Elevated serum parathormone levels are associated with myocardial iron overload in patients with β-thalassaemia major

2010 ◽  
Vol 84 (1) ◽  
pp. 64-71 ◽  
Author(s):  
Meropi Dimitriadou ◽  
Athanasios Christoforidis ◽  
Marina Economou ◽  
Ioanna Tsatra ◽  
Efthimia Vlachaki ◽  
...  
Keyword(s):  
Iron Overload ◽  
Elevated Serum ◽  
Myocardial Iron ◽  
Thalassaemia Major

Myocardial T2* in Patients with Cardiac Failure Secondary to Iron Overload.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3838-3838 ◽  
Author(s):  
Mark A. Tanner ◽  
John B. Porter ◽  
Mark A. Westwood ◽  
Sunil V. Nair ◽  
Lisa J. Anderson ◽  
...  
Keyword(s):  
Heart Failure ◽  
Iron Overload ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Iron Loading ◽  
Myocardial Iron ◽  
Thalassaemia Major ◽  
Ventricular Ejection Fraction ◽  
Symptomatic Heart Failure ◽  
Increased Risk

Abstract Introduction: Myocardial iron overload is a well established cause of heart failure in a number of haematological disorders and is the leading cause of death in β-thalassaemia major (TM). Once overt heart failure develops prognosis is very poor and it would therefore be desirable to identify patients at risk, prior to the development of symptomatic heart failure. Myocardial iron can now be rapidly and reproducibly assessed using a validated cardiac magnetic resonance (CMR) T2* technique. Left ventricular ejection fraction has been demonstrated to relate to myocardial T2* (normal >20ms), and accordingly iron overloaded patients with symptomatic heart failure are likely to have a low T2* but there are no reports of the myocardial T2* level in newly presenting patients with heart failure and the threshold for increased risk is also unknown. Purpose: To establish the distribution of myocardial T2* values in patients presenting with symptomatic heart failure secondary to iron overload. Methods: Database records of CMR T2* assessments over a 5 year period were reviewed to identify iron overloaded patients presenting with heart failure. Results: 28 patients (median age 29y, 11–79) with iron overload and documented heart failure were identified. 22 patients had thalassaemia major, 3 hereditary haemochromatosis, and 3 had miscellaneous transfusion dependent anaemias. The mean myocardial T2* in all groups was 6.8+/− 2.2ms reflecting severe iron loading by clinical criteria. See figure 1. Myocardial T2* values were similar between sub-groups as follows: TM patients 6.7+/− 2.4ms, haemochromatosis 7.7ms (6.7–7.4ms), miscellaneous 6.8ms (4.8–9.1ms). Conclusion: In this database review, the patients developing heart failure secondary to iron overload all had abnormal myocardial T2*, and overall 89% of heart failure patients had values less than 10ms, which reflects severe myocardial iron loading. This data suggests a myocardial T2* <10ms should be considered a threshold for risk of heart failure (with its attendant high mortality) and such patients should be treated aggressively with increased iron chelation. It also suggests that clinical heart failure occurs nearly exclusively in patients with severe myocardial iron loading. Distribution of myocardial T2+ values in symptomatic heart failure (n=28) Distribution of myocardial T2+ values in symptomatic heart failure (n=28)

Multi-Centre Validation of the Cardiovascular Magnetic Resonance Multi Breath-Hold T2* Technique for Myocardial Iron Quantification in Thalassaemia Major.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3828-3828
Author(s):  
Mark A. Tanner ◽  
Taigang He ◽  
Mark A. Westwood ◽  
Renzo Galanello ◽  
Gildo Matta ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Iron Overload ◽  
Breath Hold ◽  
Myocardial Iron ◽  
Thalassaemia Major ◽  
Non Invasive ◽  
Cardiac Siderosis ◽  
Iron Quantification ◽  
Siemens Sonata ◽  
Roll Out

Abstract Introduction: Beta thalassaemia major (TM) is a hereditary anaemia affecting 60 000 births worldwide each year. Survival is dependent upon lifelong blood transfusions resulting in iron overload. Cardiac siderosis can result in a cardiomyopathy which is the leading cause of death in TM. The validated cardiac magnetic resonance (CMR) T2* technique allows non-invasive and reproducible quantification of myocardial iron. Assessment of myocardial iron loading is essential in determining appropriate chelation therapy. This technique has the potential to become the new gold standard in the assessment of cardiac siderosis but is currently available at only a few sites worldwide. For maximal healthcare benefit its inter-scanner reproducibility must be demonstrated before being widely disseminated. Objective: To demonstrate that CMR T2* quantification of myocardial iron can be reproducibly transferred to MR scanners of different manufacturers in different centres. This project was sponsored by the Thalassemia International Federation. Methods: The previously described multi breath-hold gradient echo T2* technique was installed on MR scanners (all 1.5Tesla) at 6 different centres. Scanner details were as follows: Site 1, Phillips Intera (Turin, Italy), Site 2, Siemens Sonata (Philadelphia, USA), Site 3 GE Signa (Limassol, Cyprus) Site 4, Phillips Intera (Nicosia, Cyprus), Site 5, GE Signa (Cagliari, Sardinia) and Philips Intera (Genova, Italy). 34 patients (mean age 30+/− 5.7years) were scanned in total. All patients were subsequently re-scanned at the standardization centre in London, UK (Siemens Sonata, 1.5T) within 31 days of their original scan. Results: The T2* sequence was successfully installed on all 6 scanners. Myocardial T2* values ranged from 3.6ms to 51ms (14.2 +/− 11ms). The overall inter-scanner reproducibility (SD/mean) was 5.3% (figure 1). The mean difference between T2* values at the standardization centre and visited sites was 0.32ms. Conclusion: We have demonstrated that the multi breath-hold T2* technique for the quantification of myocardial iron can be reproducibly transferred to 1.5T MR scanners at different sites and of different manufacturers. There is therefore real potential to roll out this technique worldwide to facilitate maximal healthcare impact in the management of patients with iron overload conditions such as thalassaemia. Figure Figure

scholarly journals Myocardial iron overload in thalassaemia major. How early to check?

2014 ◽  
Vol 164 (4) ◽  
pp. 579-585 ◽  
Author(s):  
Caterina Borgna-Pignatti ◽  
Antonella Meloni ◽  
Giulia Guerrini ◽  
Letizia Gulino ◽  
Aldo Filosa ◽  
...  
Keyword(s):  
Iron Overload ◽  
Myocardial Iron ◽  
Thalassaemia Major

scholarly journals Restrictive lung disease in β-thalassemia major is associated with myocardial iron overload

2021 ◽  
Author(s):  
Kate Chan ◽  
Chun Ting Au ◽  
Alex Wing Kwan Leung ◽  
Albert Li ◽  
Chi-kong Li ◽  
...  
Keyword(s):  
Relaxation Time ◽  
Lung Function ◽  
Iron Overload ◽  
Myocardial Iron ◽  
Pulmonary Dysfunction ◽  
Thalassaemia Major ◽  
T2 Relaxation Time ◽  
T2 Relaxation ◽  
Restrictive Lung Disease

Background: Pulmonary dysfunction has been reported in patients with β-thalassaemia major but data are conflicting and the association with iron overload remains unclear. Objectives: To determine the pattern of pulmonary dysfunction in patients with β-thalassaemia major and their associations with iron overload. Methods: Subjects with β-thalassaemia major were recruited for lung function assessment. Serum ferritin and magnetic resonance imaging (MRI) measurements of iron status of the myocardium and the liver were used as surrogate indexes of body iron content. A subgroup of this cohort provided data on the longitudinal progress of their lung function. Results: One hundred and one patients were recruited with a mean age of 25.1 years (SD 7.9 years). Thirty-eight (38%) and five (5%) had restrictive and obstructive lung function deficits, respectively. There was a significant correlation between MRI myocardial T2* relaxation time and forced vital capacity (r=0.291, p=0.048). Higher MRI cardiac T2* relaxation time was associated with lower risk of having restrictive lung function deficit (Odds ratio (OR): 0.94; 95% CI: 0.89-0.99; p=0.023) after adjusting for age, gender and BMI. Twenty-three subjects underwent lung function reassessment with a mean follow-up duration of 13 years. Overall, they did not demonstrate significant changes in pulmonary function over time, 3 patients who had normal lung function at baseline developed restrictive abnormality at follow-up. Conclusions: Restrictive lung disease is prevalent in patients with β-thalassaemia major, and the severity correlates with myocardial iron overload. Monitoring of lung function in this group of patients is important, particularly for those with iron overload.

scholarly journals Myocardial iron overload by cardiovascular magnetic resonance native segmental T1 mapping: a sensitive approach that correlates with cardiac complications

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Antonella Meloni ◽  
Nicola Martini ◽  
Vincenzo Positano ◽  
Antonio De Luca ◽  
Laura Pistoia ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
Iron Overload ◽  
Left Ventricular ◽  
Cardiac Complications ◽  
Lv Function ◽  
Myocardial Iron ◽  
Thalassaemia Major ◽  
Native T1 ◽  
T1 And T2

Abstract Background We compared cardiovascular magnetic resonance segmental native T1 against T2* values for the detection of myocardial iron overload (MIO) in thalassaemia major and we evaluated the clinical correlates of native T1 measurements. Methods We considered 146 patients (87 females, 38.7 ± 11.1 years) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassaemia Network. T1 and T2* values were obtained in the 16 left ventricular (LV) segments. LV function parameters were quantified by cine images. Post-contrast late gadolinium enhancement (LGE) and T1 images were acquired. Results 64.1% of segments had normal T2* and T1 values while 10.1% had pathologic T2* and T1 values. In 526 (23.0%) segments, there was a pathologic T1 and a normal T2* value while 65 (2.8%) segments had a pathologic T2* value but a normal T1 and an extracellular volume (ECV) ≥ 25% was detected in 16 of 19 segments where ECV was quantified. Global native T1 was independent from gender or LV function but decreased with increasing age. Patients with replacement myocardial fibrosis had significantly lower native global T1. Patients with cardiac complications had significantly lower native global T1. Conclusions The combined use of both segmental native T1 and T2* values could improve the sensitivity for detecting MIO. Native T1 is associated with cardiac complications in thalassaemia major.

Iron Overload and Glucose Metabolism in Subjects with β-thalassaemia Major : An Overview

2013 ◽  
Vol 9 (4) ◽  
pp. 332-341 ◽  
Author(s):  
Vincenzo Sanctis ◽  
Ashraf Soliman ◽  
Mohamed Yassin
Keyword(s):  
Glucose Metabolism ◽  
Iron Overload ◽  
Thalassaemia Major

scholarly journals Clinical Manifestations of Hemochromatosis inHFEC282Y Homozygotes Identified by Screening

2008 ◽  
Vol 22 (11) ◽  
pp. 923-930 ◽  
Author(s):  
Gordon D McLaren ◽  
Christine E McLaren ◽  
Paul C Adams ◽  
James C Barton ◽  
David M Reboussin ◽  
...  
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Elevated Serum ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Joint Stiffness ◽  
Chronic Fatigue ◽  
Newly Diagnosed ◽  
Wild Type ◽  
Control Subjects

BACKGROUND: Patients with hemochromatosis may suffer organ damage from iron overload, often with serious clinical consequences.OBJECTIVE: To assess prevalences of self-reported symptoms and clinical signs and conditions in persons homozygous for the hemochromatosis gene (HFE)mutation (C282Y) identified by screening.METHODS: Participants were adults 25 years of age or older enrolled in the Hemochromatosis and Iron Overload Screening (HEIRS) Study. C282Y homozygotes (n=282) were compared with control participants without theHFEC282Y or H63D alleles (ie, wild type/wild type; n=364).RESULTS: Previously diagnosed C282Y homozygotes and newly diagnosed homozygotes with elevated serum ferritin levels had higher prevalences of certain symptoms such as chronic fatigue (OR 2.8; 95% CI 1.34 to 5.95, and OR 2.0; 95% CI 1.07 to 3.75, respectively), and had more hyperpigmentation on physical examination (OR 4.7; 95% CI 1.50 to 15.06, and OR 3.7; 95% CI 1.10 to 12.16, respectively) and swelling or tenderness of the second and third metacarpophalangeal joints (OR 4.2; 95% CI 1.37 to 13.03, and OR 3.3; 95% CI 1.17 to 9.49, respectively) than control subjects. Joint stiffness was also more common among newly diagnosed C282Y homozygotes with elevated serum ferritin than among control subjects (OR 2.7; 95% CI 1.38 to 5.30). However, the sex- and age-adjusted prevalences of self-reported symptoms and signs of liver disease, heart disease, diabetes and most other major clinical manifestations of hemochromatosis were similar in C282Y homozygotes and control subjects.CONCLUSIONS: Some symptoms and conditions associated with hemochromatosis were more prevalent among C282Y homozygotes identified by screening than among control subjects, but prevalences of most outcomes were similar in C282Y homozygotes and controls in this primary care-based study.

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