Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema

Abstract

C1 inhibitor deficiency (hereditary angioedema [HAE]) is a rare disorder for which there is a lack of consensus concerning diagnosis, therapy, and management, particularly in Canada. European initiatives have driven the approach to managing HAE with 3 C1-INH Deficiency Workshops held every 2 years in Hungary starting in 1999, with the third Workshop having recently been held in May 2003. The European Contact Board has established a European HAE Registry that will hopefully advance our knowledge of this disorder. The Canadian Hereditary Angioedema Society/Société d'Angioédème Héréditaire du Canada organized a Canadian International Consensus Conference held in Toronto, Ontario, Canada, on October 24 to 26, 2003, to foster consensus between major European and North American HAE treatment centers. Papers were presented by investigators from Europe and North America, and this consensus algorithm approach was discussed. There is a paucity of double-blind placebo-controlled trials in the treatment of HAE, making levels of evidence to support the algorithm less than optimal. Enclosed is the consensus algorithm approach recommended for the diagnosis, therapy, and management of HAE and agreed to by the authors of this article. This document is only a consensus algorithm approach and requires validation. As such, participants agreed to make this a living 2003 algorithm (ie, a work in progress) and agreed to review its content at future international HAE meetings. The consensus, however, has strength in that it was arrived at by the meeting of patient-care providers along with patient group representatives and individual patients reviewing information available to date and reaching agreement on how to approach the diagnosis, therapy, and management of HAE circa 2003. Hopefully evidence to support approaches to the management of HAE will approach the level of meta-analysis of randomized controlled trials in the near future.

Hereditary angioedema on the occasion of a pediatric case

“…Özellikle baş-boyun cerrahisi geçirecek ve dental girişim yapılacak hastalarda kısa dönem profilaksi tedavisine gerek duyulmaktadır (11). Uzun dönem profilaksi tedavisi ayda bir veya daha fazla sayıda atak olması ve hastanın hayatı tehdit edici atak geçirmiş olması durumlarında uygulanmaktadır (12). 6 yaş altındaki çocuklarda semptom sıklığının nisbeten daha az olması nedeniyle nadiren gereklidir.…”

Short‐Term Prophylaxis in Odontostomatological, Maxillofacial and ENT Procedures in Patients with Hereditary Angioedema Due to C1‐Inhibitor Deficiency

“…The 2003 Hungarian-Canadian consensus was the first consensus document on the management of C1-INH-HAE [43]. It attempted to establish a separation between minor and major DOMFOPs but did not go deeper in differentiating clearly which procedures were considered minor or major ( Figure 1) [43].…”

“…It attempted to establish a separation between minor and major DOMFOPs but did not go deeper in differentiating clearly which procedures were considered minor or major ( Figure 1) [43].…”

“…However, if pdhC1INH was not available, STP with danazol or tranexamic acid was recommended. Local injection of local anaesthetic was recognized as being able to precipitate an AE attack [43].…”

Evaluation de la prise en charge bucco-dentaire des patients atteints d’angio-œdèmes bradykiniques. A propos d’une enquête chez 72 patients

“…Après avoir éliminé un angio-oedème histaminique, lié à une hypersensibilité immédiate (oedème de Quincke), ou un emphysème cervicofacial [15], on procède à l'administration per os ou IV d'acide tranexamique en l'absence de contre-indication à ce produit ; si les symptômes s'aggravent à l'injection de concentré de C1-Inh en IV ou d'icatibant en sous-cutané [1,14].…”