We systematically explored the link of pancreatic iron with glucose metabolism and with cardiac complications in a cohort of 1,079 patients with thalassemia major (TM) enrolled in the Extension-Myocardial Iron Overload in Thalassemia (E-MIOT) project.
RESEARCH DESIGN AND METHODSMRI was used to quantify iron overload (T2* technique) and cardiac function (cine images) and to detect macroscopic myocardial fibrosis (late gadolinium enhancement technique). Glucose metabolism was assessed by the oral glucose tolerance test (OGTT).
RESULTSPatients with normal glucose metabolism showed significantly higher global pancreas T2* values than patients with impaired fasting glucose, impaired glucose tolerance, and diabetes. A pancreas T2* <13.07 ms predicted an abnormal OGTT. A normal pancreas T2* value showed a 100% negative predictive value for disturbances of glucose metabolism and for cardiac iron. Patients with myocardial fibrosis showed significantly lower pancreas T2* values. Patients with cardiac complications had significantly lower pancreas T2* values. No patient with arrhythmias/heart failure had a normal global pancreas T2*.
CONCLUSIONSPancreatic iron is a powerful predictor not only for glucose metabolism but also for cardiac iron and complications, supporting the close link between pancreatic iron and heart disease and the need to intensify iron chelation therapy to prevent both alterations of glucose metabolism and cardiac iron accumulation.Thalassemia major (TM) is a hereditary anemia characterized by ineffective erythropoiesis and hemolysis, requiring regular red blood cell transfusions to sustain life. A major drawback of this treatment is iron overload, which can cause organ dysfunction (1). The introduction of chelation therapy has improved the survival of TM patients, but cardiac complications remain the main cause of mortality, while endocrinopathies are the most frequent morbidities. Diabetes is the third-most-common endocrine complication (2). In a large cohort of well-treated and well-chelated TM patients, the
Implementation of a multidisciplinary thoracic malignancy conference increased the 1-year survival rate of patients who underwent a surgical resection for NSCLC.
Summary
We prospectively assessed the efficacy of deferasirox versus deferiprone or desferrioxamine as monotherapy in thalassaemia major (TM) patients by magnetic resonance imaging (MRI). We selected the patients enrolled in the Myocardial Iron Overload in Thalassaemia network who received only one chelator between two MRIs (deferasirox = 235, deferiprone = 142, desferrioxamine = 162). Iron overload was measured by T2* technique and biventricular function by cine images. Among the patients with baseline myocardial iron, in all three groups there was a significant improvement in global heart T2* values. The deferiprone and desferrioxamine groups showed a significant improvement in left ventricular ejection fraction (LVEF). Only the deferiprone group showed a significant improvement in right ventricular ejection fraction (RVEF). The improvement in global heart T2* was significantly lower in the deferasirox versus the deferiprone group. The improvement in the LVEF was significantly higher in the deferiprone and desferrioxamine groups than in the deferasirox group and the improvement in the RVEF was significantly higher in the deferiprone than in deferasirox group. Among the patients with baseline hepatic iron, the changes in hepatic iron were comparable in deferasirox versus the other groups. Deferasirox monotherapy was less effective than deferiprone in improving myocardial siderosis and biventricular function and less effective than desferrioxamine in improving the LVEF.
ST-segment elevation MI (STEMI) is a rare presentation in patients with coronary artery anomalies. In these patients, the identification of the culprit lesion and its treatment may be difficult, particularly in the emergency setting of primary percutaneous coronary intervention (PCI). From January 2008 to April 2011, 1015 STEMI patients received coronary artery angiography and primary PCI in our centre. Of these, 5 (0.4%) patients showed a coronary artery anomaly. In this paper we reported two rare cases: i) the first is a single coronary artery originating from right sinus of Valsalva; ii) the second is a separate origin of 3 coronary arteries originating from the right sinus of Valsalva. In conclusion, coronary artery anomalies presenting with STEMI are really uncommon, but often are a challenge. The integration between traditional coronary artery angiography and multidetector computerized tomography is crucial to optimize the interventional and medical management of these patients.
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