{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T01:17:50Z","timestamp":1790471870248,"version":"4.1.0"},"reference-count":105,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2021,3,7]],"date-time":"2021-03-07T00:00:00Z","timestamp":1615075200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Polish Ministry of Science and Higher Education","award":["ST 02-0066\/07\/253,  ST 02-0060\/07"],"award-info":[{"award-number":["ST 02-0066\/07\/253,  ST 02-0060\/07"]}]},{"name":"Austria Science Funds (FWF)","award":["Grants P32470-B and F4704-B20"],"award-info":[{"award-number":["Grants P32470-B and F4704-B20"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Mastocytosis is characterized by the pathological accumulation of mast cells (MC) in various organs. In these patients, MC may degranulate and thereby contribute to clinical symptoms, especially when a concomitant allergy is present. However, MC activation can not only be induced by high-affinity receptors for IgE, but also by anaphylatoxins, neuropeptides, IgG immune complexes, complement-components, drugs, products of bacteria or parasites, as well as physical factors such as heat, cold, vibration, stress, sun, or physical effort. Symptoms due to mediators released by activated MC may develop in adults suffering from systemic mastocytosis, but also evolve in children who usually have cutaneous mastocytosis (CM). Clinically, CM is otherwise characterized by typical brown, maculopapular skin lesions or mastocytoma associated with a positive Darier\u2019s sign. Pruritus and flushing are common and blistering may also be recorded, especially in diffuse CM (DCM). Pediatric patients with mastocytosis may also have gastrointestinal, respiratory, and neurologic complaints. Although anaphylaxis is not a typical finding, pediatric patients with massive skin involvement and high tryptase levels have a relatively high risk to develop anaphylaxis. This paper reviews MC mediator-related symptoms and anaphylaxis in children with mastocytosis, with special emphasis on risk factors, triggers, and management.<\/jats:p>","DOI":"10.3390\/ijms22052684","type":"journal-article","created":{"date-parts":[[2021,3,7]],"date-time":"2021-03-07T20:20:43Z","timestamp":1615148443000},"page":"2684","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":40,"title":["Mediator-Related Symptoms and Anaphylaxis in Children with Mastocytosis"],"prefix":"10.3390","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2775-3681","authenticated-orcid":false,"given":"Knut","family":"Brockow","sequence":"first","affiliation":[{"name":"Department of Dermatology and Allergology Biederstein, School of Medicine, Technical University of Munich, Biedersteiner Strasse 29, D-80802 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9504-9848","authenticated-orcid":false,"given":"Katarzyna","family":"Plata-Nazar","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Paediatric Gastroenterology, Allergology and Nutrition, Medical University of Gda\u0144sk, 80803 Gda\u0144sk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6967-1393","authenticated-orcid":false,"given":"Magdalena","family":"Lange","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology and Allergology, Medical University of Gda\u0144sk, 80210 Gdansk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6286-0693","authenticated-orcid":false,"given":"Bogus\u0142aw","family":"Nedoszytko","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology and Allergology, Medical University of Gda\u0144sk, 80210 Gdansk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marek","family":"Niedoszytko","sequence":"additional","affiliation":[{"name":"Department of Allergology, Medical University of Gda\u0144sk, 80210 Gdansk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0456-5095","authenticated-orcid":false,"given":"Peter","family":"Valent","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I, Division of Hematology &amp; Hemostaseology, Medical University of Vienna, 1090 Vienna, Austria"},{"name":"Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, 1090 Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,3,7]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"259","DOI":"10.2165\/11588890-000000000-00000","article-title":"Diagnosis and treatment of cutaneous mastocytosis in children: Practical recommendations","volume":"12","author":"Castells","year":"2011","journal-title":"Am. 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