{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Haddad E"],"funding":["NCATS NIH HHS","NIAID NIH HHS","NHLBI NIH HHS","NCI NIH HHS","NINDS NIH HHS"],"pagination":["1737-1749"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC6202916"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["132(17)"],"pubmed_abstract":["The Primary Immune Deficiency Treatment Consortium (PIDTC) performed a retrospective analysis of 662 patients with severe combined immunodeficiency (SCID) who received a hematopoietic cell transplantation (HCT) as first-line treatment between 1982 and 2012 in 33 North American institutions. Overall survival was higher after HCT from matched-sibling donors (MSDs). Among recipients of non-MSD HCT, multivariate analysis showed that the SCID genotype strongly influenced survival and immune reconstitution. Overall survival was similar for patients with <i>RAG</i>, <i>IL2RG</i>, or <i>JAK3</i> defects and was significantly better compared with patients with <i>ADA</i> or <i>DCLRE1C</i> mutations. Patients with <i>RAG</i> or <i>DCLRE1C</i> mutations had poorer immune reconstitution than other gen"],"journal":["Blood"],"pubmed_title":["SCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery."],"pmcid":["PMC6202916"],"funding_grant_id":["U10 HL069254","P30 CA008748","R13 AI094943","U24 CA076518","U54 AI082973","U01 TR001263","U54 NS064808","U01 HL069294"],"pubmed_authors":["Griffith LM","Pai SY","Haddad E","Bednarski JJ","Logan BR","Buckley RH","Skoda-Smith S","Abdel-Azim H","Seroogy CM","Szabolcs P","Kang EM","Cavanaugh ME","Haight AE","Shereck E","O'Reilly RJ","Malech HL","Hanson IC","Desantes KB","Taylor C","Gillio AP","Kohn DB","Prockop SE","Keller MD","Heimall J","Kletzel M","Tumlin AG","Notarangelo LD","Petrovic A","Connelly JA","Decaluwe H","Sullivan KE","Cowan MJ","Parrott RE","Martinez C","Thakar MS","Jyonouchi S","Leiding JW","Yu LC","Pulsipher MA","Moore TB","Murnane M","Bleesing JJH","Smith AR","Goldman FD","Fleisher TA","Puck JM","Porteus MH","Quigg TC","Chaisson J","Chandra S","Small TN","Craddock JA","Kapoor N","Cuvelier GDE","Shyr DC","Davila Saldana BJ","Yin Z","Knutsen AP","Vander Lugt MT","Shearer WT","Aquino V","Teira P","Burroughs L","Dvorak CC","Davis JH"],"additional_accession":[]},"is_claimable":false,"name":"SCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery.","description":"The Primary Immune Deficiency Treatment Consortium (PIDTC) performed a retrospective analysis of 662 patients with severe combined immunodeficiency (SCID) who received a hematopoietic cell transplantation (HCT) as first-line treatment between 1982 and 2012 in 33 North American institutions. Overall survival was higher after HCT from matched-sibling donors (MSDs). Among recipients of non-MSD HCT, multivariate analysis showed that the SCID genotype strongly influenced survival and immune reconstitution. Overall survival was similar for patients with <i>RAG</i>, <i>IL2RG</i>, or <i>JAK3</i> defects and was significantly better compared with patients with <i>ADA</i> or <i>DCLRE1C</i> mutations. Patients with <i>RAG</i> or <i>DCLRE1C</i> mutations had poorer immune reconstitution than other gen","dates":{"release":"2018-01-01T00:00:00Z","publication":"2018 Oct","modification":"2026-04-15T15:33:21.144Z","creation":"2019-11-05T08:04:11Z"},"accession":"S-EPMC6202916","cross_references":{"pubmed":["30154114"],"doi":["10.1182/blood-2018-03-840702"]}}