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Global epidemiology of haemoglobin disorders and derived service indicators Bernadette Modell a, Matthew Darlison a Introduction Inherited haemoglobin disorders sickle-cell disorders and thalassaemias were originally characteristic of the tropics and subtropics but are now common worldwide due to migration.
The diversity and heterogeneous distribution of haemoglobin disorders make it necessary to develop strategies at the country level. The work was initiated for WHO 78 and further developed in the United Kingdom, 910 where it is used for local needs-assessment.
Genetic terminology can be impenetrable to non-specialists. Genetic background Haemoglobin comprises four globin chains: Due to spontaneous mutation, haemoglobin gene variants are present at low prevalence carriers 1—1.
The resemblance between thalassaemia and iron deficiency can confuse the diagnosis of either disorder.
However, the life expectancy of patients treated with regular blood transfusion and iron-chelation therapy, or bone-marrow transplantation, is approaching normal.
Some cases have recently been saved by intrauterine transfusion, despite a high risk of severe mental and physical handicap. Most untreated affected children die from infection in early life, 19 but simple steps including neonatal diagnosis, prophylactic antimalarials and antibiotics, access to hospital treatment when needed, and information and support for families greatly improve quality and length of life.
Requirements are the same for thalassaemias and sickle-cell disorders. In most countries, the approach develops in three stages. Second, introduction of prenatal diagnosis for couples with affected children enables them to have a family, but has little further effect on affected birth prevalence.
Third, information and prospective carrier screening is provided for the whole population. Choice of strategy varies with social attitudes, costs and opportunities within the health system. The offer of testing in high school 2324 or before marriage 25 — 28 allows a wide range of choices and requires the least number of laboratory tests.
Methods Acquisition of data The necessary data sets are available for most countries. We gathered demographic data: Data for thalassaemias were obtained from research reviews, 34 country visits, and the former WHO Working Group on Haemoglobin Disorders.
For populations where consanguineous marriage is common, a population coefficient of consanguinity F must be included when calculating the prevalence of affected conceptions from gene frequencies.
The results are aggregated here into conceptions per of: The following five service indicators were obtained for every country by combining prevalences of carriers and affected births with demographic data.
Indicator for patient care N is the annual conceptions with a haemoglobin disorder in the absence of prevention.La storia del libro segue una serie di innovazioni tecnologiche che hanno migliorato la qualità di conservazione del testo e l'accesso alle informazioni, la portabilità e .
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Inherited haemoglobin disorders (sickle-cell disorders and thalassaemias) were originally characteristic of the tropics and subtropics but are now common worldwide due to migration.
1 – 4 Since they can be controlled cost-effectively by programmes that integrate. BibMe Free Bibliography & Citation Maker - MLA, APA, Chicago, Harvard.
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