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Secondary diabetes in children with thalassaemia major (homozygous thalassaemia)
E Karahanyan1, A Stoyanova, I Moumdzhiev
1Department of Paediatrics, University of Medicine, Plovdiv, Bulgaria.
Insights
Improved life expectancy in beta-thalassaemia major patients reveals early pancreatic dysfunction. Impaired insulin secretion, not glucose tolerance, signals beta-cell insufficiency in these children.
Area of Science:
- Endocrinology
- Hematology
- Metabolic Disorders
Background:
- Modern treatments have increased life expectancy for homozygous beta-thalassaemia patients.
- Long-term survival can lead to hemosiderosis and pancreatic dysfunction.
- Carbohydrate metabolism and pancreatic beta-cell function require investigation in this population.
Observation:
- Sixteen patients with thalassaemia major were studied for carbohydrate metabolism.
- Three patients exhibited severe diabetes mellitus; others were asymptomatic.
- Pancreatic beta-cell function was assessed via serum insulin and glucose tolerance tests.
Findings:
- Most patients had very low basal insulin levels.
- Reduced glucose tolerance was observed in only one patient.
- This patient also showed a delayed insulin response to glucose.
Implications:
- Disturbed insulin secretion appears to be an early indicator of pancreatic beta-cell insufficiency.
- This dysfunction precedes detectable changes in glucose tolerance.
- Early detection of beta-cell dysfunction is crucial for managing thalassaemia complications.
Abstract:
Life expectancy of patients suffering from homozygous beta-thalassaemia has been improved due to the modern treatment of this disease. This has allowed development of late hemosiderosis-related complications and disturbances of the endocrine and exocrine functions of the pancreas. Carbohydrate metabolism of 16 patients with thalassaemia major was studied. Three of them presented with a pronounced clinical picture and biochemical constellations of a severe diabetes mellitus. The remainder had no clinical symptoms of carbohydrate metabolism disorders. The pancreatic beta-cell function of the patients was assessed by measuring the serum concentrations of immunoreactive insulin and by a glucose tolerance test. Most patients showed very low basal insulin levels while glucose tolerance was reduced in only one of them. In this patient we also established delayed insulin response after an intravenous glucose load. We concluded that the disturbed insulin secretion found in the children studied is most likely the earliest manifestation of the pancreatic beta-cell insufficiency which precedes the changes in the glucose tolerance.