{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T17:22:45Z","timestamp":1772990565885,"version":"3.50.1"},"reference-count":9,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,1,20]],"date-time":"2026-01-20T00:00:00Z","timestamp":1768867200000},"content-version":"vor","delay-in-days":19,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2026,1]]},"abstract":"Abstract<\/jats:title>\n \n Introduction<\/jats:title>\n \n X\u2010linked inhibitor of apoptosis protein (XIAP) deficiency is an inborn error of immunity caused by pathogenic variants of\n XIAP<\/jats:italic>\n . It presents diverse symptoms, including recurrent hemophagocytic lymphohistiocytosis and inflammatory bowel disease. Previous reports established a functional analysis method that quantitatively evaluates intracellular tumor necrosis factor\u2010alpha (TNF\u2010\u03b1) production capacity following muramyl dipeptide (MDP) stimulation using flow cytometry (MDP\u2010flow TNF\u2010\u03b1) for assessing XIAP deficiency. However, this method required 2\u2009days to obtain results, which is a limitation.\n <\/jats:p>\n <\/jats:sec>\n \n Method<\/jats:title>\n We established a method to measure the downregulation of L\u2010selectin (CD62L) on the cell surface after MDP stimulation of monocytes and neutrophils from patients with XIAP deficiency using flow cytometry (MDP\u2010flow CD62L) within 4\u2009h. Moreover, we also evaluated MDP\u2010flow CD62L in patients with XIAP deficiency after allogeneic hematopoietic cell transplantation (HCT) to evaluate its usefulness in functional analysis.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Six patients with\n XIAP<\/jats:italic>\n variants, two with interleukin\u20101 receptor\u2010associated kinase 4 deficiency, and healthy controls were analyzed. The mean percent inhibition of CD62L expression (%inhibition) was evaluated in monocytes and neutrophils. The mean inhibition rates of CD62L expression in monocytes and neutrophils from patients with XIAP deficiency were 5.96% and 6.20%, respectively, significantly lower than those from healthy controls (monocytes, 85.4%; neutrophils, 85.4%). Furthermore, in three patients with XIAP deficiency after HCT, the MDP\u2010flow CD62L was evaluated post\u2010HCT, confirming improvement in accordance with donor chimerism.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n In XIAP deficiency, MDP\u2010flow CD62L enabled faster functional analysis than MDP\u2010flow TNF\u2010\u03b1. These analyses are also useful for post\u2010HCT functional assessment.<\/jats:p>\n \n \n \n image<\/jats:alt-text>\n <\/jats:graphic>\n <\/jats:boxed-text>\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/pai.70284","type":"journal-article","created":{"date-parts":[[2026,1,20]],"date-time":"2026-01-20T11:12:31Z","timestamp":1768907551000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Rapid flow cytometric diagnosis of\n XIAP<\/scp>\n deficiency"],"prefix":"10.1111","volume":"37","author":[{"given":"Ryosuke","family":"Wakatsuki","sequence":"first","affiliation":[{"name":"Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences Institute of Science Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Madoka","family":"Nishimura","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences Institute of Science Tokyo Tokyo Japan"},{"name":"Department of Pediatrics, Graduate School of Medical Sciences Kumamoto University Kumamoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dan","family":"Tomomasa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences Institute of Science Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shuhei","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences Institute of Science Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kyogo","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences Institute of Science Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koji","family":"Kawaguchi","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology Shizuoka Children's Hospital Shizuoka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryutaro","family":"Saura","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Nutrition, and Endocrinology Osaka Women's and Children's Hospital Osaka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shota","family":"Inoue","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology Osaka Women's and Children's Hospital Osaka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ichiro","family":"Takeuchi","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Center for Pediatric Inflammatory Bowel Disease National Center for Child Health and Development Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katsuhiro","family":"Arai","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Center for Pediatric Inflammatory Bowel Disease National Center for Child Health and Development Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masanaka","family":"Sugiyama","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology Tokyo Metropolitan Children's Medical Center Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuta","family":"Narishige","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology Miyagi Children's Hospital Sendai Miyagi Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akira","family":"Oshima","sequence":"additional","affiliation":[{"name":"Department of Infectious Disease & Immunology Kanagawa Children's Medical Center Yokohama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miyuki","family":"Tsumura","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Hiroshima University Hospital Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Okada","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akihiro","family":"Hoshino","sequence":"additional","affiliation":[{"name":"Department of Child Health and Development, Graduate School of Medical and Dental Sciences Institute of Science Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masatoshi","family":"Takagi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences Institute of Science Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8696-9378","authenticated-orcid":false,"given":"Hirokazu","family":"Kanegane","sequence":"additional","affiliation":[{"name":"Department of Child Health and Development, Graduate School of Medical and Dental Sciences Institute of Science Tokyo Tokyo 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