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dc.contributor.authorNepesov, Serdar
dc.contributor.authorYaman, Yöntem
dc.contributor.authorElli, Murat
dc.contributor.authorBayram, Nihan
dc.contributor.authorÖzdilli, Kürşat
dc.contributor.authorKıykım, Ayça
dc.contributor.authorAnak, Sema
dc.date.accessioned2022-08-24T11:28:29Z
dc.date.available2022-08-24T11:28:29Z
dc.date.issued2022en_US
dc.identifier.citationNepesov, S., Yaman, Y., Elli, M., Bayram, N., Özdilli, K., Kıykım, A. ... Anak, S. (2022). Hematopoietic stem cell transplantation in patients with severe combined immunodeficiency: A single-center experience. Experimed, 12(1), 24-28. http://doi.org/10.26650/experimed.2022.1077058en_US
dc.identifier.issn2667-5846
dc.identifier.urihttp://doi.org/10.26650/experimed.2022.1077058
dc.identifier.urihttps://hdl.handle.net/20.500.12511/9666
dc.description.abstractObjective: The aim of this study was to determine the factors affecting outcomes in patients who underwent hematopoietic stem cell transplantation (HSCT) with the diagnosis of severe combined immunodeficiency (SCID). Furthermore, our aim is to share our singlecenter experience of HSCT among SCID patients. Materials and Methods: The data of patients who underwent HSCT with the diagnosis of SCID between January 2014 and January 2021 in the pediatric bone marrow transplant unit of Istanbul Medipol University were retrospectively analyzed. Demographic and clinical data, treatment regimens, donor source, type of transplantation, pre- and post-transplantation infections, and complications were evaluated. Results: Among fifteen patients who underwent HSCT, 5 (33%) were female. The mean age at diagnosis was 3 months (1-6 months), and at transplantation 6 months (3-10 months). The mean time from diagnosis to transplantation was 3 months (2-9 months). There was a history of consanguineous marriage in thirteen (87%) and sibling death in eight (53%) cases. As donors, six (40%) were siblings and five (33%) were unrelated, while four (27%) patients underwent haploid transplantation. Four (27%) patients died during the first 100 days of transplantation. The median follow-up period was 23 months (9-61 months). Overall survival probability was calculated as 73%. Conclusion: SCID should be considered as an emergency in pediatrics. Devastating complications, including severe organ damage, lifethreatening infections, and even death, could appear in case of diagnostic delay. HSCT is a currently available curative treatment option. Subjects with a confirmed diagnosis should be referred to the appropriate bone marrow transplant center and treated as soon as possible.en_US
dc.language.isoengen_US
dc.publisherIstanbul Universityen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.rightsAttribution-NonCommercial 4.0 International*
dc.rights.urihttps://creativecommons.org/licenses/by-nc/4.0/*
dc.subjectBone Marrow Transplantationen_US
dc.subjectChildrenen_US
dc.subjectSevere Combined Immunodeficiencyen_US
dc.titleHematopoietic stem cell transplantation in patients with severe combined immunodeficiency: A single-center experienceen_US
dc.typearticleen_US
dc.relation.ispartofExperimeden_US
dc.departmentİstanbul Medipol Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalıen_US
dc.authorid0000-0002-4551-5433en_US
dc.authorid0000-0002-9710-8653en_US
dc.authorid0000-0002-0476-5452en_US
dc.authorid0000-0002-9688-5223en_US
dc.authorid0000-0002-7129-5024en_US
dc.authorid0000-0001-8489-7449en_US
dc.identifier.volume12en_US
dc.identifier.issue1en_US
dc.identifier.startpage24en_US
dc.identifier.endpage28en_US
dc.relation.publicationcategoryMakale - Ulusal Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.identifier.doi10.26650/experimed.2022.1077058en_US
dc.institutionauthorNepesov, Serdar
dc.institutionauthorYaman, Yöntem
dc.institutionauthorElli, Murat
dc.institutionauthorBayram, Nihan
dc.institutionauthorÖzdilli, Kürşat
dc.institutionauthorAnak, Sema


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