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    COVID-19 disease in children and adolescents following allogeneic hematopoietic stem cell transplantation: A report from the Turkish pediatric bone marrow transplantation study group
    (Wiley, 2024) Bozkurt, Ceyhun; Hazar, Volkan; Malbora, Baris; Kupesiz, Alphan; Aygunes, Utku; Fisgin, Tunc; Karakukcu, Musa
    BackgroundData on the risk factors and outcomes for pediatric patients with SARS-CoV-2 infection (COVID-19) following hematopoietic stem cell transplantation (HSCT) are limited.ObjectivesThe study aimed to analyze the clinical signs, risk factors, and outcomes for ICU admission and mortality in a large pediatric cohort who underwent allogeneic HSCT prior to COVID-19 infection.MethodIn this nationwide study, we retrospectively reviewed the data of 184 pediatric HSCT recipients who had COVID-19 between March 2020 and August 2022.ResultsThe median time from HSCT to COVID-19 infection was 209.0 days (IQR, 111.7-340.8; range, 0-3845 days). The most common clinical manifestation was fever (58.7%). While most patients (78.8%) had asymptomatic/mild disease, the disease severity was moderate in 9.2% and severe and critical in 4.4% and 7.6%, respectively. The overall mortality was 10.9% (n: 20). Deaths were attributable to COVID-19 in nine (4.9%) patients. Multivariate analysis revealed that lower respiratory tract disease (LRTD) (OR, 23.20, p: .001) and lymphopenia at diagnosis (OR, 5.21, p: .006) were risk factors for ICU admission and that HSCT from a mismatched donor (OR, 54.04, p: .028), multisystem inflammatory syndrome in children (MIS-C) (OR, 31.07, p: .003), and LRTD (OR, 10.11, p: .035) were associated with a higher risk for COVID-19-related mortality.ConclusionWhile COVID-19 is mostly asymptomatic or mild in pediatric transplant recipients, it can cause ICU admission in those with LRTD or lymphopenia at diagnosis and may be more fatal in those who are transplanted from a mismatched donor and those who develop MIS-C or LRTD. COVID-19 in children following HSCT is frequently asymptomatic/mild. Nonetheless, 12% of patients have such severe disease that they need intensive care. Adverse outcomes are expected in mismatched HSCT, lymphopenia, LRTD, and MIS-C.image
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    Endocrine and metabolic disorders in adult patients with thalassemia major
    (2020) Yilmaz, Nusret; Tazegul, Gokhan; Avsar, Esin; Kupesiz, Alphan; Sari, Ramazan; Altunbas, Hasan Ali; Balci, Mustafa Kemal
    Aim: Iron overload in tissues, despite current chelation therapies, is a major cause of organ dysfunction and serious complications in thalassemia major. Similarly, iron accumulation in endocrine tissues pave the way for various endocrinopathies. Previous reports regarding prevalence of endocrinopathies in thalassemia major varies significantly based on study population. In this study, we aimed to investigate the metabolic and endocrine disorders among the transfusion-dependent adult thalassemia major patients.Materials and Methods: Data from transfusion-dependent adult thalassemia major patients with regular follow-up were retrospectively evaluated. Former records of the patients were examined to evaluate endocrine disorders, on the basis of laboratory test results. Results: A total of 76 patients with a median age of 28 years, composed of 39 (51.3%) female and 37 (48.7%) male patients were included. Out of the entire cohort, 36.8% (n=28) had hypogonadism, 30.3% (n=23) had thyroid dysfunction, 28.9% (n=22) had a glucose metabolism disorder and 7.9% (n=6) had hypoparathyroidism. Hypogonadism was encountered in 38.5% (n=15) of females and in 35.1% (n=13) of males. Only one patient from each gender had hypergonadotropic hypogonadism, possibly related to iron overload, while the rest had hypogonadotropic hypogonadism. A positive history of delayed puberty was noted in 30.8% (n=12) of females and 24.3% (n=9) of males. Of the patients, 30.3% (n=23) had one, 18.4% (n=14) had 2, 10.5% (n=8) had 3, and 1.3% (n=1) had 4 different endocrine dysfunctions. Accordingly, 61.5% of the patients had at least one endocrine dysfunction while only 39.5% (n=30) had no endocrine dysfunction at all. Conclusion: Survival time has been prolonged in patients with thalassemia major by virtue of effective transfusion and chelation therapies. As a consequence of prolonged survival, endocrine dysfunctions commonly strike adult thalassemia major patients, therefore, endocrine functions need to be evaluated at regular intervals during follow-up.
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    Thalassemia-free and graft-versus-host-free survival: outcomes of hematopoietic stem cell transplantation for thalassemia major, Turkish experience
    (Springernature, 2022) Yesilipek, M. Akif; Uygun, Vedat; Kupesiz, Alphan; Karasu, Gulsun; Ozturk, Gulyuz; Ertem, Mehmet; Sasmaz, Ilgen
    We report the national data on the outcomes of hematopoietic stem cell transplantation (HSCT) for thalassemia major (TM) patients in Turkey on behalf of the Turkish Pediatric Stem Cell Transplantation Group. We retrospectively enrolled 1469 patients with TM who underwent their first HSCT between 1988 and 2020 in 25 pediatric centers in Turkey. The median follow-up duration and transplant ages were 62 months and 7 years, respectively; 113 patients had chronic graft versus host disease (cGVHD) and the cGVHD rate was 8.3% in surviving patients. Upon the last visit, 30 patients still had cGvHD (2.2%). The 5-year overall survival (OS), thalassemia-free survival (TFS) and thalassemia-GVHD-free survival (TGFS) rates were 92.3%, 82.1%, and 80.8%, respectively. cGVHD incidence was significantly lower in the mixed chimerism (MC) group compared to the complete chimerism (CC) group (p < 0.001). In survival analysis, OS, TFS, and TGFS rates were significantly higher for transplants after 2010. TFS and TGFS rates were better for patients under 7 years and at centers that had performed over 100 thalassemia transplants. Transplants from matched unrelated donors had significantly higher TFS rates. We recommend HSCT before 7 years old in thalassemia patients who have a matched donor for improved outcomes.