HAKL, Roman, Pavel KUKLÍNEK, Irena KRČMOVÁ, Pavlína KRÁLÍČKOVÁ, Tomáš FREIBERGER, Petr JANKŮ, Marcela VLKOVÁ and Jiří LITZMAN. Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy. Journal of Clinical Immunology. New York: Springer, 2018, vol. 38, No 7, p. 810-815. ISSN 0271-9142. doi:10.1007/s10875-018-0553-4.
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Basic information
Original name Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy
Authors HAKL, Roman (203 Czech Republic, guarantor, belonging to the institution), Pavel KUKLÍNEK (203 Czech Republic), Irena KRČMOVÁ (203 Czech Republic), Pavlína KRÁLÍČKOVÁ, Tomáš FREIBERGER (203 Czech Republic, belonging to the institution), Petr JANKŮ (203 Czech Republic), Marcela VLKOVÁ (203 Czech Republic, belonging to the institution) and Jiří LITZMAN (203 Czech Republic, belonging to the institution).
Edition Journal of Clinical Immunology, New York, Springer, 2018, 0271-9142.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30102 Immunology
Country of publisher United States of America
Confidentiality degree is not subject to a state or trade secret
Impact factor Impact factor: 4.128
RIV identification code RIV/00216224:14110/18:00104428
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.1007/s10875-018-0553-4
UT WoS 000447514500016
Keywords in English Hereditary angioedema; pregnancy; therapy; icatibant; recombinant C1 inhibitor
Tags 14110114, rivok
Tags International impact, Reviewed
Changed by Changed by: Soňa Böhmová, učo 232884. Changed: 11/2/2019 15:41.
Abstract
PurposeHereditary 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.MethodsWe present our clinical experience treating acute HAE attacks during pregnancy in six consecutive patients.ResultsDuring 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 3690g. No congenital abnormalities were detected in the neonates. No abortions occurred.ConclusionsOur 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.
Links
MUNI/A/0925/2017, internal MU codeName: Poruchy tvorby protilátek a komplementového systému
Investor: Masaryk University, Grant Agency of Masaryk University, Category A
NV15-28732A, research and development projectName: Vliv granulocytů a monocytů na vznik a rozvoj imunodeficitních chorob a dalších imunopatologických dějů
NV16-34414A, research and development projectName: Určení genových oblastí náchylných ke vzniku mutací ovlivňujících sestřih mRNA
NV18-05-00330, research and development projectName: Genetická determinace závažnosti otoků podmíněných bradykininem u pacientů s hereditárním angioedémem
Investor: Ministry of Health of the CR, Ministry of Health Research Programme 2015 - 2022
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