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Browsing Journal/Magazine Articles by Author "Fisher, C."

Browsing Journal/Magazine Articles by Author "Fisher, C."

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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 ...
  • Emerging insights in the management of hemoglobin E beta thalassemia 
    Olivieri, N. F.; Thayalsuthan, V.; O Donnell, A.; Premawardhena, A.; Rigobon, C.; Muraca, G.; Fisher, C.; Weatherall, D. J. (Wiley-Blackwell, 2010)
    Globally, hemoglobin (Hb) E beta thalassemia accounts for approximately half the severe forms of beta thalassemia. Because of its wide clinical diversity and the ability of patients with this condition to adapt unusually ...
  • 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 ...
  • A Functional element necessary for fetal hemoglobin silencing 
    Sankaran, V.G.; Xu, J.; Byron, R.; Greisman, H.A.; Fisher, C.; Weatherall, D.J.; Sabath, D.E.; Groudine, M.; Orkin, S.H.; Premawardhena, A.; Bender, M.A. (Massachusetts Medical Society, 2011)
    BACKGROUND: An improved understanding of the regulation of the fetal hemoglobin genes holds promise for the development of targeted therapeutic approaches for fetal hemoglobin induction in the β-hemoglobinopathies. Although ...
  • 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 ...
  • 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 ...
  • 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 ...
  • 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 ...
  • 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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