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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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Journal Article

Camperdown Hemoglobin Associated With β° Thalassemia In A Brazilian Child

TL;DR: It is reported that a nine-month-old Brazilian boy had a relatively more severe hypochromic and microcytic anemia in comparison to his mother’s -thalassemia trait and his Hb Camperdown heterozygous father was clinically and hematologically normal.
Journal ArticleDOI

Microcytosis and Alpha and Beta Thalassaemia in Prospective Blood Donors of East Indian Descent in Trinidad and Tobago

TL;DR: There were a high percentage of thalassaemia carriers in microcytic individuals, thus showing the importance of testing for this disorder, and a larger study is needed to determine the spectrum of α- and β-thalassaemic mutations.
Journal ArticleDOI

The Vital Role Played by Deferiprone in the Transition of Thalassaemia from a Fatal to a Chronic Disease and Challenges in Its Repurposing for Use in Non-Iron-Loaded Diseases

- 18 Jul 2023 - 
TL;DR: The iron chelating orphan drug deferiprone (L1) has been used daily by patients across the world at high doses (75-100 mg/kg) for more than 30 years with no serious toxicity as discussed by the authors .

Validity of Naked Eye Single Tube Red Cell Osmotic Fragility Test in Screening of ß-Thalassemia Trait

TL;DR: In this paper, a descriptive analytic type of study was carried out in the Department of Pediatrics of Sylhet MAG Osmani Medical College Hospital, Sylhet, from July 2006 to June 2008, where a total of 70 samples, 35 cases and 35 suitably matched controls were enrolled.

Symptomatic Complications and Quality of Life in Pakistani Patients with Iron-Deficiency Anemia: Quality of life in Iron-Deficiency Anemia Patients

TL;DR: In this article, a quantitative comparative analysis through cross-sectional research design was used to collect the data and found that Pakistani females with iron deficiency anemia report poorer quality of life, tend to maintain more health-related complications and experience greater symptoms severity than their male counterparts.
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.
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.