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 |
Turk J Haematol
January 2025
Acibadem Adana Hospital, Pediatric BMT Unit, Adana, Türkiye.
Background/aims: Preimplantation genetic diagnosis (PGD) with human leukocyte antigen (HLA) typing represents a significant advancement in treating inherited hematological disorders, particularly thalassemia major. This technology enables the birth of healthy children who can serve as compatible stem cell donors for their affected siblings. Turkey is a world leader in both PGD+HLA typing technology and hematopoietic stem cell transplantation from savior siblings born through PGD+HLA typing.
View Article and Find Full Text PDFIndian J Clin Biochem
January 2025
ICMR-National Institute of Research in Tribal Health, Jabalpur, 482003 India.
Sickle cell disease (SCD) and thalassemia are the most common hereditary disorders encountered in Central India. Timely identification of these disorders is critical to reduction in severe clinical manifestations and for identifying disease burden. Present study reports spectrum of hemoglobinopathies among the referred anemia patients to single centre in central India.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Department of General Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Sawangi Meghe, Wardha, Maharashtra, India.
Arch Gynecol Obstet
January 2025
Department of Obstetrics & Gynecology, University of Tabuk, Tabuk, Saudi Arabia.
Purpose: We explored the effect of beta-thalassemia major on pregnancy and delivery outcomes in non-endemic area, utilizing USA population database.
Methods: This is a retrospective study utilizing data from the Healthcare Cost and Utilization Project-Nationwide Inpatient Sample. A cohort of all deliveries between 2011 and 2014 was created using ICD-9 codes.
Anemia
January 2025
Department of Hematology, Mehmet Akif Inan Egitim Ve Arastirma Hastanesi, Sanliurfa, Turkey.
The effect of pretransfusion hemoglobin on transfusion burden, thrombosis, and mortality in thalassemia and myelodysplastic syndrome is unclear. We aimed to study the pretransfusion hemoglobin and erythrocyte transfusion burden and investigate the effects of these variables on each other in real-life in thalassemia and myelodysplastic syndrome. Adult patients with thalassemia and myelodysplastic syndrome who received at least one erythrocyte concentrate unit outpatient at Sanliurfa Mehmet Akif Inan Training and Research Hospital during 1 year were included in the study.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!