scholarly journals mRNA regulation of cardiac iron transporters and ferritin subunits in a mouse model of iron overload

2014 ◽  
Vol 42 (12) ◽  
pp. 1059-1067 ◽  
Author(s):  
Casey J. Brewer ◽  
Ruth I. Wood ◽  
John C. Wood
2014 ◽  
Vol 163 (2) ◽  
pp. 151-159 ◽  
Author(s):  
Casey Brewer ◽  
Maya Otto-Duessel ◽  
Ruth I. Wood ◽  
John C. Wood

2013 ◽  
Vol 386 (7) ◽  
pp. 645-650 ◽  
Author(s):  
Hong-li Shan ◽  
Yan Wang ◽  
Jian-wei Wu ◽  
Peng-zhou Hang ◽  
Xin Li ◽  
...  

2009 ◽  
Vol 33 ◽  
pp. S109
Author(s):  
L. Pascal ◽  
C. Rose ◽  
P. Fenaux ◽  
O. Ernst ◽  
H. Chiavassa ◽  
...  

2021 ◽  
Vol 10 (23) ◽  
pp. 5561
Author(s):  
Antonella Meloni ◽  
Laura Pistoia ◽  
Maria Rita Gamberini ◽  
Paolo Ricchi ◽  
Valerio Cecinati ◽  
...  

In thalassemia major, pancreatic iron was demonstrated as a powerful predictor not only for the alterations of glucose metabolism but also for cardiac iron, fibrosis, and complications, supporting a profound link between pancreatic iron and heart disease. We determined for the first time the prevalence of pancreatic iron overload (IO) in thalassemia intermedia (TI) and systematically explored the link between pancreas T2* values and glucose metabolism and cardiac outcomes. We considered 221 beta-TI patients (53.2% females, 42.95 ± 13.74 years) consecutively enrolled in the Extension–Myocardial Iron Overload in Thalassemia project. Magnetic Resonance Imaging was used to quantify IO (T2* technique) and biventricular function and to detect replacement myocardial fibrosis. The glucose metabolism was assessed by the oral glucose tolerance test (OGTT). Pancreatic IO was more frequent in regularly transfused (N = 145) than in nontransfused patients (67.6% vs. 31.6%; p < 0.0001). In the regular transfused group, splenectomy and hepatitis C virus infection were both associated with high pancreatic siderosis. Patients with normal glucose metabolism showed significantly higher global pancreas T2* values than patients with altered OGTT. A pancreas T2* < 17.9 ms predicted an abnormal OGTT. A normal pancreas T2* value showed a 100% negative predictive value for cardiac iron. Pancreas T2* values were not associated to biventricular function, replacement myocardial fibrosis, or cardiac complications. Our findings suggest that in the presence of pancreatic IO, it would be prudent to initiate or intensify iron chelation therapy to prospectively prevent both disturbances of glucose metabolism and cardiac iron accumulation.


2018 ◽  
Vol 11 (6) ◽  
pp. 471-479 ◽  
Author(s):  
Natthaphat Siri-Angkul ◽  
Siriporn C Chattipakorn ◽  
Nipon Chattipakorn

2020 ◽  
pp. 367-382
Author(s):  
Perry Elliott ◽  
Pier D. Lambiase ◽  
Dhavendra Kumar

This chapter covers inherited haematological disorders. It explains the pathophysiology, genetics, and iron overload of thalassaemia; cardiac disease in both β‎ and α‎thalassaemia; the pathophysiology, genetics, and iron overload in haemochromatosis; the evaluation of patients; and finally the management of patients at risk of cardiac iron overload.


Author(s):  
John P Carpenter ◽  
John C Wood ◽  
Dudley J Pennell

The heart is the target lethal organ in thalassaemia major. Cardiovascular magnetic resonance (CMR) measures iron using the magnetic relaxation time T2*. This allows comparison with the left ventricular function and conventional iron measurements such as liver iron and serum ferritin. The single breath-hold cardiac-gated CMR acquisition takes only 15 seconds, making it cost-efficient and relevant to developing countries. Myocardial T2* of <20 ms (increased iron) correlates with reduced left ventricular ejection fraction, but poor correlation exists with ferritin and liver iron, indicating poor capability to assess future risk. Myocardial T2* of <10 ms is present in >90% of thalassaemia patients developing heart failure, and approximately 50% of patients with T2* of <6 ms will develop heart failure within 1 year without intensified treatment. The technique is validated and calibrated against human heart iron concentration. The treatment for iron overload is iron chelation, and three major trials have been performed for the heart. The first trial showed deferiprone was superior to deferoxamine in removing cardiac iron. The second trial showed a combination therapy of deferiprone with deferoxamine was more effective than deferoxamine monotherapy. The third trial showed that deferasirox was non-inferior to deferoxamine in removing cardiac iron. Each drug in suitable doses can be used to remove cardiac iron, but their use depends on clinical circumstances. Other combination regimes are also being evaluated. Use of T2*, intensification of chelation treatment, and use of deferiprone are associated with reduced mortality (a reduction in deaths by 71% has been shown in the United Kingdom). The use of T2* and iron chelators in the heart has been summarized in recent American Heart Association guidelines.


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