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Browsing Journal/Magazine Articles by Subject "beta-Thalassemia"

Browsing Journal/Magazine Articles by Subject "beta-Thalassemia"

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  • Adaptation to anemia in hemoglobin E-beta thalassemia 
    Allen, A.; Fisher, C.; Premawardhena, A.; Peto, T.; Allen, S.J.; Arambepola, M.; Thayalsuthan, V.; Olivieri, N.; Weatherall, D. (American Society of Hematology, 2010)
    Hemoglobin E beta thalassemia is the commonest form of severe thalassemia in many Asian countries. Its remarkably variable clinical phenotype presents a major challenge to determining its most appropriate management. In ...
  • Age-related changes in adaptation to severe anemia in childhood in developing countries 
    O Donnell, A.; Premawardhena, A.; Arambepola, M.; Allen, S.J.; Peto, T.E.; Fisher, C.A.; Rees, D.C.; Olivieri, N.F.; Weatherall, D.J. (National Academy of Sciences, 2007)
    Severe forms of anemia in children in the developing countries may be characterized by different clinical manifestations at particular stages of development. Whether this reflects developmental changes in adaptation to ...
  • The association between steatosis and liver damage in transfusion-dependent beta thalassaemia patients 
    Padeniya, P.; Ediriweera, D.; de Silva, A.P.; Niriella, M.; Premawardhena, A. (Wiley-Blackwell, 2023)
    Non-alcoholic fatty liver disease (NAFLD) is a global health problem. Iron is the leading cause of liver damage in patients with transfusion-dependent thalassaemia (TDT), and data on the contribution of NAFLD to liver ...
  • A Cost-of-illness analysis of β-Thalassaemia major in children in Sri Lanka - experience from a tertiary level teaching hospital. 
    Reed-Embleton, H.; Arambepola, S.; Dixon, S.; Maldonado, B. N.; Premawardhena, A.; Arambepola, M.; Khan, J. A. M.; Allen, S. (BioMed Central., 2020)
    BACKGROUND: Sri Lanka has a high prevalence of β-thalassaemia major. Clinical management is complex and long-term and includes regular blood transfusion and iron chelation therapy. The economic burden of β-thalassaemia for ...
  • Efficacy and safety of oral hydroxyurea in transfusion-dependent β-thalassaemia: a protocol for randomised double-blind controlled clinical trial 
    Yasara, N.; Wickramarathne, N.; Mettananda, C.; Manamperi, A.; Premawardhena, A.; Mettananda, S. (BMJ Publishing Group Ltd., 2020)
    INTRODUCTION: Despite being one of the first diseases to be genetically characterised, β-thalassaemia remains a disorder without a cure in a majority of patients. Most patients with β-thalassaemia receive only supportive ...
  • Haemoglobin E beta thalassaemia in Sri Lanka 
    Premawardhena, A.; Fisher, C.A.; Olivieri, N.F.; de Silva, S.; Arambepola, M.; Perera, W.; O Donnell, A.; Peto, T.E.; Viprakasit, V.; Merson, L.; Muraca, G.; Weatherall, D.J. (Lancet Publishing Group, 2005)
    Haemoglobin E beta thalassaemia is the commonest form of severe thalassaemia in many Asian countries, but little is known about its natural history, the reasons for clinical diversity, or its management. We studied 109 Sri ...
  • Hemoglobin E-[beta] Thalassemia: Progress Report from the International Study Group 
    Premawardhena, A.; de Silva, S.; Arambepola, M.; Olivieri, N. F.; Vichinsky, E. P.; Merson, L.; Muraco, G.; Allen, A.; Fisher, C.; Peto, T.; Weatherall, D. J. (Wiley-Blackwell, 2005)
    A long-term observational study of Hb E-beta-thalassemia in Sri Lanka is beginning to define some of the genetic and environmental factors that are responsible for its remarkable phenotypic variability. In this population ...
  • Iron overload and iron-chelating therapy in haemoglobin E/beta thalassaemia 
    Olivieri, N.F.; de Silva, S.; Premawardhena, A.P.; Sharma S.; Viens, A.M.; Taylor, C.M.; Brittenham, G.M.; Weatherall, D.J. (Lippincott Williams and Wilkins, 2000)
    Whereas hemoglobin (Hb) E-beta thalassemia is recognized as probably the most common serious hemoglobinopathy worldwide, its natural history remains poorly defined. The interaction of hemoglobin E and beta-thalassemia ...
  • Is the beta thalassaemia trait of clinical importance? 
    Premawardhena, A.; Arambepola, M.; Katugaha, N.; Weatherall, D. J. (Wiley-Blackwell, 2008)
    Although the beta thalassaemia trait affects millions of people worldwide, there have been no controlled studies to determine whether it is associated with any clinical disability or abnormal physical signs. To address ...
  • Marriage patterns in Sri Lanka and the prevalence of parental consanguinity in patients with β-thalassaemia: a cross-sectional descriptive analysis 
    Premawardhena, A.P.; de Silva, S.T.; Goonatilleke, M.D.D.C.; Ediriweera, D.S.; Mettananda, S.; Rodrigo, B.K.R.P.; Allen, A.; Weatherall, D.J. (Cambridge University Press, 2020)
    Consanguineous marriages potentially play an important role in the transmission of β-thalassaemia in many communities. This study aimed to determine the rate and socio-demographic associations of consanguineous marriages ...
  • A Novel molecular basis for beta thalassemia intermedia poses new questions about its pathophysiology 
    Premawardhena, A.; Fisher, C.A.; Olivieri, N.F.; de Silva, S.; Sloane-Stanley, J.; Wood, W.G.; Weatherall, D.J. (American Society of Hematology, 2005)
    During a study of the molecular basis for severe forms of beta thalassemia in Sri Lanka, 2 patients were found to be heterozygous for beta thalassemia mutations. Further analysis revealed that one of them has a previously ...
  • Studies in haemoglobin E beta-thalassaemia 
    Olivieri, N. F.; Muraca, G. M.; O Donnell, A.; Premawardhena, A.; Fisher, C.; Weatherall, D. J. (Wiley-Blackwell, 2008)
    Haemoglobin E beta-thalassaemia is the commonest form of severe thalassaemia in many Asian countries, but little is known about its natural history, the reasons for its clinical diversity, or its optimal management. Despite ...
  • The Thal-index with the BTT prediction.exe to discriminate ß-thalassaemia traits from other microcytic anaemias 
    Nishad, A.A.N.; Pathmeswaran, A.; Wickremasinghe, A.R.; Premawardhena, A. (Pagepress, Italy, 2012)
    Several attempts have been made previously to differentiate -thalassaemia trait (BTT) from other microcytic anaemias using formulae with red cell (RC) parameters. Presently available formulae have low sensitivity and ...
  • Thalassaemia in Sri Lanka: implications for the future health burden of asian populations 
    de Silva, S.; Fisher, C.A.; Premawardhena, A.P.; Lamabadusuriya, S.P.; Peto, T.E.; Perera, G.; Old, J.M.; Clegg, J.B.; Olivieri, N.F.; Weatherall, D.J.; Sri Lanka Thalassaemia Study Group (Lancet Publishing Group, 2000)
    BACKGROUND: Thalassaemias pose an increasing problem for the Indian subcontinent and many Asian countries. We analysed the different types of thalassaemia in the Sri Lankan population, surveyed gene frequencies in ...
  • Transfusion-transmitted Hepatitis C: A cluster of cases in transfusion-dependent Thalassaemia patients in Sri Lanka 
    Perera, S.; Bonsall, D.; Niriella, M.A.; Allen, A.; Peries, A.C.; Nelumdeniya, U.B.; Dissanayake, R.; Silva, I.; de Cesare, M.; Klenerman, P.; Weatherall, D.J.; Roberts, D. J.; Premawardhena, A.P. (Blackwell Scientific Publications,, 2020)
    OBJECTIVES: To report the clinical and virologic epidemiology of a recent epidemic of hepatitis C in thalassaemia patients in Sri Lanka. BACKGROUND: Transfusion-dependent thalassaemia patients remain at risk for hepatitis ...

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