Abstract
β-thalassemia is a hereditary disorder that causes anemia as a result of the decrease in the production of β-globin chains. This disorder is caused by more than 200 point mutations, and more rarely deletions seem to be related with increased long-term morbidity and mortality, affecting the structure and function of many human organs (1). Although the disease has been characterized as a Mediterranean anemia, it seems to spread well beyond its traditional locations (Greece, Southern Italy, and France). Thus, it also appears in India and Africa, and affects certain populations in Asia and South Pacific, as well as black Americans.
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References
Rund D, Rachmilewitz E. β-thalassemia. N Engl J Med 2005;353:1135–46.
Old JM, Olivieri NF, Thein SL. Diagnosis and management of thalassemia. In: Weatherall DJ, Clegg B, eds. The Thalassemia Syndromes, 4th ed. Oxford, England: Blackwell Science 2001:630–85.
Aessopos A, Farmakis D, Karagiorga M, et al. Cardiac involvement in thalassemia intermedia: a multicenter study. Blood 2001;97:3411–16.
Kremastinos D. Heart failure in β-thalassemia. Congestive Heart Fail 2001;7:312–14.
Kremastinos D, Toutouzas P, Vyssoulis G, et al. Global and segmental left ventricular function in β-thalassemia. Cardiology 1985;72:129–39.
Kremastinos D, Tsiapras D, Tsetsos G, et al. Left ventricular diastolic Doppler characteristics in β-thalassemia major. Circulation 1993;88:1127–35.
Kremastinos D, Rentoukas E, Mavrogeni S, et al. Left ventricular filling pattern in β-thalassemia major-a Doppler echocardiographic study. Eur Heart J 1993;14:351–7.
Livrea MA, Tesoriere L, Pintaudi AM, et al. Oxidative stress and antioxidant status in β-thalassemia: iron overload and depletion of lipid-soluble antioxidants. Blood 1996;88:3608–14.
Gutteridge JMC, Halliwell B. Iron toxicity and oxygen radicals. Baillieres Clin Hematol 1989;2:195–256.
Cheung YF, Chan GC, Ha SY, et al. Arterial stiffness and endothelial function in patients with beta-thalassemia major. Circulation 2002;106:2561–66.
Lewis BS, Rachmilewitz EA, Amitai N, Halon DA, Gotsman MS. Left ventricular function in beta-thalassemia and the effect of multiple transfusions. Am Heart J 1978;96(5):636–45.
Iancu TC, Shiloh H. Morphologic observations in iron overload: an update. Adv Exp Med Biol 1994:356:255–65.
Kremastinos D, Tiniakos G, Theodorakis G, et al. Myocarditis in β-thalassemia major. A cause of heart failure. Circulation 1995;91:66–71.
Kremastinos D, Flevari P, Spyropoulou M, et al. Association of heart failure in homozygous beta-thalassemia with the major histocompatibility complex. Circulation 1999;100:2074–78.
Economou-Petersen E, Aessopos A, Kladi A, et al. Apolipoprotein E e4 allele as a genetic risk factor for left ventricular failure in homozygous β-thalassemia. Blood 1998;92:3455–59.
Hahalis G, Manolis A, Apostolopoulos D, et al. Right ventricular cardiomyopathy in beta-thalassemia major. Eur Heart J 2002;23:147–56.
Du ZD, Roguin N, Milgram E, et al. Pulmonary hypertension in patients with thalassemia major. Am Heart J 1997;134:532–7.
Eldor A, Rachmilewitz EA. The hypercoagulable state in thalassemia. Blood 2002;99:36–43.
De Sousa M. Immune cell functions in iron overload. Clin Exp Immunol 1989;75:1–6.
Gheorghiade M, De Luka L, Fonarow GC, et al. Pathophysiologic targets in the early phase of acute heart failure syndromes. Am J Cardiol 2005;96(suppl):11G–17G.
Moratelli S, De Sanctis V, Gemmati D, et al. Thrombotic risk in thalassemic patients. J Pediatr Endorinol Metab 1998;11(suppl 3):915–21.
Jensen CE, Tuck SM, Old J, et al. Incidence of endocrine complications and clinical disease severity related to genotype analysis and iron overload in patients with beta-thalassaemia. Eur J Haematol 1997;59:76–81.
Kremastinos D, Tsetsos GA, Tsiapras D, et al. Heart failure in β-thalassemia: a 5-year follow-up study. Am J Med 2001;111:349–54.
Zurlo MG, De Stefano P, Borgna-Pignatti C, et al. Survival and causes of death in thalassemia major. Lancet 1989;2:27–30.
Engle MA, Erlandson M, Smith CH. Late cardiac complications of chronic, severe, refractory anemia with hemochromatosis. Circulation 1964;30:698–705.
Hahalis G, Alexopoulos D, Kremastinos D, et al. Heart failure in beta-thalassemia syndromes: a decade of progress. Am J Med 2005;118:957–67.
Jonston DL, Rice L, Vick III GK, et al. Assessment of tissue iron overload by nuclear magnetic resonance imaging. Am J Med 1989;87:40–7.
Aessopos A, Farmakis D, Deftereos S, et al. Thalassemia heart disease: a comparative evaluation of thalassemia major and thalassemia intermedia. Chest 2005;127:1523–30.
Nienhuis AW, Hanawa H, Sawai N, et al. Development of gene therapy for hemoglobin disorders. Ann N Y Acad Sci 2003;996:101–11.
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Kremastinos, D.T., Parissis, J.T., Filippatos, G.S. (2008). Acute Heart Failure Syndromes in β-Thalassemia. In: Mebazaa, A., Gheorghiade, M., Zannad, F.M., Parrillo, J.E. (eds) Acute Heart Failure. Springer, London. https://doi.org/10.1007/978-1-84628-782-4_27
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DOI: https://doi.org/10.1007/978-1-84628-782-4_27
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