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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 ArticleDOI

Global epidemiology of haemoglobin disorders and derived service indicators

TL;DR: To demonstrate a method for using genetic epidemiological data to assess the needs for equitable and cost-effective services for the treatment and prevention of haemoglobin disorders, online databases, reference resources, and published articles are obtained.
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How malaria has affected the human genome and what human genetics can teach us about malaria.

TL;DR: The challenge for the next decade is to build the global epidemiological infrastructure required for statistically robust genomewide association analysis, as a way of discovering novel mechanisms of protective immunity that can be used in the development of an effective malaria vaccine.
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Iron bioavailability and dietary reference values

TL;DR: It would be timely to develop a range of iron bioavailability factors based not only on diet composition but also on subject characteristics, such as iron status and prevalence of obesity.
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Anaemia in low-income and middle-income countries

TL;DR: Anaemia is disproportionately concentrated in low socioeconomic groups, and that maternal anaemia is strongly associated with child anaemia, and the epidemiology, clinical assessment, pathophysiology, and consequences of anaemia in low-income and middle-income countries are reviewed.
References
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Journal Article

Prevention and control of haemoglobinopathies

TL;DR: This article summarizes the presentations and discussion made at a joint WHO/TIF meeting, held in Cyprus in April 1993, and reviews the experience of programmes in several countries for the control of haemoglobinopathies in the world.
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The Hemoglobin E Syndromes

TL;DR: One family in which HbE interacts with pyrimidine 5′ nucleotidase deficiency to cause severe anemia with Hb instability is identified, which has been well documented in Thailand.
Journal Article

The frequency of genetic disease and congenital malformation among patients in a pediatric hospital.

TL;DR: A sample of 12,801 admissions to a pediatric hospital was surveyed in 1969-70 to determine the prevalence of disease which could be classified as "genetic" in origin or related to "congenital malformation", finding that about one third of all admissions represent the effect of abnormal gene-environment interrelations.
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8 Prenatal diagnosis and screening of the haemoglobinopathies

TL;DR: This paper reviews the most important aspects of carrier detection procedures, genetic counselling, population screening and prenatal diagnosis of the thalassaemias and sickle cell anaemia and indicates that several programmes, based on carrier screening and counselling of couples at marriage, preconception or early pregnancy, are very effective.
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Pathophysiology of sickle cell disease.

TL;DR: Despite genetic identity at the site of the sickle haemoglobin mutation, all patients with sickle cell anaemia are not affected equally by this disease.