Karahanyan E, Stoyanova A, Moumdzhiev I, Ivanov I
Department of Paediatrics, University of Medicine, Plovdiv, Bulgaria.
Folia Med (Plovdiv). 1994;36(1):29-34.
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.
由于对纯合子β地中海贫血的现代治疗，该疾病患者的预期寿命已得到改善。这使得晚期铁过载相关并发症以及胰腺内分泌和外分泌功能紊乱得以发展。对16例重型地中海贫血患者的碳水化合物代谢进行了研究。其中3例表现出明显的临床症状和严重糖尿病的生化特征。其余患者无碳水化合物代谢紊乱的临床症状。通过测量免疫反应性胰岛素的血清浓度和葡萄糖耐量试验来评估患者的胰腺β细胞功能。大多数患者基础胰岛素水平非常低，而只有其中1例葡萄糖耐量降低。在该患者中，我们还确定了静脉注射葡萄糖负荷后胰岛素反应延迟。我们得出结论，在研究的儿童中发现的胰岛素分泌紊乱很可能是胰腺β细胞功能不全的最早表现，它先于葡萄糖耐量的变化。
