{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T18:02:24Z","timestamp":1787508144303,"version":"build-2736575974"},"reference-count":31,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,1,11]],"date-time":"2017-01-11T00:00:00Z","timestamp":1484092800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Immunity Inflam &amp;amp; Disease"],"published-print":{"date-parts":[[2017,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>The clinical characteristics and icatibant\u2010treatment outcomes of patients with hereditary angioedema with normal C1 inhibitor (HAE\u2010nC1 INH) are limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analyzed data from French HAE patients enrolled in the Icatibant Outcome Survey registry (from July 2009 to September 2013) to compare disease characteristics and the effectiveness and safety of acute icatibant\u2010treated angioedema attacks in patients with HAE\u2010nC1 INH, HAE with C1 INH deficiency (type I), or dysfunction (type II).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>One center in Grenoble contributed 22 patients with HAE\u2010nC1 INH and a family history of HAE while 15 centers across France contributed 153 patients with HAE type I and seven patients with HAE type II. Patients with HAE\u2010nC1 INH compared to HAE type I, respectively, were more likely to be female (88.1% vs. 63.4%), older at median age of disease onset (21 years vs. 15 years), and have a greater rate of abdominal (80% vs. 61%) and laryngeal (23% vs. 14%) attacks. Icatibant was effective in both groups though the median time to resolution of attack was significantly longer in the HAE\u2010nC1 INH group (20.0\u2009h, 37 attacks) versus the HAE type I group (14.0\u2009h, 67 attacks). Icatibant was self\u2010administered for 96.1% of attacks in patients with HAE\u2010nC1 INH and 75.8% in patients with HAE type I. No serious adverse side effects related to icatibant were reported.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>These data help further define the disease characteristics of HAE\u2010nC1 INH in the French population and extend the limited data reporting the safe and effective use of icatibant in acute treatment of angioedema in French patients diagnosed with HAE\u2010nC1 INH.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/iid3.137","type":"journal-article","created":{"date-parts":[[2017,1,11]],"date-time":"2017-01-11T02:19:00Z","timestamp":1484101140000},"page":"29-36","source":"Crossref","is-referenced-by-count":29,"title":["Hereditary angioedema with normal C1 inhibitor in a French cohort: Clinical characteristics and response to treatment with icatibant"],"prefix":"10.1002","volume":"5","author":[{"given":"Laurence","family":"Bouillet","sequence":"first","affiliation":[{"name":"Department of Internal Medicine National Reference Centre for Angioedema (CREAK) Universit\u00e9 Grenoble Alpes, Joint Unit 1036 INSERM\u2010CNRS\u2010CEA CHU Grenoble France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Boccon\u2010Gibod","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine National Reference Centre for Angioedema (CREAK) Universit\u00e9 Grenoble Alpes, Joint Unit 1036 INSERM\u2010CNRS\u2010CEA CHU Grenoble France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Launay","sequence":"additional","affiliation":[{"name":"U995 Lille Inflammation Research International Center (LIRIC) University of Lille Lille France"},{"name":"Inserm, U995 Lille France"},{"name":"d\u00e9partement de m\u00e9decine interne et immunologie clinique CHU Lille Lille France"},{"name":"National Reference Centre for Angioedema (CREAK) Lille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Gompel","sequence":"additional","affiliation":[{"name":"Gynaecology and Endocrinology Unit Paris Descartes University, Cochin\u2010Port Royal Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gisele","family":"Kanny","sequence":"additional","affiliation":[{"name":"EA 7299 Internal Medicine, Clinical Immunology and Allergology Lorraine University, University Hospital Vandoeuvre\u2010l\u00e8s\u2010Nancy France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Fabien","sequence":"additional","affiliation":[{"name":"Shire Zug Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Fain","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Saint\u2010Antoine Hospital (AP\u2010HP) Paris 6 University, DHUi2B Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for the IOS Study Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,1,11]]},"reference":[{"issue":"6","key":"e_1_2_8_2_1","first-page":"445","article-title":"Improving C1 inhibitor deficiency (type 1 and type 2 hereditary angioedema) in Latin America","volume":"24","author":"Fabiani J.","year":"2014","journal-title":"J. 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