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Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy

R. Hakl, P. Kuklínek, I. Krčmová, P. Králíčková, T. Freiberger, P. Janků, M. Vlková, J. Litzman,

. 2018 ; 38 (7) : 810-815. [pub] 20181002

Language English Country Netherlands

Document type Journal Article, Research Support, Non-U.S. Gov't

Persistent link   https://www.medvik.cz/link/bmc19045212

Grant support
NV15-28732A MZ0 CEP Register
NV16-34414A MZ0 CEP Register

PURPOSE: Hereditary angioedema (HAE) is a rare disease caused by a C1 inhibitor (C1-INH) deficit. Clinically, HAE is manifested by repeated episodes of localized subcutaneous or submucosal oedema attacks. Managing HAE patients in pregnancy is challenging, since there are only limited data on the safety and efficacy of various therapeutic approaches. METHODS: We present our clinical experience treating acute HAE attacks during pregnancy in six consecutive patients. RESULTS: During the pregnancies, 79 HAE attacks occurred. The most frequent were abdominal 53 (67.1%) followed by peripheral 21 (26.6%), facial 10 (12.7%), and laryngeal 10 (12.7%) oedemas; 13 (16.5%) attacks were combined. Fifty (63.3%) attacks were treated with recombinant human C1-INH (rhC1-INH); 17 (21.5%) with plasma-derived, pasteurized, nanofiltered C1-INH (pnfC1-INH); 13 (16.5%) with icatibant; and 1 (1.3%) with plasma-derived, nanofiltered C1-INH (nfC1-INH). Treatment had to be repeated in 5 attacks (6.3%). All six deliveries (one caesarean section and five spontaneous vaginal deliveries) were complication free. All pregnancies went to the full term and the patients delivered healthy babies with a birth weight ranging from 2850 to 3690 g. No congenital abnormalities were detected in the neonates. No abortions occurred. CONCLUSIONS: Our results show good C1-INH or icatibant treatment efficacy for HAE attacks in pregnancy. The treatment by the first drug used was effective in 93.7% of all attacks. In 6.3% of attacks, a second treatment had to be used. No adverse effects were observed.

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$a Hakl, Roman $u Department of Clinical Immunology and Allergology, St. Anne's University Hospital in Brno, Brno, Czech Republic. roman.hakl@fnusa.cz. Faculty of Medicine, Masaryk University, Brno, Czech Republic. roman.hakl@fnusa.cz.
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$a PURPOSE: Hereditary angioedema (HAE) is a rare disease caused by a C1 inhibitor (C1-INH) deficit. Clinically, HAE is manifested by repeated episodes of localized subcutaneous or submucosal oedema attacks. Managing HAE patients in pregnancy is challenging, since there are only limited data on the safety and efficacy of various therapeutic approaches. METHODS: We present our clinical experience treating acute HAE attacks during pregnancy in six consecutive patients. RESULTS: During the pregnancies, 79 HAE attacks occurred. The most frequent were abdominal 53 (67.1%) followed by peripheral 21 (26.6%), facial 10 (12.7%), and laryngeal 10 (12.7%) oedemas; 13 (16.5%) attacks were combined. Fifty (63.3%) attacks were treated with recombinant human C1-INH (rhC1-INH); 17 (21.5%) with plasma-derived, pasteurized, nanofiltered C1-INH (pnfC1-INH); 13 (16.5%) with icatibant; and 1 (1.3%) with plasma-derived, nanofiltered C1-INH (nfC1-INH). Treatment had to be repeated in 5 attacks (6.3%). All six deliveries (one caesarean section and five spontaneous vaginal deliveries) were complication free. All pregnancies went to the full term and the patients delivered healthy babies with a birth weight ranging from 2850 to 3690 g. No congenital abnormalities were detected in the neonates. No abortions occurred. CONCLUSIONS: Our results show good C1-INH or icatibant treatment efficacy for HAE attacks in pregnancy. The treatment by the first drug used was effective in 93.7% of all attacks. In 6.3% of attacks, a second treatment had to be used. No adverse effects were observed.
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$a Kuklínek, Pavel $u Department of Clinical Immunology and Allergology, St. Anne's University Hospital in Brno, Brno, Czech Republic.
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$a Krčmová, Irena $u Department of Clinical Immunology and Allergy, University Hospital, Hradec Kralove, Czech Republic.
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$a Freiberger, Tomáš $u Department of Clinical Immunology and Allergology, St. Anne's University Hospital in Brno, Brno, Czech Republic. Faculty of Medicine, Masaryk University, Brno, Czech Republic. Centre for Cardiovascular Surgery and Transplantation, Brno, Czech Republic.
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$a Janků, Petr $u Department of Obstetrics and Gynecology, University Hospital Brno, Brno, Czech Republic.
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$a Vlková, Marcela $u Department of Clinical Immunology and Allergology, St. Anne's University Hospital in Brno, Brno, Czech Republic. Faculty of Medicine, Masaryk University, Brno, Czech Republic.
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$a Litzman, Jiří $u Department of Clinical Immunology and Allergology, St. Anne's University Hospital in Brno, Brno, Czech Republic. Faculty of Medicine, Masaryk University, Brno, Czech Republic.
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