Abstract

Background: Non-invasive determination of liver iron concentration (LIC) is a valuable tool that guides iron chelation therapy in transfusion-dependent patients. Multiple methods have been utilized to measure LIC by MRI. The purpose of this study was to compare free breathing R2* (1/T2*) to whole-liver Ferriscan R2 method for estimation of LIC in a pediatric and young adult population who predominantly have hemoglobinopathies. Methods: Clinical liver and cardiac MRI scans from April 2016 to May 2018 on a Phillips 1.5 T scanner were reviewed. Free breathing T2 and T2* weighted images were acquired on each patient. For T2, multi-slice spin echo sequences were obtained. For T2*, a single mid-liver slice fast gradient echo was performed starting at 0.6 ms with 1.2 ms increments with signal averaging. R2 measurements were performed by Ferriscan analysis. R2* measurements were performed by quantitative T2* map analysis. Results: 107 patients underwent liver scans with the following diagnoses: 76 sickle cell anemia, 20 Thalassemia, 9 malignancies and 2 Blackfan Diamond anemia. Mean age was 12.5 ± 4.5 years. Average scan time for R2 sequences was 10 min, while R2* sequence time was 20 s. R2* estimation of LIC correlated closely with R2 with a correlation coefficient of 0.94. Agreement was strongest for LIC < 15 mg Fe/g dry weight. Overall bias from Bland–Altman plot was 0.66 with a standard deviation of 2.8 and 95% limits of agreement −4.8 to 6.1. Conclusion: LIC estimation by R2* correlates well with R2-Ferriscan in the pediatric age group. Due to the very short scan time of R2*, it allows imaging without sedation or anesthesia. Cardiac involvement was uncommon in this cohort.

Highlights

  • Chronic iron overload affects predominantly the liver, pancreas and heart, leading to systemic complications, including liver fibrosis, diabetes and cardiomyopathy [1,2]

  • Studies have shown that liver iron concentration (LIC) is directly proportional to total body iron stores [3]

  • We describe our real-world experience where we compare simultaneous LIC derived from R2 (Ferriscan) to that derived from free breathing single slice R2* in a cohort of pediatric and young adult patients who predominantly have hemoglobinopathies receiving chronic transfusion therapy

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Summary

Introduction

Chronic iron overload affects predominantly the liver, pancreas and heart, leading to systemic complications, including liver fibrosis, diabetes and cardiomyopathy [1,2]. Studies have shown that liver iron concentration (LIC) is directly proportional to total body iron stores [3]. Diagnostics 2020, 10, 768 of LIC is indicated prior to initiation of chelation therapy and during therapy to guide management in chronically transfused patients [4]. LIC was measured via liver biopsy [5]. Non-invasive determination of liver iron concentration (LIC) is a valuable tool that guides iron chelation therapy in transfusion-dependent patients. Multiple methods have been utilized to measure LIC by MRI. The purpose of this study was to compare free breathing R2*. (1/T2*) to whole-liver Ferriscan R2 method for estimation of LIC in a pediatric and young adult population who predominantly have hemoglobinopathies. For T2*, a single mid-liver slice fast gradient echo was performed starting at 0.6 ms with

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