Glucose Homeostasis and Effect of Chelation on β Cell Function in Children With β-Thalassemia Major

Area of Science:

• Pediatric Endocrinology
• Hematology
• Metabolic Disorders

Background:

• Beta-thalassemia major is a chronic genetic blood disorder requiring lifelong blood transfusions.
• Chronic transfusions can lead to iron overload, impacting various organs, including the endocrine system.
• Glucose metabolism abnormalities are a known complication in patients with beta-thalassemia major.

Purpose of the Study:

• To determine the prevalence of impaired glucose tolerance in children with beta-thalassemia major.
• To investigate the correlation between glucose metabolism and chelation therapy.
• To evaluate the impact of different iron chelation regimens on glucose homeostasis.

Main Methods:

• Prospective cohort study of 67 children (aged 1-20 years) with beta-thalassemia major.
• Baseline assessment included oral glucose tolerance test, serum insulin, C-peptide, and insulin resistance.
• Biochemical profiles were reassessed after 6 months of follow-up.

Main Results:

• At baseline, 11.9% had impaired fasting glucose, 10.4% had impaired glucose tolerance, and 1.4% had diabetes.
• Abnormal glucose profiles were associated with longer disease duration and higher serum ferritin levels.
• Deferiprone monotherapy showed significant improvement in glucose homeostasis compared to desferrioxamine or combination therapy.

Conclusions:

• Prolonged disease duration and elevated serum ferritin negatively impact glucose homeostasis in pediatric beta-thalassemia major patients.
• Deferiprone emerges as the most effective iron chelator for improving glucose metabolism in this population.
• Early monitoring and management of glucose metabolism are crucial in chronically transfused beta-thalassemia major patients.