Inherited haemoglobin disorders: an increasing global health problem.
TLDR
It takes considerable time to establish expertise in developing programmes for the control and management of these conditions, and the lessons learned in developed countries will need to be transmitted to those countries in which they occur at a high frequency.Abstract:
Despite major advances in our understanding of the molecular pathology, pathophysiology, and control and management of the inherited disorders of haemoglobin, thousands of infants and children with these diseases are dying through lack of appropriate medical care. This problem will undoubtedly increase over the next 20 years because, as the result of a reduction in childhood mortality due to infection and malnutrition, more babies with haemoglobin disorders will survive to present for treatment. Although WHO and various voluntary agencies have tried to disseminate information about these diseases, they are rarely mentioned as being sufficiently important to be included in setting health care priorities for the future. It takes considerable time to establish expertise in developing programmes for the control and management of these conditions, and the lessons learned in developed countries will need to be transmitted to those countries in which they occur at a high frequency.read more
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Mutation Spectrum of β-Thalassemia and Other Hemoglobinopathies in Chittagong, Southeast Bangladesh.
TL;DR: The spectrum of β-thalassemia (β-thal) alleles present in Chittagong, Southeast Bangladesh was explored, showing a relatively wide spectrum of alleles, which further demonstrates the heterogeneity of the disease in this country.
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Performance Evaluation of Automated Impedance and Optical Fluorescence Platelet Counts Compared With International Reference Method in Patients With Thalassemia.
TL;DR: Optical platelet counts and careful blood smear examination are recommended alternative platelet counting methods, depending on the clinical setting, and PLT-I was the method least correlated to IRM, with PLt-O andPLT-F comparable toIRM in patients with thalassemia.
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Thalassemia and hemoglobinopathies in an ethnic minority group in Central Vietnam: implications to health burden and relationship between two ethnic minority groups
Nga Thi Nguyen,Kanokwan Sanchaisuriya,Pattara Sanchaisuriya,Hoa Van Nguyen,Hoa Thi Thuy Phan,Goonnapa Fucharoen,Supan Fucharoen +6 more
TL;DR: Gene frequency of certain types of thalassemia among 390 women of reproductive age of the Ta-Oi ethnic minority in Vietnam revealed the same Hb CS haplotype as of the Co-Tu minority, a neighboring minority of theTa- Oi, indicating that these two minorities may share the same ancestors.
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An evaluation of concurrent G6PD (A-) deficiency and sickle cell trait in Malian populations of children with severe or uncomplicated P. falciparum malaria.
Aldiouma Guindo,Karim Traore,Seidina A. S. Diakite,Thomas E. Wellems,Ogobara K. Doumbo,Dapa A. Diallo +5 more
TL;DR: This study found that SCT provides significant protection against severe malaria in female but not male children of the villages of Kangaba and Kela, and tested whether an enhanced protective effect might be detected when the G6PD (A−) polymorphism and SCT were present in combination.
References
More filters
Journal ArticleDOI
Sickle cell disease
TL;DR: New strategies for specific therapy, including expanded use of chronic transfusions, bone marrow transplantation, and hydroxyurea, now offer hope for prevention of many or all of the hemolytic and vaso-occlusive manifestations of sickle cell disease.
World development report 1993 : investing in health
Seth Berkley,Jose Luis Bobadilla,Robert Hecht,Kenneth Hill,Dean T. Jamison,Christopher J L Murray,Philip Musgrove,Helen Saxenian,Jee-Peng Tan +8 more
TL;DR: This report examines the controversial questions surrounding health care and health policy and advocates a threefold approach to health policy for governments in developing countries and in the formerly socialist countries, based in large part on innovative research.
Journal ArticleDOI
Effect of Hydroxyurea on the Frequency of Painful Crises in Sickle Cell Anemia
Samuel Charache,Michael L. Terrin,Richard D. Moore,George J. Dover,Franca B. Barton,Susan V. Eckert,Robert P. McMahon,Duane Bonds +7 more
TL;DR: Hydroxyurea therapy can ameliorate the clinical course of sickle cell anemia in some adults with three or more painful crises per year and Maximal tolerated doses of hydroxyurea may not be necessary to achieve a therapeutic effect.
Journal ArticleDOI
Prevention of a First Stroke by Transfusions in Children with Sickle Cell Anemia and Abnormal Results on Transcranial Doppler Ultrasonography
Robert J. Adams,Virgil McKie,Lewis L. Hsu,B Files,Elliott Vichinsky,Charles H. Pegelow,Miguel R. Abboud,Dianne Gallagher,Abdullah Kutlar,Fenwick T. Nichols,Duane Bonds,Donald Brambilla +11 more
TL;DR: Transfusion greatly reduces the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcranial Doppler ultrasonography.
Journal ArticleDOI
Pathogenesis and Treatment of Sickle Cell Disease
TL;DR: A wealth of information is produced on the mechanisms by which a single base substitution in the gene encoding the human β-globin subunit, with the resulting replacement of β6 glutamic acid by valine, leads to the protean and devastating clinical manifestations of sickle cell disease.