Evidence map›Paper›PMID 42149735›Full record

ArticlePediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology2026

Characteristics and outcomes of patients with pediatric-onset non-mastocytosis mast cell activation disorders: A CEREMAST study.

M Weiss, J Rossignol, F Campeotto, E Foy, C Elbany, L Jaume, L Geroux, O Debeaupuis, A Neuraz, L Bekel and 16 more

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In one paragraph

Article in Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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

1 citing paper in PubMed.

  1. Characteristics and outcomes of patients with pediatric-onset non-mastocytosis mast cell activation disorders: A CEREMAST study.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2026
    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

26 authors.

M WeissDepartment of Dermatology, Reference Center for Genodermatoses (MAGEC), AP-HP, Necker-Children's Hospital, Paris Centre University, Paris, France.
J RossignolCEREMAST, Imagine Institute, INSERM U1163, Paris, France.
F CampeottoDepartment of Gastro-Enterology, Necker-Children's Hospital (AP-HP5), Paris-Centre University, Imagine Institute, Paris, France.
E FoyDepartment of Allergology, Necker-Children's Hospital (AP-HP5), Paris-Centre University, Imagine Institute, Paris, France.
C ElbanyDepartment of Allergology, Necker-Children's Hospital (AP-HP5), Paris-Centre University, Imagine Institute, Paris, France.
L JaumeDepartment of Dermatology, Reference Center for Genodermatoses (MAGEC), AP-HP, Necker-Children's Hospital, Paris Centre University, Paris, France.
L GerouxDepartment of Allergology, Necker-Children's Hospital (AP-HP5), Paris-Centre University, Imagine Institute, Paris, France.
O DebeaupuisCEREMAST, Imagine Institute, INSERM U1163, Paris, France.
A NeurazDepartment of Bioinformatics, Necker Branch, Necker-Children's Hospital (AP-HP5), Paris-Centre University, Imagine Institute, INSERM U1163, Paris, France.
L BekelDepartment of Dermatology, Reference Center for Genodermatoses (MAGEC), AP-HP, Necker-Children's Hospital, Paris Centre University, Paris, France.
G LezmiDepartment of Allergology, Necker-Children's Hospital (AP-HP5), Paris-Centre University, Imagine Institute, Paris, France.
N BellonDepartment of Dermatology, Reference Center for Genodermatoses (MAGEC), AP-HP, Necker-Children's Hospital, Paris Centre University, Paris, France.
C MéniDepartment of Dermatology, Reference Center for Genodermatoses (MAGEC), AP-HP, Necker-Children's Hospital, Paris Centre University, Paris, France.
C LivideanuCEREMAST, Imagine Institute, INSERM U1163, Paris, France.ORCID https://orcid.org/0000-0002-5275-6868
M MadrangeCEREMAST, Imagine Institute, INSERM U1163, Paris, France.
D CondéDepartment of Dermatology, Reference Center for Genodermatoses (MAGEC), AP-HP, Necker-Children's Hospital, Paris Centre University, Paris, France.
J AgopianCEREMAST, Imagine Institute, INSERM U1163, Paris, France.
C MichotDepartment of Genetics, National Reference Centre for Skeletal Dysplasia, Paris University, Necker-Children's Hospital (AP-HP5), Paris-Centre University, Imagine Institute, Paris, France.
F Villard-TrucDepartment of Pediatric Hepatology and Gastroenterology, Hospices Civils de Lyon, Hôpital Femme Mere Enfant and Lyon 1 University, Inserm 1111, Lyon, France.
A LachauxDepartment of Pediatric Hepatology and Gastroenterology, Hospices Civils de Lyon, Hôpital Femme Mere Enfant and Lyon 1 University, Inserm 1111, Lyon, France.
C GrecoDepartment of Pain and Palliative Care, Necker-Children's Hospital (AP-HP5), Paris-Centre University, Imagine Institute, Paris, France.ORCID https://orcid.org/0000-0003-4259-6861
S Hadj-RabiaDepartment of Dermatology, Reference Center for Genodermatoses (MAGEC), AP-HP, Necker-Children's Hospital, Paris Centre University, Paris, France.
P DubreuilCEREMAST, Imagine Institute, INSERM U1163, Paris, France.
O HermineCEREMAST, Imagine Institute, INSERM U1163, Paris, France.
C BodemerDepartment of Dermatology, Reference Center for Genodermatoses (MAGEC), AP-HP, Necker-Children's Hospital, Paris Centre University, Paris, France.
L PolivkaDepartment of Dermatology, Reference Center for Genodermatoses (MAGEC), AP-HP, Necker-Children's Hospital, Paris Centre University, Paris, France.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundMast cell (MC) activation (MCA) disorders (MCAD) include diseases in which MCs excessively release mediators leading to recurrent manifestations of MCA. MCAD encompasses MCA syndrome (MCAS) which is defined by (i) documented systemic symptoms of MCA, (ii) a 20% increase in serum tryptase level from the individual's baseline plus 2 ng/mL and (iii) a symptom response to MC-stabilizing drugs. MCAD not fulfilling mastocytosis or all MCAS criteria are referred to as MCAD not otherwise specified (MCAD-NOS). The aim was to describe the clinical, laboratory characteristics and outcomes of the first pediatric-onset non-mastocytosis MCAD cohort.

methodsAll children with the criteria of non-mastocytosis MCAD managed at the French National Referral Center for MCAD were included. Clinical, laboratory data, and outcomes were recorded.

resultsForty-four MCAD patients (mean age at onset: 4.3 years) were identified including 36 MCAD-NOS and 8 idiopathic MCAS. Hereditary-α-tryptasemia was identified in 5/33 (15.2%) patients. The spectrum of symptoms concerned most frequently both the skin (100% of children, with urticaria, angioedema, pruritus, and/or flushing) and the gastrointestinal tract (93% patients). We identified two clinical profiles, according to the age at onset. Before 3 years of age, MCAD was associated with persistent gastrointestinal symptoms triggered by various foods (leading to a drastically limited diet), and after 3 years of age with episodic, recurrent, idiopathic, anaphylactic reactions. The administration of H1 and/or H2 antihistamines drugs +/- a leukotriene receptor antagonist resulted in a marked reduction in symptoms.

conclusionThe description of the clinical profiles of pediatric-onset non-mastocytosis MCAD may help to avoid diagnostic delay.

Indexed as

Mast Cell Activation DisordersMast CellsAdolescentAge of OnsetChildChild, PreschoolFemaleHumansInfantMaleTryptasesTryptaseshereditary alpha tryptasemiaKIT mutationmast cellmast cell activation syndrometryptase

Identifiers

PMID42149735
PMCPMC13183001

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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.