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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 Article

A web-based educational intervention module to improve knowledge and attitudes towards thalassaemia prevention in Malaysian young adults.

TL;DR: An interactive web-based educational module in the Malay language wherein videos were combined with text and pictorial visual cues appears effective in improving knowledge and attitudes towards thalassaemia prevention and its incorporation in thalassedemia prevention programs is potentially useful in Malaysia and countries with a high internet penetration rate.
Journal ArticleDOI

Genetics background of β-thalassemia (3.5 kb deletion) in Southern Thailand: Haplotype analysis using novel reverse dot blot hybridization.

TL;DR: In this paper, a study aimed to determine genetic diversity in subjects with β-thal (3.5 kb deletion) alleles and to ascertain the origin of this mutation using haplotype and phylogenetic analysis.
Journal ArticleDOI

Molecular analysis of alpha- and beta-thalassemia in Meizhou region and comparison of gene mutation spectrum with different regions of southern China.

TL;DR: In this article, the molecular characterization of α- and β-thalassemia in Meizhou city of China was studied, where a total of 22,401 individuals were collected; hematological and hemoglobin electrophoresis analysis and thalassemia genetic testing were performed.
Journal ArticleDOI

Role of red cell distribution width in screening for Hb E trait in population screening for hemoglobin disorders

TL;DR: Red cell distribution width (RDW) cutoff values would help in increasing the sensitivity of screening for Hb E trait, and the importance of taking RDW into consideration for screening is highlighted.
Dissertation

Screening for anaemia and haemoglobinopathy before and during pregnancy. A question of ethnicity

S.M.P.J. Jans
TL;DR: Pregnant women of non-Northern European descent are a specific group in terms of anaemia and are at high risk of a low haemoglobin level for all cut-off values; they have a higher chance of becoming anaemic in pregnancy than women of Northern European descent.
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.
Journal ArticleDOI

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.