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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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Frequency of haemoglobinopathies: a single-centre, cross-sectional study from Islamabad, Pakistan.

TL;DR: The frequency of haemoglobinopathies among 504 consecutive cases visiting Islamabad Diagnostic Centre from July 2010 to February 2011 provides support for continuing efforts towards early detection and characterization of thalassaemia trait and major to control the affected births in Pakistan.
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Evaluation of the URIT-2900 automated hematology analyzer for screening of thalassemia and hemoglobinopathies in Southeast Asian populations.

TL;DR: The combined blood cell counting using the URIT-2900 Automated Hematology Analyzer and dichlorophenolindophenol test is suitable for population screening of thalassemia and hemoglobinopathies in Southeast Asia.
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Drépanocytose et fratrie : regard croisé du vécu d’une sœur et d’un frère d’un enfant malade

TL;DR: In this paper, the authors investigate the effect of a maladie genetique on the dynamique of a family in the face of crisis and depenses onereuses concerning ses soins.

A fisiopatologia da anemia falciforme

TL;DR: A anemia falciforme and a doenca hereditaria monogenica mais comum, no Brasil, are prevalecer em individuos descendentes de populacoes do Mediterrâneo, Caribe, America Central e Sul, Arabia e India as mentioned in this paper.
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Primary stroke prevention for sickle cell disease in north-east Italy: the role of ethnic issues in establishing a Transcranial Doppler screening program

TL;DR: The preliminary data set the base to further evaluate the implementation of a primary stroke prevention program in the setting of HbS/HbS African immigrants and HBS/beta thalassemia Italians and long prospective trials are needed to verify their efficacy in defining stroke risk in this setting.
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.
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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.