{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,10]],"date-time":"2026-01-10T22:26:52Z","timestamp":1768084012561,"version":"3.49.0"},"reference-count":32,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2022,4,22]],"date-time":"2022-04-22T00:00:00Z","timestamp":1650585600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2022,4,22]],"date-time":"2022-04-22T00:00:00Z","timestamp":1650585600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"funder":[{"DOI":"10.13039\/501100008386","name":"Marmara \u00dcniversitesi","doi-asserted-by":"publisher","award":["SAG-C-TUP-250919-0288"],"award-info":[{"award-number":["SAG-C-TUP-250919-0288"]}],"id":[{"id":"10.13039\/501100008386","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Clin Immunol"],"published-print":{"date-parts":[[2022,7]]},"DOI":"10.1007\/s10875-022-01262-0","type":"journal-article","created":{"date-parts":[[2022,4,22]],"date-time":"2022-04-22T07:05:50Z","timestamp":1650611150000},"page":"1036-1050","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Clinical and Laboratory Factors Affecting the Prognosis of Severe Combined Immunodeficiency"],"prefix":"10.1007","volume":"42","author":[{"given":"Elif","family":"Ozturk","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mehmet Cihangir","family":"Catak","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayca","family":"Kiykim","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dilek","family":"Baser","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sevgi","family":"Bilgic Eltan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koray","family":"Yalcin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nurhan","family":"Kasap","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ercan","family":"Nain","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alper","family":"Bulutoglu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gamze","family":"Akgun","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasemin","family":"Can","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Asena Pinar","family":"Sefer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Royala","family":"Babayeva","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suar","family":"Caki-Kilic","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gulsun","family":"Tezcan Karasu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akif","family":"Yesilipek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ahmet","family":"Ozen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elif","family":"Karakoc-Aydiner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4730-9422","authenticated-orcid":false,"given":"Safa","family":"Baris","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,4,22]]},"reference":[{"issue":"2 Suppl 2","key":"1262_CR1","doi-asserted-by":"publisher","first-page":"S182","DOI":"10.1016\/j.jaci.2009.07.053","volume":"125","author":"LD Notarangelo","year":"2010","unstructured":"Notarangelo LD. Primary immunodeficiencies. J Allergy Clin Immunol. 2010;125(2 Suppl 2):S182\u201394.","journal-title":"J Allergy Clin Immunol"},{"issue":"1","key":"1262_CR2","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1007\/s10875-019-00737-x","volume":"40","author":"SG Tangye","year":"2020","unstructured":"Tangye SG, Al-Herz W, Bousfiha A, Chatila T, Cunningham-Rundles C, Etzioni A, et al. Human inborn errors of immunity: 2019 update on the classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2020;40(1):24\u201364.","journal-title":"J Clin Immunol"},{"issue":"3","key":"1262_CR3","doi-asserted-by":"publisher","first-page":"666","DOI":"10.1007\/s10875-021-00980-1","volume":"41","author":"SG Tangye","year":"2021","unstructured":"Tangye SG, Al-Herz W, Bousfiha A, Cunningham-Rundles C, Franco JL, Holland SM, et al. The ever-increasing array of novel inborn errors of immunity: an interim update by the IUIS committee. J Clin Immunol. 2021;41(3):666\u201379.","journal-title":"J Clin Immunol"},{"issue":"3","key":"1262_CR4","doi-asserted-by":"publisher","first-page":"316","DOI":"10.1007\/s10875-019-00610-x","volume":"39","author":"A Ikinciogullari","year":"2019","unstructured":"Ikinciogullari A, Cagdas D, Dogu F, Tugrul T, Karasu G, Haskologlu S, et al. Clinical features and HSCT outcome for SCID in Turkey. J Clin Immunol. 2019;39(3):316\u201323.","journal-title":"J Clin Immunol"},{"issue":"1","key":"1262_CR5","doi-asserted-by":"publisher","first-page":"405","DOI":"10.1016\/j.jaci.2018.08.027","volume":"143","author":"CC Dvorak","year":"2019","unstructured":"Dvorak CC, Haddad E, Buckley RH, Cowan MJ, Logan B, Griffith LM, et al. The genetic landscape of severe combined immunodeficiency in the United States and Canada in the current era (2010\u20132018). J Allergy Clin Immunol. 2019;143(1):405\u20137.","journal-title":"J Allergy Clin Immunol"},{"issue":"4","key":"1262_CR6","doi-asserted-by":"publisher","first-page":"1092","DOI":"10.1016\/j.jaci.2013.09.044","volume":"133","author":"WT Shearer","year":"2014","unstructured":"Shearer WT, Dunn E, Notarangelo LD, Dvorak CC, Puck JM, Logan BR, et al. Establishing diagnostic criteria for severe combined immunodeficiency disease (SCID), leaky SCID, and Omenn syndrome: the Primary Immune Deficiency Treatment Consortium experience. J Allergy Clin Immunol. 2014;133(4):1092\u20138.","journal-title":"J Allergy Clin Immunol"},{"issue":"6","key":"1262_CR7","doi-asserted-by":"publisher","first-page":"646","DOI":"10.1007\/s10875-018-0537-4","volume":"38","author":"OM Delmonte","year":"2018","unstructured":"Delmonte OM, Schuetz C, Notarangelo LD. RAG deficiency: two genes, many diseases. J Clin Immunol. 2018;38(6):646\u201355.","journal-title":"J Clin Immunol"},{"issue":"17","key":"1262_CR8","doi-asserted-by":"publisher","first-page":"1737","DOI":"10.1182\/blood-2018-03-840702","volume":"132","author":"E Haddad","year":"2018","unstructured":"Haddad E, Logan BR, Griffith LM, Buckley RH, Parrott RE, Prockop SE, et al. SCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery. Blood. 2018;132(17):1737\u201349.","journal-title":"Blood"},{"issue":"25","key":"1262_CR9","doi-asserted-by":"publisher","first-page":"2718","DOI":"10.1182\/blood-2017-05-781849","volume":"130","author":"J Heimall","year":"2017","unstructured":"Heimall J, Logan BR, Cowan MJ, Notarangelo LD, Griffith LM, Puck JM, et al. Immune reconstitution and survival of 100 SCID patients post-hematopoietic cell transplant: a PIDTC natural history study. Blood. 2017;130(25):2718\u201327.","journal-title":"Blood"},{"issue":"5","key":"1262_CR10","doi-asserted-by":"publisher","first-page":"434","DOI":"10.1056\/NEJMoa1401177","volume":"371","author":"SY Pai","year":"2014","unstructured":"Pai SY, Logan BR, Griffith LM, Buckley RH, Parrott RE, Dvorak CC, et al. Transplantation outcomes for severe combined immunodeficiency, 2000\u20132009. N Engl J Med. 2014;371(5):434\u201346.","journal-title":"N Engl J Med"},{"issue":"11","key":"1262_CR11","doi-asserted-by":"publisher","first-page":"1029","DOI":"10.1080\/1744666X.2017.1381558","volume":"13","author":"J Heimall","year":"2017","unstructured":"Heimall J, Cowan MJ. Long term outcomes of severe combined immunodeficiency: therapy implications. Expert Rev Clin Immunol. 2017;13(11):1029\u201340.","journal-title":"Expert Rev Clin Immunol"},{"issue":"6","key":"1262_CR12","doi-asserted-by":"publisher","first-page":"727","DOI":"10.1007\/s10875-018-0540-9","volume":"38","author":"IJ Abd Hamid","year":"2018","unstructured":"Abd Hamid IJ, Slatter MA, McKendrick F, Pearce MS, Gennery AR. Long-term health outcome and quality of life post-HSCT for IL7Ralpha-, Artemis-, RAG1- and RAG2-deficient severe combined immunodeficiency: a single center report. J Clin Immunol. 2018;38(6):727\u201332.","journal-title":"J Clin Immunol"},{"issue":"2","key":"1262_CR13","doi-asserted-by":"publisher","first-page":"879","DOI":"10.1182\/blood-2004-03-0960","volume":"105","author":"K Rao","year":"2005","unstructured":"Rao K, Amrolia PJ, Jones A, Cale CM, Naik P, King D, et al. Improved survival after unrelated donor bone marrow transplantation in children with primary immunodeficiency using a reduced-intensity conditioning regimen. Blood. 2005;105(2):879\u201385.","journal-title":"Blood"},{"key":"1262_CR14","doi-asserted-by":"publisher","first-page":"625","DOI":"10.1146\/annurev.immunol.22.012703.104614","volume":"22","author":"RH Buckley","year":"2004","unstructured":"Buckley RH. Molecular defects in human severe combined immunodeficiency and approaches to immune reconstitution. Annu Rev Immunol. 2004;22:625\u201355.","journal-title":"Annu Rev Immunol"},{"key":"1262_CR15","doi-asserted-by":"crossref","unstructured":"Delmonte OM, Castagnoli R, Yu J, Dvorak CC, Cowan MJ, Davila Saldana BJ, et al. Poor T-cell receptor beta repertoire diversity early post-transplant for severe combined immunodeficiency predicts failure of immune reconstitution. J Allergy Clin Immunol. 2021.","DOI":"10.1016\/j.bbmt.2019.12.406"},{"issue":"8","key":"1262_CR16","doi-asserted-by":"publisher","first-page":"2790","DOI":"10.1016\/j.jaip.2019.06.011","volume":"7","author":"A Kiykim","year":"2019","unstructured":"Kiykim A, Ogulur I, Dursun E, Charbonnier LM, Nain E, Cekic S, et al. Abatacept as a long-term targeted therapy for LRBA deficiency. J Allergy Clin Immunol Pract. 2019;7(8):2790-800.e15.","journal-title":"J Allergy Clin Immunol Pract"},{"issue":"3","key":"1262_CR17","doi-asserted-by":"publisher","first-page":"228","DOI":"10.1159\/000504598","volume":"181","author":"I Ogulur","year":"2020","unstructured":"Ogulur I, Kiykim A, Baser D, Karakoc-Aydiner E, Ozen A, Baris S. Lymphocyte subset abnormalities in pediatric-onset common variable immunodeficiency. Int Arch Allergy Immunol. 2020;181(3):228\u201337.","journal-title":"Int Arch Allergy Immunol"},{"key":"1262_CR18","doi-asserted-by":"crossref","unstructured":"Kolukisa B, Baser D, Akcam B, Danielson J, Bilgic Eltan S, Haliloglu Y, et al. Evolution and long-term outcomes of combined immunodeficiency due to CARMIL2 deficiency. Allergy. 2021.","DOI":"10.1111\/all.15010"},{"issue":"1","key":"1262_CR19","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1007\/s10875-012-9763-3","volume":"33","author":"SS Kilic","year":"2013","unstructured":"Kilic SS, Ozel M, Hafizoglu D, Karaca NE, Aksu G, Kutukculer N. The prevalences and patient characteristics of primary immunodeficiency diseases in Turkey\u2013two centers study. J Clin Immunol. 2013;33(1):74\u201383.","journal-title":"J Clin Immunol"},{"key":"1262_CR20","doi-asserted-by":"crossref","unstructured":"Firtina S, Yin Ng Y, Hatirnaz Ng O, Kiykim A, Aydiner E, Nepesov S, et al. Mutational landscape of severe combined immunodeficiency patients from Turkey. Int J Immunogenet. 2020.","DOI":"10.1111\/iji.12496"},{"key":"1262_CR21","doi-asserted-by":"publisher","first-page":"23","DOI":"10.3389\/fimmu.2019.00023","volume":"10","author":"J Aluri","year":"2019","unstructured":"Aluri J, Desai M, Gupta M, Dalvi A, Terance A, Rosenzweig SD, et al. Clinical, immunological, and molecular findings in 57 patients with severe combined immunodeficiency (SCID) from India. Front Immunol. 2019;10:23.","journal-title":"Front Immunol"},{"issue":"4","key":"1262_CR22","doi-asserted-by":"publisher","first-page":"1302","DOI":"10.1016\/j.jaci.2016.07.040","volume":"139","author":"C Speckmann","year":"2017","unstructured":"Speckmann C, Doerken S, Aiuti A, Albert MH, Al-Herz W, Allende LM, et al. A prospective study on the natural history of patients with profound combined immunodeficiency: an interim analysis. J Allergy Clin Immunol. 2017;139(4):1302-10 e4.","journal-title":"J Allergy Clin Immunol"},{"issue":"1","key":"1262_CR23","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1007\/s10875-020-00865-9","volume":"41","author":"MJ Dorsey","year":"2021","unstructured":"Dorsey MJ, Wright NAM, Chaimowitz NS, D\u00e1vila Salda\u00f1a BJ, Miller H, Keller MD, et al. Infections in infants with SCID: isolation, infection screening, and prophylaxis in PIDTC centers. J Clin Immunol. 2021;41(1):38\u201350.","journal-title":"J Clin Immunol"},{"issue":"3","key":"1262_CR24","doi-asserted-by":"publisher","first-page":"602","DOI":"10.1016\/j.jaci.2010.06.015","volume":"126","author":"AR Gennery","year":"2010","unstructured":"Gennery AR, Slatter MA, Grandin L, Taupin P, Cant AJ, Veys P, et al. Transplantation of hematopoietic stem cells and long-term survival for primary immunodeficiencies in Europe: entering a new century, do we do better? J Allergy Clin Immunol. 2010;126(3):602-10.e1-11.","journal-title":"J Allergy Clin Immunol"},{"issue":"17","key":"1262_CR25","doi-asserted-by":"publisher","first-page":"4114","DOI":"10.1182\/blood-2008-09-177923","volume":"113","author":"B Neven","year":"2009","unstructured":"Neven B, Leroy S, Decaluwe H, Le Deist F, Picard C, Moshous D, et al. Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency. Blood. 2009;113(17):4114\u201324.","journal-title":"Blood"},{"issue":"1","key":"1262_CR26","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1093\/bmb\/ldaa017","volume":"135","author":"AR Gennery","year":"2020","unstructured":"Gennery AR. The challenges presented by haematopoietic stem cell transplantation in children with primary immunodeficiency. Br Med Bull. 2020;135(1):4\u201315.","journal-title":"Br Med Bull"},{"issue":"4","key":"1262_CR27","doi-asserted-by":"publisher","first-page":"892","DOI":"10.1016\/j.jaci.2007.08.007","volume":"120","author":"E Mazzolari","year":"2007","unstructured":"Mazzolari E, Forino C, Guerci S, Imberti L, Lanfranchi A, Porta F, et al. Long-term immune reconstitution and clinical outcome after stem cell transplantation for severe T-cell immunodeficiency. J Allergy Clin Immunol. 2007;120(4):892\u20139.","journal-title":"J Allergy Clin Immunol"},{"key":"1262_CR28","doi-asserted-by":"crossref","unstructured":"Lankester AC, Albert MH, Booth C, Gennery AR, Gungor T, Honig M, et al. EBMT\/ESID inborn errors working party guidelines for hematopoietic stem cell transplantation for inborn errors of immunity. Bone Marrow Transplant. 2021.","DOI":"10.1038\/s41409-021-01378-8"},{"issue":"4","key":"1262_CR29","doi-asserted-by":"publisher","first-page":"994","DOI":"10.1016\/j.jaci.2013.01.047","volume":"131","author":"E Haddad","year":"2013","unstructured":"Haddad E, Leroy S, Buckley RH. B-cell reconstitution for SCID: should a conditioning regimen be used in SCID treatment? J Allergy Clin Immunol. 2013;131(4):994\u20131000.","journal-title":"J Allergy Clin Immunol"},{"issue":"4","key":"1262_CR30","doi-asserted-by":"publisher","first-page":"1152","DOI":"10.1016\/j.jaci.2016.01.053","volume":"138","author":"K Rao","year":"2016","unstructured":"Rao K, Adams S, Qasim W, Allwood Z, Worth A, Silva J, et al. Effect of stem cell source on long-term chimerism and event-free survival in children with primary immunodeficiency disorders after fludarabine and melphalan conditioning regimen. J Allergy Clin Immunol. 2016;138(4):1152\u201360.","journal-title":"J Allergy Clin Immunol"},{"issue":"24","key":"1262_CR31","doi-asserted-by":"publisher","first-page":"4872","DOI":"10.1200\/JCO.2004.02.189","volume":"22","author":"M Eapen","year":"2004","unstructured":"Eapen M, Horowitz MM, Klein JP, Champlin RE, Loberiza FR Jr, Ringden O, et al. Higher mortality after allogeneic peripheral-blood transplantation compared with bone marrow in children and adolescents: the Histocompatibility and Alternate Stem Cell Source Working Committee of the International Bone Marrow Transplant Registry. J Clin Oncol. 2004;22(24):4872\u201380.","journal-title":"J Clin Oncol"},{"issue":"3","key":"1262_CR32","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1111\/j.1365-2249.2007.03378.x","volume":"148","author":"H Okamoto","year":"2007","unstructured":"Okamoto H, Arii C, Shibata F, Toma T, Wada T, Inoue M, et al. Clonotypic analysis of T cell reconstitution after haematopoietic stem cell transplantation (HSCT) in patients with severe combined immunodeficiency. Clin Exp Immunol. 2007;148(3):450\u201360.","journal-title":"Clin Exp Immunol"}],"updated-by":[{"DOI":"10.1007\/s10875-023-01455-1","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2023,3,11]],"date-time":"2023-03-11T00:00:00Z","timestamp":1678492800000}}],"container-title":["Journal of Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10875-022-01262-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10875-022-01262-0\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10875-022-01262-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,3,12]],"date-time":"2023-03-12T07:04:08Z","timestamp":1678604648000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10875-022-01262-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,22]]},"references-count":32,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2022,7]]}},"alternative-id":["1262"],"URL":"https:\/\/doi.org\/10.1007\/s10875-022-01262-0","relation":{"correction":[{"id-type":"doi","id":"10.1007\/s10875-023-01455-1","asserted-by":"object"}]},"ISSN":["0271-9142","1573-2592"],"issn-type":[{"value":"0271-9142","type":"print"},{"value":"1573-2592","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4,22]]},"assertion":[{"value":"1 November 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 March 2022","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 April 2022","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 March 2023","order":4,"name":"change_date","label":"Change Date","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Correction","order":5,"name":"change_type","label":"Change Type","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"A Correction to this paper has been published:","order":6,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"https:\/\/doi.org\/10.1007\/s10875-023-01455-1","URL":"https:\/\/doi.org\/10.1007\/s10875-023-01455-1","order":7,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The study was approved by the Ethics Committee of Marmara University, School of Medicine (09.2019.511).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics Approval"}},{"value":"Informed consent for participation was obtained from all individuals.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to Participate"}},{"value":"Informed publication consent was obtained from all participants.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for Publication"}},{"value":"The authors declare no competing interests.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of Interest"}}]}}