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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Recent advances in the management of transfusional hemosiderosis in adolescence
TL;DR: It is expected that the proper use of three chelators will lead to further advances in chelation therapy with respect to compliance, prevention and treatment of iron overload, reduction of complications, and improvement in the survival and quality of life of transfused patients.
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Baseline Hemoglobin, Hepcidin, Ferritin, and Total Body Iron Stores are Equally Strong Diagnostic Predictors of a Hemoglobin Response to 12 Weeks of Daily Iron Supplementation in Cambodian Women.
Lulu X Pei,Hou Kroeun,Suzanne Vercauteren,Susan I. Barr,Timothy J. Green,Arianne Albert,Crystal D Karakochuk +6 more
TL;DR: In this article, the authors investigated the predictive power of ten baseline hematological biomarkers towards a 12-week Hb response to iron supplementation and found that Hb as measured with a hematology analyzer, inflammation-adjusted ferritin, hepcidin, and inflammation adjusted TBIS had the best combined predictive ability (AUCROC=0.86).
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Molecular epidemiological investigation of abnormal hemoglobin in Shaokwan region, southern China
TL;DR: The study of hemoglobin disorders in this special Hakka Chinese population will contribute considerably to the understanding of the historical, emigrational, and genetic relationships among different ethnic group in this region.
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Clinico-haematological study of iron deficiency anaemia and β-thalassemia traitpredictive value of sehgal index and various complete blood count indices to discriminate between iron deficiency anaemia and β-thalassemia trait and confirmation using haemoglobin electrophoresis/hplc
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Cord blood 25-hydroxy Vitamin D and Fluoride in newborn with congenital anomalies
Abhra Ghosh,Jagriti Bhardwaj +1 more
TL;DR: Serum 25-hydroxy Vitamin D levels were significantly decreased in babies with congenital anomalies and show a significant negative association with fluoride, suggesting that association of it with excess fluoride can cause congenital anomaly.
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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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
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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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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.