SHORT STATURE IN PATIENTS WITH ß-THALASSEMIA MAJOR .

Shiva, S and Sarisorkhabi, R (2008) SHORT STATURE IN PATIENTS WITH ß-THALASSEMIA MAJOR . J Urmia Univ Med Sci., 19 (2). pp. 125-131.

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Abstract

S Shiva , MD R Sarisorkhabi , MD Received: 24 Oct, 2007 Accepted: 12 March, 2008 Abstract Background & Aims: Combination of chelating therapy regimens with regular blood transfusion has significantly improved the life expectancy of thalassemic patients. Despite progresses in treatment of these patients, growth failure, and short stature are found in significant number of them. Factors such as iron overload, desferal toxicity, and endocrine disorders play roles in growth failure, but other unknown factors may contribute too. We aimed to study short stature and its contributing factors in our thalassemic patients. Materials & Methods: This descriptive-analytic study was conducted in 2006, on 71 patients with ß-thalassemia major who had regular visits and complete medical recordings. The patients were evaluated for stature, endocrine disorders, serum ferritin, pretransfusion hemoglobin, and liver enzymes. They were compared in two ( short stature and non short stature) groups. Results: 45 boys and 26 girls with mean age of 12.9±5.2 years were studied. 45.1% of patients were short stature and negative linear correlation between advancing of age and height SDS observed. Patients with endocrine anomaly were shorter and those with multiple endocrine anomalies had very severe growth retardation. Cases of hypersplenism were significant in short stature group, but there were no significant differences in pretransfusion hemoglobin and serum ferritin levels between the groups. Although liver enzymes increased in significant number of patients, we didn't find any meaningful differences between two groups. Coclusion: Growth failure of patients with ß-thalassemia major begins from first decades of life and aggravated with age advancement. For prevention of becoming short stature, they need to be evaluated regularly for height velocity, and contributing factors in growth failure should be recognized earlier and treated appropriately.

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