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Quantitative MR in Paediatric Patients with Wilson Disease: A Case Series Review
Wilson disease (WD) is a liver disorder characterized by improper copper metabolism. Although non-invasive tools are currently used to support diagnosis and management, this is still an area of unmet need, as patients present with a wide range of symptoms. Our aim was to investigate the potential ut...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9139168/ https://www.ncbi.nlm.nih.gov/pubmed/35626790 http://dx.doi.org/10.3390/children9050613 |
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author | Janowski, Kamil Shumbayawonda, Elizabeth Kelly, Matt Ferreira, Carlos Pronicki, Maciej Grajkowska, Wieslawa Naorniakowska, Magdalena Pawliszak, Piotr Chełstowska, Sylwia Jurkiewicz, Elżbieta Banerjee, Rajarshi Socha, Piotr |
author_facet | Janowski, Kamil Shumbayawonda, Elizabeth Kelly, Matt Ferreira, Carlos Pronicki, Maciej Grajkowska, Wieslawa Naorniakowska, Magdalena Pawliszak, Piotr Chełstowska, Sylwia Jurkiewicz, Elżbieta Banerjee, Rajarshi Socha, Piotr |
author_sort | Janowski, Kamil |
collection | PubMed |
description | Wilson disease (WD) is a liver disorder characterized by improper copper metabolism. Although non-invasive tools are currently used to support diagnosis and management, this is still an area of unmet need, as patients present with a wide range of symptoms. Our aim was to investigate the potential utility of multiparametric magnetic resonance imaging (mpMRI) and quantitative magnetic resonance cholangiopancreatography (MRCP+) to support patient management. MRI examinations of 7 children and young adults aged 8–16 years (six at diagnosis) were performed alongside a standard of care clinical and histological examination. Images were quantitatively analyzed to derive metrics of liver (corrected T1 (cT1; fibro-inflammation), MR liver fat (proton density fat fraction; PDFF)), and biliary health (MRCP+). MRI–PDFF provided a more dynamic characterization of fat compared with that provided by ultrasound. Those with elevated histological scores of fibrosis, inflammation, and steatosis had elevated mpMRI values. MRCP+ managed to identify dilatations in the biliary tree which were not observed during the standard of care examination. mpMRI and MRCP+ metrics show early promise as markers to assess both liver and biliary health in Wilson disease. Investigations to understand and explore the utility of these markers are warranted and should be performed. |
format | Online Article Text |
id | pubmed-9139168 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-91391682022-05-28 Quantitative MR in Paediatric Patients with Wilson Disease: A Case Series Review Janowski, Kamil Shumbayawonda, Elizabeth Kelly, Matt Ferreira, Carlos Pronicki, Maciej Grajkowska, Wieslawa Naorniakowska, Magdalena Pawliszak, Piotr Chełstowska, Sylwia Jurkiewicz, Elżbieta Banerjee, Rajarshi Socha, Piotr Children (Basel) Case Report Wilson disease (WD) is a liver disorder characterized by improper copper metabolism. Although non-invasive tools are currently used to support diagnosis and management, this is still an area of unmet need, as patients present with a wide range of symptoms. Our aim was to investigate the potential utility of multiparametric magnetic resonance imaging (mpMRI) and quantitative magnetic resonance cholangiopancreatography (MRCP+) to support patient management. MRI examinations of 7 children and young adults aged 8–16 years (six at diagnosis) were performed alongside a standard of care clinical and histological examination. Images were quantitatively analyzed to derive metrics of liver (corrected T1 (cT1; fibro-inflammation), MR liver fat (proton density fat fraction; PDFF)), and biliary health (MRCP+). MRI–PDFF provided a more dynamic characterization of fat compared with that provided by ultrasound. Those with elevated histological scores of fibrosis, inflammation, and steatosis had elevated mpMRI values. MRCP+ managed to identify dilatations in the biliary tree which were not observed during the standard of care examination. mpMRI and MRCP+ metrics show early promise as markers to assess both liver and biliary health in Wilson disease. Investigations to understand and explore the utility of these markers are warranted and should be performed. MDPI 2022-04-25 /pmc/articles/PMC9139168/ /pubmed/35626790 http://dx.doi.org/10.3390/children9050613 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Case Report Janowski, Kamil Shumbayawonda, Elizabeth Kelly, Matt Ferreira, Carlos Pronicki, Maciej Grajkowska, Wieslawa Naorniakowska, Magdalena Pawliszak, Piotr Chełstowska, Sylwia Jurkiewicz, Elżbieta Banerjee, Rajarshi Socha, Piotr Quantitative MR in Paediatric Patients with Wilson Disease: A Case Series Review |
title | Quantitative MR in Paediatric Patients with Wilson Disease: A Case Series Review |
title_full | Quantitative MR in Paediatric Patients with Wilson Disease: A Case Series Review |
title_fullStr | Quantitative MR in Paediatric Patients with Wilson Disease: A Case Series Review |
title_full_unstemmed | Quantitative MR in Paediatric Patients with Wilson Disease: A Case Series Review |
title_short | Quantitative MR in Paediatric Patients with Wilson Disease: A Case Series Review |
title_sort | quantitative mr in paediatric patients with wilson disease: a case series review |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9139168/ https://www.ncbi.nlm.nih.gov/pubmed/35626790 http://dx.doi.org/10.3390/children9050613 |
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