A Young Child with Sickle Cell Beta Thalassemia: A Case Report and Review of Literatures

Mollah, Md. Abid Hossain; Rahman, Ekhlasur; Islam, Saiful; Morshed, Akm Amirul; Munmun, Farzana; Shohel, Mohammad

doi:10.3329/jdmc.v21i2.15368

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“…2). Among the 8 studies selected outside India in South Asia, there were 7 case reports with 2 case studies each from Pakistan, Sri Lanka, Bangladesh and one from Nepal [22][23][24][25][26][27][28]. No eligible study was identified from Maldives and Bhutan.…”

Section: Resultsmentioning

confidence: 99%

“…Moreover, the usage of HU for infants and children age 9 months or older who are asymptomatic or having infrequent pain episode has not adequately analysed among Indian patients. Therapeutic usage of HU for SCD have also been noted in couple of case reports from Pakistan, Bangladesh, Nepal and one descriptive cross-sectional study from Sri Lanka [21,22,26,28]. However, particulars of different dosing regimens in practice, toxicities and detailed response to HU therapy is largely unknown.…”

Section: Discussionmentioning

confidence: 99%

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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: Resultsmentioning

confidence: 99%

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

View full text Add to dashboard Cite

show abstract

A Young Child with Sickle Cell Beta Thalassemia: A Case Report and Review of Literatures

Abstract: J Dhaka Medical College, Vol. 21, No. 2, October, 2012, Page 245-249 DOI: http://dx.doi.org/10.3329/jdmc.v21i2.15368

Cited by 1 publication

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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

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