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回顾性评估法乐氏家族性地中海热患者的产科处理过程:一家三级风湿病诊所的三年经验。

Retrospective evaluation of obstetric processes in patients with Familial Mediterranean Fever's disease: The three years experience of a tertiary rheumatology clinic.

机构信息

University of Health Sciences, Başakşehir Çam and Sakura City Hospital, Department of Rheumatology, İstanbul, Turkey; Marmara University, School of Medicine, Faculty of Health Science, Department of Medical Biology and Genetics, İstanbul, Turkey.

İstanbul Medipol University School of Medicine, Department of Gynecology and Obstetrics, İstanbul, Turkey.

出版信息

Taiwan J Obstet Gynecol. 2024 Nov;63(6):900-903. doi: 10.1016/j.tjog.2024.09.008.

DOI:10.1016/j.tjog.2024.09.008
PMID:39482000
Abstract

OBJECTIVES

Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease affecting both genders in reproductive age. In this study, we aimed to investigate the relation between FMF and pregnancy on both maternal and fetal aspects.

MATERIAL AND METHODS

In this retrospective, single-center, descriptive study we analysed total of 95 pregnancies of 40 FMF patients. Clinical and demographic data were obtained from patients' records. To prevent recall bias, only the last pregnancy of each patient was evaluated for disease activity and use or revision of medications during pregnancy.

RESULTS

The median age of the patients at diagnosis was 22 and the first pregnancy age was 26 years. The median duration of FMF at last pregnancy was 8 (0-23) years. Eight (20%) patients had at least 1 pregnancy via assisted reproductive techniques (IVF), while 34 (85%) patients had at least 1 spontaneous pregnancy. While 32 patients were in remission (80%) before pregnancy, 8 were clinically active (20%). Improvement in clinical course and attack frequency during pregnancy was observed in 23 patients (57.5%), stable course in 10 (25.0%), and worsening in 7 (17.5%). The rate of live birth was 70.0%, abortus was 28.9%, preterm labor was 8.1%, pre-eclampsia was 5.0%, and only 1 achondroplasia as congenital fetal abnormality was observed.

CONCLUSION

FMF did not constitute a contraindication for pregnancy. The most important obstetric problems, complications, and negative fetal outcomes in the course of pregnancy are increased IVF requirement, abortion, and cesarean rates. There is no increase in the risk of congenital malformations due to FMF itself or use of colchicine.

摘要

目的

家族性地中海热(FMF)是一种影响生育年龄两性的遗传性自身炎症性疾病。本研究旨在探讨 FMF 与妊娠对母婴双方的影响。

材料和方法

在这项回顾性、单中心、描述性研究中,我们分析了 40 名 FMF 患者的 95 例妊娠。临床和人口统计学数据来自患者的病历。为了防止回忆偏倚,仅评估每位患者的最后一次妊娠的疾病活动以及妊娠期间药物的使用或修订。

结果

患者诊断时的中位年龄为 22 岁,首次妊娠年龄为 26 岁。最后一次妊娠时 FMF 的中位病程为 8 年(0-23 年)。8 名(20%)患者通过辅助生殖技术(IVF)至少有一次妊娠,34 名(85%)患者至少有一次自然妊娠。32 名患者在妊娠前处于缓解状态(80%),8 名患者处于临床活动状态(20%)。23 名患者(57.5%)妊娠期间临床病程和发作频率改善,10 名患者(25.0%)病情稳定,7 名患者(17.5%)病情恶化。活产率为 70.0%,流产率为 28.9%,早产率为 8.1%,子痫前期发生率为 5.0%,仅观察到 1 例胎儿软骨发育不全的先天性胎儿异常。

结论

FMF 并不构成妊娠的禁忌证。妊娠过程中最重要的产科问题、并发症和不良胎儿结局是增加 IVF 需求、流产和剖宫产率。FMF 本身或秋水仙碱的使用并不会增加先天性畸形的风险。

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