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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Dissertation
Hemoglobinopatias e malária : obstáculos com potencial futuro
TL;DR: Almost 70 years since Haldane's Malaria Hypothesis, there are still roads to uncover in the relationship between haemoglobinopathies and Malaria, and the need for further understanding of this process urges new investigations, which could be the way to develop new therapeutics and new preventive methods.
Journal ArticleDOI
Red Blood Cell Immune Complex Binding Capacity in Children with Sickle Cell Trait (HbAS) Living in P. falciparum Malaria Holoendemic Region of Western Kenya
TL;DR: A study to determine the competence of the red blood cells of children with HbAS to bind immune complexes (ICs) and compared this with normal hemoglobin (HbAA) found it to be less predisposed to getting severe manifestations of malaria.
Dissertation
Investigations on potential digenic HAMP (hepcidin) and HFE haemochromatosis gene mutations in the development of iron overload in Irish patients with dilated cardiomyopathy
TL;DR: The forms, pathophysiology, and genotypic expressions of HFE and hepcidin (HAMP) gene mutations focusing on the possibility of digenic occurrence that could lead to potential development of iron overload cardiomyopathy among Irish patients and their direct family members are described.
Journal ArticleDOI
Quality of life of adolescents living with sickle cell anaemia in Ondo State, Nigeria
TL;DR: Knowledge of adolescents with SCD that participated in the study was relatively low and their quality of life moderate and effort should be geared towards improving their knowledge about SCD and providing supportive care that will improve theirquality of life.
Neonatal screening and clinical care programmes for sickle cell disorders in sub-Saharan African countries: Which lessons from two pilot studies?
Léon Tshilolo,Eleonore Kafando,M. Sawadogo,Frédéric Cotton,F. Vertongen,A. Ferster,B. Gulbis +6 more
TL;DR: The purpose of this review is to evaluate the need for a neonatal screening programme for Sickle cell disease in sub-Saharan African countries and to propose a realistic healthcare programme for sickle cellnewborns in those countries based on personal experiences in Kinshasa and Ouagadougou as well as from a review of the literature.
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
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.
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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.
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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.
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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.