{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T08:41:07Z","timestamp":1761986467999},"reference-count":16,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2012,7,17]],"date-time":"2012-07-17T00:00:00Z","timestamp":1342483200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2013,2]]},"abstract":"<jats:p>Kanegane H, Taneichi H, Nomura K, Wada T, Yachie A, Imai K, Ariga T, Santisteban I, Hershfield MS, Miyawaki T. Successful bone marrow transplantation with reduced intensity conditioning in a patient with delayed\u2010onset adenosine deaminase deficiency.<\/jats:p><jats:p><jats:bold>Abstract:\u2002<\/jats:bold> In this case report, we describe successful BMT with RIC in a patient with delayed\u2010onset ADA deficiency. A three\u2010yr\u2010old Japanese boy was diagnosed with delayed\u2010onset ADA deficiency because of recurrent bronchitis, bronchiectasia, and lymphopenia. In addition, autoimmune thyroiditis and neutropenia were present. At four yr of age, he underwent BMT with a RIC regimen, including busulfan and fludarabine, from an HLA\u2010identical healthy sister. Engraftment after BMT was uneventful without GVHD. Decreased ADA levels in blood immediately increased following BMT, and the patient was disease\u2010free 13\u2003months after BMT. These results suggest that BMT with RIC may sufficiently restore immune regulation in delayed\u2010onset ADA deficiency. A longer follow\u2010up period is needed to confirm these observations.<\/jats:p>","DOI":"10.1111\/j.1399-3046.2012.01762.x","type":"journal-article","created":{"date-parts":[[2012,7,19]],"date-time":"2012-07-19T07:05:45Z","timestamp":1342681545000},"source":"Crossref","is-referenced-by-count":4,"title":["Successful bone marrow transplantation with reduced intensity conditioning in a patient with delayed\u2010onset adenosine deaminase deficiency"],"prefix":"10.1111","volume":"17","author":[{"given":"Hirokazu","family":"Kanegane","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiromichi","family":"Taneichi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keiko","family":"Nomura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Taizo","family":"Wada","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akihiro","family":"Yachie","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kohsuke","family":"Imai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tadashi","family":"Ariga","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ines","family":"Santisteban","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael S.","family":"Hershfield","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshio","family":"Miyawaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2012,7,17]]},"reference":[{"key":"e_1_2_7_2_2","first-page":"480","volume-title":"Immunologic Disorders in Infants and Children","author":"Hershfield MS","year":"2004"},{"key":"e_1_2_7_3_2","first-page":"169","volume-title":"Primary Immunodeficiency Diseases: A Molecular and Genetic Approach","author":"Hirshhorn R","year":"2007"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-06-189209"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/s12185-012-1055-4"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2009.05.026"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2011.01.052"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1086\/652752"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V99.3.1005"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1086\/302054"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0952-7915(03)00104-3"},{"key":"e_1_2_7_12_2","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1016\/S0140-6736(03)12513-5","article-title":"Long\u2010term survival and transplantation of haematopoietic stem cells for immunodeficiencies: Report of the European experience 1968\u201399","volume":"361","author":"Antoine C","year":"2003","journal-title":"Lancet"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2009.08.041"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0805817"},{"key":"e_1_2_7_15_2","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2009.1.682"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2011.04.030"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2010.025098"}],"container-title":["Pediatric Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1399-3046.2012.01762.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1399-3046.2012.01762.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T22:25:17Z","timestamp":1698704717000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1399-3046.2012.01762.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,7,17]]},"references-count":16,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2013,2]]}},"alternative-id":["10.1111\/j.1399-3046.2012.01762.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1399-3046.2012.01762.x","archive":["Portico"],"relation":{},"ISSN":["1397-3142","1399-3046"],"issn-type":[{"value":"1397-3142","type":"print"},{"value":"1399-3046","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,7,17]]}}}