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Open AccessJournal ArticleDOI

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.

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Journal ArticleDOI

Molecular heterogeneity of β‐thalassemia variants in the Eastern region of Morocco

TL;DR: The molecular profile of β‐thalassemia in the Eastern region of Morocco is presented for the first time, and it is shown that each ethnic group has its own mutation spectrum.
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A 10 month old girl with fever and cough

TL;DR: A 10 month old baby girl, hailing from the Cumilla (a city in the Chattogram Division), Bangladesh was brought to the Department of Hematology, Armed Forces Institute of Pathology, Dhaka Cantonment, Dh Bangladesh with the complaints of fever and cough for the last seven days.
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Silent cerebral infarct in sickle cell anemia patients of southern Turkey

TL;DR: The presence of homozygote HbSS/Sβ0 genotype, high MCV, and HbS are risk factors for SCI, and SCI is not uncommon among SCA patients in Turkey.
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Xmn I polymorphism in Egyptian patients with β-thalassemia major and its correlation with the HbF level

TL;DR: The study found that the presence of the XmnI polymorphism influences HbF concentration and ameliorate the clinical severity of the disease.
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Nature and severity of dental malocclusion in children suffering from transfusion-dependent (-thalassemia major

TL;DR: A cross-sectional study was conducted on 200 transfusion-dependent children diagnosed with homozygous β-thalassemia and 200 healthy school children aged 11-17 years.
References
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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

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.
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Effect of Hydroxyurea on the Frequency of Painful Crises in Sickle Cell Anemia

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

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.