Spectrum of Types of Thalassemias and Hemoglobinopathies: Study in a Tertiary Level Children Hospital in Bangladesh

Khan, Waqar Ahmed; Banu, Bilquis; Sadiya, Salma; Sarwardi, Golam

doi:10.4081/thal.2017.6354

Cited by 4 publications

(9 citation statements)

References 15 publications

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“…One reason for this paucity of data could be the low prevalence of sickle haemoglobin in some countries in South Asia. For instance, studies from Sri Lanka and Bangladesh have shown that the prevalence of sickle haemoglobin was relatively lower than that of other haemoglobinopathies in these regions [63][64][65]. Sickle haemoglobin has been reported at comparatively higher prevalence from the Tharu community of Western Nepal and Pakistan [66,67].…”

Section: Discussionmentioning

confidence: 99%

Hydroxyurea and blood transfusion therapy for Sickle cell disease in South Asia: inconsistent treatment of a neglected disease

Darshana

Rees

Premawardhena

2021

Orphanet J Rare Dis

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Background Hydroxyurea and blood transfusion therapies remain the main therapeutic strategies for Sickle cell disease. Preliminary data suggest substantial variation and inconsistencies in practice of these two therapeutic modalities in South Asia. In this systematic review we searched Medline, Cochrane library and Scopus for articles on usage of hydroxyurea and blood transfusion therapies for sickle cell disease in South Asia published in English between October 2005 and October 2020. Results We selected 41 papers: 33 from India, 3 from Sri Lanka, 2 each from Pakistan and Bangladesh and one from Nepal. Only 14 prospective trials focused on hydroxyurea therapy from which majority (n = 10; 71.4%) adopted fixed low dose (10 mg/kg/day) regimen. With hydroxyurea therapy, 12 and 9 studies reported significant reductions in vaso-occlusive crises and transfusion requirement respectively. Severe anaemia (haemoglobin level < 6–7 g/dl) was the commonest indicator (n = 8) for transfusion therapy followed by vaso-occlusive crisis. Conclusions Published data on the hydroxyurea and transfusion therapies in South Asia are limited and heterogeneous. A clear gap of knowledge exists about the nature of the sickle cell disease in the Indian subcontinent particularly from countries outside India necessitating further evidence-based assessments and interventions.

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Section: Discussionmentioning

confidence: 99%

Hydroxyurea and blood transfusion therapy for Sickle cell disease in South Asia: inconsistent treatment of a neglected disease

Darshana

Rees

Premawardhena

2021

Orphanet J Rare Dis

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“…1a, the first-generation dendrimer (G1-Cys) was synthesized stepwise from b-CD via the Williams and Sharpless reactions, referring to our previous study. 35 G1-Cys was characterized by the 1 H NMR spectrum, which could be ascribed to the terminal allyl groups, glycerol ether, and D-galactose of b-CD moieties in the dendrimers (Fig. 1b).…”

Section: Characterizationmentioning

confidence: 99%

“…The first-generation dendrimer (G1-Cys) was synthesized via the Williams and Sharpless reactions as in our previous research. 35 Briefly, NaH and dried N,N-dimethylformamide (DMF) were mixed. b-Cyclodextrin (b-CD) in DMF solution was then added dropwise.…”

Section: Synthesismentioning

confidence: 99%

Novel manganese and polyester dendrimer-based theranostic nanoparticles for MRI and breast cancer therapy

Zhou

et al. 2023

J. Mater. Chem. B

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“…Thalassemias and hemoglobinopathies are the most common inheritant hemolytic congenital disorders in Bangladesh. 1 Haemoglobinopathies denote genetic defects collectively affecting either haem structure or synthesis. 1 They are divided into two categories.…”

Section: Introductionmentioning

confidence: 99%

Pattern of Thalassemia and other Hemoglobinopathies in Sylhet, Bangladesh

Chowdhury MZR,

Benzamin M,

Chowdhury T

et al. 2024

JSWMC

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Background: Thalassemias and hemoglobinopathies are the most common inheritant hemolytic congenital disorders in Bangladesh.There is no well precise validated data available about the prevalence of Thalassaemia and related hemoglobin disorders in Bangladesh. Method: This retrospective, cross-sectional observational study was carried out in Dr.Benzamin’s Pediatric Liver Research Centre and Nutrition Clinic, Sylhet, Bangladesh. We reviewed the data record software of the hematology section of the Popular Diagnostic Centre and Mount Adora Hospital, Sylhet, from March, 2022 to July, 2023 and collected all the Hemoglobin electrophoresis reports.A total of 783patients data were evaluated, and the data were entered into Microsoft Excel and analyzed by Statistical Package for Social Sciences (SPSS) software version 22. The aim of the study was to identify the pattern of Thalassemia and other Hemoglobinopathies in Sylhet, Bangladesh. Result: Out of 783 patients, 291(37.2%) were male, 492 (62.8%) female, pediatric population that is under 18 years 387 (44.4%) and 18 years or more 396(50.6%). Among these, 262 (33.46%) reports showed Hemoglobinopathies. Before 6 months, 10 patients were advised for Hb electrophoresis. Overall, in study population, most common hemoglobinopathies were Beta thalassemia trait (118, 15.1%), followed by Hb E trait (45, 11.6%), Hb E Beta thalassemia (34, 4.43%), Beta thalassemia major (8, 0.9%). Same trend followed in pediatric age group, Beta thalassemia trait (53, 13.7%), Hb E tarit (84, 10.7%), Hb E Beta thalassemia (28,7.24%), Beta thalassemia major (8, 1.8 %). In adult age group, Beta thalassemia trait (65, 16.4%), Hb E tarit (39, 9.8%), Hb E Beta thalassemia (6, 1.5%%). Conclusion: Hemoglobinopathies among the anaemic population of Sylhet is very common. Beta thalassaemia trait, Hb E tarit, Hb E Beta thalassemia, Beta thalassemia major are common variants. So, during anaemia evaluation, hemoglobinopathies should be kept in mind

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Spectrum of Types of Thalassemias and Hemoglobinopathies: Study in a Tertiary Level Children Hospital in Bangladesh

Cited by 4 publications

References 15 publications

Hydroxyurea and blood transfusion therapy for Sickle cell disease in South Asia: inconsistent treatment of a neglected disease

Hydroxyurea and blood transfusion therapy for Sickle cell disease in South Asia: inconsistent treatment of a neglected disease

Novel manganese and polyester dendrimer-based theranostic nanoparticles for MRI and breast cancer therapy

Pattern of Thalassemia and other Hemoglobinopathies in Sylhet, Bangladesh

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