Tıp Fakültesi / Faculty of Medicine

Permanent URI for this collectionhttps://hdl.handle.net/11727/1403

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search.filters.applied.f.language: search.filters.language.engSubject: search.filters.subject.Acute chest syndrome

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    East Mediterranean Region Sickle Cell Disease Mortality Trial: Retrospective Multicenter Cohort Analysis of 735 Patients
    (2016) Karacaoglu, Pelin Kardas; Asma, Suheyl; Korur, Asli; Solmaz, Soner; Buyukkurt, Nurhilal Turgut; Gereklioglu, Cigdem; Kasar, Mutlu; Ozbalci, Demircan; Unal, Selma; Kaya, Hasan; Gurkan, Emel; Yeral, Mahmut; Sariturk, Cagla; Boga, Can; Ozdogu, Hakan; https://orcid.org/0000-0002-7459-7167; https://orcid.org/0000-0001-5335-7976; https://orcid.org/0000-0002-0895-4787; https://orcid.org/0000-0003-3856-7005; https://orcid.org/0000-0002-9580-628X; https://orcid.org/0000-0002-4130-1059; https://orcid.org/0000-0002-8902-1283; 27068408; HKF-1250-2023; AAI-7831-2021; AAL-6544-2020; AAE-1457-2021; AAL-3906-2021; ABC-4148-2020; AAS-7129-2021; AAD-6222-2021; AAD-5542-2021
    Sickle cell disease (SCD), one of the most common genetic disorders worldwide, is characterized by hemolytic anemia and tissue damage from the rigid red blood cells. Although hydroxyurea and transfusion therapy are administered to treat the accompanying tissue injury, whether either one prolongs the lifespan of patients with SCD is unknown. SCD-related mortality data are available, but there are few studies on mortality-related factors based on evaluations of surviving patients. In addition, ethnic variability in patient registries has complicated detailed analyses. The aim of this study was to investigate mortality and mortality-related factors among an ethnically homogeneous population of patients with SCD. The 735 patients (102 children and 633 adults) included in this retrospective cohort study were of Eti-Turk origin and selected from 1367 patients seen at 5 regional hospitals. A central population management system was used to control for records of patient mortality. Data reliability was checked by a data supervision group. Mortality-related factors and predictors were identified in univariate and multivariate analyses using a Cox regression model with stepwise forward selection. The study group included patients with homozygous hemoglobin S (Hgb S) disease (67 %), Hb S-beta(0) thalassemia (17 %), Hgb S-beta(+) thalassemia (15 %), and Hb S-alpha thalassemia (1 %). They were followed for a median of 66 +/- 44 (3-148) months. Overall mortality at 5 years was 6.1 %. Of the 45 patients who died, 44 (6 %) were adults and 1 (0.1 %) was a child. The mean age at death was 34.1 +/- 10 (18-54) years for males, 40.1 +/- 15 (17-64) years for females, and 36.6 +/- 13 (17-64) years overall. Hydroxyurea was found to have a notable positive effect on mortality (p = 0.009). Mortality was also significantly related to hypertension and renal damage in a univariate analysis (p = 0.015 and p = 0.000, respectively). Acute chest syndrome, splenic sequestration, and prolonged painful-crisis-related multiorgan failure were the most common causes of mortality. In a multivariate analysis of laboratory values, only an elevated white blood cell count was related to mortality (p = 0.009). These data show that despite recent progress in the treatment of SCD, disease-related factors continue to result in mortality in young adult patients. Our results highlight the importance of evaluating curative treatment options for patients who have an appropriate stem cell donor in addition to improving patient care and patient education.
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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.