title: International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency.
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.
authors: 3rd Department of Internal Medicine, Hungarian Angioedema Center, Semmelweis University, Budapest, Hungary.
authors: H
authors: H
authors: Farkas
authors: Hemophilia Center Rhine Main, Moerfelden-Walldorf, Germany.
authors: I
authors: I
authors: Martinez-Saguer
authors: Department of Dermatology, University Medical Center Mainz, Mainz, Germany.
authors: K
authors: K
authors: Bork
authors: Departments of Medicine and Paediatrics, University of Calgary, Calgary, AB, Canada.
authors: T
authors: T
authors: Bowen
authors: Department of Medicine, Pediatrics and Graduate Studies, Penn State University, Hershey, PA, USA.
authors: T
authors: T
authors: Craig
authors: Department of Pediatrics, Duke University Medical Center, Durham, NC, USA.
authors: M
authors: M
authors: Frank
authors: Department of Immunology and Histocompatibility, School of Health Sciences, Faculty of Medicine, University of Thessaly, Larissa, Greece.
authors: A E
authors: AE
authors: Germenis
authors: Outpatient Group of Recurrent Infections, Faculty of Medicine ABC, Santo Andre, SP, Brazil.
authors: A S
authors: AS
authors: Grumach
authors: 1st Department of Pediatrics, Semmelweis University, Budapest, Hungary.
authors: A
authors: A
authors: Luczay
authors: 3rd Department of Internal Medicine, Hungarian Angioedema Center, Semmelweis University, Budapest, Hungary.
authors: L
authors: L
authors: Varga
authors: Department of Biomedical and Clinical Sciences "Luigi Sacco", University of Milan, ASST Fatebenefratelli Sacco, Milan, Italy.
authors: A
authors: A
authors: Zanichelli
mesh_terms: D000367:Age Factors; D000465:Algorithms; D015415:Biomarkers; D003131:Combined Modality Therapy; D015897:Comorbidity; D019468:Disease Management; D005260:Female; D056829:Hereditary Angioedema Types I and II / Q000175:diagnosis* / Q000517:prevention & control / Q000628:therapy*; D006801:Humans; D008297:Male; D015201:Meta-Analysis as Topic; D009092:Mucous Membrane / Q000473:pathology; D012307:Risk Factors; D012720:Severity of Illness Index; D063189:Symptom Assessment
chemical_list: D015415:Biomarkers
keywords: C1 inhibitor deficiency; diagnosis; hereditary angioedema; management; pediatric