Treatment with hydroxycarbamide for intermedia thalassaemia: decrease of efficacy in some patients during long-term follow up.
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Biomedical subjects
Publications and source records attributed to Paolo Rigano.
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Seventeen TM patients with impaired glucose tolerance (IGT) or non-insulin dependent diabetes mellitus (NIDDM) and hyperinsulinism were treated for 12 months with acarbose (100 mg. orally with breakfast, lunch and evening meals). An improvement in glucose tolerance was observed in 2 out of 11 TM patients with IGT and in all TM patients with NIDDM. Acarbose does not appear to directly improve insulin resistance but may have an indirect effect delaying the absorption of glucose of complex carbohydrates and disaccharides. It may be concluded that acarbose may represent a useful first-line therapy for improving glycemic control in TM patients with abnormalities of glucose homeostasis and hyperinsulinism.
The pathogenesis of diabetes in thalassaemia is complex and multifactorial. Understanding the sequence of abnormalities in the progression from normal glucose tolerance to impaired glucose tolerance may help in the formulation of ways to intervene in this process. In our study, we assessed the effects of acarbose, an alpha-glucosidase inhibitor, in five young adult thalassaemic patients with hyperinsulinism and normal oral glucose tolerance test (OGTT). A decrease of fasting insulin levels, insulin peak and area under the curve (AUC) after OGTT, were observed in thalassaemic patients receiving acarbose therapy. These values remained unchanged in an untreated group of eight thalassaemic patients. We believe that acarbose may have a potential role in the treatment of abnormalities of glucose homeostasis and insulin release.
The purpose of this study was to evaluate if the variations of heart magnetic resonance imaging in beta-thalassemia major patients treated with Deferoxamine B mesylate (DF) or Deferiprone (L1) chelation therapy is a useful tool of the indirect myocardial iron content determination. For this reason, a prospective study was carried out. Seventy-two consecutive patients with beta-thalassemia major (35 treated with DF and 37 with L1) were studied. The main outcome results were laboratory parameters including determination of the liver iron concentration (LIC) and magnetic resonance imaging (MRI) of the heart and liver. The heart to muscle signal intensity ratios (HSIRs) were significantly increased in both the DF (t = -2.8; p < 0.01) and L1 (t = -3.1; p < 0.01) groups after one year of treatment No statistically significant difference in the values of HSIRs was present between the two groups at the beginning of treatment (p = 0.25; t = 1.13), and after one year of treatment (p = 0.20; t = 1.28). The HSIR were inversely correlated to the LIC (r = -0.52; p < 0.001) but not with ferritin levels (r = 0.10; p = 0.18). A positive correlation was found between the variation of HSIRs and that of the liver signal intensity ratios (r=0.52; p < 0.001), and a mild correlation (r = 0.40; p < 0.001) was found between the gamma glutamyltransferase (gammaGt) levels and the HSIRs values. Our data confirm that heart MRI is sensitive enough to detect significant variations of the mean HSIR during iron chelation with DF or L1.