International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency. Issue 2 (8th September 2016)

• Record Type: Journal Article
• Authors: Farkas, H.; Martinez‐Saguer, I.; Bork, K.; Bowen, T.; Craig, T.; Frank, M.; Germenis, A. E.; Grumach, A. S.; Luczay, A.; Varga, L.; Zanichelli, A.
• Other Names: Aberer Werner investigator.; Andrejevic Sladjana investigator.; Aygoeren‐Pürsün Emel investigator.; Banerji Alena investigator.; Bara Noemi‐Anna investigator.; Bas Murat investigator.; Bernstein Jonathan investigator.; Betschel Stephen investigator.; Björkander Janne investigator.; Boccon‐Gibod Isabelle investigator.; Bouillet Laurence investigator.; Bova Maria investigator.; Boysen Henrik Halle investigator.; Branco‐Ferreira Manuel investigator.; Bygum Anette investigator.; Caballero Teresa investigator.; Cancian Mauro investigator.; Castaldo Anthony investigator.; Christiansen Sandra investigator.; Cicardi Marco investigator.; Drouet Christian investigator.; Fabiani Jose investigator.; Gompels Mark investigator.; Gonzalez‐Quevedo Maria Teresa investigator.; Gooi Jimmy investigator.; Gower Richard investigator.; Gökmen Nihal Mete investigator.; Grivcheva‐Panovska Vesna investigator.; Guilarte Mar investigator.; Gülbahar Okan investigator.; Hack Erik investigator.; Hakl Roman investigator.; Harmat György investigator.; Jeseňák Miloš investigator.; Jolles Stephen investigator.; Kaplan Allen investigator.; Katelaris Connie investigator.; Kosnik Mitja investigator.; Kőhalmi Kinga Viktória investigator.; Leibovich Iris investigator.; Levi Marcel investigator.; Li Henry investigator.; Longhurst Hilary J. investigator.; Lumry William investigator.; Magerl Markus investigator.; Malbran Alejandro investigator.; Martin Ludovic investigator.; Maurer Marcus investigator.; Mihály Enikő investigator.; Moldovan Dumitru investigator.; Murdjeva Mariana investigator.; Nagy Imola Beatrix investigator.; Nielsen Erik W. investigator.; Nieto Sandra investigator.; Nordenfelt Patrik investigator.; Obtulowitzc Kristine investigator.; Pedrosa Maria investigator.; Porębski Grzegorz investigator.; Prior Nieves investigator.; Reshef Avner investigator.; Riedl Marc A. investigator.; Rosenkranz Bernd investigator.; Schmid‐Grendelmeier Peter investigator.; Péter Spath investigator.; Speletas Matthaios investigator.; Staevska Maria investigator.; Stobiecki Marcin investigator.; Triggiani Massimo investigator.; Veszeli Nóra investigator.; Wuillemin Walter investigator.; Xiang Zhi Yu investigator.; Yamamoto Beverley investigator.; Zuraw Bruce investigator.;
• Is Part Of: Allergy. Volume 72:Issue 2(2017:Feb.)
• Page Start: 300
• Page End: 313
• Publication Date: 2016-09-08
• Subjects: C1 inhibitor deficiency -- diagnosis -- hereditary angioedema -- management -- pediatric; Allergy -- Periodicals; 616.97
• DOI: 10.1111/all.13001
• Languages: English
• ISSNs: 0105-4538

Abstract

Abstract: Background: The consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1‐INH‐HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagnosis and management of pediatric patients with C1‐INH‐HAE. Methods: During an expert panel meeting that took place during the 9th C1 Inhibitor Deficiency Workshop in Budapest, 2015 (www.haenet.hu ), pediatric data were presented and discussed and a consensus was developed by voting. Results: The symptoms of C1‐INH‐HAE often present in childhood. Differential diagnosis can be difficult as abdominal pain is common in pediatric C1‐INH‐HAE, but also commonly occurs in the general pediatric population. The early onset of symptoms may predict a more severe subsequent course of the disease. Before the age of 1 year, C1‐INH levels may be lower than in adults; therefore, it is advisable to confirm the diagnosis after the age of one year. All neonates/infants with an affected C1‐INH‐HAE family member should be screened for C1‐INH deficiency. Pediatric patients should always carry a C1‐INH‐HAE information card and medicine for emergency use. The regulatory approval status of the drugs for prophylaxis and for acute treatment is different in each country. Plasma‐derived C1‐INH, recombinant C1‐INH, and ecallantide are the only agents licensed for the acute treatment of pediatric patients. Clinical trials are underway with additional drugs. It is recommended to follow up patients in an HAE comprehensive care center. Conclusions: The pediatric‐focused international consensus for the diagnosis and management of C1‐INH‐HAE patients was created.