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Pancreatic iron overload by T2*MRI in a large cohort of well treated thalassemia major patients: Can it tell us heart iron distribution and function?

AMERICAN JOURNAL OF HEMATOLOGY (2015)

Journal

AMERICAN JOURNAL OF HEMATOLOGY

Volume 90, Issue 9, Pages E189-E190

Publisher

WILEY

DOI: 10.1002/ajh.24081

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Quantification of pancreatic iron overload and fat infiltration and their correlation with glucose disturbance in pediatric thalassemia major patients

Jingwen Huang, Jun Shen, Qihua Yang, Ziliang Cheng, Xiaodong Chen, Taihui Yu, Jinglian Zhong, Yun Su, Hua Guo, Biling Liang

Summary: Pediatric TM patients can have homogeneous iron siderosis and heterogeneous fat infiltration in the pancreas as measured by gradient-echo imaging, both of which are risk factors for diabetes.

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Quantitative T2*MRI for bone marrow iron overload: normal reference values and assessment in thalassemia major patients

Antonella Meloni, Laura Pistoia, Gennaro Restaino, Massimiliano Missere, Vincenzo Positano, Anna Spasiano, Tommaso Casini, Antonella Cossu, Liana Cuccia, Antonella Massa, Francesco Massei, Filippo Cademartiri

Summary: This study evaluated the feasibility and reproducibility of bone marrow T2* measurements in a cohort of healthy subjects and established the lower limit of normal values. In patients with thalassemia major (TM), significantly lower bone marrow T2* values were observed. These values were inversely correlated with serum ferritin levels and hepatic iron load. Incorporating bone marrow T2* measurements into clinical practice may provide new insights into iron deposition.

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A general framework for hepatic iron overload quantification using MRI

Ahmed Karam Eldaly, Ayman M. Khalifa

Summary: This article proposes a general framework for estimating iron overload in the liver using magnetic resonance imaging (MRI), considering various liver models. The iron overload estimation is formulated as a minimization problem with regularization functions assigned to unknown model parameters. An alternating direction method of multipliers (ADMM) is applied for parameter estimation. Three models were tested and compared using synthetic datasets, showing good correlation with ground truth T2* measurements. The proposed approaches were also evaluated using MRI scans of patients, demonstrating better performance than existing methods.

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Article Cardiac & Cardiovascular Systems

National networking in rare diseases and reduction of cardiac burden in thalassemia major

Alessia Pepe, Laura Pistoia, Maria Rita Gamberini, Liana Cuccia, Roberto Lisi, Valerio Cecinati, Aurelio Maggio, Francesco Sorrentino, Aldo Filosa, Rosamaria Rosso, Giuseppe Messina, Massimiliano Missere, Riccardo Righi, Stefania Renne, Antonino Vallone, Stefano Dalmiani, Vincenzo Positano, Massimo Midiri, Antonella Meloni

Summary: The tailored chelation therapy guided by MRI in the MIOT network has shown significant improvement in the prognosis of TM patients. Continuous monitoring of cardiac iron levels and personalized treatment can reduce cardiac burden, improve cardiac function, and reduce complications and death from heart failure related to myocardial iron overload.

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Diagnostic value of T2 relaxation time for hepatic iron grading in rat model of fatty and fibrotic liver

Mingli Jin, Yin Jiang, Qi Zhao, Zhihua Pan, Fang Xiao

Summary: The study evaluates the diagnostic value of T2 relaxation time for determining liver iron grades in the presence of fat and fibrosis. The results show that T2 relaxation time can assess histologically determined liver iron grades, regardless of coexisting liver steatosis or fibrosis. It is more accurate for higher iron grading.

PLOS ONE (2022)

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Increased myocardial extracellular volume is associated with myocardial iron overload and heart failure in thalassemia major

Antonella Meloni, Laura Pistoia, Vincenzo Positano, Antonio De Luca, Nicola Martini, Anna Spasiano, Ilaria Fotzi, Pier Paolo Bitti, Domenico Visceglie, Gianna Alberini, Gianfranco Sinagra, Alessia Pepe, Filippo Cademartiri

Summary: This study evaluated the association between myocardial extracellular volume (ECV) and demographics, cardiovascular magnetic resonance (CMR) findings, and cardiac involvement in patients with thalassemia major. The results showed that increased ECV was associated with myocardial iron overload and heart failure, potentially indicating diffuse interstitial fibrosis.

EUROPEAN RADIOLOGY (2023)

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Pattern and clinical correlates of renal iron deposition in adult beta-thalassemia major patients

Antonella Meloni, Luigi Barbuto, Vincenzo Positano, Laura Pistoia, Anna Spasiano, Tommaso Casini, Francesco Massei, Crocetta Argento, Piera Giovangrossi, Angelica Barone, Luigia Romano, Filippo Cademartiri

Summary: We evaluated the pattern and clinical correlates of renal T2* measurements in adult β-thalassemia major (β-TM) patients. A total of 11.1% of β-TM patients showed renal iron overload, which was associated with liver iron concentration (LIC) values and pancreas T2* values. Renal iron overload was more common in patients with cardiac iron overload. MRI LIC and lactate dehydrogenase (LDH) were strong predictors of global kidney T2* values. An MRI LIC > 4.83 mg/g dw predicted the presence of renal iron overload.

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The Importance of Cardiac T2*Magnetic Resonance Imaging for Monitoring Cardiac Siderosis in Thalassemia Major Patients

Narumol Chaosuwannakit, Pattarapong Makarawate, Chinnadol Wanitpongpun

Summary: Cardiac T2* MRI is a valuable tool for monitoring cardiac iron overload in thalassemia major patients, allowing for early diagnosis and treatment of patients at risk of cardiac siderosis, thus reducing cardiac mortality associated with myocardial siderosis.

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Left ventricular deformation mechanics over time in patients with thalassemia major with and without iron overload

Michael J. Bonios, Epameinontas Fountas, Polyxeni Delaporta, Stamatis Kyrzopoulos, Antonis Kattamis, Stamatis N. Adamopoulos, Dimitris Tsiapras

Summary: The study investigated advanced echocardiography parameters for early identification of myocardial dysfunction in TM patients, finding GLS to be potentially more efficient than LVEF in detecting hemochromatosis-related cardiac dysfunction earlier.

BMC CARDIOVASCULAR DISORDERS (2021)

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Iron Overload in Chronic Kidney Disease: Less Ferritin, More T2*MRI

Abdulqadir J. Nashwan, Mohamed A. Yassin, Mohamed Izham Mohamed Ibrahim, Hanan F. Abdul Rahim, Mujahed Shraim

Summary: Currently, there is no agreement on the most reliable marker of iron status in chronic kidney disease (CKD) patients. Serum ferritin, commonly used for this purpose, may provide misleading information about iron overload. The success of T2* MRI in monitoring iron overload in patients with hemoglobinopathies can be beneficial in monitoring CKD patients.

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Vitamin D levels and left ventricular function in beta-thalassemia major with iron overload

Mrudula Pala, Kamalakshi G. Bhat, Sharath Manya, Nitin Joseph, Sindhu Harish

Summary: This study aimed to determine the association between vitamin D and left ventricular function in patients with beta-thalassemia major with iron overload. A cross-sectional study measured the vitamin D and ferritin levels, and left ventricular function was assessed using echocardiography. The study found a significant negative correlation between vitamin D and serum ferritin levels, and a significant positive association between vitamin D levels and EF and FS.

EUROPEAN JOURNAL OF PEDIATRICS (2023)

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Comparison of Two Alternative Procedures to Obtain Packed Red Blood Cells for β-Thalassemia Major Transfusion Therapy

Davide Schiroli, Lucia Merolle, Eleonora Quartieri, Roberta Chicchi, Tommaso Fasano, Tiziana De Luca, Giuseppe Molinari, Stefano Pulcini, Thelma A. Pertinhez, Erminia Di Bartolomeo, Rino Biguzzi, Roberto Baricchi, Chiara Marraccini

Summary: This study compared two methods for preparing optimal P-RBCs products for beta-thalassemia major patients, finding that method 1 resulted in higher RBC count and Hb content, while method 2 produced P-RBCs with lower levels of K+, iron, and storage lesions markers. Further clinical studies are needed to determine the impact of these differences on transfusion-related complications and the quality of life of beta TM patients.

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Perturbations in lipid metabolism and gut microbiota composition precede cardiac dysfunction in a mouse model of thalassemia

Ying Liu, Carine Fillebeen, Anik Forest, Amy Botta, Thibault V. Varin, Andre Marette, Yan Burelle, Christine Des Rosiers, Kostas Pantopoulos, Gary Sweeney

Summary: This study using the Th3/+ mouse model found that altered lipid metabolism and gut microbiota may be related to the development of cardiomyopathy in thalassemia. These findings are important for understanding the early biomarkers and therapeutic targets for the development of cardiomyopathy in thalassemia.

FASEB JOURNAL (2023)

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The Link of Pancreatic Iron with Glucose Metabolism and Cardiac Iron in Thalassemia Intermedia: A Large, Multicenter Observational Study

Antonella Meloni, Laura Pistoia, Maria Rita Gamberini, Paolo Ricchi, Valerio Cecinati, Francesco Sorrentino, Liana Cuccia, Massimo Allo, Riccardo Righi, Priscilla Fina, Ada Riva, Stefania Renne, Giuseppe Peritore, Stefano Dalmiani, Vincenzo Positano, Emilio Quaia, Filippo Cademartiri, Alessia Pepe

Summary: The study revealed a significant association between pancreatic iron overload and disturbances in glucose metabolism and heart disease in patients with thalassemia intermedia. Pancreatic iron overload was more common in regularly transfused patients and was linked to splenectomy and hepatitis C virus infection. A normal pancreas T2* value demonstrated a 100% negative predictive value for cardiac iron overload.

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Circulating miRNAs and tissue iron overload in transfusion-dependent β-thalassemia major: novel predictors and follow-up guide

Nashwa El-Khazragy, Safa Matbouly, Demiana H. Hanna, Nievin Ahmed Mahran, Sally Abdallah Mostafa, Bassam A. Abdelrehim, Yasmeen K. Farouk, Soha Abuelela

Summary: The study identifies an association between three iron-related miRNAs and tissue iron overload in transfusion-dependent beta-thalassemia major patients. Results suggest that circulating miR-let-7d and miR-200b are downregulated, while miR-122 is upregulated in TDT patients. Aberrant expression of miRNAs is significantly associated with increased iron accumulation in hepatic and cardiac tissues, making them potential predictive biomarkers for tissue iron excess.

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Deep Learning Staging of Liver Iron Content From Multiecho MR Images

Vincenzo Positano, Antonella Meloni, Maria Filomena Santarelli, Laura Pistoia, Anna Spasiano, Liana Cuccia, Tommaso Casini, Maria Rita Gamberini, Massimo Allo, Pier Paolo Bitti, Alessia Pepe, Filippo Cademartiri

Summary: This study developed a deep-learning method for unsupervised classification of liver iron content (LIC) from TEE multiecho MR images. The results showed that the HippoNet-Ensemble network achieved higher accuracy, sensitivity, and specificity in LIC staging compared to other networks.

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Photon-Counting Computed Tomography (PCCT): Technical Background and Cardio-Vascular Applications

Antonella Meloni, Francesca Frijia, Daniele Panetta, Giulia Degiorgi, Carmelo De Gori, Erica Maffei, Alberto Clemente, Vincenzo Positano, Filippo Cademartiri

Summary: Photon-counting computed tomography (PCCT) is a new advanced imaging technique that offers improved spatial and contrast resolution, reduced image noise and artifacts, reduced radiation exposure, and multi-energy/multi-parametric imaging based on tissue properties. It has the potential to transform the standard clinical use of computed tomography (CT) imaging.

DIAGNOSTICS (2023)

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Article Medicine, General & Internal

Multi-Parametric Cardiac Magnetic Resonance for Prediction of Heart Failure Death in Thalassemia Major

Antonella Meloni, Laura Pistoia, Maria Rita Gamberini, Liana Cuccia, Roberto Lisi, Valerio Cecinati, Paolo Ricchi, Calogera Gerardi, Gennaro Restaino, Riccardo Righi, Vincenzo Positano, Filippo Cademartiri

Summary: We evaluated the prognostic value of multiparametric cardiovascular magnetic resonance (CMR) in predicting death from heart failure in patients with thalassemia major (TM). Iron overload, ventricular dysfunction, ventricular dilation, and replacement myocardial fibrosis were identified as significant predictors of heart failure death. Patients with all four markers had a significantly higher risk of dying from heart failure compared to patients without markers or with one to three markers.

DIAGNOSTICS (2023)

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Sex Differences in Repolarization Markers: Telemonitoring for Chronic Heart Failure Patients

Federica Moscucci, Susanna Sciomer, Silvia Maffei, Antonella Meloni, Ilaria Lospinuso, Myriam Carnovale, Andrea Corrao, Ilaria Di Diego, Cristina Caltabiano, Martina Mezzadri, Anna Vittoria Mattioli, Sabina Gallina, Pietro Rossi, Damiano Magri, Gianfranco Piccirillo

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JOURNAL OF CLINICAL MEDICINE (2023)

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Phenotypic Clustering of Beta-Thalassemia Intermedia Patients Using Cardiovascular Magnetic Resonance

Antonella Meloni, Michela Parravano, Laura Pistoia, Alberto Cossu, Emanuele Grassedonio, Stefania Renne, Priscilla Fina, Anna Spasiano, Alessandra Salvo, Sergio Bagnato, Calogera Gerardi, Zelia Borsellino, Filippo Cademartiri, Vincenzo Positano

Summary: An unsupervised clustering method integrating demographic, clinical, and cardiac magnetic resonance (CMR) data was used to identify distinct phenogroups of beta-thalassemia intermedia patients. Three mutually exclusive phenogroups were identified based on unsupervised hierarchical clustering. PG2 exhibited a significantly higher risk of future cardiovascular events than PG1 and PG3. Clustering emerged as a useful tool for risk stratification in beta-thalassemia intermedia.

JOURNAL OF CLINICAL MEDICINE (2023)

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Article Medicine, General & Internal

Pancreatic T2*Magnetic Resonance Imaging for Prediction of Cardiac Arrhythmias in Transfusion-Dependent Thalassemia

Antonella Meloni, Laura Pistoia, Paolo Ricchi, Vincenzo Positano, Filomena Longo, Zelia Borsellino, Valerio Cecinati, Giuseppe Messina, Elisabetta Corigliano, Rosamaria Rosso, Riccardo Righi, Giuseppe Peritore, Stefania Renne, Antonino Vallone, Filippo Cademartiri

Summary: The value of pancreatic T2* magnetic resonance imaging (MRI) for predicting cardiac events in transfusion-dependent thalassemia patients was assessed. The study found that pancreatic T2* values were a significant predictor of future arrhythmia-related events, suggesting that monitoring pancreatic iron overload may help improve patient outcomes.

JOURNAL OF CLINICAL MEDICINE (2023)

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Article Cardiac & Cardiovascular Systems

Left ventricular global function index is associated with myocardial iron overload and heart failure in thalassemia major patients

Antonella Meloni, Vincenzo Positano, Laura Pistoia, Riccardo Righi, Massimiliano Missere, Emanuele Grassedonio, Nicolo Schicchi, Antonino Vallone, Maria Rita Gamberini, Liana Cuccia, Roberto Lisi, Valerio Cecinati, Anna Spasiano, Filippo Cademartiri

Summary: This study explored the association between left ventricular global function index (LVGFI) and myocardial iron overload (MIO), left ventricular ejection fraction (LVEF), myocardial fibrosis, and heart failure (HF) in patients with beta-thalassemia major (TM). The results showed that LVGFI and LVEF were significantly lower in patients with significant MIO, and LVGFI was a stronger predictor of HF compared to LVEF. LVGFI has incremental diagnostic value for HF detection in TM patients.

INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING (2023)

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Article Radiology, Nuclear Medicine & Medical Imaging

Impact of the COVID-19 Pandemic on Iron Overload Assessment by MRI in Patients with Hemoglobinopathies: The E-MIOT Network Experience

Antonella Meloni, Laura Pistoia, Amalia Lupi, Riccardo Righi, Antonino Vallone, Massimiliano Missere, Stefania Renne, Priscilla Fina, Ada Riva, Maria Rita Gamberini, Valerio Cecinati, Francesco Sorrentino, Rosamaria Rosso, Giuseppe Messina, Paolo Ricchi, Vincenzo Positano, Sophie Mavrogeni, Emilio Quaia, Filippo Cademartiri, Alessia Pepe

Summary: The COVID-19 pandemic had a strong negative impact on MRI services for patients with hemoglobinopathies in the Italian E-MIOT Network. The total number of MRI scans significantly declined, and some patients refused MRI exams due to fear of the virus.

TOMOGRAPHY (2023)

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Correction Hematology