Understanding Beta-Thalassemia with Focus on the Indian Subcontinent and the Middle East

Lahiry, Piya; Al-Attar, S.A.; Ra, Hegele

doi:10.2174/1874276900802010005

Cited by 16 publications

(6 citation statements)

References 65 publications

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“… 1 - 3 Individuals belonging to the South-East Asian, Middle East, and Mediterranean region were affected mainly with thalassemia. 4 , 5 As per the World Health Organization (WHO) statement, among all hemoglobinopathies, beta-thalassemia is the most fatal worldwide and requires strict attention. 6 In different regions of the world, the prevalence of thalassemia has been discovered to be varied.…”

Section: Introductionmentioning

confidence: 99%

Red Blood Cell Alloimmunization in Pediatric group with Beta Thalassemia: A Five-Year Experience

Minhas

Ejaz

Tukruna³

et al. 2022

Global Pediatric Health

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Beta-thalassemia is one of the most frequently occurring hematological disorders in [Removed for blinded peer-review]. Regular blood transfusion is required in almost all cases for management. However, this is associated with significant major complications like red blood cell (RBC) alloimmunization. This retrospective cross-sectional is conducted to evaluate the RBC alloimmunization frequency in children with beta-thalassemia aged between 6 months and 16 years in [Removed for blinded peer-review]. Antibody screening was performed using the Dia clon3 cell antigen panel. If the screening came back positive, a detailed panel was created for the identification of specific antibody. In our sample, the frequency of RBC alloimmunization was found in 22 (26.19%) patients. Of these 22 patients, the Rhesus system was found in most patients 17 (77.3%), followed by Kell 5 (22.7%). RBC alloimmunization was significantly associated with a family history of a blood disorder and splenectomy.

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Section: Introductionmentioning

confidence: 99%

Red Blood Cell Alloimmunization in Pediatric group with Beta Thalassemia: A Five-Year Experience

Minhas

Ejaz

Tukruna³

et al. 2022

Global Pediatric Health

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“…The only treatment for β-thalassaemia is bone marrow transplantation, which is expensive and risky; moreover, it is difficult to identify a suitable donor, and the required infrastructure is unavailable in most Middle Eastern countries. The cost of blood transfusions for β-thalassaemia major patients in the Middle East has been estimated at USD 3,200 annually per patient, with lifetime costs of approximately USD 284,145 [6,7]. β-Thalassaemia is inherited, and couples where both partners are carriers (i.e.…”

Section: Introductionmentioning

confidence: 99%

Exploring the Effectiveness of Mandatory Premarital Screening and Genetic Counselling Programmes for β-Thalassaemia in the Middle East: A Scoping Review

Saffi

Howard

2015

Public Health Genomics

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Background: β-Thalassaemia is a common genetic blood disorder in the Middle Eastern region. Mandatory premarital screening and genetic counselling (PMSGC) programmes are implemented in 8 Middle East countries to reduce at-risk marriages and thus disease prevalence. A scoping review was conducted to explore the effectiveness of these programmes. Methods: The 6-stage scoping framework of Arksey and O'Malley [Int J Soc Res Methodol 2005;8:19-32] was used. Reported outcomes were analysed per country, with success defined as achieving a 65% reduction in at-risk marriages and/or thalassaemia-affected births. Emergent enablers and barriers were analysed thematically. Results: Twenty-one sources were included from the 1,348 identified, discussing 7 country programmes, with 95% (20/21) published during 2003-2013. Five publications each were included for Iran and Saudi Arabia, 3 for Turkey, 2 each for Bahrain and Iraq (Kurdistan), and 1 for the United Arab Emirates, plus 2 multi-country evaluations. No programme achieved a 65% at-risk marriage cancellation rate. Though data on thalassaemia-affected birth reductions were minimal, programmes in Iran, Turkey and Iraq reported at least 65% reductions. A thematic analysis found that screening timing, access to prenatal detection and abortion, socio-religious issues, awareness and counselling affected decisions. Conclusion: This review found that PMSGC programmes were unsuccessful in discouraging at-risk marriages but successful in reducing the prevalence of affected births in countries providing prenatal detection and therapeutic abortion. A life cycle approach to prevention, incorporation of school screening, awareness campaigns, reconsideration of therapeutic abortion, and screening and counselling of couples married prior to programme inception are likely to improve the effectiveness of such programmes in the Middle Eastern region.

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“…Thalassemia can be classified into three different clinical categories based on its severity: major, which requires lifelong blood transfusions; intermedia, which causes non-fatal anemia on its own; and mild thalassemia, also known as thalassemia trait, which typically presents with no observable symptoms [6][7][8] . The type of gene mutation responsible for thalassemia is linked to its phenotype.…”

Section: Introductionmentioning

confidence: 99%

Role of blood hepcidin alteration as a biomarker in β-thalassemia patients’ diagnosis

Al-dulaimy,

Sahab,

Mohammed

et al. 2024

Trends Immunother.

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The objective of this research was to determine the change in hepcidin levels and other biochemical markers, including total iron binding capacity (TIBC), serum iron, testosterone, and specific vitamins in the blood of individuals with β-Thalassemia. Here, 140 participants were involved in the study, of whom 110 were affected by β-thalassemia and 30 were healthy. The samples were obtained from Baqubah Teaching Hospital, the blood bank and blood donation center. The study was carried out from May 2022 to August 2022, and the patients were housed in Diyala Provence, Baquba City and its suburbs. The participants in this investigation were split into three groups: A: 55 male patients with β-thalassemia; B: 55 female patients with β-thalassemia; and C: 30 healthy individuals that were set as a control group. The findings of the study showed that the levels of HbA1 in males and females were 93.44 ± 0.71 and 93.53 ± 0.91, respectively, while the levels of HbA2 in males and females were 4.99 ± 0.59 and 5.29 ± 0.72, respectively. In contrast, the control group had HbA1 levels of 97.33 ± 0.40 in males and 97.66 ± 0.46 in females, but HbA2 levels were 2.32 ± 0.33 in males and 2.24 ± 0.24 in females. The study revealed remarkable differences (P < 0.05) between these variables. Hematological measures, such as hemoglobin concentration and percentages of mean corpuscular volume (MCV), packed cell volume (PCV), and mean corpuscular hemoglobin (MCH) were substantially reduced (P < 0.05) in β-thalassemia patients when compared to the controls. Serum iron and TIBC were significantly increased (P < 0.05), while Hepcidin levels were markedly decreased (P < 0.05) in the serum of β-thalassemia patients compared to controls. Therefore, as a conclusion the reduction of hepcidin levels and increase in iron levels are correlated with β-thalassemia and can be used as a biomarkers in monitoring β-thalassemia disease.

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Understanding Beta-Thalassemia with Focus on the Indian Subcontinent and the Middle East

Cited by 16 publications

References 65 publications

Red Blood Cell Alloimmunization in Pediatric group with Beta Thalassemia: A Five-Year Experience

Red Blood Cell Alloimmunization in Pediatric group with Beta Thalassemia: A Five-Year Experience

Exploring the Effectiveness of Mandatory Premarital Screening and Genetic Counselling Programmes for β-Thalassaemia in the Middle East: A Scoping Review

Role of blood hepcidin alteration as a biomarker in β-thalassemia patients’ diagnosis

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