International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

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RESEARCH PRODUCT

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

H Farkas I Martinez Saguer K Bork T Bowen T Craig M Frank Ae Germenis As Grumach A Luczay L Varga A Zanichelli W Aberer S Andrejevic E Aygoeren Pürsün A Banerji Na Bara M Bas J Bernstein S Betschel J Björkander I Boccon Gibod L Bouillet M Bova Hh Boysen M Branco Ferreira A Bygum T Caballero M Cancian A Castaldo S Christiansen M Cicardi C Drouet J Fabiani M Gompels Gonzalez Quevedo Mt J Gooi R Gower Nm Gökmen V Grivcheva Panovska M Guilarte O Gülbahar E Hack R Hakl G Harmat M Mjeseňák S Jolles A Kaplan C Katelaris M Kosnik Kv Kőhalmi I Leibovich M Levi H Li Hj Longhurst W Lumry M Magerl A Malbran L Martin M Maurer E Mihály D Moldovan M Murdjeva Ib Nagy Ew Nielsen S Nieto P Nordenfelt K Obtulowitzc M Pedrosa G Porębski N Prior A Reshef Ma Riedl B Rosenkranz P Schmid Grendelmeier S Péter M Speletas M Staevska M Stobiecki Massimo Triggiani N Veszeli W Wuillemin Zy Xiang B Yamamoto B. Zuraw

subject

Male Abdominal pain Pediatrics diagnosis Comorbidity Disease Severity of Illness Index Ecallantide 0302 clinical medicine Risk Factors Diagnosis Immunology and Allergy heterocyclic compounds 030212 general & internal medicine Hereditary angioedema Pediatric Hereditary Angioedema Types I and II Age Factors Disease Management Combined Modality Therapy Immunodeficiencies Management Hereditary angioedema Female Original Article Symptom Assessment medicine.symptom management Algorithms medicine.drug medicine.medical_specialty C1 inhibitor deficiency Immunology 03 medical and health sciences Meta-Analysis as Topic medicine Humans Mucous Membrane Adult patients business.industry Original Articles C1 inhibitor deficiency; Diagnosis; Hereditary angioedema; Management; Pediatric; Immunology and Allergy; Immunology bacterial infections and mycoses medicine.disease hereditary angioedema respiratory tract diseases Clinical trial pediatric 030228 respiratory system C1 inhibitor deficiency Differential diagnosis business Biomarkers

description

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.

year	journal	country	edition	language
2016-08-06

10.1111/all.13001 http://hdl.handle.net/11386/4685338