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Cognitive Function in Children With b-Thalassemia Major

Arun K Kurian1, Monika Sharma2, P V Varughese2

  • 1Department of Pediatrics, Christian Medical College, Ludhiana, Punjab. Correspondence to: Dr Arun K Kurian, Senior Resident, Department of Pediatrics, Christian Medical College, Ludhiana, Punjab. arunkkurian@gmail.com.

Indian Pediatrics
|January 12, 2023
PubMed

Insights

Intelligence Quotient (IQ) in children with transfusion-dependent beta-thalassemia major is generally average. Higher IQ scores correlate with earlier diagnosis and better pre-transfusion hemoglobin levels, emphasizing timely medical intervention.

Area of Science:

  • Pediatric Hematology
  • Neurodevelopmental Pediatrics
  • Medical Psychology

Background:

  • Beta-thalassemia major is a severe inherited blood disorder requiring lifelong blood transfusions.
  • Transfusion-dependent patients face potential complications, including effects on cognitive function.
  • Assessing intelligence quotient (IQ) is crucial for understanding the neurodevelopmental impact of this condition.

Purpose of the Study:

  • To evaluate the intelligence quotient (IQ) in transfusion-dependent beta-thalassemia major patients.
  • To correlate IQ components (verbal, performance, full scale) with serum ferritin and blood transfusion needs.
  • To investigate the relationship between cognitive function and disease management parameters.

Main Methods:

  • A cross-sectional study involving 100 transfusion-dependent beta-thalassemia patients aged 6 to 15 years 11 months.
  • Intelligence Quotient (IQ) assessment was performed using the Malin Intelligence Scale for Indian Children (MISIC).
  • Statistical analysis was employed to correlate IQ scores with serum ferritin levels, annual blood transfusion requirements, and age at diagnosis.

Main Results:

  • The mean full-scale IQ was 95.96 (SD 7.23), with most patients exhibiting average IQ.
  • Significant negative correlations were found between serum ferritin and object assembly (PIQ component), and between annual blood requirements and general comprehension (VIQ component).
  • Positive correlations were observed between Performance IQ (PIQ) and Full Scale IQ (FSIQ) with pre-transfusion hemoglobin levels.

Conclusions:

  • Cognitive function in transfusion-dependent beta-thalassemia major patients is influenced by the age at diagnosis and average annual pre-transfusion hemoglobin levels.
  • Maintaining satisfactory hemoglobin levels and ensuring early diagnosis are critical for optimizing neurodevelopmental outcomes.
  • These findings underscore the importance of comprehensive care, including monitoring cognitive health in these patients.
Abstract

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