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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

Prenatal Diagnosis of Hemoglobinopathies in Hacettepe University, Turkey

TL;DR: The results of the study showed that β gene mutations in hemoglobinopathies have a very broad spectrum and the termination of 261 affected fetuses provided both psychological and economic relief for the parents and was economically beneficial for the country in the long term.
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Manifestations of HbSE sickle cell disease: a systematic review.

TL;DR: In this paper, the authors performed a systematic review according to the PRISMA guidelines using PubMed, SCOPUS, and Google Scholar databases, and synthesized data on demographics, manifestations, and management of HBSE disease.
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Impaired Collateral Vessel Formation in Sickle Cell Disease.

TL;DR: Dysfunctional collateral vessel formation in SS mice after vascular injury is suggested and the role of neutrophil depletion or treatment with the antioxidant N-acetylcysteine reduced oxidative stress and improved functional collateral formation in the SS mice.
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The Prevalence and Spectrum of α-Thalassemia in Guizhou Province of South China

TL;DR: The results of this study will be useful in genetic counseling and prenatal diagnosis of α-thal in Guizhou Province.
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Mechanisms of and obstacles to iron cardiomyopathy in thalassemia.

TL;DR: The mechanisms underlying the development of heart disease in thalassemia are reviewed and strategies for therapeutic intervention in TM patients with congestive heart failure are reviewed.
References
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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.