Wos Açık Erişimli Yayınlar

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Author: search.filters.author.Boga, CanSubject: search.filters.subject.Hemoglobinopathy

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    Comparison of the clinical course of COVID-19 infection in sickle cell disease patients with healthcare professionals
    (2021) Boga, Can; Asma, Suheyl; Leblebisatan, Goksel; Sen, Nazan; Tombak, Anil; Demiroglu, Yusuf Ziya; Yeral, Mahmut; Akin, Sule; Yesilagac, Hasan; Habesoglu, Mehmet Ali; Aribogan, Anis; Kasar, Mutlu; Korur, Asli; Ozdogu, Hakan; 0000-0002-9866-2197; 34032899; AAZ-9711-2021; AAY-2668-2021
    It is highly expected that COVID-19 infection will have devastating consequences in sickle cell disease (SCD) patients due to endothelial activation and decreased tissue and organ reserve as a result of microvascular ischemia and continuous inflammation. In this study, we aimed to compare the clinical course of COVID-19 in adult SCD patients under the organ injury mitigation and clinical care improvement program (BASCARE) with healthcare professionals without significant comorbid conditions. The study was planned as a retrospective, multicenter and cross-sectional study. Thirty-nine SCD patients, ages 18 to 64 years, and 121 healthcare professionals, ages 21 to 53, were included in the study. The data were collected from the Electronic Health Recording System of PRANA, where SCD patients under the BASCARE program had been registered. The data of other patients were collected from the Electronic Hospital Data Recording System and patient files. In the SCD group, the crude incidence of COVID-19 was 9%, while in healthcare professionals at the same period was 23%. Among the symptoms, besides fever, loss of smell and taste were more prominent in the SCD group than in healthcare professionals. There was a significant difference between the two groups in terms of development of pneumonia, hospitalization, and need for intubation (43 vs 5%, P < 0.00001; 26 vs 7%, P = 0.002; and 10 vs 1%, P = 0.002, respectively). Prophylactic low molecular weight heparin and salicylate were used more in the SCD group than in healthcare professionals group (41 vs 9% and 28 vs 1%; P < 0.0001 for both). The 3-month mortality rate was demonstrated as 5% in the SCD group, while 0 in the healthcare professionals group. One patient in the SCD group became continously dependent on respiratory support. The cause of death was acute chest syndrome in the first case, hepatic necrosis and multi-organ failure in the second case. In conclusion, these observations supported the expectation that the course of COVID-19 in SCD patients will get worse. The BASCARE program applied in SCD patients could not change the poor outcome.
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    Organ damage mitigation with the Baskent Sickle Cell Medical Care Development Program (BASCARE)
    (2018) Boga, Can; Ozdogdu, Hakan; Asma, Suheyl; Kozanoglu, Ilknur; Gereklioglu, Cigdem; Yeral, Mahmut; Buyukkurt, Nurhilal Turgut; Solmaz, Soner; Korur, Asli; Aytan, Pelin; Maytalman, Erkan; Kasar, Mutlu; 0000-0002-5086-5593; 0000-0002-0895-4787; 0000-0002-8902-1283; 0000-0003-3856-7005; 0000-0001-5335-7976; 0000-0002-5268-1210; 0000-0002-2553-7715; 0000-0001-5284-7439; 0000-0002-9680-1958; 0000-0002-9580-628X; 29419693; AAD-5616-2021; AAE-1457-2021; AAL-6544-2020; AAD-6222-2021; AAD-5542-2021; AAL-3906-2021; AAI-7831-2021; AAE-1241-2021; AAE-3833-2019; ABC-4148-2020; F-6265-2019
    The 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.