Tıp Fakültesi / Faculty of Medicine
Permanent URI for this collectionhttps://hdl.handle.net/11727/1403
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Item Pregnancy and Sickle Cell Disease: A Review of the Current Literature(2016) Boga, Can; Ozdogu, Hakan; https://orcid.org/0000-0002-8902-1283; 26672916; AAD-6222-2021; AAD-5542-2021Sickle cell disease (SCD) is the most common hereditary disease worldwide, presenting with anemia and intermittent severe pain. Pregnancy in a patient with SCD is associated with high levels of maternal and fetal morbidity and mortality; the maternal and fetal death rates during pregnancy can attain 11.4% and 20%, respectively. Patient care has improved over time. However, certain management issues remain unresolved; these include the optimum policy in terms of prophylactic blood transfusion, and the use of antiplatelet drugs. Such issues are attributable to the heterogeneous nature of clinical SCD features, and the limitations of uncontrolled and prospective trials. In this review, we seek to facilitate a better understanding of relevant issues via creation of a comparative data profile extracted from current studies. This report may also encourage the drafting of standard operating procedure for management of pregnancy in SCD patients. (C) 2015 Elsevier Ireland Ltd. All rights reserved.Item 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-5542-2021Sickle 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.Item Red Blood Cell Exchange Followed by Plasma Exchange in Patients with Intrahepatic Cholestasis due to Sickle Cell Disease(2016) Buyukkurt, Nurhilal; Kozanoglu, Ilknur; Boga, Can; Ozdogu, Hakan; Yeral, Mahmut; 0000-0002-0895-4787; 0000-0002-5268-1210; 0000-0002-8902-1283; 0000-0002-9580-628X; 0000-0002-9680-1958; AAE-1457-2021; AAE-1241-2021; AAD-6222-2021; AAD-5542-2021; ABC-4148-2020Intrahepatic cholestasis, a rare complication of sickle cell anemia, is characterized by marked hyperbilirubinemia, acute hepatic failure, and an often fatal course. In this report, we present patients with homozygous hemoglobin S and one patient with sickle-beta thalassemia disease who have intrahepatic cholestasis. Despite automated red blood cell exchange transfusion, which successfully decreased the hemoglobin S level to less than 30% of total hemoglobin level in the peripheral blood, basic biochemical laboratory examination revealed signs of cholestasis with a serum bilirubin level of up to 50 mg/dL in the first case, 30 mg/dL in the second, and 10 mg/dL in the third. The patients underwent plasma exchange, which improved their clinical condition except one. These cases suggest that plasma exchange may have a role in improving the clinical condition of patients with sickle cell disease complicated with intrahepatic cholestasis (SCIC) that fails to respond to automated red blood cell exchange therapyItem Corticosteroid-Induced Vaso-Occlusive Events May Be Prevented by Lowering Hemoglobin S Levels in Adults With Sickle Cell Disease(2017) Yeral, Mahmut; Boga, Can; Aytan, Pelin; Ozdogu, Hakan; 0000-0002-9580-628X; 0000-0002-2553-7715; 0000-0002-8902-1283; 28988593; ABC-4148-2020; AAD-6222-2021; AAE-3833-2019; AAD-5542-2021Item Use of Red Blood Cell Exchange for Treating Acute Complications of Sickle Cell Disease(2018) Kozanoglu, Ilknur; Ozdogu, Hakan; https://orcid.org/0000-0002-5268-1210; https://orcid.org/0000-0002-8902-1283; 29525569; AAE-1241-2021; AAD-5542-2021Sickle cell disease (SCD) is a life-threatening chronic condition primarily caused by genetic mutation. The disease is characterized by intermittent vaso-occlusive events and chronic hemolytic anemia. Acute complications in patients with SCD are difficult to manage due to the pathophysiological nature of the disease. Transfusion therapy is the cornerstone of management of acute complications and significantly reduces SCD morbidity and mortality. Red cell exchange (RCE), which is characterized by low iron accumulation and volume overload, has been widely used for transfusion therapy in recent years. (C) 2018 Published by Elsevier Ltd.Item 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-2668-2021It 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.Item Red blood cell alloimmunization in patients with sickle cell disease in Turkey: a single center retrospective cohort study(2016) Solmaz, Soner; Karacaoglu, Pelin; Gereklioglu, Cigdem; Asma, Suheyl; Korur, Asli; Buyukkurt, Nurhilal; Kasar, Mutlu; Yeral, Mahmut; Kozanoglu, Ilknur; Boga, Can; Ozdogu, Hakan; 0000-0002-5086-5593; 0000-0002-8902-1283; 0000-0003-3856-7005; 0000-0002-0895-4787; 0000-0002-5268-1210; 0000-0002-9680-1958; AAD-5616-2021; AAD-5542-2021; AAL-3906-2021; AAE-1457-2021; ABC-4148-2020; AAD-6222-2021; AAE-1241-2021Purpose: We aimed to investigate erythrocyte alloimmunization frequency and related factors in our region where SCD is common Material and Methods: This study was planned as a single center, cross-sectional and retrospective cohort study. A total of 216 patients who had been followed up due to SCD [Hemoglobin (Hb) SS, Hb S-beta thalassemia, Hb S-alpha thalassemia] were included in this study. Patients were divided to two groups according to amount of transfusion. The patients who had received less than 6 transfusions per year and who did not have the history of erythropheresis were allocated to Group 1, and the patients who had received 6 or more simple transfusion per year or who had undergone erythrocyte exchange were allocated to Group 2 Results: Of 216 SCD patients included in the study. Alloimmunization was detected in 67 (31.0%) out of 216 patients who underwent transfusion, and in 17 (30.4%) out of 56 patients in Group 1 and in 50 (31.3%) out of 160 patients in Group 2. When the patients were analyzed according to alloimmunization development, our study revealed that neither SCD complications are a risk factor for alloimmunization nor alloimmunization increases mortality rates Conclusion: High alloimmunization frequency found in our study suggests the insufficient adherence of alloimmunization-prevention policies in RBC transfusions performed except experienced institutions. Therefore alloimmunization may be reduced or prevented through performing extended red cell typing among SCD patients