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Browsing Journal/Magazine Articles by Author "Allen, A."

Browsing Journal/Magazine Articles by Author "Allen, A."

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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 ...
  • Alpha thalassaemia and extended alpha globin genes in Sri Lanka 
    Suresh, S.; Fisher, C.; Ayyub, H.; Premawardhena, A.; Allen, A.; Perera, A.; Bandara, D.; Olivieri, N.; Weatherall, D. (Elsevier-Academic Press, 2013)
    The α-globin genes were studied in nine families with unexplained hypochromic anaemia and in 167 patients with HbE β thalassaemia in Sri Lanka. As well as the common deletion forms of α(+) thalassaemia three families from ...
  • The Evolutionary and clinical implications of the uneven distribution of the frequency of the inherited haemoglobin variants over short geographical distances 
    Premawardhena, A.; Allen, A.; Piel, F.; Fisher, C.; Perera, L.; Rodrigo, R.; Goonathilaka, G.; Ramees, L.; Peto, T.; Olivieri, N.; Weatherall, D. (Wiley-Blackwell, 2017)
    Studies of the frequency of heterozygous carriers for common inherited diseases of haemoglobin in over 7500 adolescent children in 25 districts in Sri Lanka have disclosed a highly significant variation over very short ...
  • Genotype-phenotype association analysis identifies the role of α globin genes in modulating disease severity of β thalassaemia intermedia in Sri Lanka 
    Perera, S.; Allen, A.; Silva, I.; Hapugoda, M.; Wickramarathne, M.N.; Wijesiriwardena, I.; Allen, S.; Rees, D.; Efremov, D.G.; Fisher, C.A.; Weatherall, D.J.; Premawardhena, A. (Nature Publishing Group, 2019)
    β thalassaemia intermedia (βTI) are a heterogeneous group of disorders known to be extremely phenotypically diverse. This group is more complex to manage as no definitive treatment guidelines exist unlike for β thalassaemia ...
  • Haemoglobin variants, iron status and anaemia in Sri Lankan adolescents with low red cell indices: A cross sectional survey 
    Rodrigo, R.; Allen, A.; Manamperi, A.; Perera, L.; Fisher, C.A.; Allen, S.; Weatherall, D.J.; Premawardhena, A. (Academic Press, 2018)
    Iron deficiency complicates the use of red cell indices to screen for carriers of haemoglobin variants in many populations. In a cross sectional survey of 7526 secondary school students from 25 districts of Sri Lanka, 1963 ...
  • 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 ...
  • Hepcidin detects iron deficiency in Sri Lankan adolescents with a high burden of hemoglobinopathy: A diagnostic test accuracy study 
    Wray, K.; Allen, A.; Evans, E.; Fisher, C.; Premawardhena, A.; Perera, L.; Rodrigo, R.; Goonathilaka, G.; Ramees, L.; Webster, C.; Armitage, A.E.; Prentice, A.M.; Weatherall, D.J.; Drakesmith, H.; Pasricha, S.R. (Wiley-Blackwell, 2017)
    Anemia affects over 800 million women and children globally. Measurement of hepcidin as an index of iron status shows promise, but its diagnostic performance where hemoglobinopathies are prevalent is unclear. We evaluated ...
  • Hepcidin is suppressed by erythropoiesis in hemoglobin E β-thalassemia and β-thalassemia trait 
    Jones, E.; Pasricha, S.R.; Allen, A.; Evans, P.; Fisher, C.A.; Wray, K.; Premawardhena, A.; Bandara, D.; Perera, A.; Webster, C.; Sturges, P.; Olivieri, N.F.; St Pierre, T.; Armitage, A.E.; Porter, J.B.; Weatherall, D.J.; Drakesmith, H. (American Society of Hematology, 2015)
    Hemoglobin E (HbE) β-thalassemia is the most common severe thalassemia syndrome across Asia, and millions of people are carriers. Clinical heterogeneity in HbE β-thalassemia is incompletely explained by genotype, and the ...
  • Iron status and anaemia in Sri Lankan secondary school children: A cross-sectional survey 
    Allen, A.; Allen, S.; Rodrigo, R.; Perera, L.; Shao, W.; Li, C.; Wang, D.; Oliviery, N.; Weatherall, D.J.; Premawardhena, A.P. (Public Library of Science, 2017)
    BACKGROUND: Iron deficiency, the most common micronutrient disorder and cause of anaemia globally, impairs growth, cognition, behaviour and resistance to infection. METHODS/RESULTS: As part of a national survey of inherited ...
  • Leg ulcers: A report in patients with hemoglobin E beta thalassemia and review of the literature in severe beta Thalassemia 
    Mehta, V.; Kirubarajan, A.; Sabouhanian, A.; Jayawardena, S.M.; Chandrakumaran, P.; Thangavelu, N.; Cader, R.; Mettananda, S.; Bandara, D.; Khan, S.; Weatherall, D.J.; Allen, A.; Premawardhena, A.P.; Olivieri, N.F. (Basel, Karger., 2022)
    BACKGROUND: Leg ulcers are a frequent complication in patients with the inherited hemoglobin disorders. In thalassemia, the literature is limited, and factors associated with the development of leg ulcers in HbE beta ...
  • 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 ...
  • Methemoglobinemia and ascorbate deficiency in hemoglobin E β thalassemia: metabolic and clinical implications. 
    Allen, A.; Fisher, C.; Premawardhena, A.; Bandara, D.; Perera, A.; Allen, S.; St Pierre, T.; Olivieri, N.; Weatherall, D. (American Society of Hematology., 2012)
    ABSTRACT: During investigations of the phenotypic diversity of hemoglobin (Hb) E β thalassemia, a patient was encountered with persistently high levels of methemoglobin associated with a left-shift in the oxygen dissociation ...
  • 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 ...
  • A "One-Stop" screening protocol for hemoglobinopathy traits and iron deficiency in Sri Lanka 
    Allen, A.; Perera, S.; Perera, L.; Rodrigo, R.; Mettananda, S.; Matope, A.; Silva, I.; Hameed, N.; Fisher, C. A.; Olivieri, N.; Weatherall, D. J.; Allen, S.; Premawardhena, A. (Lausanne : Frontiers Media S.A., 2019)
    INTRODUCTION: The high frequencies of carriers of severe haemoglobinopathies and of iron deficiency in Southeast Asia require reliable and affordable tests to improve on current screening procedures. OBJECTIVES: We evaluate ...
  • Oxidative status in the β-thalassemia syndromes in Sri Lanka; a cross-sectional survey 
    Allen, A.; Perera, S.; Mettananda, S.; Rodrigo, R.; Perera, L.; Darshana, T.; Moggach, F.; Crawford, A.J.; Heirene, L.; Fisher, C.; Olivieri, N.; Rees, D.; Premawardhena, A.; Allen, S. (Elsevier Science-Pergamon Press, 2021)
    ABSTRACT: In the β-thalassemias, oxidative stress, resulting from chronic hemolysis, globin chain imbalance, iron overload and depleted antioxidant defences, likely contributes to cell death, organ damage, anemia, hypoxia ...
  • The p.H63D allele of the HFE gene protects against low iron stores in Sri Lanka. 
    Allen, A.; Premawardhena, A.; Allen, S.; Rodrigo, R.; Manamperi, A.; Perera, L.; Wray, K.; Armitage, A.; Fisher, C.; Drakesmith, A.; Robson, K.; Weatherall, D. (Academic Press, 2019)
    In hereditary hemochromatosis, iron overload is associated with homozygosity for the p.C282Y mutation. A second mutation, p.H63D, occurs at significant frequencies in Europe, North Africa, the Middle East and Asia. Early ...
  • Pitfalls in the diagnosis of β-Thalassemia Intermedia 
    Perera, S.; Allen, A.; Rees, DC.; Premawardhena, A. (Informa Healthcare, 2021)
    We present case histories of three patients who had β-thalassemia (β-thal) trait with 'unusual severity' managed as β-thal intermedia (β-TI) where the basis of disease severity could not be explained with routine hematological ...
  • Sickle cell disease in Sri Lanka: clinical and molecular basis and the unanswered questions about disease severity 
    Darshana, T.; Bandara, D.; Nawarathne, U.; de Silva, U.; Costa, Y.; Pushpakumara, K.; Pathirage, S.; Basnayake, S.; Epa, C.; Dilrukshi, P.; Wijayawardena, M.; Anthony, A. A.; Rodrigo, R.; Manamperi, A.; Smith, F.; Allen, A.; Menzel, S.; Rees, D.; Premawardhena, A. (BioMed Central., 2020)
    BACKGROUND: Though case reports and limited case series of Sickle cell disease in Sri Lanka have been reported previously, no attempt has been made hitherto to undertake a comprehensive genotypic-phenotypic analysis of ...
  • 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 ...

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