Evidence map›Paper›PMID 41618059›Full record

GuidelineAllergy2026

International Guideline on the Diagnosis and Management of Pediatric Patients With Hereditary Angioedema.

Henriette Farkas, Inmaculada Martinez-Saguer, Konrad Bork, Anastasios E Germenis, Anete S Grumach, Hanga Réka Horváth, Andrea Luczay, Andrea Zanichelli, Markus Magerl, Stephen Betschel and 23 more

Abstract readReviewPractice Guideline
In one paragraph

Guideline in Allergy, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed, 2 pooled it
–field-weighted citation impact
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

7 citing papers in PubMed, 2 syntheses or guidelines pooled it.

  1. Pooled it
  2. Guideline
  3. Review
  4. Review
  5. Article
  6. Article
  7. Article
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

33 authors.

Henriette FarkasHungarian Angioedema Centre of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
Inmaculada Martinez-SaguerHZRM Heamophilia Centre Rein Main, Frankfurt, Germany.
Konrad BorkDepartment of Dermatology, University Medical Center, Johannes Gutenberg University, Mainz, Germany.
Anastasios E GermenisDepartment of Immunology & Histocombatibility, School of Medicine, University of Thessaly, Larissa, Greece.ORCID https://orcid.org/0000-0002-3315-6947
Anete S GrumachClinical Immunology, Centro Universitario Faculdade de Medicina do ABC, Santo Andre, Brazil.
Hanga Réka HorváthHungarian Angioedema Centre of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
Andrea LuczayPediatric Center Semmelweis University, Budapest, Hungary.
Andrea ZanichelliOperative Unit of Medicine, Angioedema Center, IRCCS Policlinico San Donato, Milan, Italy.
Markus MagerlInstitute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität Zu Berlin, Berlin, Germany.ORCID https://orcid.org/0000-0001-9218-5468
Stephen BetschelDivision of Clinical Immunology and Allergy, Department of Medicine, Unity Health, University of Toronto, Toronto, Ontario, Canada.
Emel Aygören-PürsünDepartment of Pediatrics, University Hospital, Goethe University, Frankfurt am Main, Germany.
Jonathan A BernsteinDepartment of Internal Medicine, Division of Rheumatology, Allergy and Immunology, University of Cincinnati College of Medicine, Partner of Advanced Allergy Services and Bernstein Clinical Research Center, Cincinnati, Ohio, USA.ORCID https://orcid.org/0000-0002-3476-1196
Isabelle Boccon-GibodClinical Immunology and Internal Medicine Department, National Reference Centre for Angioedema (CREAK) and ACARE Centre of Reference and Excellence, Grenoble Alpes University Hospital, Grenoble, France.
Teresa CaballeroDepartment of Allergy, Hospital Universitario La Paz, Hospital La Paz Health Research Institute (IdiPAZ), biomedical Research Network on Rare Diseases (CIBERER, U754), Madrid, Spain.
Mauro CancianDepartment of Systems Medicine, University Hospital of Padua, Padua, Italy.ORCID https://orcid.org/0000-0003-2246-9144
Sandra ChristiansenDivision of Allergy & Immunology, Department of Medicine, University of California San Diego, La Jolla, California, USA.
Danny M CohnAmsterdam UMC, Cardiovascular Sciences, Department of Vascular Medicine, University of Amsterdam, Amsterdam, the Netherlands.
Francisco ContrerasAllergy Service, National Institute of Pediatrics, Mexico City, Mexico.
Sansanee CraigDepartment of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Camelia IsaicHAE Junior Patient Organization, Prague, Czech Republic.
Ankur JindalPediatric Clinical Immunology and Rheumatology, Department of Pediatrics, Manipal Hospital, Bengaluru, India.ORCID https://orcid.org/0000-0002-7954-0661
Constance H KatelarisImmunology & Allergy Unit Campbelltown Hospital and Western Sydney University, Sydney, New South Wales, Australia.
Hilary J LonghurstDepartment of Immunology, Auckland City Hospital and University of Auckland, Auckland, New Zealand.
Andrew MacGinnitieDivision of Allergy, Asthma, and Immunology, Children's Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Jonny PeterDivision of Allergy and Clinical Immunology, Groote Schuur Hospital, University of Cape Town, Rondebosch, South Africa.ORCID https://orcid.org/0000-0002-2658-0723
Grzegorz PorebskiDepartment of Clinical and Environmental Allergology, Jagiellonian University Medical College, Krakow, Poland.ORCID https://orcid.org/0000-0002-6146-0188
Avner ReshefAngioedema Research Center, Barzilai University Medical Center, Ashkelon, Israel.ORCID https://orcid.org/0000-0002-3324-7072
Dinh Van NguyenVinmec-VinUni Institute of Immunology, VinUniversity, Hanoi, Vietnam.
Bruce ZurawDivision of Allergy & Immunology, Department of Medicine, University of California San Diego, La Jolla, California, USA.
Anthony J CastaldoHereditary Angioedema International, USA.
Henrik Balle BoysenHereditary Angioedema International, USA.
Timothy CraigDepartment of Medicine, Pediatrics, OB/GYN MFM, and Biomedical Sciences, Penn State University, Hershey, Pennsylvania, USA.ORCID https://orcid.org/0000-0002-7774-4855
Hereditary Angioedema Working Group (HAWK Group)

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Hereditary angioedema (HAE) with C1 inhibitor deficiency is a rare disease characterized by unpredictable episodes of tissue swelling (angioedema), which, in most cases, occur first under the age of 18 years, and entail a significant burden of disease not only for the patients but also for their families. Clinical symptoms of HAE are not specific, which may cause difficulties in differential diagnosis. Additionally, if not appropriately treated, HAE attacks can be life-threatening. The international HAE guidelines published so far have focused mainly on adults. A guideline that refers to the age-specific characteristics of pediatric patients, both in terms of diagnosis and management, was therefore needed. The International Steering Committee and Taskforce developed recommendations and provided evidence-based grading based on expert opinion and strength of evidence. Recommendations were presented to, discussed, and electronically voted by healthcare professionals during the 14th C1 Inhibitor Deficiency and Angioedema Workshop in Budapest, Hungary, 2025. This international guideline will ensure early diagnosis, standardized and up-to-date treatment, and promote the availability of effective therapies for all pediatric patients affected with this rare disease. It also draws attention to the importance of establishing HAE centers and registries, which solicit specialist care and research of the disease.

Indexed as

Angioedemas, HereditaryAdolescentChildComplement C1 Inhibitor ProteinDiagnosis, DifferentialDisease ManagementHumansComplement C1 Inhibitor Proteinacute treatmentbradykininC1 inhibitor deficiencycomplement testcomprehensive caregenetic testinghereditary angioedemalong‐term prophylaxispediatric guidelineshort‐term prophylaxis

Identifiers

PMID41618059
PMCPMC13466006

What Socratic holds

Textmetadata
Read underepoch 390

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 Socratic graph.