Title |
Hematopoietic stem cell transplantation for people with ß-thalassaemia major
|
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Published by |
John Wiley & Sons, Ltd, October 2014
|
DOI | 10.1002/14651858.cd008708.pub3 |
Pubmed ID | |
Authors |
Jagannath, Vanitha A, Fedorowicz, Zbys, Al Hajeri, Amani, Sharma, Akshay |
Abstract |
Thalassemia is an inherited blood disorder, caused by mutations in regulatory genes and transmitted as an autosomal recessive disorder, which results in a reduced rate of synthesis of one of the globin chains that make up haemoglobin. In ß-thalassaemia major there is an underproduction of ß-globin chains combined with excess of free α-globin chains. The excess free α-globin chains damage the red blood cell membranes, leading to their destruction and a phenomenon termed ineffective erythropoiesis. The conventional approach to treatment is based on the correction of haemoglobin status through regular blood transfusions and iron chelation therapy for iron overload. Although conventional treatment has the capacity to improve the quality of life of people with ß-thalassaemia major, allogeneic hematopoietic stem cell transplantation is the only currently available procedure which has the potential to definitively cure the disease. |
X Demographics
Geographical breakdown
Country | Count | As % |
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United Kingdom | 1 | 50% |
Unknown | 1 | 50% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 2 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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Italy | 1 | 3% |
Unknown | 29 | 97% |
Demographic breakdown
Readers by professional status | Count | As % |
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Student > Master | 5 | 17% |
Student > Bachelor | 4 | 13% |
Student > Ph. D. Student | 3 | 10% |
Researcher | 2 | 7% |
Other | 2 | 7% |
Other | 5 | 17% |
Unknown | 9 | 30% |
Readers by discipline | Count | As % |
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Medicine and Dentistry | 8 | 27% |
Psychology | 3 | 10% |
Nursing and Health Professions | 2 | 7% |
Agricultural and Biological Sciences | 2 | 7% |
Engineering | 2 | 7% |
Other | 4 | 13% |
Unknown | 9 | 30% |