{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T12:42:28Z","timestamp":1753879348823,"version":"3.41.2"},"reference-count":14,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2019,4,29]],"date-time":"2019-04-29T00:00:00Z","timestamp":1556496000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2019,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>CHARGE syndrome is a rare congenital malformation syndrome which may share symptoms with DiGeorge syndrome. Complete DiGeorge syndrome (cDGS) is a severe form of DiGeorge syndrome, characterized by a CD3+ T\u2010cell count of &lt;50\/mm<jats:sup>3<\/jats:sup> due to athymia, and is fatal without immunologic intervention. We performed peripheral blood lymphocyte transfusion (PBLT) from an HLA\u2010identical sibling without pretransplant conditioning in a CHARGE\/cDGS patient with a novel <jats:italic>CHD7<\/jats:italic> splice site mutation. Cyclosporine and short\u2010term methotrexate were used for graft versus host disease (GVHD) prophylaxis, and neither acute nor chronic GVHD was observed. After PBLT, T\u2010cell proliferative response to phytohemagglutinin and concanavalin A recovered, and intractable diarrhea improved. EBV infection, evidenced by a gradual increase in the viral genome copy number to a maximum of 2861 copies\/\u03bcgDNA on day 42 after PBLT, resolved spontaneously. HLA A2402 restricted, EBV\u2010specific CTLs were detected from peripheral blood on day 148, and EBV seroconversion was observed on day 181. Thus, EBV\u2010specific immunity was successfully established by PBLT. Our results indicate that PBLT is a simple and effective therapy to reconstitute immune systems in CHARGE\/DiGeorge syndrome.<\/jats:p>","DOI":"10.1111\/petr.13424","type":"journal-article","created":{"date-parts":[[2019,4,30]],"date-time":"2019-04-30T13:01:58Z","timestamp":1556629318000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Establishment of immunity against Epstein\u2010Barr virus infection in a patient with CHARGE\/complete DiGeorge syndrome after peripheral blood lymphocyte transfusion"],"prefix":"10.1111","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2792-1235","authenticated-orcid":false,"given":"Sho","family":"Hosaka","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of Tsukuba Hospital Tsukuba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chie","family":"Kobayashi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Tsukuba Hospital Tsukuba Japan"},{"name":"Department of Child Health, Faculty of Medicine University of Tsukuba Tsukuba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hirota","family":"Saito","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Tsukuba Hospital Tsukuba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayako","family":"Imai-Saito","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Tsukuba Hospital Tsukuba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryoko","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Tsukuba Hospital Tsukuba Japan"},{"name":"Department of Child Health, Faculty of Medicine University of Tsukuba Tsukuba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Atsushi","family":"Iwabuchi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Tsukuba Hospital Tsukuba Japan"},{"name":"Department of Child Health, Faculty of Medicine University of Tsukuba Tsukuba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiaki","family":"Kato","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Tsukuba Hospital Tsukuba Japan"},{"name":"Department of Child Health, Faculty of Medicine University of Tsukuba Tsukuba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takahiro","family":"Jimbo","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery University of Tsukuba Hospital Tsukuba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nobuyuki","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Department of Immunology National Research Institute for Child Health and Development Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masafumi","family":"Onodera","sequence":"additional","affiliation":[{"name":"Department of Immunology National Research Institute for Child Health and Development Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ken\u2010Ichi","family":"Imadome","sequence":"additional","affiliation":[{"name":"Department of Advanced Medicine for Infections, National Center for Child Health and Development (NCCHD) Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kouji","family":"Masumoto","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery University of Tsukuba Hospital Tsukuba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toru","family":"Nanmoku","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine University of Tsukuba 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