Genetic Determined Iron Starvation Signature in Friedreich's Ataxia.

BACKGROUND: Early studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking. OBJECTIVES: The objective is to characterize systemic iron metabolism, body iron storages, and intracellular iron regulation in FA patients. METHODS: In FA patients and matched healthy controls, we assessed serum iron parameters, regulatory hormones as well as the expression of regulatory proteins and iron distribution in peripheral blood mononuclear cells (PBMCs). We applied magnetic resonance imaging with R2*-relaxometry to quantify iron storages in the liver, spleen, and pancreas. Across all evaluations, we assessed the influence of the genetic severity as expressed by the length of the shorter GAA-expansion (GAA1). RESULTS: We recruited 40 FA patients (19 women). Compared to controls, FA patients displayed lower serum iron and transferrin saturation. Serum ferritin, hepcidin, mean corpuscular hemoglobin and mean corpuscular volume in FA inversely correlated with the GAA1-repeat length, indicating iron deficiency and restricted availability for erythropoiesis with increasing genetic severity. R2*-relaxometry revealed a reduction of splenic and hepatic iron stores in FA. Liver and spleen R2* values inversely correlated with the GAA1-repeat length. FA PBMCs displayed downregulation of ferritin and upregulation of transferrin receptor and divalent metal transporter-1 mRNA, particularly in patients with >500 GAA1-repeats. In FA PBMCs, intracellular iron was not increased, but shifted toward mitochondria. CONCLUSIONS: We provide evidence for a previously unrecognized iron starvation signature at systemic and cellular levels in FA patients, which is related to the underlying genetic severity. These findings challenge the use of systemic iron lowering therapies in FA. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Association of dysglycaemia with persistent infarct core iron in patients with acute ST-segment elevation myocardial infarction.

BACKGROUND: Dysglycaemia increases the risk of myocardial infarction and subsequent recurrent cardiovascular events. However, the role of dysglycaemia in ischemia/reperfusion injury with development of irreversible myocardial tissue alterations remains poorly understood. In this study we aimed to investigate the association of ongoing dysglycaemia with persistence of infarct core iron and their longitudinal changes over time in patients undergoing primary percutaneous coronary intervention (PCI) for acute ST-segment elevation myocardial infarction (STEMI). METHODS: We analyzed 348 STEMI patients treated with primary PCI between 2016 and 2021 that were included in the prospective MARINA-STEMI study (NCT04113356). Peripheral venous blood samples for glucose and glycated hemoglobin (HbA1c) measurements were drawn on admission and 4 months after STEMI. Cardiac magnetic resonance (CMR) imaging including T2 * mapping for infarct core iron assessment was performed at both time points. Associations of dysglycaemia with persistent infarct core iron and iron resolution at 4 months were calculated using multivariable regression analysis. RESULTS: Intramyocardial hemorrhage was observed in 147 (42%) patients at baseline. Of these, 89 (61%) had persistent infarct core iron 4 months after infarction with increasing rates across HbA1c levels (<5.7%: 33%, ≥5.7: 79%). Persistent infarct core iron was independently associated with ongoing dysglycaemia defined by HbA1c at 4 months (OR: 7.87 [95% CI: 2.60-23.78]; p < 0.001), after adjustment for patient characteristics and CMR parameters. The independent association was present even after exclusion of patients with diabetes (pre- and newly diagnosed, n = 16). CONCLUSIONS: In STEMI patients treated with primary PCI, ongoing dysglycaemia defined by HbA1c is independently associated with persistent infarct core iron and a lower likelihood of iron resolution. These findings suggest a potential association between ongoing dysglycaemia and persistent infarct core iron, which warrants further investigation for therapeutic implications.

Treatment of cartilage defects in the patellofemoral joint with matrix-associated autologous chondrocyte implantation effectively improves pain, function, and radiological outcomes after 5-7 years.

INTRODUCTION: The aim of the present study was to evaluate midterm outcomes 5-7 years after matrix-associated autologous chondrocyte implantation (MACI) in the patellofemoral joint. MATERIALS AND METHODS: Twenty-six patients who had undergone MACI using the Novocart® 3D scaffold were prospectively evaluated. Clinical outcomes were determined by measuring the 36-Item Short-Form Health Survey (SF-36) and International Knee Documentation Committee (IKDC) scores and the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) values preoperatively and 3, 6, and 12 months, and a mean of 6 years postoperatively. At the final follow-up, the Magnetic Resonance Observation of Cartilage Repair Tissue (MOCART) score was evaluated. RESULTS: Twenty-two patients with 23 focal cartilage defects (19 patella and four trochlea) were available for the final follow-up. The mean defect size was 4.0 ± 1.9 cm2 (range 2.4-9.4 cm2). All clinical outcome scores improved significantly until 5-7 years after MACI (SF-36 score, 61.2 ± 19.6 to 83.2 ± 11.6; P = 0.001; IKDC score, 47.5 ± 20.6 to 74.7 ± 15.5; P < 0.001; and WOMAC, 29.8 ± 15.7 to 8.2 ± 10.3; P < 0.001). The mean MOCART score was 76.0 ± 11.0 at the final follow-up. Nineteen of the 22 patients (86.4%) were satisfied with the outcomes after 5-7 years and responded that they would undergo the procedure again. CONCLUSION: MACI in the patellofemoral joint demonstrated good midterm clinical results with a significant reduction in pain, improvement in function, and high patient satisfaction. These clinical findings are supported by radiological evidence from MOCART scores. LEVEL OF EVIDENCE: IV-case series.

Quantification of Liver Iron Overload with MRI: Review and Guidelines from the ESGAR and SAR.

Accumulation of excess iron in the body, or systemic iron overload, results from a variety of causes. The concentration of iron in the liver is linearly related to the total body iron stores and, for this reason, quantification of liver iron concentration (LIC) is widely regarded as the best surrogate to assess total body iron. Historically assessed using biopsy, there is a clear need for noninvasive quantitative imaging biomarkers of LIC. MRI is highly sensitive to the presence of tissue iron and has been increasingly adopted as a noninvasive alternative to biopsy for detection, severity grading, and treatment monitoring in patients with known or suspected iron overload. Multiple MRI strategies have been developed in the past 2 decades, based on both gradient-echo and spin-echo imaging, including signal intensity ratio and relaxometry strategies. However, there is a general lack of consensus regarding the appropriate use of these methods. The overall goal of this article is to summarize the current state of the art in the clinical use of MRI to quantify liver iron content and to assess the overall level of evidence of these various methods. Based on this summary, expert consensus panel recommendations on best practices for MRI-based quantification of liver iron are provided.

E-AHPBA-ESSO-ESSR Innsbruck consensus guidelines for preoperative liver function assessment before hepatectomy.

BACKGROUND: Posthepatectomy liver failure (PHLF) contributes significantly to morbidity and mortality after liver surgery. Standardized assessment of preoperative liver function is crucial to identify patients at risk. These European consensus guidelines provide guidance for preoperative patient assessment. METHODS: A modified Delphi approach was used to achieve consensus. The expert panel consisted of hepatobiliary surgeons, radiologists, nuclear medicine specialists, and hepatologists. The guideline process was supervised by a methodologist and reviewed by a patient representative. A systematic literature search was performed in PubMed/MEDLINE, the Cochrane library, and the WHO International Clinical Trials Registry. Evidence assessment and statement development followed Scottish Intercollegiate Guidelines Network methodology. RESULTS: Based on 271 publications covering 4 key areas, 21 statements (at least 85 per cent agreement) were produced (median level of evidence 2- to 2+). Only a few systematic reviews (2++) and one RCT (1+) were identified. Preoperative liver function assessment should be considered before complex resections, and in patients with suspected or known underlying liver disease, or chemotherapy-associated or drug-induced liver injury. Clinical assessment and blood-based scores reflecting liver function or portal hypertension (for example albumin/bilirubin, platelet count) aid in identifying risk of PHLF. Volumetry of the future liver remnant represents the foundation for assessment, and can be combined with indocyanine green clearance or LiMAx® according to local expertise and availability. Functional MRI and liver scintigraphy are alternatives, combining FLR volume and function in one examination. CONCLUSION: These guidelines reflect established methods to assess preoperative liver function and PHLF risk, and have uncovered evidence gaps of interest for future research.

Liver surgery is an effective treatment for liver tumours. Liver failure is a major problem in patients with a poor liver quality or having large operations. The treatment options for liver failure are limited, with high death rates. To estimate patient risk, assessing liver function before surgery is important. Many methods exist for this purpose, including functional, blood, and imaging tests. This guideline summarizes the available literature and expert opinions, and aids clinicians in planning safe liver surgery.

1.5-T MR relaxometry in quantifying splenic and pancreatic iron: retrospective comparison of a commercial 3D-Dixon sequence and an established 2D multi-gradient echo sequence.

OBJECTIVES: To compare the quantitative measurement of splenic and pancreatic iron content using a commercial 3D-Dixon sequence (qDixon) versus an established fat-saturated R2* relaxometry method (ME-GRE). METHODS: We analyzed splenic and pancreatic iron levels in 143 MR examinations (1.5 T) using the qDixon and a ME-GRE sequence (108 patients: 65 males, 43 females, mean age 61.31 years). Splenic and pancreatic R2* values were compared between both methods using Bland-Altman plots, concordance correlation coefficients (CCC), and linear regression analyses. Iron overload (R2* > 50 1/s) was defined for both organs and compared using contingency tables, overall agreement, and Gwet's AC1 coefficient. RESULTS: Of all analyzable examinations, the median splenic R2* using the qDixon sequence was 25.75 1/s (range: 5.6-433) and for the ME-GRE sequence 35.35 1/s (range: 10.9-400.8) respectively. Concerning the pancreas, a median R2* of 29.93 1/s (range: 14-111.45) for the qDixon and 31.25 1/s (range: 14-97) for the ME-GRE sequence was found. Bland-Altman analysis showed a mean R2* difference of 2.12 1/s with a CCC of 0.934 for the spleen and of 0.29 1/s with a CCC of 0.714 for the pancreas. Linear regression for the spleen/pancreas resulted in a correlation coefficient of 0.94 (p < 0.001)/0.725 (p < 0.001). Concerning iron overload, the proportion of overall agreement between the two methods was 91.43% for the spleen and 93.18% for the pancreas. CONCLUSIONS: Our data show good concordance between R2* values obtained with a commercial qDixon sequence and a validated ME-GRE relaxometry method. The 3D-qDixon sequence, originally intended for liver assessment, seems to be a reliable tool for non-invasive evaluation of iron content also in the spleen and the pancreas. KEY POINTS: • A 3D chemical shift imaging sequence and 2D multi-gradient echo sequence show good conformity quantifying splenic and pancreatic R2* values. • The 3D chemical shift imaging sequence allows a reliable analysis also of splenic and pancreatic iron status. • In addition to the liver, the analysis of the spleen and pancreas is often helpful for further differential diagnostic clarification and patient guidance regarding the iron status.

Tissue iron distribution in patients with anemia of inflammation: Results of a pilot study.

Anemia of inflammation (AI) is frequently present in subjects with inflammatory disorders, primarily caused by inflammation-driven iron retention in macrophages. So far, only limited data on qualitative and quantitative estimates of tissue iron retention in AI patients exist. We performed a prospective cohort study analyzing splenic, hepatic, pancreatic, and cardiac iron content with MRI-based R2*-relaxometry in AI patients, including subjects with concomitant true iron deficiency (AI+IDA) hospitalized between 05/2020-01/2022. Control groups were individuals without inflammation. Spleen R2* values in AI patients with ferritin ≤200 µg/L (AI+IDA) were comparable with those found in controls. In AI patients with ferritin >200 µg/L, spleen (47.6 s-1 vs. 19.3 s-1 , p < .001) and pancreatic R2* values (32.5 s-1 vs. 24.9 s-1 , p = .011) were significantly higher compared with controls, while liver and heart R2*-values did not differ. Higher spleen R2* values were associated with higher ferritin, hepcidin, CRP, and IL-6 concentrations. Spleen R2* values normalized in AI patients after recovery (23.6 s-1 vs. 47.6 s-1 , p = .008), while no changes were found in patients with baseline AI+IDA. This is the first study investigating tissue iron distribution in patients with inflammatory anemia and AI with concomitant true iron deficiency. The results support the findings in animal models demonstrating iron retention in macrophages, which are primarily accumulating in the spleen under inflammatory conditions. MRI-related iron measurement may help to better characterize actual iron needs and to define better biomarker thresholds in the diagnosis of true ID in patients with AI. It may qualify as a useful diagnostic method to estimate the need for iron supplementation and to guide therapy.

Operative versus conservative treatment of acute Achilles tendon ruptures: preliminary results of clinical outcome, kinematic MRI and contrast-enhanced ultrasound.

INTRODUCTION: There is no uniform consensus on the gold standard therapy for acute Achilles tendon rupture. The aim of this pilot study was to compare operative and conservative treatment regarding imaging findings and clinical outcome. MATERIALS AND METHODS: Surgically or conservatively treated patients with acute Achilles tendon rupture were retrospectively evaluated. Differences in tendon length and diameter with and without load were analysed using kinematic MRI, tendon perfusion, structural alterations, movement and scar tissue by means of grey-scale and contrast-enhanced ultrasound (CEUS). Intra- and interobserver agreement were recorded. RESULTS: No significant difference was detected regarding clinical outcome, B mode ultrasonography, contrast-enhanced sonography or MRI findings, although alterations in MRI-based measurements of tendon elasticity were found for both groups. Considerable elongation and thickening of the injured tendon were detected in both groups. CONCLUSION: Both, conservative and surgical treatment showed comparable outcomes in our preliminary results and may suggest non-inferiority of a conservative approach.

Neurodegeneration in Hepatic and Neurologic Wilson's Disease.

Clinical presentation of Wilson disease (WD) includes hepatic and neurologic manifestations. This study compares subcortical brain regions by magnetic resonance imaging in patients with WD and without neurological symptoms. Distinct atrophy affecting the basal ganglia, accumbens, and hippocampus was present in neurological WD. Cerebellar atrophy was observed in hepatic WD without neurological symptoms.

MRI-Based Iron Phenotyping and Patient Selection for Next-Generation Sequencing of Non-Homeostatic Iron Regulator Hemochromatosis Genes.

BACKGROUND AND AIMS: High serum ferritin is frequent among patients with chronic liver disease and commonly associated with hepatic iron overload. Genetic causes of high liver iron include homozygosity for the p.Cys282Tyr variant in homeostatic iron regulator (HFE) and rare variants in non-HFE genes. The aims of the present study were to describe the landscape and frequency of mutations in hemochromatosis genes and determine whether patient selection by noninvasive hepatic iron quantification using MRI improves the diagnostic yield of next-generation sequencing (NGS) in patients with hyperferritinemia. APPROACH AND RESULTS: A cohort of 410 unselected liver clinic patients with high serum ferritin (defined as ≥200 µg/L for women and ≥300 µg/L for men) was investigated by HFE genotyping and abdominal MRI R2*. Forty-one (10%) patients were homozygous for the p.Cys282Tyr variant in HFE. Of the remaining 369 patients, 256 (69%) had high transferrin saturation (TSAT; ≥45%) and 199 (53%) had confirmed hepatic iron overload (liver R2* ≥70 s-1 ). NGS of hemochromatosis genes was carried out in 180 patients with hepatic iron overload, and likely pathogenic variants were identified in 68 of 180 (38%) patients, mainly in HFE (79%), ceruloplasmin (25%), and transferrin receptor 2 (19%). Low spleen iron (R2* <50 s-1 ), but not TSAT, was significantly associated with the presence of mutations. In 167 patients (93%), no monogenic cause of hepatic iron overload could be identified. CONCLUSIONS: In patients without homozygosity for p.Cys282Tyr, coincident pathogenic variants in HFE and non-HFE genes could explain hyperferritinemia with hepatic iron overload in a subset of patients. Unlike HFE hemochromatosis, this type of polygenic hepatic iron overload presents with variable TSAT. High ferritin in blood is an indicator of the iron storage disease, hemochromatosis. A simple genetic test establishes this diagnosis in the majority of patients affected. MRI of the abdomen can guide further genetic testing.

Injuries to the anterolateral ligament are observed more frequently compared to lesions to the deep iliotibial tract (Kaplan fibers) in anterior cruciate ligamant deficient knees using magnetic resonance imaging.

PURPOSE: To determine the accuracy of detection, injury rate and inter- and intrarater reproducibility in visualizing lesions to the anterolateral ligament (ALL) and the deep portion of the iliotibial tract (dITT) in anterior cruciate ligament (ACL) deficient knees. METHODS: Ninety-one consecutive patients, out of those 25 children (age 14.3 ± 3.5 years), with diagnosed ACL tears were included. Two musculoskeletal radiologists retrospectively reviewed MRI data focusing on accuracy of detection and potential injuries to the ALL or dITT. Lesion were diagnosed in case of discontinued fibers in combination with intra- or peri-ligamentous edema and graded as intact, partial or complete tears. Cohen's Kappa and 95% confidence intervals (95% CI) were determined for inter- and intrarater reliability measures. RESULTS: The ALL and dITT were visible in 52 (78.8%) and 56 (84.8%) of adult-and 25 (100%) and 19 (76.0%) of pediatric patients, respectively. The ALL was injured in 45 (58.5%; partial: 36.4%, compleate: 22.1%) patients. Partial and comleate tears, where visualized in 21 (40.4%) and 16 (30.8%) adult- and seven (28.0%) and one (4%) peditric patients. A total of 16 (21.3%; partial: 13.3%, compleate: 8.0%) dITT injuries were identified. Partal and complete lesions were seen in seven (12.5%) and five (8.9%) adult- and three (15.8%) and one (5.3%) pediatric patients. Combined injuries were visualized in nine (12.7%) patients. Inter-observer (0.91-0.95) and intra-observer (0.93-0.95) reproducibility was high. CONCLUSION: In ACL injured knees, tears of the ALL are observed more frequently compared to lesions to the deep iliotibial tract. Combined injuries of both structures are rare. Clinically, the preoperative visualization of potentially injured structures of the anterolateral knee is crucial and is important for a more personalized preoperative planning and tailored anatomical reconstruction. The clinical implication of injuries to the anterolateral complex of the knee needs further investigation. LEVEL OF EVIDENCE: II.

Performance of different Dixon-based methods for MR liver iron assessment in comparison to a biopsy-validated R2* relaxometry method.

OBJECTIVES: To prospectively evaluate a 3D-multiecho-Dixon sequence with inline calculation of proton density fat fraction (PDFF) and R2* (qDixon), and an improved version of it (qDixon-WIP), for the MR-quantification of hepatic iron in a clinical setting. METHODS: Patients with increased serum ferritin underwent 1.5-T MRI of the liver for the evaluation of hepatic iron overload. The imaging protocol for R2* quantification included as follows: (1) a validated, 2D multigradient-echo sequence (initial TE 0.99 ms, R2*-ME-GRE), (2) a 3D-multiecho-Dixon sequence with inline calculation of PDFF and R2* (initial TE 2.38 ms, R2*-qDixon), and optionally (3) a prototype (works-in-progress, WIP) version of the latter (initial TE 1.04 ms, R2*-qDixon-WIP) with improved water/fat separation and noise-corrected parameter fitting. For all sequences, three manually co-registered regions of interest (ROIs) were placed in the liver. R2* values were compared and linear regression analysis and Bland-Altman plots calculated. RESULTS: Forty-six out of 415 patients showed fat-water (F/W) swap with qDixon and were excluded. A total of 369 patients (mean age 52 years) were included; in 203/369, the optional qDixon-WIP was acquired, which showed no F/W swaps. A strong correlation was found between R2*-ME-GRE and R2*-qDixon (r2 = 0.92, p < 0.001) with Bland-Altman revealing a mean difference of - 3.82 1/s (SD = 21.26 1/s). Correlation between R2*-GRE-ME and R2*-qDixon-WIP was r2 = 0.95 (p < 0.001) with Bland-Altman showing a mean difference of - 0.125 1/s (SD = 30.667 1/s). CONCLUSIONS: The 3D-multiecho-Dixon sequence is a reliable tool to quantify hepatic iron. Results are comparable with established relaxometry methods. Improvements to the original implementation eliminate occasional F/W swaps and limitations regarding maximum R2* values. KEY POINTS: • The 3D-multiecho-Dixon sequence for 1.5 T is a reliable tool to quantify hepatic iron. • Results of the 3D-multiecho-Dixon sequence are comparable with established relaxometry methods. • An improved version of the 3D-multiecho-Dixon sequence eliminates minor drawbacks.

Hide-and-seek: Neurotropes Plattenepithelkarzinom der periorbitalen Region - eine Fallserie und Übersichtsarbeit aktueller Literatur.

Das Plattenepithelkarzinom ist nach dem Basalzellkarzinom das zweithäufigste Malignom der Haut und wird vorwiegend an sonnenexponierten Stellen wie der Gesichtshaut diagnostiziert. Diese meist lokal destruktiv wachsende Malignität kann durchaus auch invasives Wachstumsverhalten, wie perineurale Ausbreitungsmechanismen, aufweisen. Das Plattenepithelkarzinom der periorbitalen Region ist in bis zu 14 % der Fälle mit perineuraler Invasion assoziiert. Vor allem in diesem Bereich birgt die anatomische Nähe zu den Hirnnerven das Risiko einer Progression Richtung zentrales Nervensystem, was mit einer schlechteren Prognose assoziiert ist. Der klinisch unauffällige Charakter dieser Entität resultiert oft in einer Verzögerung der definitiven Diagnosestellung, wodurch die vollständige Resektion und anschließende Rekonstruktion erschwert werden. Eine aufmerksame klinische Evaluierung kann bereits vor Erlangen histologischer Befunde Hinweise für ein perineurales Wachstum liefern. Neben fünf herausfordernden Fällen analysiert diese Arbeit Risikofaktoren, klinische als auch histologische Merkmale und Behandlungsoptionen des periorbitalen Plattenepithelkarzinoms mit perineuraler Invasion.

Hide-and-seek: Neurotropic squamous cell carcinoma of the periorbital region - a series of five cases and review of the literature.

Squamous cell carcinoma is the second most common malignancy of the skin after basal cell carcinoma and mainly found in sun-exposed areas such as the face. This mostly locally destructive malignancy may show invasive growth and insidious mechanisms of dissemination such as perineural invasion. Periorbital squamous cell carcinoma is associated with perineural invasion in up to 14 % of cases. Specifically in this region, the proximity to cranial nerves and therefore the associated risk of progression to the central nervous system are associated with poor prognosis. The clinically concealed character of this entity often leads to a delay in diagnosis and consequently makes complete resection and reconstruction demanding. Careful clinical evaluation often hints at perineural invasion before obtaining histology. Aside from presenting five challenging cases, this work analyzes risk factors, clinical as well as histological features, and treatment options for periorbital squamous cell carcinoma with perineural invasion.

Reduced iron export associated with hepcidin resistance can explain the iron overload spectrum in ferroportin disease.

BACKGROUND & AIMS: Ferroportin disease (FD) and hemochromatosis type 4 (HH4) are associated with variants in the ferroportin-encoding gene SLC40A1. Both phenotypes are characterized by iron overload despite being caused by distinct variants that either mediate reduced cellular iron export in FD or resistance against hepcidin-induced inactivation of ferroportin in HH4. The aim of this study was to assess if reduced iron export also confers hepcidin resistance and causes iron overload in FD associated with the R178Q variant. METHODS: The ferroportin disease variants R178Q andA77D and the HH4-variant C326Y were overexpressed in HEK-293T cells and subcellular localization was characterized by confocal microscopy and flow cytometry. Iron export and cytosolic ferritin were measured as markers of iron transport and radioligand binding studies were performed. The hepcidin-ferroportin axis was assessed by ferritin/hepcidin correlation in patients with different iron storage diseases. RESULTS: In the absence of hepcidin, the R178Q and A77D variants exported less iron when compared to normal and C326Y ferroportin. In the presence of hepcidin, the R178Q and C326Y, but not the A77D-variant, exported more iron than cells expressing normal ferroportin. Regression analysis of serum hepcidin and ferritin in patients with iron overload are compatible with hepcidin deficiency in HFE hemochromatosis and hepcidin resistance in R178Q FD. CONCLUSIONS: These results support a novel concept that in certain FD variants reduced iron export and hepcidin resistance could be interlinked. Evasion of mutant ferroportin from hepcidin-mediated regulation could result in uncontrolled iron absorption and iron overload despite reduced transport function.

Magnetic resonance cholangiopancreatography with compressed sensing at 1.5 T: clinical application for the evaluation of branch duct IPMN of the pancreas.

OBJECTIVES: To evaluate magnetic resonance cholangiopancreatography (MRCP) with compressed sensing (CS) for the assessment of branch duct intraductal papillary mucinous neoplasm (BD-IPMN) of the pancreas. For this purpose, conventional navigator-triggered (NT) sampling perfection with application-optimized contrast using different flip angle evolutions (SPACE) MRCP was compared with various CS-SPACE-MRCP sequences in a clinical setting. METHODS: A total of 41 patients (14 male, 27 female, mean age 68 years) underwent 1.5-T MRCP for the evaluation of BD-IPMN. The MRCP protocol consisted of the following sequences: conventional NT-SPACE-MRCP, CS-SPACE-MRCP with long (BHL, 17 s) and short single breath-hold (BHS, 8 s), and NT-CS-SPACE-MRCP. Two board-certified radiologists evaluated image quality, duct sharpness, duct visualization, lesion conspicuity, confidence, and communication with the main pancreatic duct in consensus using a 5-point scale (1-5), with higher scores indicating better quality/delineation/confidence. Maximum intensity projection reconstructions and originally acquired data were used for evaluation. Wilcoxon signed-rank test was used to compare the intra-individual difference between sequences. RESULTS: BHS-CS-SPACE-MRCP had the highest scores for image quality (3.85 ± 0.79), duct sharpness (3.81 ± 1.05), and duct visualization (3.81 ± 1.01). There was a significant difference compared with NT-CS-SPACE-MRCP (p < 0.05) but no significant difference to the standard NT-SPACE-MRCP (p > 0.05). Concerning diagnostic quality, BHS-CS-SPACE-MRCP had the highest scores in lesion conspicuity (3.95 ± 0.92), confidence (4.12 ± 1.08), and communication (3.8 ± 1.06), significantly higher compared with NT-SPACE-MRCP, BHL-SPACE-MRCP, and NT-CS-SPACE-MRCP (p = <0.05). CONCLUSIONS: MRCP with CS 3D SPACE for the evaluation of BD-IPMN at 1.5 T provides the best results using a short breath-hold sequence. This approach is feasible and an excellent alternative to standard NT 3D MRCP sequences. KEY POINTS: • 1.5-T MRCP with compressed sensing for the evaluation of branch duct IPMN is a feasible method. • Short breath-hold sequences provide the best results for this purpose.

Practical guide to quantification of hepatic iron with MRI.

Our intention is to demystify the MR quantification of hepatic iron (i.e., the liver iron concentration) and give you a step-by-step approach by answering the most pertinent questions. The following article should be more of a manual or guide for every radiologist than a classic review article, which just summarizes the literature. Furthermore, we provide important background information for professional communication with clinicians. The information regarding the physical background is reduced to a minimum. After reading this article, you should be able to perform adequate MR measurements of the LIC with 1.5-T or 3.0-T scanners. KEY POINTS: • MRI is widely accepted as the primary approach to non-invasively determine liver iron concentration (LIC). • This article is a guide for every radiologist to perform adequate MR measurements of the LIC. • When using R2* relaxometry, some points have to be considered to obtain correct measurements-all explained in this article.

Non-contrast MRI protocol for TAVI guidance: quiescent-interval single-shot angiography in comparison with contrast-enhanced CT.

OBJECTIVES: To prospectively compare unenhanced quiescent-interval single-shot MR angiography (QISS-MRA) with contrast-enhanced computed tomography angiography (CTA) for contrast-free guidance in transcatheter aortic valve intervention (TAVI). METHODS: Twenty-six patients (mean age 83 ± 5 years, 15 female [58%]) referred for TAVI evaluation underwent QISS-MRA for aortoiliofemoral access guidance and non-contrast three-dimensional (3D) "whole heart" MRI for prosthesis sizing on a 1.5-T system. Contrast-enhanced CTA was performed as imaging gold standard for TAVI planning. Image quality was assessed by a 4-point Likert scale; continuous MRA and CTA measurements were compared with regression and Bland-Altman analyses. RESULTS: QISS-MRA and CTA-based measurements of aortoiliofemoral vessel diameters correlated moderately to very strong (r = 0.572 to 0.851, all p ≤ 0.002) with good to excellent inter-observer reliability (intra-class correlation coefficient (ICC) = 0.862 to 0.999, all p < 0.0001) regarding QISS assessment. Mean diameters of the infrarenal aorta and iliofemoral vessels differed significantly (bias 0.37 to 0.98 mm, p = 0.041 to < 0.0001) between the two modalities. However, inter-method decision for transfemoral access route was comparable (κ = 0.866, p < 0.0001). Aortic root parameters assessed by 3D whole heart MRI strongly correlated (r = 0.679 to 0.887, all p ≤ 0.0001) to CTA measurements. CONCLUSION: QISS-MRA provides contrast-free access route evaluation in TAVI patients with moderate to strong correlations compared with CTA and substantial inter-observer agreement. Despite some significant differences in minimal vessel diameters, inter-method agreement for transfemoral accessibility is strong. Combination with 3D whole heart MRI facilitates unenhanced TAVI guidance. KEY POINTS: • QISS-MRA and CTA inter-method agreement for transfemoral approach is strong. • QISS-MRA is a very good alternative to CTA and MRA especially in patients with Kidney Disease Outcomes Quality Initiativestages 4 and 5. • Combination of QISS-MRA and 3D "whole heart" MRI facilitates fully unenhanced TAVI guidance.

Thermal ablation of CT 'invisible' liver tumors using MRI fusion: a case control study.

Purpose: To evaluate whether 'invisible' liver tumors on CT can be treated by stereotactic radiofrequency ablation (SRFA) using fusion of pre-ablation MRI.Methods: In this retrospective case-control study, 60 patients (14 with Hepatocellular carcinoma (HCC) and 46 with metastatic liver tumors) with CT 'invisible' lesions underwent SRFA using MRI-fusion between June 2005 and June 2018 ('fusion group'). For comparison, 60 patients who underwent SRFA without image fusion were selected using nearest neighbor propensity score matching ('control group'). Endpoints consisted of local tumor control, safety, overall and disease-free survival.Results: Major complications occurred in 6/69 ablations (8.7%) in the fusion group and in 6/89 ablations (6.7%) in the control group (p = 0.434). Primary technical efficacy rate (i.e., successful initial ablation) was 96.6% (28/29) for HCC and 97.9% (166/170) for metastatic disease in the fusion group and 100% (33/33) and 93.3% (184/194) in the control group, respectively (p = 0.468 and 0.064). Local recurrence (LR) was observed in 1/29 (3.5%) HCCs and in 6/170 metastases (4.0%) in the fusion group and 1/33 (3.0%) and 21/196 (10.7%) in the control group, respectively. The LR rate of metastasis in the control group was significantly higher (p = 0.007), although differences in OS and DFS did not reach statistical significance.Conclusions: Image fusion using pre-procedural MRI allows for ablation of CT-'invisible' liver tumors that are otherwise untreatable. Moreover, local oncological control was higher in metastatic liver tumors versus matched controls which suggests it could be useful tool for all stereotactic radiofrequency ablation procedures.

[Magnetic resonance elastography of the liver : Worth knowing for clinical routine]. / Magnetresonanzelastographie der Leber : Wissenswertes zur klinischen Routine.

BACKGROUND: Magnetic resonance elastography (MRE) is a noninvasive, quantitative, MRI-based method to evaluate liver stiffness. Beside biopsy and ultrasound elastography, this imaging method plays in many places a significant role in the detection and additive characterization of chronic liver disease. OBJECTIVES, MATERIALS AND METHODS: Based on the literature, a brief review of the underlying method and the commercially available products is given. Furthermore, the practical procedure, the analysis, and the interpretation of clinically relevant questions are illustrated and a comparison with ultrasound elastography is provided. RESULTS: This relative "young" MRI method allows extensive evaluation of mechanical properties of the liver and is an important diagnostic tool especially in follow-up examinations. The MRE of the liver is with a maximum technical failure rate of 5.8% a robust technique with high accuracy and an excellent re-test reliability as well as intra- and interobserver reproducibility. There is a high diagnostic certainty within the framework of most important clinical indications, the quantification of fibrosis, and with a very good correlation with the "gold standard" biopsy. CONCLUSION: Based on its rising clinical relevance and the broad usage, MRE of the liver is increasingly used in many centers and in routine liver protocols. Therefore, basic knowledge of this method is essential for every radiologist.