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.read more
Citations
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Molecular Heterogeneity of Thalassemia among Pregnant Laotian Women
Kasama Wongprachum,Kanokwan Sanchaisuriya,Maneelay Dethvongphanh,Boualay Norcharoen,Bousanit Htalongsengchan,Virack Vidamaly,Pattara Sanchaisuriya,Supan Fucharoen,Goonnapa Fucharoen,Frank P. Schelp +9 more
TL;DR: The remarkable genotype-phenotype diversity of thalassemia indicates a need for careful laboratory interpretation in order to provide appropriate genetic counseling and care to the Laotian population.
Journal ArticleDOI
Novel Use of Hydroxyurea in an African Region With Malaria: Protocol for a Randomized Controlled Clinical Trial
Juliana N. Anyanwu,Olatundun Williams,Casey Sautter,Phillip Kasirye,Heather Hume,Robert O. Opoka,Teresa Latham,Christopher M. Ndugwa,Russell E. Ware,Chandy C. John +9 more
TL;DR: This is the first prospective randomized, placebo-controlled clinical trial investigating the use of hydroxyurea for children with SCA in a malaria-endemic region within Africa and has the potential to significantly advance understanding of how to safely and effectively use the medication.
Journal ArticleDOI
A call for policy action in sub-Saharan Africa to rethink diagnostics for pregnancy affected by sickle cell disease: differential views of medical doctors, parents and adult patients predict value conflicts in Cameroon
Ambroise Wonkam,Samia Hurst +1 more
TL;DR: The data underscore the urgency of policy action to place emphasis on: premarital screening, early detection and care of SCD, socio-economic measures to assist SCD-affected families, appropriateness to consider maternal distress due to fetal anomalies in medical abortion legislation.
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Calcium channel blockers for preventing cardiomyopathy due to iron overload in people with transfusion-dependent beta thalassaemia
TL;DR: The available evidence does not clearly suggest that the use of calcium channel blockers is associated with a reduction in myocardial iron in people with transfusion-dependent beta thalassaemia, although a potential for this was seen.
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Negative epistasis between α+ thalassaemia and sickle cell trait can explain interpopulation variation in South Asia.
TL;DR: In this paper, the relationship between α+ thalassaemia and sickle cell in South Asian populations was investigated, and it was shown that very high levels of α+thalaemia combined with varying levels of malaria selection can explain why sickle cells has penetrated certain populations but not others.
References
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Sickle cell disease
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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.
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Prevention of a First Stroke by Transfusions in Children with Sickle Cell Anemia and Abnormal Results on Transcranial Doppler Ultrasonography
Robert J. Adams,Virgil McKie,Lewis L. Hsu,B Files,Elliott Vichinsky,Charles H. Pegelow,Miguel R. Abboud,Dianne Gallagher,Abdullah Kutlar,Fenwick T. Nichols,Duane Bonds,Donald Brambilla +11 more
TL;DR: Transfusion greatly reduces the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcranial Doppler ultrasonography.
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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.