{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T23:17:07Z","timestamp":1789514227883,"version":"build-2803163510"},"reference-count":49,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[2015,11,1]],"date-time":"2015-11-01T00:00:00Z","timestamp":1446336000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2015,11,1]],"date-time":"2015-11-01T00:00:00Z","timestamp":1446336000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","jacionline.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Allergy and Clinical Immunology"],"published-print":{"date-parts":[[2015,11]]},"DOI":"10.1016\/j.jaci.2015.04.027","type":"journal-article","created":{"date-parts":[[2015,6,6]],"date-time":"2015-06-06T19:43:50Z","timestamp":1433619830000},"page":"1178-1185","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":53,"title":["Radiation-sensitive severe combined immunodeficiency: The arguments for and against conditioning before hematopoietic cell transplantation\u2014what to do?"],"prefix":"10.1016","volume":"136","author":[{"given":"Morton J.","family":"Cowan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew R.","family":"Gennery","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jaci.2015.04.027_bib1","doi-asserted-by":"crossref","first-page":"620","DOI":"10.1016\/j.dnarep.2013.04.015","article-title":"DNA repair mechanisms in dividing and non-dividing cells","volume":"12","author":"Iyama","year":"2013","journal-title":"DNA Repair (Amst)"},{"key":"10.1016\/j.jaci.2015.04.027_bib2","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1007\/BF03195638","article-title":"Mystery of DNA repair: the role of the MRN complex and ATM kinase in DNA damage repair","volume":"49","author":"Czornak","year":"2008","journal-title":"J Appl Genet"},{"key":"10.1016\/j.jaci.2015.04.027_bib3","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1093\/hmg\/dds448","article-title":"Bone marrow transplantation improves the outcome of Atm-deficient mice through the migration of ATM-competent cells","volume":"22","author":"Pietzner","year":"2013","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.jaci.2015.04.027_bib4","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1146\/annurev-genet-110711-155540","article-title":"Modernizing the nonhomologous end-joining repertoire: alternative and classical NHEJ share the stage","volume":"47","author":"Deriano","year":"2013","journal-title":"Annu Rev Genet"},{"key":"10.1016\/j.jaci.2015.04.027_bib5","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1016\/S0092-8674(01)00309-9","article-title":"Artemis, a novel DNA double-strand break repair\/V(D)J recombination protein, is mutated in human severe combined immune deficiency","volume":"105","author":"Moshous","year":"2001","journal-title":"Cell"},{"key":"10.1016\/j.jaci.2015.04.027_bib6","first-page":"91","article-title":"A DNA-PKcs mutation in a radiosensitive T-B- SCID patient inhibits Artemis activation and nonhomologous end-joining","volume":"119","author":"van der Burg","year":"2009","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jaci.2015.04.027_bib7","doi-asserted-by":"crossref","first-page":"5060","DOI":"10.4049\/jimmunol.176.8.5060","article-title":"A severe form of human combined immunodeficiency due to mutations in DNA ligase IV","volume":"176","author":"Enders","year":"2006","journal-title":"J Immunol"},{"key":"10.1016\/j.jaci.2015.04.027_bib8","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1016\/j.cell.2005.12.030","article-title":"Cernunnos, a novel nonhomologous end-joining factor, is mutated in human immunodeficiency with microcephaly","volume":"124","author":"Buck","year":"2006","journal-title":"Cell"},{"key":"10.1016\/j.jaci.2015.04.027_bib9","doi-asserted-by":"crossref","first-page":"8889","DOI":"10.1073\/pnas.1323649111","article-title":"Differential role of nonhomologous end joining factors in the generation, DNA damage response, and myeloid differentiation of human induced pluripotent stem cells","volume":"111","author":"Felgentreff","year":"2014","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2015.04.027_bib10","doi-asserted-by":"crossref","first-page":"1250","DOI":"10.1002\/eji.200425555","article-title":"Damaging-agent sensitivity of Artemis-deficient cell lines","volume":"35","author":"Musio","year":"2005","journal-title":"Eur J Immunol"},{"key":"10.1016\/j.jaci.2015.04.027_bib11","doi-asserted-by":"crossref","first-page":"1303","DOI":"10.1093\/hmg\/ddl050","article-title":"Radiation-induced delayed cell death in a hypomorphic Artemis cell line","volume":"15","author":"Evans","year":"2006","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.jaci.2015.04.027_bib12","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1038\/nrm2514","article-title":"Ataxia-telangiectasia: from a rare disorder to a paradigm for cell signalling and cancer","volume":"10","author":"Lavin","year":"2008","journal-title":"Nat Rev Mol Cell Biol"},{"key":"10.1016\/j.jaci.2015.04.027_bib13","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1038\/bmt.2012.207","article-title":"Long-term survival after allogeneic-matched sibling PBSC transplantation with conditioning consisting of low-dose busilvex and fludarabine in a 3-year-old boy with ataxia-telangiectasia syndrome and ALL","volume":"48","author":"Ussowicz","year":"2013","journal-title":"Bone Marrow Transplant"},{"key":"10.1016\/j.jaci.2015.04.027_bib14","doi-asserted-by":"crossref","first-page":"994","DOI":"10.1016\/j.jaci.2013.01.047","article-title":"B-cell reconstitution for SCID: should a conditioning regimen be used in SCID treatment?","volume":"131","author":"Haddad","year":"2013","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2015.04.027_bib15","doi-asserted-by":"crossref","first-page":"4114","DOI":"10.1182\/blood-2008-09-177923","article-title":"Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency","volume":"113","author":"Neven","year":"2009","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2015.04.027_bib16","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1056\/NEJMoa1401177","article-title":"Transplantation outcomes for severe combined immunodeficiency, 2000-2009","volume":"371","author":"Pai","year":"2014","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.jaci.2015.04.027_bib17","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1007\/s10875-012-9797-6","article-title":"Post-transplantation B cell function in different molecular types of SCID","volume":"33","author":"Buckley","year":"2013","journal-title":"J Clin Immunol"},{"key":"10.1016\/j.jaci.2015.04.027_bib18","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1016\/j.jaci.2014.06.021","article-title":"Comparison of outcomes of hematopoietic stem cell transplantation without chemotherapy conditioning by using matched sibling and unrelated donors for treatment of severe combined immunodeficiency","volume":"134","author":"Dvorak","year":"2014","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2015.04.027_bib19","doi-asserted-by":"crossref","first-page":"1125","DOI":"10.1016\/j.bbmt.2008.07.008","article-title":"Megadose CD34(+) cell grafts improve recovery of T cell engraftment but not B cell immunity in patients with severe combined immunodeficiency disease undergoing haplocompatible nonmyeloablative transplantation","volume":"14","author":"Dvorak","year":"2008","journal-title":"Biol Blood Marrow Transplant"},{"key":"10.1016\/j.jaci.2015.04.027_bib20","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1182\/blood-2013-01-476432","article-title":"SCID patients with ARTEMIS vs RAG deficiencies following HCT: increased risk of late toxicity in ARTEMIS-deficient SCID","volume":"123","author":"Schuetz","year":"2014","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2015.04.027_bib21","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1111\/petr.12310","article-title":"A trial of Alemtuzumab adjunctive therapy in allogeneic hematopoietic cell transplantation with minimal conditioning for severe combined immunodeficiency","volume":"18","author":"Dvorak","year":"2014","journal-title":"Pediatr Transplant"},{"key":"10.1016\/j.jaci.2015.04.027_bib22","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1001\/jama.2014.9132","article-title":"Newborn screening for severe combined immunodeficiency in 11 screening programs in the United States","volume":"312","author":"Kwan","year":"2014","journal-title":"JAMA"},{"key":"10.1016\/j.jaci.2015.04.027_bib23","doi-asserted-by":"crossref","first-page":"1219","DOI":"10.1016\/j.jaci.2008.08.031","article-title":"Omenn syndrome is associated with mutations in DNA ligase IV","volume":"122","author":"Grunebaum","year":"2008","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2015.04.027_bib24","doi-asserted-by":"crossref","first-page":"1175","DOI":"10.1016\/S1097-2765(01)00408-7","article-title":"DNA ligase IV mutations identified in patients exhibiting developmental delay and immunodeficiency","volume":"8","author":"O'Driscoll","year":"2001","journal-title":"Mol Cell"},{"key":"10.1016\/j.jaci.2015.04.027_bib25","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1186\/1750-1172-2-5","article-title":"Successful bone marrow transplantation in a patient with DNA ligase IV deficiency and bone marrow failure","volume":"2","author":"Gruhn","year":"2007","journal-title":"Orphanet J Rare Dis"},{"key":"10.1016\/j.jaci.2015.04.027_bib26","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1002\/eji.200535401","article-title":"Severe combined immunodeficiency and microcephaly in siblings with hypomorphic mutations in DNA ligase IV","volume":"36","author":"Buck","year":"2006","journal-title":"Eur J Immunol"},{"key":"10.1016\/j.jaci.2015.04.027_bib27","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1172\/JCI26121","article-title":"A new type of radiosensitive T-B-NK+ severe combined\u00a0immunodeficiency caused by a LIG4 mutation","volume":"116","author":"van der Burg","year":"2006","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jaci.2015.04.027_bib28","doi-asserted-by":"crossref","first-page":"912","DOI":"10.1016\/S0140-6736(09)60945-4","article-title":"Haemopoietic stem-cell transplantation with antibody-based minimal-intensity conditioning: a phase 1\/2 study","volume":"374","author":"Straathof","year":"2009","journal-title":"Lancet"},{"key":"10.1016\/j.jaci.2015.04.027_bib29","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1002\/pbc.22031","article-title":"A novel mutation in a family with DNA ligase IV deficiency syndrome","volume":"53","author":"Unal","year":"2009","journal-title":"Pediatr Blood Cancer"},{"key":"10.1016\/j.jaci.2015.04.027_bib30","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1111\/j.1399-3046.2008.01028.x","article-title":"Unrelated hematopoietic stem cell transplantation for Cernunnos-XLF deficiency","volume":"13","author":"Faraci","year":"2009","journal-title":"Pediatr Transplant"},{"key":"10.1016\/j.jaci.2015.04.027_bib31","doi-asserted-by":"crossref","first-page":"2462","DOI":"10.1073\/pnas.0437964100","article-title":"Nonhomologous end joining and V(D)J recombination require an additional factor","volume":"100","author":"Dai","year":"2003","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2015.04.027_bib32","doi-asserted-by":"crossref","first-page":"E167","DOI":"10.1111\/j.1399-3046.2011.01491.x","article-title":"Two\u00a0SCID cases with Cernunnos-XLF deficiency successfully treated by hematopoietic stem cell transplantation","volume":"16","author":"\u00c7a\u011fda\u015f","year":"2012","journal-title":"Pediatr Transplant"},{"key":"10.1016\/j.jaci.2015.04.027_bib33","doi-asserted-by":"crossref","first-page":"2686","DOI":"10.1128\/JCM.01077-13","article-title":"Atypical strain of Toxoplasma gondii causing fatal reactivation after hematopoietic stem cell transplantation in a patient with an underlying immunological deficiency","volume":"51","author":"Ivovi\u0107","year":"2013","journal-title":"J Clin Microbiol"},{"key":"10.1016\/j.jaci.2015.04.027_bib34","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1038\/bmt.2009.207","article-title":"Successful SCT for Nijmegen breakage syndrome","volume":"45","author":"Albert","year":"2010","journal-title":"Bone Marrow Transplant"},{"key":"10.1016\/j.jaci.2015.04.027_bib35","doi-asserted-by":"crossref","first-page":"E51","DOI":"10.1111\/petr.12420","article-title":"Reduced-intensity conditioning umbilical cord blood transplantation in Nijmegen breakage syndrome","volume":"19","author":"Wo\u017aniak","year":"2015","journal-title":"Pediatr Transplant"},{"key":"10.1016\/j.jaci.2015.04.027_bib36","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1038\/sj.bmt.1702831","article-title":"Bone marrow\u00a0transplantation for T-B- severe combined immunodeficiency disease in\u00a0Athabascan-speaking native Americans","volume":"27","author":"O'Marcaigh","year":"2001","journal-title":"Bone Marrow Transplant"},{"key":"10.1016\/j.jaci.2015.04.027_bib37","doi-asserted-by":"crossref","first-page":"892","DOI":"10.1016\/j.jaci.2007.08.007","article-title":"Long-term immune reconstitution and clinical outcome after stem cell transplantation for severe T-cell immunodeficiency","volume":"120","author":"Mazzolari","year":"2007","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2015.04.027_bib38","doi-asserted-by":"crossref","first-page":"4575","DOI":"10.1182\/blood-2006-07-029090","article-title":"Long-term T-cell reconstitution after hematopoietic stem-cell transplantation in primary T-cell-immunodeficient patients is associated with myeloid chimerism and possibly the primary disease phenotype","volume":"109","author":"Cavazzana-Calvo","year":"2007","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2015.04.027_bib39","doi-asserted-by":"crossref","first-page":"2969","DOI":"10.1172\/JCI67349","article-title":"PRKDC mutations in a SCID patient with profound neurological abnormalities","volume":"123","author":"Woodbine","year":"2013","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jaci.2015.04.027_bib40","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1038\/sj.bmt.1704872","article-title":"Outcome of boost haemopoietic stem cell transplant for decreased donor chimerism or graft dysfunction in primary immunodeficiency","volume":"35","author":"Slatter","year":"2005","journal-title":"Bone Marrow Transplant"},{"key":"10.1016\/j.jaci.2015.04.027_bib41","doi-asserted-by":"crossref","first-page":"1608","DOI":"10.1016\/j.bbmt.2013.08.014","article-title":"Effect of weight and maturation on busulfan clearance in infants and small children undergoing hematopoietic cell transplantation","volume":"19","author":"Savic","year":"2013","journal-title":"Biol Blood Marrow Transplant"},{"key":"10.1016\/j.jaci.2015.04.027_bib42","doi-asserted-by":"crossref","first-page":"1296","DOI":"10.1126\/science.1149726","article-title":"Efficient transplantation via antibody-based clearance of hematopoietic stem cell niches","volume":"318","author":"Czechowicz","year":"2007","journal-title":"Science"},{"key":"10.1016\/j.jaci.2015.04.027_bib43","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1007\/s002800050410","article-title":"Natural\u00a0killer cell activity in chronic lymphocytic leukemia patients treated with fludarabine","volume":"37","author":"Robertson","year":"1996","journal-title":"Cancer Chemother Pharmacol"},{"key":"10.1016\/j.jaci.2015.04.027_bib44","doi-asserted-by":"crossref","first-page":"1996","DOI":"10.1016\/j.bbmt.2014.08.020","article-title":"Treosulfan-based conditioning and hematopoietic cell transplantation for nonmalignant diseases: a prospective multicenter trial","volume":"12","author":"Burroughs","year":"2014","journal-title":"Biol Blood Marrow Transplant"},{"key":"10.1016\/j.jaci.2015.04.027_bib45","doi-asserted-by":"crossref","first-page":"834","DOI":"10.1016\/j.jpeds.2009.07.049","article-title":"Long-term clinical outcome of patients with severe combined immunodeficiency who received related donor bone marrow transplants without pretransplant chemotherapy or post-transplant GVHD prophylaxis","volume":"155","author":"Railey","year":"2009","journal-title":"J Pediatr"},{"key":"10.1016\/j.jaci.2015.04.027_bib46","first-page":"1705","article-title":"B-cell recovery after stem cell transplantation of Artemis-deficient SCID requires elimination of autologous bone marrow precursor-B-cells","volume":"91","author":"van der Burg","year":"2006","journal-title":"Haematologica"},{"key":"10.1016\/j.jaci.2015.04.027_bib47","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1182\/blood.V99.3.872","article-title":"Hematopoietic stem cell transplantation for severe combined immunodeficiency in the neonatal period leads to superior thymic output and improved survival","volume":"99","author":"Myers","year":"2002","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2015.04.027_bib48","doi-asserted-by":"crossref","first-page":"16406","DOI":"10.1073\/pnas.0608130103","article-title":"Complete correction of murine Artemis immunodeficiency by lentiviral vector-mediated gene transfer","volume":"103","author":"Mostoslavsky","year":"2006","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jaci.2015.04.027_bib49","doi-asserted-by":"crossref","first-page":"1490","DOI":"10.1038\/mt.2008.118","article-title":"Stable and functional lymphoid reconstitution in Artemis-deficient mice following lentiviral artemis gene transfer into hematopoietic stem cells","volume":"16","author":"Benjelloun","year":"2008","journal-title":"Mol Ther"}],"container-title":["Journal of Allergy and Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674915005898?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674915005898?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,25]],"date-time":"2025-09-25T08:54:20Z","timestamp":1758790460000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0091674915005898"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,11]]},"references-count":49,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2015,11]]}},"alternative-id":["S0091674915005898"],"URL":"https:\/\/doi.org\/10.1016\/j.jaci.2015.04.027","relation":{},"ISSN":["0091-6749"],"issn-type":[{"value":"0091-6749","type":"print"}],"subject":[],"published":{"date-parts":[[2015,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Radiation-sensitive severe combined immunodeficiency: The arguments for and against conditioning before hematopoietic cell transplantation\u2014what to do?","name":"articletitle","label":"Article Title"},{"value":"Journal of Allergy and Clinical Immunology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jaci.2015.04.027","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2015 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}