{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T12:06:35Z","timestamp":1791893195535,"version":"4.3.4"},"reference-count":29,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T00:00:00Z","timestamp":1779062400000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Mast 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\u2009ng\/mL and (iii) a symptom response to MC\u2010stabilizing drugs. MCAD not fulfilling mastocytosis or all MCAS criteria are referred to as MCAD not otherwise specified (MCAD\u2010NOS). The aim was to describe the clinical, laboratory characteristics and outcomes of the first pediatric\u2010onset non\u2010mastocytosis MCAD cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>All children with the criteria of non\u2010mastocytosis MCAD managed at the French National Referral Center for MCAD were included. Clinical, laboratory data, and outcomes were recorded.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Forty\u2010four MCAD patients (mean age at onset: 4.3\u2009years) were identified including 36 MCAD\u2010NOS and 8 idiopathic MCAS. Hereditary\u2010\u03b1\u2010tryptasemia 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\u2009years of age, MCAD was associated with persistent gastrointestinal symptoms triggered by various foods (leading to a drastically limited diet), and after 3\u2009years of age with episodic, recurrent, idiopathic, anaphylactic reactions. The administration of H1 and\/or H2 antihistamines drugs +\/\u2212 a leukotriene receptor antagonist resulted in a marked reduction in symptoms.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The description of the clinical profiles of pediatric\u2010onset non\u2010mastocytosis MCAD may help to avoid diagnostic delay.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/pai.70373","type":"journal-article","created":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T16:45:43Z","timestamp":1779122743000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Characteristics and outcomes of patients with pediatric\u2010onset non\u2010mastocytosis mast cell activation disorders: A\n                    <scp>CEREMAST<\/scp>\n                    study"],"prefix":"10.1111","volume":"37","author":[{"given":"M.","family":"Weiss","sequence":"first","affiliation":[{"name":"Department of Dermatology Reference Center for Genodermatoses (MAGEC), AP\u2010HP, Necker\u2010Children's Hospital, Paris Centre University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Rossignol","sequence":"additional","affiliation":[{"name":"CEREMAST, Imagine Institute, INSERM U1163  Paris France"},{"name":"Department of Hematology Necker\u2010Children's Hospital (AP\u2010HP5), Paris\u2010Centre University, Imagine Institute  Paris France"},{"name":"Paris\u2010Centre University, Imagine Institute, Laboratory of Molecular Mechanisms of Hematologic Disorders and Therapeutic Implications, INSERM UMR 1163  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Campeotto","sequence":"additional","affiliation":[{"name":"Department of Gastro\u2010Enterology Necker\u2010Children's Hospital (AP\u2010HP5), Paris\u2010Centre University, Imagine Institute  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Foy","sequence":"additional","affiliation":[{"name":"Department of Allergology Necker\u2010Children's Hospital (AP\u2010HP5), Paris\u2010Centre University, Imagine Institute  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Elbany","sequence":"additional","affiliation":[{"name":"Department of Allergology Necker\u2010Children's Hospital (AP\u2010HP5), Paris\u2010Centre University, Imagine Institute  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Jaume","sequence":"additional","affiliation":[{"name":"Department of Dermatology Reference Center for Genodermatoses (MAGEC), AP\u2010HP, Necker\u2010Children's Hospital, Paris Centre University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Geroux","sequence":"additional","affiliation":[{"name":"Department of Allergology Necker\u2010Children's Hospital (AP\u2010HP5), Paris\u2010Centre University, Imagine Institute  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"O.","family":"Debeaupuis","sequence":"additional","affiliation":[{"name":"CEREMAST, Imagine Institute, INSERM U1163  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Neuraz","sequence":"additional","affiliation":[{"name":"Department of Bioinformatics Necker Branch, Necker\u2010Children's Hospital (AP\u2010HP5), Paris\u2010Centre University, Imagine Institute, INSERM U1163  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Bekel","sequence":"additional","affiliation":[{"name":"Department of Dermatology Reference Center for Genodermatoses (MAGEC), AP\u2010HP, Necker\u2010Children's Hospital, Paris Centre University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Lezmi","sequence":"additional","affiliation":[{"name":"Department of Allergology Necker\u2010Children's Hospital (AP\u2010HP5), Paris\u2010Centre University, Imagine Institute  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Bellon","sequence":"additional","affiliation":[{"name":"Department of Dermatology Reference Center for Genodermatoses (MAGEC), AP\u2010HP, Necker\u2010Children's Hospital, Paris Centre University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"M\u00e9ni","sequence":"additional","affiliation":[{"name":"Department of Dermatology Reference Center for Genodermatoses (MAGEC), AP\u2010HP, Necker\u2010Children's Hospital, Paris Centre University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5275-6868","authenticated-orcid":false,"given":"C.","family":"Livideanu","sequence":"additional","affiliation":[{"name":"CEREMAST, Imagine Institute, INSERM U1163  Paris France"},{"name":"Department of Dermatology Toulouse University Medical Hospital  Toulouse France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Madrange","sequence":"additional","affiliation":[{"name":"CEREMAST, Imagine Institute, INSERM U1163  Paris France"},{"name":"Paris\u2010Centre University, Imagine Institute, Laboratory of Molecular Mechanisms of Hematologic Disorders and Therapeutic Implications, INSERM UMR 1163  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Cond\u00e9","sequence":"additional","affiliation":[{"name":"Department of Dermatology Reference Center for Genodermatoses (MAGEC), AP\u2010HP, Necker\u2010Children's Hospital, Paris Centre University  Paris France"},{"name":"CEREMAST, Imagine Institute, INSERM U1163  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Agopian","sequence":"additional","affiliation":[{"name":"CEREMAST, Imagine Institute, INSERM U1163  Paris France"},{"name":"CRCM [Signaling, Hematopoiesis and Mechanism of Oncogenesis, CEREMAST, AFIRMM], Inserm U1068, Institut Paoli\u2010Calmettes, Aix\u2010Marseille University, UM105  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Michot","sequence":"additional","affiliation":[{"name":"Department of Genetics National Reference Centre for Skeletal Dysplasia, Paris University, Necker\u2010Children's Hospital (AP\u2010HP5), Paris\u2010Centre University, Imagine Institute  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Villard\u2010Truc","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hepatology and Gastroenterology Hospices Civils de Lyon, H\u00f4pital Femme Mere Enfant and Lyon 1 University, Inserm 1111  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Lachaux","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hepatology and Gastroenterology Hospices Civils de Lyon, H\u00f4pital Femme Mere Enfant and Lyon 1 University, Inserm 1111  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4259-6861","authenticated-orcid":false,"given":"C.","family":"Greco","sequence":"additional","affiliation":[{"name":"Department of Pain and Palliative Care Necker\u2010Children's Hospital (AP\u2010HP5), Paris\u2010Centre University, Imagine Institute  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Hadj\u2010Rabia","sequence":"additional","affiliation":[{"name":"Department of Dermatology Reference Center for Genodermatoses 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