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患有补体基因变异介导的血栓性微血管病的肾移植受者的妊娠情况

Pregnancies in kidney transplant recipients with complement gene variant-mediated thrombotic microangiopathy.

作者信息

Haninger-Vacariu Natalja, Aigner Christof, Gaggl Martina, Kain Renate, Prohászka Zoltán, Böhmig Georg A, Sunder-Plassmann Raute, Sunder-Plassmann Gere, Schmidt Alice

机构信息

Department of Medicine III, Division of Nephrology and Dialysis, Medical University of Vienna, Vienna, Austria.

Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria.

出版信息

Clin Kidney J. 2020 Aug 20;14(4):1255-1260. doi: 10.1093/ckj/sfaa113. eCollection 2021 Apr.

Abstract

BACKGROUND

Pregnancies in patients with complement gene variant-mediated thrombotic microangiopathy (cTMA) are challenging, and pregnancies in such patients after kidney transplantation (KTX) are even more so.

METHODS

We identified nine pregnancies following KTX of three genetically high-risk cTMA patients enrolled in the Vienna thrombotic microangiopathy cohort. Preventive plasma therapy was used in three pregnancies, and one patient had ongoing eculizumab (ECU) therapy during two pregnancies.

RESULTS

Seven out of nine pregnancies (78%) resulted in the delivery of healthy children. The other two included one early abortion at gestational Week 12 during ongoing ECU therapy and one late foetal death at gestational Week 33 + 3, most likely not related to complement dysregulation. Kidney transplant function after delivery remained stable in all but one pregnancy. In the aforementioned case, a severe cTMA flare occurred after delivery despite use of preventive plasma infusions. Kidney graft function could be rescued in this patient by ECU. As such, successful pregnancies can be accomplished in kidney transplant recipients (KTRs) with a history of cTMA. We used preemptive plasma therapy or ongoing ECU treatment in selected cases.

CONCLUSIONS

Thus, becoming pregnant can be encouraged in KTRs with native kidney cTMA. Extensive preconception counselling, however, is mandatory in such cases.

摘要

背景

补体基因变异介导的血栓性微血管病(cTMA)患者的妊娠具有挑战性,而肾移植(KTX)后此类患者的妊娠更是如此。

方法

我们在维也纳血栓性微血管病队列中确定了3名遗传高危cTMA患者肾移植后的9次妊娠。3次妊娠采用了预防性血浆治疗,1名患者在两次妊娠期间持续接受依库珠单抗(ECU)治疗。

结果

9次妊娠中有7次(78%)分娩出健康婴儿。另外两次包括1次在持续ECU治疗期间妊娠第12周的早期流产和1次妊娠第33 + 3周的晚期胎儿死亡,极有可能与补体失调无关。除1次妊娠外,所有分娩后肾移植功能均保持稳定。在上述病例中,尽管使用了预防性血浆输注,但分娩后仍发生了严重的cTMA发作。该患者通过ECU挽救了肾移植功能。因此,有cTMA病史的肾移植受者(KTRs)可以实现成功妊娠。我们在特定病例中采用了抢先性血浆治疗或持续ECU治疗。

结论

因此,可以鼓励患有原发性肾cTMA的KTRs怀孕。然而,在这种情况下,进行广泛的孕前咨询是必不可少的。

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Women's Reproductive Health for the Nephrologist.肾脏病学家与女性生殖健康
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Syndromes of thrombotic microangiopathy.血栓性微血管病的综合征。
N Engl J Med. 2014 Aug 14;371(7):654-66. doi: 10.1056/NEJMra1312353.

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