Tıp Fakültesi / Faculty of Medicine
Permanent URI for this collectionhttps://hdl.handle.net/11727/1403
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Item Short-term Central Venous Catheter Complications in Patients with Sickle Cell Disease Who Undergo Apheresis(2014) Yeral, Mahmut; Boga, Can; Oguzkurt, Levent; Asma, Suheyl; Kasar, Mutlu; Kozanoglu, Ilknur; https://orcid.org/0000-0002-9580-628X; https://orcid.org/0000-0001-5335-7976; https://orcid.org/0000-0003-3856-7005; https://orcid.org/0000-0002-5268-1210; 23504572; ABC-4148-2020; AAD-6222-2021; AAI-7831-2021; AAL-3906-2021; AAE-1241-2021Patients with sickle cell disease (SCD) are prone to develop thrombosis and infection due to their inflammatory and immune deficiency state. These patients require red cell exchange therapy for treatment or prevention of hemoglobin S associated complications. Owing to vascular access problems, adult patients need central venous catheterization (CVC) for exchange procedures. Procedure related complications have been reported for long-term CVCs in pediatric patients. However, short-term CVC complications in adult patients are not clear. This report represents the results of documented complications of short-term CVCs in patients with SCD who undergo apheresis. A total of 142 non-tunneled catheters with average median diameter of 9 F (range 8-16 F) were implanted for apheresis. The catheters were mainly inserted through the right internal jugular vein (66.2 %). Total days of catheter were 412. Results were reported as a complication rate and event according to 1,000 catheter days and compared to a control group including 37 healthy stem cell donors. In the patient group, 1 (1 %) hematoma and 1 (1 %) infection were observed for internal jugular vein catheterization (3.7 hemorrhages and 3.7 infections according to 1,000 catheter days), whereas four (8.9 %) cases of thrombosis and 1 (2.2 %) infection (27 and 6.9 according to 1,000 catheter days) developed in femoral vein. There was a significant difference in terms of thrombosis (P = 0.009). In the control group, only individual developed thrombosis in internal jugular vein. Short-term CVC inserted through to the internal jugular vein seems to be safer than femoral vein in patients with SCD.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 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 Hematopoietic Stem Cell Transplantation in Adult Sickle Cell Disease: Problems and Solutions(2015) Ozdogdu, Halkan; Boga, Can; 25912490Sickle cell disease-related organ injuries cannot be prevented despite hydroxyurea use, infection prophylaxis, and supportive therapies. As a consequence, disease-related mortality reaches 14% in adolescents and young adults. Hematopoietic stem cell transplantation is a unique curative therapeutic approach for sickle cell disease. Myeloablative allogeneic hematopoietic stem cell transplantation is curative for children with sickle cell disease. Current data indicate that long-term disease-free survival is about 90% and overall survival about 95% after transplantation. However, it is toxic in adults due to organ injuries. In addition, this curative treatment approach has several limitations, such as difficulties to find donors, transplant-related mortality, graft loss, graft-versus-host disease (GVHD), and infertility. Engraftment effectivity and toxicity for transplantations performed with nonmyeloablative reduced-intensity regimens in adults are being investigated in phase 1/2 trials at many centers. Preliminary data indicate that GVHD could be prevented with transplantations performed using reduced-intensity regimens. It is necessary to develop novel regimens to prevent graft loss and reduce the risk of GVHD.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 patientsItem Significance of electronic health records: A comparative study of vaccination rates in patients with sickle cell disease(2017) Korur, Asli; Asma, Suheyl; Gereklioglu, Cigdem; Solmaz, Soner; Boga, Can; Ozsahin, Akatli Kursat; Kut, Altug; 0000-0002-5086-5593; 0000-0001-8844-3946; 0000-0002-8902-1283; 0000-0001-5335-7976; 0000-0003-0776-8349; 0000-0002-9680-1958; 28811769; AAD-5616-2021; AAL-6544-2020; AAN-3522-2021; AAD-5542-2021; AAD-6222-2021; AAI-7831-2021; A-2550-2015Objective: In this study, we investigated the influence of electronic health records (EHR) and electronic vaccination schedule applications on the vaccination status of patients who were admitted to our Center for the treatment of sickle cell disease (SCD). Methods: The vaccination status against influenza and pneumococcus infection was determined in 93 patients who were admitted to the hematology outpatient clinic, Baskent University Adana Hospital from April 2004 to March 2009. The vaccination status was then re-evaluated following establishment of EHR and electronic vaccination schedules in 2012. Results: Of the 93 patients with SCD 21.5% (n=20) were vaccinated against pneumococcus and 21.5% (n=20) were regularly vaccinated against influenza. When the vaccination rates of 59 of 93 patients who presented for their regular control examinations were analyzed following establishment of EHR and vaccination schedules in 2012, these rates were 49.2% (n=29) and 50.8% (n=30) for influenza and pneumococcus, respectively, after EHR; there were 23.7% (n=14) and 20.3% (n=12), respectively, before EHR. A statistically significant difference was found between the vaccination rates before and after EHR (p < 0.05). Conclusion: Although viral and bacterial infections are life-threatening health problems in patients with SCD, the vaccination rates were low in high-risk patients. However, these rates increased after application of electronic vaccination schedules.Item 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-6265-2019The 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.