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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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Overview on thalassemias: a review article

TL;DR: Clinical features of thalassemia are reviewed, focusing on pathophysiology, clinical features, complication, management, screening and diagnosis, by hematologic tests, hemoglobin electrophoresis, and DNA analysis.
Journal ArticleDOI

Pandora's box: ethics of PGD for inherited risk of late-onset disorders

TL;DR: There is concern that the Human Fertilisation and Embryology Authority has approved extension of PGD to late-onset multifactorial diseases without clear guidelines for its use.
Journal Article

False positive rates of thalassemia screening in rural clinical setting: 10-year experience in Thailand.

TL;DR: The combination of the OF and DCIP test, which has been the strategy for screening of thalassemia and HbE in pregnant women throughout this country, resulted in a large economic burden in terms of high cost and workload associated with further hemoglobin and DNA analyses of false positive samples.
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[Evaluation of hemogram in patients with homozygous sickle cell disease: about 87 cases].

TL;DR: L’objectif est de déterminer le profil des paramètres hématologiques du drépanocytaire homozygote (SS) marocain au cours des stades stationnaires.
Journal ArticleDOI

Treatment of dental and orthodontic complications in thalassaemia

TL;DR: This review highlights the need for conducting and appropriate reporting, of high-quality randomised controlled trials investigating the effectiveness of various treatment modalities for dental and orthodontic complications in people with thalassaemia.
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.
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.