Cognitive Impairment in Children with Beta-Thalassaemia Major at a Tertiary Care Centre, Rajasthan, India: A Cross-sectional Study

Abstract

Introduction: Thalassaemia is one of the most common genetic disorders. Iron overload due to repeated blood transfusions and chelating therapy leads to oxidative stress and possible irreversible brain tissue damage, causing cognitive impairment in these children. Aim: To study cognitive impairment in children with beta thalassaemia major in a tertiary care centre in Bikaner, Rajasthan, India. Materials and Methods: This was a hospital-based cross-sectional observational study conducted in the Department of Paediatrics, Sardar Patel Medical College, Bikaner, Rajasthan, India from September 2019 to August 2020. Sixty-five children with thalassaemia and 65 children without thalassaemia (as controls) were recruited and assessed using modified Mini-Mental State Examination (MMSE) for cognitive impairment. Results: Majority of children belonged to age group 6-11 years (67.7% in cases and 68.8% in controls). The mean MMSE scores were significantly lower among children with thalassaemia (26.82±6.45) compared to the controls (29.08±5.71). There was a significantly higher prevalence of cognitive dysfunction in thalassaemia children (72.3%) as compared to controls (33.8%). Children who were compliant with chelation medications (n=30) were found to have a lesser prevalence of cognitive dysfunction (60%) than those who were not compliant (n=35) with medications (82.86%) (p-value=0.013). Among cases, the mean ferritin levels were higher in children with cognitive dysfunction (3399.00±1489.18) compared to those with normal cognitive function (2412.38±1117.26) (p-value=0.046). There was moderate positive correlation between thalassaemia child’s Body Mass Index (BMI) and modified MMSE score. Conclusion: This study highlights the higher prevalence of cognitive impairment in children with thalassaemia (72.3%). The key contributing factors were- lack of compliance to chelation therapy, higher serum ferritin levels and low BMI.

Keywords

• body mass index
• chelation
• modified mini-mental state examination
• serum ferritin