4.6 Review

Elastography of the Liver in Wilson's Disease

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Application of attenuation coefficient in the assessment of hepatic involvement in children and adolescents with Wilson's disease

Jiajia Wang et al.

Summary: This study aimed to investigate the use of the attenuation coefficient (ATT) as a noninvasive index to assess liver involvement in children and adolescents with Wilson's disease (WD). The results showed that ATT can be used as a noninvasive index for evaluating liver steatosis in WD patients, and the combination of ATT with APRI, FIB-4, and SWM may improve diagnostic efficacy.

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Accuracy of the ultrasound attenuation coefficient for the evaluation of hepatic steatosis: a systematic review and meta-analysis of prospective studies

Jong Keon Jang et al.

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Clinical relevance of shear wave elastography compared with transient elastography and other markers of liver fibrosis

Oyekoya T. Ayonrinde et al.

Summary: This study compares the application of SWE, TE, and clinical markers in patients with various liver diseases and finds that SWE is at least comparable to TE and other non-invasive tests for liver fibrosis. SWE is highly accurate in predicting liver-related portal hypertension.

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Roberto de Franchis et al.

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Liver fibrosis assessment: MR and US elastography

Arinc Ozturk et al.

Summary: Elastography has become a preferred non-invasive imaging technique for clinical assessment of liver fibrosis, providing liver stiffness measurement as a surrogate biomarker for fibrosis burden. Both ultrasound and MRI-based elastography methods have shown to be accurate in detecting liver diseases.

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Correlation Between QElaXto Techniques and Supersonic Imagine for Liver Stiffness Quantification in Chronic Liver Disease

Matteo Garcovich et al.

Summary: The study investigated the agreement between different equipment for liver stiffness quantification, showing a very good correlation between QElaXto techniques and SSI in LS measurements. Cut-off thresholds for fibrosis staging in non-HBV-related CLD were identified using two QElaXto techniques for the first time.

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Simplification of 2D shear wave elastography by enlarged SWE box and multiple regions of interest in one acquisition

Moritz Weiher et al.

Summary: This study improves and compares the method of using Two-dimensional Shear Wave Elastography (SWE) to evaluate liver stiffness. The results show that there is good reliability between one acquisition with increased size of the SWE-box and ten different ROI measurements and ten separate acquisitions with a single ROI measurement, if the mean shear-wave-speed is below 1.6m/s (7.7kPa).

PLOS ONE (2022)

Article Gastroenterology & Hepatology

Evaluation of the Liver and Pancreas by 2D Shear Wave Elastography in Pediatric Wilson's Disease

Sibel Yavuz et al.

Summary: The study aimed to investigate parenchymal changes in the liver and pancreas related to copper accumulation in pediatric patients with Wilson's disease and explore the effectiveness of two-dimensional shear wave elastography in diagnosing organ involvement. The results showed increased liver tissue stiffness and decreased pancreatic tissue stiffness in Wilson's disease patients compared to healthy controls. Two-dimensional shear wave elastography is an easy to use, non-invasive, and promising method for objectively monitoring the early changes in tissue stiffness in Wilson's disease patients.

TURKISH JOURNAL OF GASTROENTEROLOGY (2022)

Article Gastroenterology & Hepatology

Evaluation of liver fibrosis in patients with Wilson's disease

Adam Przybylkowski et al.

Summary: CPA is correlated with Ishak score, while transient elastography results are highly correlated with SWE measurements. However, transient elastography or SWE did not show significant correlation with Ishak score or CPA.

EUROPEAN JOURNAL OF GASTROENTEROLOGY & HEPATOLOGY (2021)

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Fibroscan® probe selection for lean adults

Vanessa Stadlbauer et al.

Summary: The study aimed to compare liver stiffness assessments using Fibroscan (R) S+ and M+ probes, as well as controlled attenuation parameter (only available for M+ probe), in healthy volunteers with a thoracic circumference <= 75 cm and >75 cm. Results showed that intake of a light meal had no relevant influence on liver stiffness or CAP measurements in both groups, indicating that lean adults with a thoracic circumference below 75 cm can be assessed with either probe and may eat before assessment.

JGH OPEN (2021)

Article Gastroenterology & Hepatology

Long-term outcome of Wilson's disease complicated by liver disease

Satoko Arai et al.

Summary: This study observed the long-term liver disease status of Wilson's disease patients and found that continuous treatment could effectively prolong survival, prevent liver function deterioration, and reduce the risk of hepatocellular carcinoma. Treatment interruption should be avoided to achieve better prognosis.

JGH OPEN (2021)

Article Pharmacology & Pharmacy

HR-MS Based Untargeted Lipidomics Reveals Characteristic Lipid Signatures of Wilson's Disease

Yixiao Zhi et al.

Summary: The study found that in Wilson's disease patients, the levels of saturated triglycerides increase while other unsaturated lipids decrease. Some serum lipid species may be used as biomarkers for Wilson's disease. Additionally, a similar lipid profile was also found in relatives, suggesting that these changes may be related to mutations in the ATP7B gene.

FRONTIERS IN PHARMACOLOGY (2021)

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Diagnostic accuracy of elastography and magnetic resonance imaging in patients with NAFLD: A systematic review and meta-analysis

Emmanuel Anandraj Selvaraj et al.

Summary: Non-invasive tests measuring liver stiffness or using MRI have acceptable diagnostic accuracy for advanced fibrosis and cirrhosis. The potential clinical impact of these tests cannot be fully assessed due to lack of intention-to-diagnose analyses and validation of pre-specified thresholds.

JOURNAL OF HEPATOLOGY (2021)

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EASL Clinical Practice Guidelines on non-invasive tests for evaluation of liver disease severity and prognosis-2021 update

Annalisa Berzigotti et al.

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Attenuation coefficient (ATT) measurement for liver fat quantification in chronic liver disease

Nobuharu Tamaki et al.

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Systematic Review with Meta-Analyses: Diagnostic Accuracy of FibroMeter Tests in Patients with Non-Alcoholic Fatty Liver Disease

Anne-Marieke van Dijk et al.

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Association of shear-wave elastography with clinical outcomes post-liver transplantation

Tom Boeken et al.

Summary: In this study, liver-stiffness values on 2D-SWE were found to be significantly higher in liver transplant patients without complications during the first 90 days post-transplantation compared to after 1 year. Patients with biliary complications had significantly higher liver-stiffness values on 2D-SWE compared to those without complications.

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Liver pathology in Wilson's disease: From copper overload to cirrhosis

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