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Blood, 1 July 2004, Vol. 104, No. 1, pp. 34-39. Prepublished online as a Blood First Edition Paper on February 26, 2004; DOI 10.1182/blood-2003-09-3167. This Article Full Text (PDF) All Versions of this Article: 2003-09-3167v1 104/1/34    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Cunningham, M. J Articles by Cohen, A. R Search for Related Content PubMed PubMed Citation Articles by Cunningham, M. J Articles by Cohen, A. R Social Bookmarking                   What's this? Submitted September 15, 2003 Accepted January 23, 2004 Complications of -thalassemia major in North America Melody J Cunningham, Eric A Macklin, Ellis J Neufeld*, and Alan R Cohen Division of Hematology, Children's Hospital Boston and Dana Farber Cancer Institute, Boston, MA, USA; Department of Pediatrics, Harvard Medical School, Boston, MA, USA New England Research Institutes, Watertown, MA, USA Children's Hospital of Philadelphia, Philadelphia, PA, USA * Corresponding author; email: ellis.neufeld{at}childrens.harvard.edu. Treatment of patients with beta thalassemia major has improved dramatically during the past 40 years; however, the current clinical status of these patients remains poorly characterized. We performed a cross-sectional study of 342 patients in the Registry of the NIH-sponsored Thalassemia Clinical Research Network. Evidence of hepatitis C exposure was present in 35% of tested patients, was associated with age, and had a rate of spontaneous viral clearance of 33%. Ferritin levels ranged from 147 to 11,010 ng/mL (median 1696 ng/mL). Median hepatic iron content was 7.8 mg/g dry wt and 23% had values 15 mg/g dry wt. No patients 15 years or younger and 5% of patients 16-24 years had heart disease requiring medication. Ten percent had cirrhosis on biopsy. Endocrinologic complications were common among adults. Seventy-four (22%) patients had recent implantable central venous access devices (CVAD). Among 80 episodes of bacteremia in 38 patients, 90% were attributable to CVAD. Among 330 patients who had received deferoxamine chelation therapy, 224 (68%) reported no complications. We conclude that Hepatitis C, iron-related organ dysfunction and complications of iron chelation are strongly age-dependent in North American -thalassemia patients. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: A. Roghi, S. Dellegrottaglie, P. Pedrotti, S. Pedretti, E. Cassinerio, and M. D. Cappellini Unexpected myocarditis in thalassaemia major patient screened for iron load cardiomyopathy BMJ Case Reports, February 16, 2009; 2009(feb04_1): bcr0820080811 - bcr0820080811. [Full Text] P. Harmatz, M. M. Jonas, J. L. Kwiatkowski, E. C. Wright, R. Fischer, E. Vichinsky, P. J. Giardina, E. J. Neufeld, J. Porter, N. Olivieri, et al. Safety and efficacy of pegylated interferon {alpha}-2a and ribavirin for the treatment of hepatitis C in patients with thalassemia Haematologica, August 1, 2008; 93(8): 1247 - 1251. [Abstract] [Full Text] [PDF] V. Di Marco, M. Capra, F. Gagliardotto, Z. Borsellino, D. Cabibi, F. Barbaria, D. Ferraro, L. Cuccia, G. B. Ruffo, F. Bronte, et al. Liver disease in chelated transfusion-dependent thalassemics: the role of iron overload and chronic hepatitis C Haematologica, August 1, 2008; 93(8): 1243 - 1246. [Abstract] [Full Text] [PDF] R. Setiabudy, P. A. Wahidiyat, and L. 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