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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 ArticleDOI

Prevalence of Thalassemia and Glucose-6-Phosphate Dehydrogenase Deficiency in Newborns and Adults at the Ramathibodi Hospital, Bangkok, Thailand.

TL;DR: The results indicated that heterozygous Hb E and G6PD Viangchan are predominant both in newborns and adults in this study.
Dissertation

The development of molecular assays for the detection and identification of inherited disorders of haemoglobin in a highly heterogeneous population

Andrew Turner
TL;DR: A number of examples of two very rare haemoglobins, Hb Fontainebleau and Hb Handsworth were encountered during the course of this work, and the diagnostic features of these two rare variants are described.
Journal ArticleDOI

Thalassemia in Malaysia

TL;DR: The Malaysian National Programme for Thalassemia Prevention and Control was launched in 2004 and consisted of mass public education campaigns, public awareness and health education, Malaysian Thalassaemia Registry, population screening, laboratory diagnosis and comprehensive patient management.
Journal ArticleDOI

Modulatory effect of single nucleotide polymorphism in Xmn1, BCL11A and HBS1L-MYB loci on foetal haemoglobin levels in β-thalassemia major and Intermedia patients.

TL;DR: There was found a clear association between foetal haemoglobin level and single nucleotide polymorphisms in HBG2 (rs7482144) and BCL11A (rs766432) genes, and this correlation was additive and was seen both in thalassemia major and thAlassemia intermedia cohorts.
Journal ArticleDOI

Sociodemographic profile and oral health status of thalassemic patients attending the National Thalassaemia Centre, Kurunegala, Sri Lanka.

TL;DR: Families of the thalassemic patients attending the National Thalassaemia Centre are from a low socioeconomic background and their DMFT and periodontal health are significantly better than those of healthy individuals.
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.

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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.