Short-term Prophylaxis for Delivery in Pregnant Women with Hereditary Angioedema with Normal C1-Inhibitor

Abstract

Objective To verify the efficacy of short-term prophylaxis for vaginal or cesarean section childbirth with plasma-derived C1-inhibitor concentrate in pregnant women. They should have hereditary angioedema (HAE) and normal plasma C1-inhibitor.

Methods Case report of pregnant women diagnosed with HAE with normal C1-inhibitor who had been treated with intravenous C1-inhibitor concentrate for prophylaxis of angioedema attacks when hospitalized for delivery. The exon 9 of the Factor 12 (F12) genotyping gene was performed by automatic sequencing in all patients.

Results Three cases of pregnant women with HAE with normal serum level of C1-inhibitor are reported. The genetic test detected the presence of a pathogenic mutation in the F12 gene. Deliveries occurred uneventfully and patients had no HAE symptoms in the following 72 hours.

Conclusion C1-inhibitor concentrate could be useful to prevent angioedema attacks during and after delivery.

Resumo

Objetivo Verificar a eficácia da profilaxia de curto prazo para o parto vaginal ou cesáreo com inibidor de C1 derivado de plasma concentrado em mulheres grávidas. Eles devem ter angioedema hereditário e inibidor normal de C1 no plasma.

Métodos Relato de caso de gestantes diagnosticadas com angioedema hereditário com inibidor de C1 normal que foram tratadas com inibidor intravenoso de concentrado de C1 para profilaxia de ataques de angioedema quando hospitalizadas para o parto. O exon 9 do gene de genotipagem do fator 12 (F12) foi realizado por sequenciamento automático em todos os pacientes.

Resultados Três casos de gestantes com angioedema hereditário com nível sérico normal de inibidor de C1 são relatados. O teste genético detectou a presença de uma mutação patogênica no gene F12. Os partos ocorreram sem intercorrências e as pacientes não apresentaram sintomas hereditários de angioedema nas 72 horas seguintes.

Conclusão O concentrado de inibidor de C1 pode ser útil para prevenir ataques de angioedema durante e após o parto.

Keywords

angioedema - estrogens - edema - pregnancy - prophylaxis

Palavras-chave

angioedema - estrogênios - edema - gravidez - profilaxia

Publication History

Received: 18 February 2020

Accepted: 17 September 2020

Article published online:
21 December 2020

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