在遗传性血管性水肿病例中,使用促黄体激素释放激素注射的体外受精导致健康三胞胎的发生率未增加。

Pub Date : 2018-02-13 eCollection Date: 2018-01-01 DOI:10.1155/2018/2706751
Ceyda Tunakan Dalgıç, Fatma Düşünür Günsen, Gökten Bulut, Emine Nihal Mete Gökmen, Aytül Zerrin Sin
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引用次数: 4

摘要

c1抑制剂缺乏引起的遗传性血管性水肿(C1-INH-HAE)是一种罕见的常染色体显性遗传病。妊娠期C1-INH-HAE患者的管理对医生来说是一个挑战。静脉注射血浆源性纳米过滤C1-INH (pdC1INH)是妊娠、产后和哺乳期唯一推荐的选择。为了提高怀孕率,医生使用受精疗法来增加雌激素的内源性水平。因此,这些技术会增加C1-INH-HAE患者水肿发作的次数和严重程度。我们的患者是一名32岁的女性,自2004年以来被诊断为C1-INH-HAE 1型。患者服用达那唑50- 200mg /天9年。由于她在2013年有怀孕计划,所以停用了那那唑。PdC1INH被定期用于预防目的。使用促黄体生成素释放激素(LHRH)进行体外受精发生三胞胎妊娠。在我们的患者中,在体外受精过程中进行了四次LHRH注射,没有引起任何严重的攻击。在怀孕和分娩期间,由于预防性静脉注射pdC1INH,血管水肿没有恶化。根据发作频率和严重程度,三个妊娠期之间没有差异。据我们所知,这是首次发表的C1-INH-HAE在妊娠和分娩期间接受体外受精治疗而无血管性水肿发作的病例,并在预防性静脉注射pdC1INH的情况下最终生下了健康的三胞胎。
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In Vitro Fertilization Using Luteinizing Hormone-Releasing Hormone Injections Resulted in Healthy Triplets without Increased Attack Rates in a Hereditary Angioedema Case.

Hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. The management of pregnant patients with C1-INH-HAE is a challenge for the physician. Intravenous plasma-derived nanofiltered C1-INH (pdC1INH) is the only recommended option throughout pregnancy, postpartum, and breastfeeding period. In order to increase pregnancy rates, physicians use fertilization therapies increasing endogen levels of estrogens. Therefore, these techniques can provoke an increase in the number and severity of edema attacks in C1-INH-HAE. Our patient is a 32-year-old female, diagnosed with C1-INH-HAE type 1 since 2004. She had been taking danazol 50-200 mg/day for 9 years. Due to her pregnancy plans in 2013, danazol was discontinued. PdC1INH was prescribed regularly for prophylactic purpose. Triplet pregnancy occurred by in vitro fertilization using luteinizing hormone-releasing hormone (LHRH) injections. In our patient, LHRH injections were done four times without causing any severe attack during in vitro fertilization. Angioedema did not worsen during pregnancy and delivery due to the prophylactic use of intravenous pdC1INH in our patient. According to the attack frequency and severity, there was no difference between the three pregnancy trimesters. To our knowledge, this is the first published case of C1-INH-HAE receiving in vitro fertilization therapies without any angioedema attacks during pregnancy and delivery and eventually having healthy triplets with the prophylactic use of intravenous pdC1INH.

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