[Hepatic pathology in beta-thalassemia major]

Area of Science:

• Hepatology
• Hematology
• Pediatrics

Context:

• Beta-thalassemia major is a severe inherited blood disorder requiring lifelong transfusions.
• Chronic blood transfusions lead to iron overload and potential liver damage.
• Elevated liver enzymes (transaminases) indicate hepatic dysfunction in these patients.

Purpose:

• To evaluate liver function in pediatric patients with beta-thalassemia major and persistently high transaminase levels.
• To investigate the correlation between liver enzyme levels, iron overload, and viral hepatitis.
• To assess liver histology and its relationship with clinical and biochemical parameters.

Summary:

• 74 beta-thalassemia major patients (9 months-19 years) with elevated transaminases underwent liver function evaluation.
• Elevated GPT levels (66.33 U/L) were common, correlating with age, ferritin, and transfusional iron.
• 60% had HBV infection; liver biopsies showed siderosis, fibrosis, and chronic hepatitis, particularly in those with abnormal BSP clearance tests.

Impact:

• Highlights the high prevalence of liver dysfunction and pathology in beta-thalassemia major.
• Underscores the role of iron overload and HBV infection in disease progression.
• Informs clinical management strategies for preventing and treating liver complications in these patients.