IJSR International Journal of Scientific Research 2277 - 8179 Indian Society for Health and Advanced Research ijsr-6-6-11217 Original Research Paper GROWTH PROFILE AMONG CHILDREN WITH THALASSEMIA MAJOR ON REGULAR TRANSFUSIONS AND ORAL CHELATION THERAPY G. Ravindranath Dr. Dr. V Sobha Rani Dr. June 2017 6 6 01 02 ABSTRACT

 Background: The inherited haemoglobin disorders are the most common single gene defect in man.The frequency of the carrier state has been estimated to be 270/million with about 400,000 annual births a year. The Thalassemia’s are the most common genetic disorder in a worldwide basis. 3% of world population carries genes of β thalassemia3.In India over 20 million people have thalassemia gene.It has been estimated that over 6000–8000 children, who are homozygotes of ß–thalassemia are born in India every year and unfortunately most of these children die either undiagnosed.Objectives: 1.To assess the growth pattern in Children with Thalassemia major on oral Iron chelation therapy 2.To compare the growth patterns across children with mean pre–transfusion Haemoglobin <8 g/dl and ≥ 8g/dl.Materials and Methods: The study was done in Transfusion dependent thalassemia major children on oral Iron Chelation Therapy between 2–10 yrs of age who are attending ASRAM Medical College, Eluru for regular transfusions. A total of 100 children was studied based on sample size calculated taking prevalence of 10% with 95% CL and absolute precision of 6%. Results: Out of 100 children, 70 were male and 30 were female. Mean age of the study population is 88.75 ± 22.18 months. Majority of the children are born out of 2nddegree consanguity 51% (n=51), followed by Non–consanguineous 39 % (n=39) and 3rddegree Consanguity 10%. Most of the children are on oral Deferiprone 63%(n=63),and 37%(n=37)of the children are on oral Deferasirox. In children with Mean pre transfusion Hb<8g/dl, the average Serum ferritin Concentration is 3501.36 ± 1390.76 ng/ml. In children with Mean pre transfusion Hb ≥8g/dl, the average Serum ferritin concentration is 3455.87 ± 1466.52ng/ml.Conclusion: Thalassemia syndromes are important cause of morbidity and mortality in children. Majority of thalassemic children were born to consanguineous marriage. Most of the children had mean Pre transfusion hemoglobin of less than 8gm% indicating inadequate blood transfusion. Oral chelation was not found to be effective in reducing the iron over load. Although the patients were on regular blood transfusions, they were maintaining low hemoglobin levels which is evident from positive association with growth parameters.