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@article{1466116,
author = {Hakl, Roman and Kuklínek, Pavel and Krčmová, Irena and Králíčková, Pavlína and Freiberger, Tomáš and Janků, Petr and Vlková, Marcela and Litzman, Jiří},
article_location = {New York},
article_number = {7},
doi = {http://dx.doi.org/10.1007/s10875-018-0553-4},
keywords = {Hereditary angioedema; pregnancy; therapy; icatibant; recombinant C1 inhibitor},
language = {eng},
issn = {0271-9142},
journal = {Journal of Clinical Immunology},
title = {Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy},
volume = {38},
year = {2018}
}
TY - JOUR
ID - 1466116
AU - Hakl, Roman - Kuklínek, Pavel - Krčmová, Irena - Králíčková, Pavlína - Freiberger, Tomáš - Janků, Petr - Vlková, Marcela - Litzman, Jiří
PY - 2018
TI - Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy
JF - Journal of Clinical Immunology
VL - 38
IS - 7
SP - 810-815
EP - 810-815
PB - Springer
SN - 02719142
KW - Hereditary angioedema
KW - pregnancy
KW - therapy
KW - icatibant
KW - recombinant C1 inhibitor
N2 - 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.
ER -
HAKL, Roman, Pavel KUKLÍNEK, Irena KRČMOVÁ, Pavlína KRÁLÍČKOVÁ, Tomáš FREIBERGER, Petr JANKŮ, Marcela VLKOVÁ a Jiří LITZMAN. Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy. \textit{Journal of Clinical Immunology}. New York: Springer, 2018, roč.~38, č.~7, s.~810-815. ISSN~0271-9142. Dostupné z: https://dx.doi.org/10.1007/s10875-018-0553-4.
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