Evidence map›Paper›PMID 27503784›Full record

ArticleAllergy2017

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, A E Germenis, A S Grumach, A Luczay, L Varga and 2 more

Open access · hybridAbstract readConsensus Statement
In one paragraph

Article in Allergy, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 64 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
64citing papers in PubMed, 2 pooled it
13.7field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

64 citing papers in PubMed, 2 syntheses or guidelines pooled it, 196 citations in OpenAlex.

  1. Guideline
  2. Pooled it
  3. Trial
  4. Recombinant human C1 esterase inhibitor treatment for hereditary angioedema attacks in children.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2019
    Trial
  5. A randomized trial of human C1 inhibitor prophylaxis in children with hereditary angioedema.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2019
    Trial
  6. Review
  7. Article
  8. Article
  9. The International/Canadian hereditary angioedema guideline.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2026
    Review
  10. Article
  11. Hereditary Angioedema (HAE) in China: Advancing Awareness, Access, Advocacy and Alliances From the Greater Bay Area to the Global HAE Community.Clinical and experimental allergy : journal of the British Society for Allergy and Clinical Immunology · 2025
    Review
  12. Article
  13. Article
  14. Article
  15. Article
  16. Hereditary angioedema in children: Review and practical perspective for clinical management.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2024
    Review
  17. Review
  18. Article
  19. Article
  20. Pathogenic variant inBMJ case reports · 2023
    Article

4 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

12 authors at 9 institutions in 7 countries.

H Farkas3rd Department of Internal Medicine, Hungarian Angioedema Center, Semmelweis University, Budapest, Hungary.
I Martinez-SaguerHemophilia Center Rhine Main, Moerfelden-Walldorf, Germany.
K BorkDepartment of Dermatology, University Medical Center Mainz, Mainz, Germany.
T BowenDepartments of Medicine and Paediatrics, University of Calgary, Calgary, AB, Canada.
T CraigDepartment of Medicine, Pediatrics and Graduate Studies, Penn State University, Hershey, PA, USA.
M FrankDepartment of Pediatrics, Duke University Medical Center, Durham, NC, USA.
A E GermenisDepartment of Immunology and Histocompatibility, School of Health Sciences, Faculty of Medicine, University of Thessaly, Larissa, Greece.
A S GrumachOutpatient Group of Recurrent Infections, Faculty of Medicine ABC, Santo Andre, SP, Brazil.
A Luczay1st Department of Pediatrics, Semmelweis University, Budapest, Hungary.
L Varga3rd Department of Internal Medicine, Hungarian Angioedema Center, Semmelweis University, Budapest, Hungary.
A ZanichelliDepartment of Biomedical and Clinical Sciences "Luigi Sacco", University of Milan, ASST Fatebenefratelli Sacco, Milan, Italy.
HAWK
Semmelweis University · HUASST Fatebenefratelli Sacco · ITDuke University Hospital · USFaculdade de Medicina do ABC · BRPenn State Milton S. Hershey Medical Center · USSystems, Applications & Products in Data Processing (Germany) · DEUniversity Medical Center of the Johannes Gutenberg University Mainz · DEUniversity of Calgary · CAUniversity of Thessaly · GR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundThe 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.

methodsDuring 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.

resultsThe 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.

conclusionsThe pediatric-focused international consensus for the diagnosis and management of C1-INH-HAE patients was created.

Indexed as

Age FactorsAlgorithmsBiomarkersCombined Modality TherapyComorbidityDisease ManagementFemaleHereditary Angioedema Types I and IIHumansMaleMeta-Analysis as TopicMucous MembraneRisk FactorsSeverity of Illness IndexSymptom AssessmentBiomarkersC1 inhibitor deficiencydiagnosishereditary angioedemamanagementpediatric

Identifiers

PMID27503784
PMCPMC5248622
OpenAlexW2518027570

What OpenQuestion holds

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LicenceCC BY-NC
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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.