ASSESSMENT OF CARDIAC COMPLICATIONS AND MORTALITY RATE OF BETA THALASSEMIA MAJOR IN HAZRAT ALI ASGHAR CHILDREN HOSPITAL IN THE PAST TEN YEARS

Ansari, SH; Vosough, P; Razavi, M.R; Nojoomi, M

Volume 10, Issue 36 (12-2003) RJMS 2003, 10(36): 497-502 | Back to browse issues page

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Ansari S, Vosough P, Razavi M, Nojoomi M. ASSESSMENT OF CARDIAC COMPLICATIONS AND MORTALITY RATE OF BETA THALASSEMIA MAJOR IN HAZRAT ALI ASGHAR CHILDREN HOSPITAL IN THE PAST TEN YEARS. RJMS 2003; 10 (36) :497-502
URL: http://rjms.iums.ac.ir/article-1-192-en.html

ASSESSMENT OF CARDIAC COMPLICATIONS AND MORTALITY RATE OF BETA THALASSEMIA MAJOR IN HAZRAT ALI ASGHAR CHILDREN HOSPITAL IN THE PAST TEN YEARS

SH Ansari

, P Vosough

, M.R Razavi

, M Nojoomi

Abstract: (8985 Views)

Cardiac symptoms and premature death from cardiac causes are still major problem with beta thalassemia despite chelation therapy. Heart complications are the leading causes of mortality in the absence of effective iron chelation therapy. Many patients develop evidence of iron-induced myocardial damage with cardiac failure, cardiac arrhythmia, sudden death or death from progressive congestive failure. The characteristic lesion in the heart is caused by iron deposition in the myofibrils with myofibrillar fragmentation and diminished mitochondrial volume in myocyte. The basic cardiological assessment including 12-lead electrocardiogram, chest X-ray and echocardiogram is done from 7 years of age annually or more if it is needed. This study was undertaken on beta thalassemia patients who were under treatment in Hazrat Ali Asghar Children Hospital in the past ten years. In total of 1069 cases, 16 patients(1.5%) died from severe cardiac impairment(CHF) and 19 patients with severe cardiac problems, who were treated with monitoring cardiac function and intensive chelation therapy, are alive and relatively well. 106(9.9%) cases are asyptomatic with moderate cardiac impairment and 985(92.1%) cases of thalassemia are asymptomatic patients with normal heart condition.

Keywords: Key Words: 1)Beta thalassemia complications 2) Hemochromatosis metabolism

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Type of Study: Research | Subject: Pediatric Cardiology