{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,14]],"date-time":"2026-04-14T19:18:05Z","timestamp":1776194285863,"version":"3.50.1"},"reference-count":34,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2023,9,11]],"date-time":"2023-09-11T00:00:00Z","timestamp":1694390400000},"content-version":"vor","delay-in-days":10,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>A few studies assessed the clinical and immunological features of selective IgM deficiency (SIgMD), especially in the pediatric age. We aimed to characterize the clinical and immunological phenotypes of a cohort of pediatric patients with SIgMD according to the different diagnostic criteria available.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this multicenter study, we evaluated pediatric SIgMD patients diagnosed at the Pediatric Clinic in Pavia, Italy, or through the Italian Primary Immunodeficiency NETwork (IPINET) and monitored changes in their diagnosis over a time frame that ranges from several months to several years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010eight patients with SIgMD were included (mean serum IgM: 33\u2009mg\/dL). The most common clinical manifestations were recurrent infections (67%) and allergies (48%). Subgroup analysis according to SIgMD definition criteria of the European Society for Immunodeficiencies (ESID) showed no significant difference in clinical manifestations, also considering the group with additional immunological abnormalities. Sixteen patients had long\u2010term follow\u2010up, during which 87% preserved their SIgMD diagnosis, while two patients showed a reduction in IgA in addition to low IgM.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our data suggest that the identification of a reduction in serum IgM in children should lead to a complete immunological work\u2010up to obtain a comprehensive clinical and immunological characterization of the patient. The follow\u2010up of these patients is fundamental to define the disease evolution and appropriate management.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pai.14015","type":"journal-article","created":{"date-parts":[[2023,9,11]],"date-time":"2023-09-11T11:06:18Z","timestamp":1694430378000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Clinical and immunological phenotypes of selective <scp>IgM<\/scp> deficiency in children: Results from a multicenter study"],"prefix":"10.1111","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0029-9383","authenticated-orcid":false,"given":"Riccardo","family":"Castagnoli","sequence":"first","affiliation":[{"name":"Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences University of Pavia  Pavia Italy"},{"name":"Pediatric Clinic Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0372-523X","authenticated-orcid":false,"given":"Ivan","family":"Taietti","sequence":"additional","affiliation":[{"name":"Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences University of Pavia  Pavia Italy"},{"name":"Pediatric Clinic Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martina","family":"Votto","sequence":"additional","affiliation":[{"name":"Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences University of Pavia  Pavia Italy"},{"name":"Pediatric Clinic Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matteo","family":"Naso","sequence":"additional","affiliation":[{"name":"Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences University of Pavia  Pavia Italy"},{"name":"Pediatric Clinic Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"De\u00a0Filippo","sequence":"additional","affiliation":[{"name":"Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences University of Pavia  Pavia Italy"},{"name":"Pediatric Clinic Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessia","family":"Marseglia","sequence":"additional","affiliation":[{"name":"Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences University of Pavia  Pavia Italy"},{"name":"Pediatric Clinic Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lorenza","family":"Montagna","sequence":"additional","affiliation":[{"name":"Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences University of Pavia  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mara","family":"De\u00a0Amici","sequence":"additional","affiliation":[{"name":"Pediatric Clinic Fondazione IRCCS Policlinico San Matteo  Pavia Italy"},{"name":"Laboratory of Immuno\u2010Allergology of Clinical Chemistry Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Antonietta","family":"Avanzini","sequence":"additional","affiliation":[{"name":"Immunology and Transplantation Laboratory, Cell Factory, Pediatric Hematology Oncology Unit, Department of Maternal and Children's Health Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniela","family":"Montagna","sequence":"additional","affiliation":[{"name":"Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences University of Pavia  Pavia Italy"},{"name":"Immunology and Transplantation Laboratory, Cell Factory, Pediatric Hematology Oncology Unit, Department of Maternal and Children's Health Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3662-0159","authenticated-orcid":false,"given":"Gian Luigi","family":"Marseglia","sequence":"additional","affiliation":[{"name":"Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences University of Pavia  Pavia Italy"},{"name":"Pediatric Clinic Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1773-6482","authenticated-orcid":false,"given":"Amelia","family":"Licari","sequence":"additional","affiliation":[{"name":"Pediatric Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences University of Pavia  Pavia Italy"},{"name":"Pediatric Clinic Fondazione IRCCS Policlinico San Matteo  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"the Italian Primary Immunodeficiency NETwork (IPINET)","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,9,11]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-017-0464-9"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-022-01289-3"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-022-01352-z"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.4.5579.583"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-013-8375-x"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2017.01056"},{"key":"e_1_2_10_8_1","unstructured":"Indian Pediatrics \u2013 Editorial. Accessed February 22 2023.https:\/\/www.indianpediatrics.net\/sep2005\/sep\u2010961\u2010962.htm"},{"issue":"3","key":"e_1_2_10_9_1","first-page":"214","article-title":"Selective immunoglobulin M deficiency in an adult with Streptococcus pneumoniae sepsis and invasive aspergillosis","volume":"18","author":"Hong R","year":"2008","journal-title":"J Investig Allergol Clin Immunol"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)60962-3"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1159\/000222682"},{"issue":"6","key":"e_1_2_10_12_1","first-page":"547","article-title":"IgM deficiency: clinical spectrum and immunologic assessment","volume":"62","author":"Guill MF","year":"1989","journal-title":"Ann Allergy"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(70)90288-6"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/pai.13497"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.20639"},{"issue":"1","key":"e_1_2_10_16_1","first-page":"21","article-title":"Analysis of subsets of B cells, Breg, CD4Treg and CD8Treg cells in adult patients with primary selective IgM deficiency","volume":"5","author":"Louis AG","year":"2016","journal-title":"Am J Clin Exp Immunol"},{"issue":"6","key":"e_1_2_10_17_1","first-page":"55","article-title":"Comprehensive clinical and immunological features of 62 adult patients with selective primary IgM deficiency","volume":"8","author":"Lucuab\u2010Fegurgur DL","year":"2019","journal-title":"Am J Clin Exp Immunol"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1155\/2008\/624850"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-019-00641-4"},{"key":"e_1_2_10_20_1","unstructured":"Educational Materials\/Library\/JMF. Accessed June 22 2023.https:\/\/info4pi.org\/library\/educational\u2010materials\/"},{"key":"e_1_2_10_21_1","unstructured":"ESID \u2013 European Society for Immunodeficiencies. Accessed March 25 2023.https:\/\/esid.org\/Working\u2010Parties\/Registry\u2010Working\u2010Party\/Diagnosis\u2010criteria"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/cei.13065"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/sji.12763"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(97)70200-2"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2010.04206.x"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2249.1998.00589.x"},{"issue":"1","key":"e_1_2_10_27_1","first-page":"112","article-title":"IgM deficiency","volume":"11","author":"Hobbs JR","year":"1975","journal-title":"Birth Defects Orig Artic Ser"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/cei.12281"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-017-0420-8"},{"issue":"3","key":"e_1_2_10_30_1","first-page":"630","article-title":"Selective IgM deficiency in adults: phenotypically and functionally altered profiles of peripheral blood lymphocytes","volume":"68","author":"Ohno T","year":"1987","journal-title":"Clin Exp Immunol"},{"issue":"3","key":"e_1_2_10_31_1","first-page":"670","article-title":"Functional assessment of T and B lymphocytes in patients with selective IgM deficiency","volume":"49","author":"De la Concha EG","year":"1982","journal-title":"Clin Exp Immunol"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-3083.1989.tb02445.x"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.iac.2015.07.001"},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1399-3038.2002.02088.x"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1182\/hematology.2019002062"}],"container-title":["Pediatric Allergy and Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pai.14015","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,25]],"date-time":"2023-09-25T15:36:31Z","timestamp":1695656191000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pai.14015"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,9]]},"references-count":34,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2023,9]]}},"alternative-id":["10.1111\/pai.14015"],"URL":"https:\/\/doi.org\/10.1111\/pai.14015","archive":["Portico"],"relation":{},"ISSN":["0905-6157","1399-3038"],"issn-type":[{"value":"0905-6157","type":"print"},{"value":"1399-3038","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,9]]},"assertion":[{"value":"2023-04-27","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-08-14","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-09-11","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e14015"}}