Abstract
Thalassemias represent the most common single-gene disorder causing a major public health problem in India. Thalassemia and hemoglobinopathies probably developed over 7000 years ago as a defense against malaria. In simple terms, thalassemia is caused by a mutation in either the â-globin chain or the á-globin chain which combine equally in red cells to form hemoglobin. These mutations lead to varying degree of anemia resulting into thalassemia minor, intermedia or major. Present write up relates to advances in the management of â-thalassemia major.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Cunningham MJ, Macklin EA, Newfeld EJ, Cohen AR. Complications of â-thalassaemia major in North America. Blood 2004; 104:34–39.
De SV. Growth and puberty and its management in thalassaemia. Horm Res 2002;58Suppl 1:72–79.
Hui L, Leung MP, Ha SY, Chau AKT, Cheung YF. Early detection of left ventricular dysfunction in patients with beta thal major by dobutamine stress echocardiography. Heart 2003; 89:669–670.
Westwood M, Anderson LJ, Pennell DJ. Treatment of cardiac iron overload in thalassaemia major (Ed). Haematologica 2003;88:481–482.
Jensen PD. Evaluation of iron overload. Br J Haematol 2004;124:697–711.
Fischer R, Longo F, Nielsen P, Engelhardt R, Hider RC, Piga A. Monitoring long-term efficacy of iron chelation therapy by deferiprone and desferrioxamine in patients with beta thalassaemia major: Application of SQUID biomagnetic liver susceptometry. Br J Haematol 2003;121:938–948.
Anderson LJ, Davis HB, Prescott E, Charrier CC, Bunce NH, Firmin DN et al. Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload. Eur Heart J 2001;22:2171–2179.
Jensen PD, Jansen FT, Christensen T, Eiskjaer H, Baandrup U, Nielsen JL. Evaluation of myocardial iron by magnetic resonance imaging during iron chelation therapy with desferrioxamine indication of close relation between myocardial iron content and chelation iron pool. Blood 2003; 101:4632–4639.
Wanless IR, Sweeney G, Dhillon AP, Guido M, Piga A, Galanello R et al. Lack of progressive hepatic fibrosis during long-term therapy with deferiprone in subjects with transfusion-dependent beta-thalassaemia. Blood 2002; 100:1566–1569.
Agarwal MB. ICL 670: A new oral chelator: A major breakthrough in treatment of thalassaemia. Ind J Hematol & Bl Trans 2003; 31: 26–28.
Cheung YF, Chan GCF, Ha SY. Arterial stiffness and endothelial function in patients with beta-thalassaemia major. Circulation 2002;106:2561–2566.
Voskardou E, Terpos E, Spina G, Palermos J, Rahemtulla A, Loutradi A et al. Pamidronate is an effective treatment for osteoporosis in patients with beta thalassaemia. Br J Haematol 2003; 123: 730–737.
Borgna-Pignatti C, Vergine G, Lombardo T et al. Hepatocellular carcinoma in the thal syndromes. Br J Haematol 2004;124:114–117.
Erer B, Lucarelli G. Bone marrow transplantation in thalassaemia. Turk J Hematol 1999;16:147–159.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Agarwal, M. Advances in management of Thalassemia. Indian J Pediatr 76, 177–184 (2009). https://doi.org/10.1007/s12098-009-0048-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12098-009-0048-7