Thalassaemia Prevention: Bangladesh Perspective - A Current Update

Tahura, Sarabon; Selimuzzaman,; Khan, Waqar Ahmed

doi:10.3329/bjch.v40i1.31553

Cited by 18 publications

(19 citation statements)

References 26 publications

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“…About 10-19 million people of this country (6-12% of the population) carry a thalassemia gene (10). According to World Health Organization (WHO) estimates, approximately 3% of the population (3.6 million) carries β-thalassemia and 4% (4.8 million) carries hemoglobin E (HbE) in Bangladesh (5,11,12). It is assumed that over 7000 children are born with thalassemia each year in Bangladesh (13).…”

Section: Introductionmentioning

confidence: 99%

Public Perceptions and Attitudes of Bangladeshi Population towards Thalassemia Prevention: A Nationwide Study

Alam

Islam

Suriea

et al. 2021

Preprint

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Background: Thalassemia is one of the most common life-threatening yet preventable congenital hemoglobin disorders especially in South Asian regions like Bangladesh. It has become a rising public health concern for Bangladesh as 6-12% of the population are carriers and many of them are unaware of it. The purpose of the study is to inspect the knowledge and attitude towards thalassemia among the general people of Bangladesh. Methods: A cross-sectional survey was conducted in eight administrative regions of Bangladesh between January and October of 2020. A self-administrative close-ended questionnaire was designed to collect information about thalassemia and socio-demographic characteristics. Pearson’s chi-square (χ2) test and One-way ANOVA were performed to assess the association between the demographic variables with knowledge and practice regarding thalassemia. A p-value <0.05 was considered significant.Results: Of the 1623 participants, only 44.7% (726/1623) had heard of thalassemia. The mean knowledge of thalassemia was scored 4.75 ± 2.05 out of a total possible score of 10. Half of the participants had no idea that thalassemia was not a transfusion transmitted disease. About 73.1% knew that blood tests are a diagnosis process to determine thalassemia. The urban residing participants had the highest (5.10 ± 1.99) and participants with primary education had the lowest (3.38 ± 1.37) mean score of knowledge. Participants' knowledge score varied significantly by marital status, living pace, literacy and occupation (p< 0.05). However, about 68.2% and 85.5% of the participants showed a positive attitude towards premarital screening of themselves or their family members and donating blood to thalassemia patients, respectively. Conclusion: The study shows that there is a need to disseminate the information on thalassemia since the knowledge gap is huge among people. These findings will assist the implementation of significant steps such as educational programs, health counseling, premarital screening, campaigning, etc. to increase the awareness of thalassemia.

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Section: Introductionmentioning

confidence: 99%

Public Perceptions and Attitudes of Bangladeshi Population towards Thalassemia Prevention: A Nationwide Study

Alam

Islam

Suriea

et al. 2021

Preprint

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“…Bangladesh is a developing country with culturally sensitive people who lack literacy related to the consequences of thalassemia and its potential prevention. 11 However, its prevention can largely be achieved through building awareness before marital engagement. Thalassemia transmission reduction is somewhat unimaginable in Bangladesh because less studies or initiatives are conducted to screen thalassemia or build awareness of its consequences.…”

Section: Introductionmentioning

confidence: 99%

Distribution of β-Thalassemia and Other Hemoglobinopathies in Bangladeshi University Students and Ready-Made Garment Workers

Islam¹,

Hossain²,

Sakib³

et al. 2021

RMHP

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Background The inheritable thalassemia and other structural hemoglobinopathies are prevalent globally. In Bangladesh, there is a scarcity of studies concerning thalassemia and other structural hemoglobinopathies. Hence, the present study investigated the prevalence and distribution of β-thalassemia and other hemoglobinopathies among the university students and ready-made garment (RMG) workers. Methods A cross-sectional study was conducted, whereas a total of 989 subjects were selected randomly from 10 garments industries (n=495) and five universities (n=494) for diagnosis and counseling from April 2018 to August 2018. Basic information related to the study and venous blood was collected and subjected to capillary Hb-electrophoresis for each participant. Results The present study identified thalassemia carriers and other hemoglobinopathies among 13.6% of the participants (14.1% university students and 13.2% RMG workers). The most common form of hemoglobin formation disorder was the Hb-E trait (10.5%), considering both participants’ groups. Other forms of hemoglobin formation disorders observed were a β-thalassemia trait, Hb-D trait, Hb-E disease, and β-thalassemia major. The hemoglobinopathies were significantly associated with the individuals who had a family history of thalassemia and were not vaccinated against hepatitis B in the total sample. Although not statistically suggestive, the most considerable portion (87.3%) of the carriers was unmarried in the present findings. Conclusion The present study findings are anticipated to help the public health authorities by providing novel data (of a neglected cohort, ie, RGM workers), facilitating the upcoming comprehensive national thalassemia prevention programs.

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“…[15][16][17] It is estimated that more than 300,000 infants are born with major haemoglobinopathies worldwide each year of whom 60,000 to 70,000 are β-thalassaemia major cases especially in the Mediterranean area, Middle East, Far East, and East Asia. [18][19][20] Globally every year, severe form of β-thalassaemia accounts for 50,000 to 100,000 deaths in all age group and about 0.5%-0.9% deaths of under-5 children of low or middle income countries. 21 According to Thalassaemia International Federation (TIF), about 23,000 children are born with transfusiondependent β-thalassaemia major each year, while a smaller ill-defined number have the nontransfusion dependent thalassaemia (NTDT), a form of β-thalassaemia intermedia.…”

Section: Introductionmentioning

confidence: 99%

Role of XmnI polymorphism in HbF induction in HbE/β and β-thalassaemia patients

Mannoor

Hossain

Noor

et al. 2019

Bangladesh Med Res Counc Bull

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Background: Thalassaemia is one of the most common genetic blood disorders worldwide. Patients with β-thalassaemia major and HbE/β-thalassaemia are blood transfusion dependent. Foetal haemoglobin or HbF can play a role in disease manifestations in these patients and there is evidence that a homozygous state for XmnI polymorphic site, associated with increased expression of Gγ-gene, may play an important role among other factors in ameliorating the clinical severity of homozygous β-thalassaemia and thalassaemia intermedia. The aim of this review was to provide a comprehensive review of the role of XmnI polymorphic site for increased HbF production in HbE/β and β-thalassaemia patients. Methods: Published literatures were reviewed on the allelic frequency of Xmn1 polymorphism and its effect on HbF induction among thalassaemia patients of different countries. Results: In all β-thalassaemias, Hb F levels are relatively increased due to the selective survival of the erythroid precursors that synthesize relatively more γ-chains. The expression of HbF level is dominated by three different loci: HBG2: γ -158C>T, BCL11A, and HBS1L-MYB intergenic region. Genetic determinants influencing Hb F response can be within the β-globin complex or trans-acting. The published literature showed that the C>T substitution (rs7482144) at position –158 of the Gγ-globin gene, referred to as the XmnI-Gγ polymorphism, is a common sequence variant in all population groups, present at a frequency of 0.32 to 0.35. It was found in some studies, response to Hydroxyurea (HU) has been shown to be largely associated with the presence of the C>T polymorphism at -158 XmnI site (HBG2:c.- 53-158C>T) upstream of the Gγ-globin gene and HU therapy exerts a 2- to 9- fold increase in γ-mRNA expression in β-thalassaemia patients. Conclusion: A number of various study groups around the world suggests that XmnI polymorphism is an important key regulator of disease severity of HbE/β and β-thalassaemia patients.

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Thalassaemia Prevention: Bangladesh Perspective - A Current Update

Abstract: Thalassaemias are a group of autosomal recessive disorder and the most common inherited disease worldwide with a wide geographical variation in incidence. Bangladesh, a

Cited by 18 publications

References 26 publications

Public Perceptions and Attitudes of Bangladeshi Population towards Thalassemia Prevention: A Nationwide Study

Public Perceptions and Attitudes of Bangladeshi Population towards Thalassemia Prevention: A Nationwide Study

Distribution of β-Thalassemia and Other Hemoglobinopathies in Bangladeshi University Students and Ready-Made Garment Workers

Role of XmnI polymorphism in HbF induction in HbE/β and β-thalassaemia patients

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