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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Distribution and frequency of β‐thalassemia mutations in northwestern and central Greece
Ioannis Georgiou,Alexandros Makis,Aristidis Chaidos,I. Bouba,E. Hatzi,V. Kranas,C. Zilidis,Bourantas Kl +7 more
TL;DR: The aim of the present study was to identify the distribution of the most common β‐thalassemia mutations in the population of northwestern and central Greece.
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External quality assessment of hemoglobin A2 measurement: data from an Italian pilot study with fresh whole blood samples and commercial HPLC systems.
TL;DR: Quality in the measurement of HbA2 should be improved, and the overall interlaboratory coefficient of variation was 8.0%, 6.0% and 7.9% for samples with low, high and intermediate H bA2 levels, respectively.
Journal ArticleDOI
Avascular necrosis of the femoral head in sickle cell disease patients.
Marcos Almeida Matos Matos,Luanne Lisle dos Santos Silva,Rony Brito Fernandes,Cristiane Dias Malheiros,Bruno Vieira Pinto da Silva +4 more
TL;DR: The attention of physicians treating SCD patients must also be drawn to the possibility of ANFH in order to prevent or avoid this disastrous complication, especially in younger patients presenting with frequent hemolytic crises.
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Solid fuel use is associated with anemia in children
TL;DR: It is found that countries in which the population uses solid fuel the most have over three times higher anemia rates in children than countries with the lowest prevalence of solid fuels use.
Journal ArticleDOI
Anxiety and depression affects life and sleep quality in adults with beta-thalassemia.
Bashir Hajibeigi,Azita Azarkeyvan,Seyed Moayed Alavian,Maryam Moghani Lankarani,Shervin Assari +4 more
TL;DR: Screening for anxiety and depression in patients with thalassemia is essential and further studies should test if appropriate treatment of these conditions may improve patients HRQoL and sleep quality or not.
References
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Sickle cell disease
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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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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.