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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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The Homozygous Hemoglobin EE Variant Is Associated with Poorer Riboflavin Status in Cambodian Women of Reproductive Age

TL;DR: Although riboflavin biomarker deficiency or marginal status is widely prevalent in Cambodian women, lower rib oflavin status was observed more frequently in women with the Hb EE genotype than in womenWith normal Hb AA.
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Fatal pulmonary thromboembolism associated with hemoglobin SC disease in a 15 year-old boy

TL;DR: The autopsy was very instructive, revealing the florid morphologic aspect of sickle cell disease and its complications, and the importance of considering the hypercoagulability in these patients.
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A 65–year–old male with moderate anemia, low-grade fever and weight loss

TL;DR: A 65-year-old male hailing from Lolua, Tangail north bordering district of Dhaka, Bangladesh, reported to the Department of Hematology, Armed Forces Institute of Pathology with the complaints of marked weight loss, generalized weakness, low-grade fever and anorexia.
Journal Article

Public Health Challenges of Hemoglobinopathies in Tribal Land in India: A Necessity of Introducing Genetic Services in the Health Care Systems Approach

TL;DR: This epidemiological review related to public health genetics, highlights the genetic burden/magnitude of hemoglobinopathies having glimpses of reproductive and child health, and neonatal/infant mortality in afflicted couples in the three states of India, namely, Chhattisgarh, Madhya Pradesh, and Odisha.
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Emergencies in Patients With Inherited Hemoglobin Disorders—An Emergency Department Perspective

TL;DR: In a case-based format, the clinical presentation, assessment, and management of the Hb disorders seen in the ED will be discussed to help clinicians practice in an evidence-based fashion.
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.