Wos İndeksli Yayınlar Koleksiyonu

Permanent URI for this collectionhttps://hdl.handle.net/11727/4807

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    Hematopoietic Stem Cell Transplantation in Adult Sickle Cell Disease: Problems and Solutions
    (2015) Ozdogdu, Halkan; Boga, Can; 25912490
    Sickle 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.
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    A Rare Complication Developing After Hematopoietic Stem Cell Transplantation: Wernicke's Encephalopathy
    (2015) Solmaz, Soner; Gereklioglu, Cigdem; Tan, Meliha; Demir, Senay; Yeral, Mahmut; Korur, Asli; Boga, Can; Ozdogu, Hakan; 25912759
    Thiamine is a water-soluble vitamin. Thiamine deficiency can present as a central nervous system disorder known as Wernicke's encephalopathy, which classically manifests as confusion, ataxia, and ophthalmoplegia. Wernicke's encephalopathy has rarely been reported following hematopoietic stem cell transplantation. Herein, we report Wernicke's encephalopathy in a patient with acute myeloid leukemia who had been receiving prolonged total parenteral nutrition after haploidentical allogeneic hematopoietic stem cell transplantation. To the best of our knowledge, this is the first case reported from Turkey in the literature.
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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.