Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy
Language English Country Netherlands Media print-electronic
Document type Journal Article, Research Support, Non-U.S. Gov't
PubMed
30280305
DOI
10.1007/s10875-018-0553-4
PII: 10.1007/s10875-018-0553-4
Knihovny.cz E-resources
- Keywords
- Hereditary angioedema, icatibant, pregnancy, recombinant C1 inhibitor, therapy,
- MeSH
- Anti-Inflammatory Agents, Non-Steroidal therapeutic use MeSH
- Bradykinin analogs & derivatives therapeutic use MeSH
- Adult MeSH
- Angioedemas, Hereditary diagnosis drug therapy genetics MeSH
- Complement C1 Inhibitor Protein administration & dosage therapeutic use MeSH
- Pregnancy Complications * MeSH
- Humans MeSH
- Young Adult MeSH
- Disease Progression MeSH
- Recombinant Proteins administration & dosage therapeutic use MeSH
- Risk Factors MeSH
- Pregnancy MeSH
- Pregnancy Trimesters MeSH
- Delivery, Obstetric MeSH
- Treatment Outcome MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Young Adult MeSH
- Pregnancy MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Names of Substances
- Anti-Inflammatory Agents, Non-Steroidal MeSH
- Bradykinin MeSH
- icatibant MeSH Browser
- Complement C1 Inhibitor Protein MeSH
- Recombinant Proteins MeSH
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.
Centre for Cardiovascular Surgery and Transplantation Brno Czech Republic
Department of Clinical Immunology and Allergy University Hospital Hradec Kralove Czech Republic
Department of Obstetrics and Gynecology University Hospital Brno Brno Czech Republic
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