literature search of Pubmed, Medline and Scopus for articles reporting successful pregnancies in CAH other than 21-hydroxylase deficiency, and found 25 studies reporting 39 pregnancies covering deficiency in steroidogenic acute regulatory protein, 17α-hydroxylase/17,20-lyase, 11β-hydroxylase, P450 oxidoreductase, cytochrome b5 and 3β-hydroxysteroid dehydrogenase. We summarized various clinical manifestations and tailored reproduction strategy for each subtype. Furthermore, a meta-analysis was performed to evaluate the pregnancy complications of CAH patients. A total of 19 cross-sectional or cohort studies involving 1311 pregnancies of classic and non-classic CAH patients were included. Surprisingly, as high as 5.5% (95% CI 2.3%-9.7%) of pregnancies were electively aborted, and the risk was significantly higher in those studies with a larger proportion of classic CAH than those with only non-classical patients (8.43% (4.1%-13.81%) VS 3.75%(1.2%-7.49%)), which called for better family planning. Pooled incidence of miscarriage was 18.2% (13.4%-23.4%) with a relative risk (RR) of 1.86 (1.27-2.72) compared to control. Glucocorticoid treatment in non-classical CAH patients significantly lowered the miscarriage rate when compared to the untreated group (RR 0.25 (0.13-0.47)). CAH patients were also more susceptible to gestational diabetes mellitus, with a prevalence of 7.3% (2.4%-14.1%) and a RR 2.57 (1.29-5.12). However, risks of preeclampsia, preterm birth and small for gestational age were not significantly different. 67.8% (50.8%-86.9%) CAH patients underwent Cesarean delivery, 3.86 (1.66-8.97) times the risk of the control group. These results showed that fertility is possible for CAH patients but special care was necessary when planning, seeking and during pregnancy.
PROSPERO https://www.crd.york.ac.uk/PROSPERO/display_record.php?RecordID=342642, CRD42022342642.