Β-Thalassemia MAJOR

Abstract

… Background: Β-thalassemia is a common single genetic disorder in Pakistan with about 8% gene frequency and roughly 10 million carriers. Growth impairment leading to short stature in thalassemic patients is an important cause of morbidity. Objectives: To determine the frequency of short stature in children with muti-transfused β-Thalassemia major. Study Design: Descriptive cross sectional study. Place and Duration of Study: Pediatric Department, Allied Hospital, Faisalabad from December 2015 to May 2016. Patient & Methods:Ninety multi-transfused β-thalassemia major patients diagnosed by hemoglobin electrophoresis between 06 to 10 years of age of either gender were included. Patients with β-thalassemia major with a concomitant chronic illness like congenital heart disease, tuberculosis, celiac disease and immunodeficiency and those with familial short stature as determined by history and mid-parental height were excluded. Results: Out of 90 cases, 56.67% (n=51) were between 6-8 years of age while 43.33% (n=39) between 9-10 years of age, mean + SD was 7.85+1.50 years, 51.11%(n=46) male and 48.89%(n=44) were females. Frequency of short stature in children with β-thalassemia major receiving multiple transfusion was 41.11% (n=37) while 58.89% (n=53) had normal stature. Conclusion: The frequency of short stature is high among β-thalassemic multi-transfused children. It is recommended that every patient with β-Thalassemia major, should be sort out for short stature. However, surveillance of growth and development in these patients is important.