Pregnancy and obstetrical outcomes in pseudoxanthoma elasticum
Pregnancy and obstetrical outcomes in pseudoxanthoma elasticum
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DOI：
10.1111/j.1365-2133.2004.06183.x
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发表时间：
2004-11-01
影响因子：
10.3
通讯作者：
Weinstock, MA
中科院分区：
文献类型：
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作者：
Bercovitch, L;Leroux, T;Weinstock, MA
背景弹性纤维假黄瘤（Pseudoxanthoma elasticum，PXE）是一种遗传性多系统疾病，以皮肤、视网膜和动脉中的钙化性营养不良弹性纤维为特征。许多关于妊娠PXE的早期文献包含严重并发症的报告，目的探讨妊娠合并PXE妇女的产科结局和妊娠并发症的发生率，并确定妊娠是否与疾病进程的不良影响有关。方法对2004年10月至2005年12月在我院就诊的100例PXE妇女（n = 10）进行回顾性分析，并对妊娠合并PXE妇女的临床资料进行统计学分析。结果妊娠合并PXE妇女的产科结局与妊娠并发症的发生率无显著性差异（P > 0.05）。结论妊娠合并PXE妇女的产科结局与妊娠并发症的发生率有显著性差异（P < 0.05）。407）回答了关于生育史和怀孕以及疾病过程的详细问卷。确定报告的妊娠结局和并发症的频率。结果在306例有妊娠史的PXE患者中，有795例妊娠。其中，83%为活产，1%为死产。中位出生体重在正常范围内，妊娠期低出生体重的发生率较低。10%的孕妇发生高血压，胃出血和视网膜并发症<1%，12%的孕妇伴有皮肤症状恶化。在40岁以上的女性中，妊娠和临床严重程度对皮肤（P = 0.07）、眼部（P = 0.59）或心脏（P = 0.42）的PXE表现没有影响，妊娠也不会对这些临床严重程度指数产生不利影响。101名妇女谁从来没有怀孕过，17%的决定，因为他们建议不要怀孕的医疗保健专业人员和11%没有怀孕，因为他们担心在他们的怀孕或diseases.Conclusions不良后果PXE是不相关的显着增加胎儿丢失或不良生殖结果。胃出血的发生率，虽然可能高于未受影响的人群，但比以前报道的要低得多，视网膜并发症也不常见。虽然少数妊娠与皮肤表现恶化有关，但妊娠或曾经妊娠与皮肤、眼部或心血管表现的最终严重程度无关。建议患有PXE的妇女避免怀孕是没有依据的，大多数PXE妊娠都不复杂。
Background Pseudoxanthoma elasticum (PXE) is a genetic multisystem disorder characterized by calcified dystrophic elastic fibres in skin, retina and arteries. Much of the earlier literature on pregnancy in PXE contained reports of severe complications, leading some healthcare providers to advise women with PXE against becoming pregnant and some women with PXE to avoid pregnancy.Objectives To evaluate the obstetrical outcomes and the incidence of pregnancy complications in women with PXE and to determine if pregnancy is associated with an adverse effect on the course of the disease.Methods Women with PXE (n = 407) answered detailed questionnaires regarding reproductive history and pregnancy as well as the course of their disease. The frequency of reported pregnancy outcomes and complications was determined. Severity indices for the major clinical manifestations of PXE were developed and correlated with gravidity of affected women aged 40 years or over.Results Among the 306 respondents with PXE who had ever been pregnant, there were 795 pregnancies. Of these, 83% ended in live births and 1% in stillbirth. The median birth weight was within the normal range and the incidence of low birth weight for gestation was low. Hypertension occurred in 10% of pregnancies, gastric bleeding and retinal complications in < 1%, and 12% of pregnancies were associated with worsening of skin manifestations. There was no effect of gravidity and clinical severity on cutaneous (P = 0.07), ocular (P = 0.59) or cardiac (P = 0.42) manifestations of PXE in women aged 40+ years, nor did ever having been pregnant adversely affect these clinical severity indices. Of the 101 women who had never been pregnant, 17% made the decision because they were advised against becoming pregnant by a healthcare professional and 11% did not become pregnant because they feared an adverse outcome either in their pregnancy or disease.Conclusions PXE is not associated with markedly increased fetal loss or adverse reproductive outcomes. The incidence of gastric bleeding, although probably higher than in the unaffected population, is much lower than previously reported, and retinal complications are uncommon. Although a few pregnancies were associated with worsening of skin manifestations, there was no correlation of either gravidity or ever having been pregnant with ultimate severity of skin, ocular or cardiovascular manifestations. There is no basis for advising women with PXE to avoid becoming pregnant, and most pregnancies in PXE are uncomplicated.