Fox James, Vegh Arthur B, Martinez-Saguer Inmaculada, Wuillemin Walter A, Edelman Jonathan, Williams-Herman Debora, Rojavin Mikhail, Rosenberg Tanja
Allergy Asthma Proc. 2017 May 1;38(3):216-221. doi: 10.2500/aap.2017.38.4038.
Increased estrogen levels during pregnancy can exacerbate hereditary angioedema (HAE), yet disease and treatment ramifications remain poorly studied in pregnant women.
Data from the international Berinert Patient Registry were used to evaluate outcomes of pregnancies exposed to plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH) during routine HAE management.
This observational registry, conducted between 2010 and 2014 at 30 U.S. and 7 European sites, gathered data on 318 subjects and 15,000 pnfC1-INH infusions. Whenever possible, the subjects who used pnfC1-INH during pregnancy were followed up to term to assess neonatal outcomes and to collect maternal adverse events (AE) that occurred up to 1 month after pnfC1-INH administration.
The registry data base included 11 pregnancies in 10 subjects who used pnfC1-INH for HAE attack treatment and/or prophylaxis (>261 doses during pregnancy). Eight pregnancies concluded in the birth of a healthy baby. Of the remaining three pregnancies: one was voluntarily terminated at 9 weeks of gestation; a second ended as a first-trimester spontaneous abortion 1 week after the subject's most recent pnfC1-INH infusion and was considered unrelated to pnf-C1INH treatment; and the third occurred in a subject who exited the registry approximately 2 months before her due date, with no further follow up. As assessed for 30 days after each pnfC1-INH infusion, there were no AEs that were considered related to pnfC1-INH therapy.
Administration of pnfC1-INH during pregnancy was generally safe and not associated with any treatment-related AEs. In all registry pregnancies followed up to term, the birth of a healthy baby was reported.
孕期雌激素水平升高会加重遗传性血管性水肿（HAE），但孕妇疾病及治疗的影响仍研究不足。
利用国际贝林妥欧单抗患者登记处的数据，评估在常规HAE管理期间暴露于血浆来源、巴氏杀菌、纳滤C1抑制剂浓缩物（pnfC1-INH）的妊娠结局。
该观察性登记研究于2010年至2014年在美国30个地点和欧洲7个地点进行，收集了318名受试者和15000次pnfC1-INH输注的数据。只要有可能，对孕期使用pnfC1-INH的受试者进行随访至足月，以评估新生儿结局，并收集在pnfC1-INH给药后1个月内发生的母亲不良事件（AE）。
登记数据库包括10名使用pnfC1-INH治疗HAE发作和/或预防（孕期>261剂）的受试者的11次妊娠。8次妊娠以健康婴儿出生告终。其余3次妊娠中：1次在妊娠9周时自愿终止；第2次在受试者最近一次pnfC1-INH输注后1周发生早期自然流产，被认为与pnf-C1INH治疗无关；第3次发生在一名在预产期前约2个月退出登记的受试者身上，未进行进一步随访。在每次pnfC1-INH输注后30天进行评估，没有AE被认为与pnfC1-INH治疗相关。
孕期给予pnfC1-INH总体安全，且与任何治疗相关的AE无关。在所有随访至足月的登记妊娠中，均报告了健康婴儿的出生。
