Evidence map›Paper›PMID 39655944›Full record

ReviewPediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology2024

Hereditary angioedema in children: Review and practical perspective for clinical management.

Anne Pagnier, Angelina Dermesropian, Charlotte Kevorkian-Verguet, Mélisande Bourgoin-Heck, Cyrille Hoarau, Héloïse Reumaux, Frédérique Nugues, Christine Audouin-Pajot, Sibylle Blanc, Aurélia Carbasse and 5 more

Abstract readReview
In one paragraph

Review in Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
13citing papers in PubMed, 1 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

13 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Guideline
  2. Review
  3. Article
  4. Review
  5. Article
  6. The International/Canadian hereditary angioedema guideline.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2026
    Review
  7. Article
  8. Article
  9. Article
  10. Article
  11. Article
  12. Review
  13. 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
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

15 authors.

Anne PagnierDepartment of Pediatrics, CREAK, University Hospital Grenoble, Grenoble, France.
Angelina DermesropianAllée des Mitaillières, bâtiment A, Meylan, France.
Charlotte Kevorkian-VerguetDepartment of General Pediatrics, Grenoble Alpes University Hospital, Grenoble, France.
Mélisande Bourgoin-HeckDepartment of Pediatric Allergology, Armand Trousseau University Hospital, Sorbonne University, AP-HP, Paris, France;, Paris, France.ORCID https://orcid.org/0000-0001-6497-4830
Cyrille HoarauDepartment of Allergology and Clinical Immunology, Inserm UMR1327 ISCHEMIA, University of Tours, Tours, France.
Héloïse ReumauxDepartment of Pediatric Rheumatology, Hôpital Jeanne de Flandre, CHU Lille, Lille, France.
Frédérique NuguesDepartment of Pediatric Imagery, Couple-Child Hospital, University Hospital Grenoble, Grenoble, France.
Christine Audouin-PajotDepartment of Nephrology-Internal Medicine-Rheumatology, Children's Hospital, Purpan University Hospital, Toulouse, France.
Sibylle BlancPediatric Pulmonology and Allergology Department, Lenval University Hospital, Nice, France.
Aurélia CarbasseDepartment of Pediatrics, Arnaud de Villeneuve University Hospital, Montpellier, France.
Anne-Laure JurquetDepartment of Pediatrics, Assistance Publique des Hôpitaux de Marseille (AP-HM), North University Hospital, Marseille, France.
Mélanie VoideyDepartment of Children Respiratory and Allergic Diseases, Besançon University Hospital, Besançon, France.
Mona VilledieuBiocryst France SAS, Boulogne-Billancourt, France.
Laurence BouilletFrench National Reference Center for Angioedema (CREAK), Internal Medicine Department, Grenoble University Hospital, France, Grenoble, France.
Isabelle Boccon-GibodFrench National Reference Center for Angioedema (CREAK), Center of Excellence and Reference (ACARE), Internal Medicine Department, Grenoble Alpes University Hospital, Grenoble, France.

Funding

BioCryst
6 · The paper itself

Abstract

backgroundHereditary angioedema (HAE) in children has specific features and requires multidisciplinary management.

methodsWe performed a literature search and underwent in-depth discussions to provide practical tools for physicians.

resultsHAE is a rare, life-threatening genetic disorder. Its epidemiology is poorly documented in children. Clinical manifestations usually appear during childhood or early adolescence. Classical signs, often preceded by prodromal symptoms, include transient, localized, non-pitting, non-pruritic swelling of deep dermal/subcutaneous or mucosal/submucosal tissues, leading to oedema of the extremities, face, lips, tongue, trunk and genitals, recurring gastrointestinal symptoms and laryngeal edema possibly causing asphyxiation and death. Diagnosis is often delayed due to low awareness in the medical community, and particularly challenging in case of isolated abdominal crises or atypical presentation and in neonates or infants. It relies on biological tests (measurement of serum/plasma levels of C1INH function, C1INH protein, and C4), genetic testing in selected cases, and imaging for differential diagnosis of acute abdominal crises. Main differential diagnosis for peripheral oedema is mast cell-mediated oedema that accounts for 95% of angioedema in clinical practice. Quality of life can be significantly impaired. Disease management includes treatment of attacks, short-term and long-term prophylaxis, psychological support, avoidance of triggers, patients' and parents' education and coordination of all stakeholders, ideally within a specialized healthcare network. New plasma kallikrein inhibitors, namely lanadelumab (subcutaneous route) and berotralstat (oral route) have facilitated long-term prophylaxis thanks to improved usability.

conclusionDiagnostic and treatment of HAE are particularly challenging in children and require specific management by multiple stakeholders.

Indexed as

Angioedemas, HereditaryAdolescentAntibodies, Monoclonal, HumanizedChildChild, PreschoolComplement C1 Inhibitor ProteinDiagnosis, DifferentialDisease ManagementHumansInfantInfant, NewbornQuality of LifeAntibodies, Monoclonal, HumanizedComplement C1 Inhibitor Proteinlanadelumabdaily diaryiatrogenicsuicide ideation

Identifiers

PMID39655944
PMCPMC11629734

What OpenQuestion holds

Textmetadata
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.