SynthesisOrphanet journal of rare diseases2021
Hydroxyurea and blood transfusion therapy for Sickle cell disease in South Asia: inconsistent treatment of a neglected disease.
Synthesis in Orphanet journal of rare diseases, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 14 citations in OpenAlex.
- Epidemiology of vaso-occlusive crisis among sickle cell disease patients in India: A community-based multi-centric study.The Indian journal of medical research · 2026Article
- Sickle cell disease in Jharkhand: A registry-based evaluation of disease burden and healthcare needs.The Indian journal of medical research · 2026Review
- Sickle cell health awareness, perspectives and experiences (SHAPE) survey: perspectives on sickle cell disease (SCD) from healthcare providers in India, Malaysia, and Thailand.Orphanet journal of rare diseases · 2025Article
- A retrospective research of adverse event reporting system events for voxelotor based on the FAERS database.BMC pharmacology & toxicology · 2025Article
- Sickle Cell Disease Update: New Treatments and Challenging Nutritional Interventions.Nutrients · 2024Review
- Current challenges and new approaches to implementing optimal management of sickle cell disease in sub-Saharan Africa.Seminars in hematology · 2023Article
- Defining global strategies to improve outcomes in sickle cell disease: a Lancet Haematology Commission.The Lancet. Haematology · 2023Review
- Review
- Treatment Options That Reduce the Duration of Sickle Cell Vaso-Occlusive Crises: A Systematic Review.Cureus · 2022Review
- Sickle cell disease: More than a century of progress. Where do we stand now?The Indian journal of medical research · 2021Article
Corrections and comments
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Authors and funding
3 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHydroxyurea 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.
resultsWe 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.
conclusionsPublished 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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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.