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dc.contributor.authorBoga, Can
dc.contributor.authorOzdogdu, Hakan
dc.contributor.authorAsma, Suheyl
dc.contributor.authorKozanoglu, Ilknur
dc.contributor.authorGereklioglu, Cigdem
dc.contributor.authorYeral, Mahmut
dc.contributor.authorBuyukkurt, Nurhilal Turgut
dc.contributor.authorSolmaz, Soner
dc.contributor.authorKorur, Asli
dc.contributor.authorAytan, Pelin
dc.contributor.authorMaytalman, Erkan
dc.contributor.authorKasar, Mutlu
dc.date.accessioned2019-05-21T08:36:04Z
dc.date.available2019-05-21T08:36:04Z
dc.date.issued2018
dc.identifier.issn0025-7974
dc.identifier.urihttps://insights.ovid.com/crossref?an=00005792-201802090-00034
dc.identifier.urihttp://hdl.handle.net/11727/3294
dc.description.abstractThe Eastern Mediterranean is among the regions where sickle cell disease (SCD) is common. The morbidity and mortality of this disease can be postponed to adulthood through therapies implemented in childhood. The present study focuses on the organ damage-reducing effects of the Baskent Sickle Cell Medical Care Development Program (BASCARE), which was developed by a team who lives in this region and has approximately 25 years of experience. The deliverables of the program included the development of an electronic health recording system (PRANA) and electronic vaccination system; the use of low citrate infusion in routine prophylactic automatic erythrocyte exchange (ARCE) programs including pregnant women; the use of leukocyte-filtered and irradiated blood for transfusion; the use of magnetic resonance imaging methods (T2(*)) for the management of transfusion-related hemosiderosis; and the implementation of an allogeneic hematopoietic stem cell transplantation protocol for adult patients. The sample was composed of 376 study subjects and 249 control subjects. The hospital's Data Management System and the central population operating system were used for data collection. BASCARE enabled better analysis and interpretation of complication and mortality data. Vaccination rates against influenza and pneumococcal disease improved (21.5% vs 50.8% and 21.5% vs 49.2%, respectively). Effective and safe ARCE with low citrate infusion were maintained in 352 subjects (1003 procedures). Maternal and fetal mortality was prevented in 35 consecutive pregnant patients with ARCE. Chelating therapy rates reduced from 6.7% to 5%. Successful outcomes could be obtained in all 13 adult patients who underwent allogeneic peripheral stem cell transplantation from a fully matched, related donor. No patients died by day 100 or after the first year. Cure could be achieved without graft loss, grades III to IV acute graft versus host disease, extensive chronic graft versus host disease, or other major complications. The BASCARE program significantly improved patient care and thereby prolonged the life span of SCD patients (42 +/- 13 years vs 29 +/- 7 years, P < .001). We may recommend using such individualized programs in centers that provide health care for patients with SCD, in accordance with holistic approach due to the benign nature but malignant course of the disease.en_US
dc.language.isoengen_US
dc.relation.isversionof10.1097/MD.0000000000009844en_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectChelationen_US
dc.subjectErythrocyte exchangeen_US
dc.subjectHematopoietic stem cell transplantationen_US
dc.subjectHemoglobinopathyen_US
dc.subjectMortalityen_US
dc.subjectSickle cell diseaseen_US
dc.titleOrgan damage mitigation with the Baskent Sickle Cell Medical Care Development Program (BASCARE)en_US
dc.typearticleen_US
dc.relation.journalMEDICINEen_US
dc.identifier.volume97en_US
dc.identifier.issue6en_US
dc.identifier.wos000428576300034en_US
dc.identifier.scopus2-s2.0-85042064364en_US
dc.contributor.pubmedID29419693en_US
dc.contributor.orcID0000-0002-5086-5593en_US
dc.contributor.orcID0000-0002-0895-4787en_US
dc.contributor.orcID0000-0002-8902-1283en_US
dc.contributor.orcID0000-0003-3856-7005en_US
dc.contributor.orcID0000-0001-5335-7976en_US
dc.contributor.orcID0000-0002-5268-1210en_US
dc.contributor.orcID0000-0002-2553-7715en_US
dc.contributor.orcID0000-0001-5284-7439en_US
dc.contributor.orcID0000-0002-9680-1958en_US
dc.contributor.orcID0000-0002-9580-628Xen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergien_US
dc.contributor.researcherIDAAD-5616-2021en_US
dc.contributor.researcherIDAAE-1457-2021en_US
dc.contributor.researcherIDAAL-6544-2020en_US
dc.contributor.researcherIDAAD-6222-2021en_US
dc.contributor.researcherIDAAD-5542-2021en_US
dc.contributor.researcherIDAAL-3906-2021en_US
dc.contributor.researcherIDAAI-7831-2021en_US
dc.contributor.researcherIDAAE-1241-2021en_US
dc.contributor.researcherIDAAE-3833-2019en_US
dc.contributor.researcherIDABC-4148-2020en_US
dc.contributor.researcherIDF-6265-2019en_US


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