To check the amount of cellular damage caused by serial transfusions of blood in thalassemia patients. Methods: A cross-sectional study was conducted in the University of Lahore, Lahore, Pakistan between August 2012 and December 2012. A total of 43 thalassemia patients underwent at least 10 blood transfusions. Comprehensive biochemical analysis of blood was performed to record the levels of creatinine, urea, uric acid, albumin, liver function tests, malondialdehyde (MDA), and ferritin. Results: Serum creatinine (0.732±0.23mg/dl) and uric acid (6.7±0.94mg/dl, p less than 0.05) were significantly higher in patient groups as compared with the control. Ferritin levels were significantly higher in patients as compared with the control (3103.9±1747.4, p less than 0.05). Hemoglobin levels were observed in controls 14±1.3g/dl and in patients 7.1±1.03g/dl. No clear relationship exists between age and hematological parameters of thalassemic patients. Serum ferritin level is positively related with serum alanine transaminase, aspartate aminotransferase, and alkaline phosphatase and MDA (p less than 0.05). Conclusion: Serum MDA and serum ferritin of patients (r=0.593, p less than 0.05) reflects that both are crucial parameters estimating the cellular damage in patients suffering from thalassemia.
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http://dx.doi.org/10.15537/smj.2016.11.16242 | DOI Listing |
J Clin Med
January 2025
Department of Life Sciences and Biotechnology, Ferrara University, 44121 Ferrara, Italy.
: Cellular biobanks are of great interest for performing studies finalized in the development of personalized approaches for genetic diseases, including β-thalassemia and sickle cell disease (SCD), important diseases affecting the hematopoietic system. These inherited genetic diseases are characterized by a global distribution and the need for intensive health care. The aim of this report is to present an update on the composition of a cellular Thal-Biobank, to describe its utilization since 2016, to present data on its application in studies on fetal hemoglobin induction and on gene editing, and to discuss its employment as a "unique tool" during and after the COVID-19 pandemic.
View Article and Find Full Text PDFMol Biotechnol
January 2025
Department of Pharmaceutical Chemistry, Faculty of Pharmacy, University of Tabuk, Tabuk, Saudi Arabia.
Clustered, regularly interspaced short palindromic repeats (CRISPR)-CRISPR-associated protein (Cas) system is a new gene editing tool that represents a revolution in gene therapy. This study aimed to review the clinical trials conducted to evaluate the efficacy and safety of the CRISPR/Cas9 system in treating thalassemia and sickle cell disease (SCD). We searched relevant literature using "CRISPR Cas", "thalassemia", "sickle cell" and "clinical trial" as subject terms in PubMed, Cochrane, Web of Science, and Google Scholar up to December 3rd, 2023.
View Article and Find Full Text PDFTrends Mol Med
January 2025
Université Paris Cité, Imagine Institute, Laboratory of chromatin and gene regulation during development, INSERM UMR 1163, 75015, Paris, France.
Beta-thalassemia is a severe, hereditary blood disorder characterized by anemia, transfusion dependence, reduced life expectancy, and poor quality of life. Allogeneic transplantation of hematopoietic stem cells (HSCs) is the only curative treatment for transfusion-dependent β-thalassemia, but a lack of compatible donors prevents the use of this approach for most patients. Over the past 20 years, the rise of gene therapy and the development of lentiviral vectors and genome-editing tools has extended curative options to a broader range of patients.
View Article and Find Full Text PDFJ Family Med Prim Care
December 2024
Department of Pediatrics, Lady Hardinge Medical College, New Delhi, India.
Background: Our study aimed to determine the prevalence of Peripheral Neuropathy (using nerve conduction studies (NCS)) in children with transfusion-dependent thalassemia aged between 5 to 18 years and to study its correlation with chronic anemia, ferritin levels, chelation status, annual transfusion requirement, deficiency of serum Vitamin B12, and Folate levels.
Methods: In this hospital-based cross-sectional study, 100 eligible children were enrolled in a tertiary care teaching hospital in New Delhi, India. Neurological examinations focusing on peripheral neuropathy followed by NCS were performed on all the patients.
Neuroimage
January 2025
Department of Diagnostic Radiology, City of Hope National Medical Center, 1500 E Duarte, CA 91010, USA.
Background: The aim of this study was to establish an iron overload rat model to simulate the elevated iron levels in patients with thalassemia and to investigate the potential association between hippocampal iron deposition and cognition.
Methods: Two groups of iron overloaded rats and one group of control rats were used for this study. The Morris water maze (MWM) was used to test spatial reference memory indicated by escape latency time and number of MWM platform crossings.
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