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Background: Renal dysfunction is an underestimated complication of thalassemia major.
Objectives: The aim of this study is to compare the glomerular and tubular functions in children with β- Thalassemia major (β- TM) with healthy controls and assess the oxidative stress caused by high ferritin levels.
Design And Setting: This prospective cross-sectional study was conducted in tertiary care hospital.
Methods: Complete blood count (CBC), calcium (Ca), urea, creatinine (Cr), serum cystatin C before transfusion and urinary calcium (uCa), creatinine (uCr), protein (UPr) levels were analyzed in fresh samples. Beta-2-microglobulin (uβ2-MG), N- acetylglucosaminidase (uNAG), retinol binding protein (uRBP), malonedialdehyde (uMDA) secretion and creatinine levels were analyzed. Serum total antioxidant capacity (sTAC) and total oxidant capacity (sTOC) were measured with colorimetric micro-ELISA method. Last four serum ferritin values were recorded and the mean value was used for statistical analyzes.
Results: Data from 47 patients and 32 controls were analyzed. The urinary RBP/Cr, Ca/Cr and Protein/Cr, were significantly higher in β-TM group. A statistically insignificant increase in urinary β2MG/Cr, uNAG/Cr, MDA/Cr was also found in the TM group. Proteinuria was present in 46 % (n: 22) and hypercalciuria in 34 % (n: 16) of the patients with β- TM. Serum total antioxidant capacity and total oxidant status (TOS) levels were significantly elevated in the patient group. Serum ferritin was significantly correlated with proteinuria, cystatin C levels, urinary Protein/Cr and uRBP/Cr.
Conclusion: Asymptomatic renal dysfunction is prevalent in β- TM patients that necessitate regular screening. Urinary RBP may be useful for early diagnosis.
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http://dx.doi.org/10.1016/j.transci.2020.102746 | DOI Listing |
Hematology
December 2025
Thalassaemia International Federation (TIF), Nicosia, Cyprus.
Objective: The complexity of β-thalassaemia with associated morbidity, lifelong daily expensive treatment, and multidisciplinary care results in a considerable disease burden. Our study aimed to revisit the β-thalassaemia burden using epidemiological, clinical, and financial indicators related to patients, families, and healthcare systems.
Methods: Patient density measures, transfusion indices, complication rates, universal health coverage, and other indicators were tabulated by country and region.
Clin Chim Acta
September 2025
Department of Medical Laboratory Sciences, Faculty of Allied Health Sciences, University of Jaffna, Jaffna 40000, Sri Lanka. Electronic address:
Screening for β-thalassemia trait (βTT) is crucial for preventing β-thalassemia major in offspring. Although hematological discrimination formulae (HDF), developed using complete blood count parameters, are cost-effective tools for screening βTT, their performance varies across different populations. This study evaluated the performance of 32 HDF for screening βTT in the Sri Lankan population.
View Article and Find Full Text PDFJ Hematol
August 2025
Department of Pathology, Microbiology and Immunology, University of Nebraska Medical Center, Omaha, NE, USA.
Background: Thalassemias are inherited red blood cell disorders characterized by defective globin production, resulting in microcytic hypochromic anemia. Severe variants lead to transfusion dependence and consequent iron overload, often despite chelation therapy. The role of automated red blood cell exchange (RBCX) for transfusion-dependent thalassemia (TDT) is unclear and previously there was no specific apheresis parameters specific for thalassemia defined.
View Article and Find Full Text PDFCrit Rev Oncol Hematol
September 2025
Student Research Committee, Department of Hematology and Blood Banking, School of Allied Medical Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran.. Electronic address:
Epigenetic regulation is fundamental to hematopoiesis, influencing stem cell fate, lineage commitment, and the development of hematologic diseases. Recent technological innovations have transitioned from traditional genetic editing towards programmable, reversible epigenetic modulation without altering the DNA sequence. This review explores the evolution of epigenetic editing platforms, from zinc finger proteins and TALEs to the transformative CRISPR-dCas9 system, and introduces next-generation technologies leveraging dCas12, dCas13, and modular RNA-guided systems.
View Article and Find Full Text PDFBlood Transfus
August 2025
Department of Pathology, University of Virginia, Charlottesville, VA, United States of America.