Background: Regular and frequent red blood cell transfusions have significantly increased the life expectancy of patients with β-thalassemia major (β-TM). However, when no appropriate chelation therapy is available, patients accumulate iron in the heart, liver, spleen, pancreas, and endocrine glands, leading to progressive organ dysfunction.
Objective and hypotheses: To assess the correlation between cardiac and hepatic T2*MRI findings with the endocrine and exocrine pancreatic functions in known β-TM patients.
Method: A total of 44 children and adolescents β-TM patients and 44 healthy controls were investigated via: serum amylase, lipase, triglyceride index, oral glucose tolerance test, and T2* MRI to assess iron content in the heart and liver.
Results: Overt diabetes was found in 9.4% and 45.5% of patients had impaired fasting glucose. Median cardiac T2* was 22 ms (1231 ms) and LIC was 6 ms (49 ms). Cardiac T2* was less than 10 ms in 21.4% indicating heavy load with iron in cardiac tissues. There is a significant decrease in serum amylase (87.5 vs 63.5 IU/l, P=0.003) and lipase (94 vs70 IU/l, P=0.056) among enrolled patients in comparison to control group. Thalassemic diabetic showed low serum amylase (32.5 vs 59.5, P=0.0005), serum lipase (39.5 vs 68, P=0.0007), low cardiac T2* was found (7 vs 22 ms, P=0.0006) and low LIC (2 vs 6 ms, P=0.0006) than other β-TM patients without diabetes. Inverse correlation was found between triglyceride index with cardiac T2* (r=−0.376, P=0.014) and low LIC (r=−0.376, P=0.014 respectively) but not with serum lipase (r=−0.099, P=0.533), (r=−0.222, P=0.1570) and serum amylase (r=−0.191, P=0.225), (r=−0.053, P=0.738) respectively.
Conclusion: Follow up of thalassemic patients with impaired fasting glucose together with intensive chelation therapy may help to prevent the development of cardiac and hepatic siderosis.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology