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Browsing by Author "Arambepola, M."

Browsing by Author "Arambepola, M."

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  • 20 year follow up and survival analysis in a cohort of patients with Haemoglobin E beta Thalassaemia. 
    Olivieri, N.F.; Premawardhena, A.P.; Amir-Arsalan, S.; Ediriweera, D.; Mettananda, S.; Bandara, W.D.; Arambepola, M.; de Silva, S.; Refai, M.A.C.M.; Allen, A. (Sri Lanka Medical Association., 2019)
    INTRODUCTION & OBJECTIVES: Haemoglobin E beta thalassaemia (EBT) is the commonest beta thalassaemia syndrome in the world and is extremely phenotypically variable. Unlike for transfusion dependent thalassaemia (TDT) there ...
  • 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 ...
  • 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 ...
  • The Global distribution of length polymorphisms of the promoters of the gucuronosyltransferase I gene(UGTIAI): hematologic and evolutionary implications 
    Premawardhena, A.P.; Fisher, C.A.; Liu, Y.T.; Verma, I.C.; de Silva, S.; Arambepola, M.; Clegg, J.B.; Weatherall, D.J. (Academic Press, 2003)
    The promoter region of the UDP glucuronosyltransferase 1 gene (UGT1A1) contains a run of thymine-adenine (TA) repeats, usually six (TA)(6). As well as its relationship to Gilbert's syndrome, homozygosity for the extended ...
  • 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 ...
  • Hemoglobin E-beta-thalassemia: Progress report from the international study group 
    Premawardhena, A.; de Silver, S.; Arambepola, M.; Olivieri, N.F.; Vichinsky, E.P.; Merson, L.; Muraco, G.; Allen, A.; Fisher, C.; Peto, T.; Weatherall, D.J. (Blackwell Publishing, 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 ...
  • Interaction of malaria with a common form of severe thalassemia in an Asian population 
    O Donnell, A.; Premawardhena, A.; Arambepola, M.; Samaranayake, R.; Allen, S.J.; Peto, T.E.; Fisher, C.A.; Cook, J.; Corran, P.H.; Olivieri, N.F.; Weatherall, D.J. (National Academy of Sciences, 2009)
    In many Asian populations, the commonest form of severe thalassemia results from the coinheritance of HbE and beta thalassemia. The management of this disease is particularly difficult because of its extreme clinical ...
  • 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 ...
  • A nationwide survey of hospital-based thalassemia patients and standards of care and a preliminary assessment of the national prevention program in Sri Lanka 
    Premawardhena, A.P.; Mudiyanse, R.; de Silva, S.T.; Jiffry, N.; Nelumdeniya, U.; de Silva, U.; Lamabadusuriya, S.P.; Pushpakumara, K.; Dissanayaka, R.; Jansz, M.; Rifaya, I.; Navarathne, U.; Thirukumaran, V.; Arambepola, M.; Bandara, W.D.; Vaidyanatha, U.; Mendis, D.; Weerasekara, K.; de Silva, N**.; Kumara, D.K.S.; Amarasena, S.D.; Hemantha, K. K.; Refai, M.A.C.M.; Silva, I.; Hameed, N.; Rajiyah, F.; Mettananda, S.; Allen, A.; Weatherall, D. J.; Oliveri, N. F. (Public Library of Science, 2019)
    OBJECTIVES:Our aim was to describe the numbers and distribution of patients with different types of thalassemia and to assess the standards of care in all thalassemia treatment centers throughout Sri Lanka and the success ...
  • Non-adherence to chelation therapy and associated psychosocial factors among transfusion-dependent thalassaemia patients in Kandy, Sri Lanka 
    Baminiwatta, A.; Gunathilake, R.; Arambepola, S.; Arambepola, M. (Sri Lanka College of Paediatricians, 2021)
    BACKGROUND: Poor adherence to iron chelation may underlie the detrimental levels of iron overload previously reported among transfusion-dependent thalassaemia (TDT) patients in Sri Lanka. Given the many challenges faced ...
  • Paraspinal extramcduallry erythropoiesis- a rare cause of spinal cord compression: two case reports 
    Arambepola, M.; Premawardhena, A.P.; de Silva, S.; Olivieri, N.; Weatherall, D.J. (Sri Lanka Medical Association, 2004)
    INTRODUCTION: Extrameduallry erythropoiesis (EME) is characterized by the appearance of haemopoietic tissue outside the bone marrow. When EME occurs, albeit rarely, outside the liver and spleen problems may occur. We ...
  • Reappraisal of symptoms and signs of uncomplicated β thalassemia trait 
    Premawardhena, A.; Malewana, T.; Weerasinghe, M.; Arambepola, M. (Sri Lanka Medical Association, 2005)
    INTRODUCTION: Although considered a mild disorder, J3 thalassemia trait has been reportedly associated with symptoms of anaemia, while in other series hepatosplenomegaly has been reported as associated with this diagnosis. ...
  • Response of jaundice to phenobarbitone in thalassaemic patients co-inheriting Gilbert syndrome 
    Premawardhena, A.P.; Arambepola, M.; Fisher, C.A.; Oliveiri, N.F.; Weatherall, D.J. (Sri Lanka Medical Association, 2004)
    INTRODUCTION: Genetic mutations causing Gilbert's syndrome are found in up to 20% of Sri Lankans. The co-existence of Gilbert's syndrome together with haemolytic anaemias can lead to significantly higher level of serum ...
  • Survival and complications in patients with haemoglobin E thalassaemia in Sri Lanka: a prospective, longitudinal cohort study. 
    Premawardhena, A.P.; Ediriweera, D.S.; Sabouhanian, A.; Allen, A.; Rees, D.; de Silva, S.; Perera, W.; Katugaha, N.; Arambepola, M.; Yamashita, R.C.; Mettananda, S.; Jiffry, N.; Mehta, V.; Cader, R.; Bandara, D.; St Pierre, T.; Muraca, G.; Fisher, C.; Kirubarajan, A.; Khan, S.; Allen, S.; Lamabadusuriya, S.P.; Weatherall, D.J.; Olivieri, N.F. (Elsevier Ltd, 2022)
    Background: Worldwide, haemoglobin E β-thalassaemia is the most common genotype of severe β-thalassaemia. The paucity of long-term data for this form of thalassaemia makes evidence-based management challenging. We did a ...
  • Symptoms and signs of β thalassaemia trait: results of the first comparative study 
    Premawardhena, A.P.; Weerasinghe, M.; Kottachchi, D.; Arambepola, M.; Katugaha, N.; Samarakoon, S.; Otivieri, N.F.; Weatherall, D.J. (Sri Lanka Medical Association, 2007)
    OBJECTIVE: Thalassaemia trait is considered a mild and asymptomatic condition. However patients with this disease may be symptomatic. Should these be attributed to the disease? Does pre-knowledge about the diagnosis affect ...
  • Thalassemia in Sri Lanka: a progress report 
    Premawardhena, A.P.; de Silva, S.; Arambepola, M.; Olivieri, N.; Merson, L.; Muraco, J.; Allen, A.; Fisher, C.A.; Peto, T.; Vichinsky, E.; Weatherall, D.J. (Oxford University Press, 2004)
    The thalassemias pose an increasing burden for health-care services in many Asian countries. In order to conserve rare resources, it is essential to determine the reasons for the remarkable phenotypic heterogeneity and ...
  • Using red cell indices as a screening test for the detection of haemoglobin E trait in population screening for haemoglobin disorders 
    Premawardhena, A.; Samarakoon, S.; Perera, U.; Samaranayake, R.; Arambepola, M. (Sri Lanka Medical Association, 2005)
    INTRODUCTION: When screening populations for haemoglobin E trait, which is found in up to 5% in some regions in Sri Lanka, some believe that the full blood count with red cell indices, demonstrating hypochromasia and ...
  • The variable phenotypes of haemoglobin D in Sri Lankan patients 
    Premawardhena, A.; Arambepola, M.; Weatherall, D. (Sri Lanka Medical Association, 2006)
    INTRODUCTION: There are only few reports of the occurrence of the variant haemoglobin, haemoglobin D Punjab (β 121 Glu-Gln) in Sri Lanka and its clinical spectrum has not been well documented. MATERIALS AND METHODS: During ...

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