Cargando…
Wilson Disease and Alpha1-Antitrypsin Deficiency: A Review of Non-Invasive Diagnostic Tests
Wilson disease and alpha1-antitrypsin deficiency are two rare genetic diseases that may impact predominantly the liver and/or the brain, and the liver and/or the lung, respectively. The early diagnosis of these diseases is important in order to initiate a specific treatment, when available, ideally...
Autores principales: | , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9857715/ https://www.ncbi.nlm.nih.gov/pubmed/36673066 http://dx.doi.org/10.3390/diagnostics13020256 |
_version_ | 1784873929364471808 |
---|---|
author | Guillaud, Olivier Dumortier, Jérôme Couchonnal-Bedoya, Eduardo Ruiz, Mathias |
author_facet | Guillaud, Olivier Dumortier, Jérôme Couchonnal-Bedoya, Eduardo Ruiz, Mathias |
author_sort | Guillaud, Olivier |
collection | PubMed |
description | Wilson disease and alpha1-antitrypsin deficiency are two rare genetic diseases that may impact predominantly the liver and/or the brain, and the liver and/or the lung, respectively. The early diagnosis of these diseases is important in order to initiate a specific treatment, when available, ideally before irreversible organ damage, but also to initiate family screening. This review focuses on the non-invasive diagnostic tests available for clinicians in both diseases. These tests are crucial at diagnosis to reduce the potential diagnostic delay and assess organ involvement. They also play a pivotal role during follow-up to monitor disease progression and evaluate treatment efficacy of current or emerging therapies. |
format | Online Article Text |
id | pubmed-9857715 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-98577152023-01-21 Wilson Disease and Alpha1-Antitrypsin Deficiency: A Review of Non-Invasive Diagnostic Tests Guillaud, Olivier Dumortier, Jérôme Couchonnal-Bedoya, Eduardo Ruiz, Mathias Diagnostics (Basel) Review Wilson disease and alpha1-antitrypsin deficiency are two rare genetic diseases that may impact predominantly the liver and/or the brain, and the liver and/or the lung, respectively. The early diagnosis of these diseases is important in order to initiate a specific treatment, when available, ideally before irreversible organ damage, but also to initiate family screening. This review focuses on the non-invasive diagnostic tests available for clinicians in both diseases. These tests are crucial at diagnosis to reduce the potential diagnostic delay and assess organ involvement. They also play a pivotal role during follow-up to monitor disease progression and evaluate treatment efficacy of current or emerging therapies. MDPI 2023-01-10 /pmc/articles/PMC9857715/ /pubmed/36673066 http://dx.doi.org/10.3390/diagnostics13020256 Text en © 2023 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 | Review Guillaud, Olivier Dumortier, Jérôme Couchonnal-Bedoya, Eduardo Ruiz, Mathias Wilson Disease and Alpha1-Antitrypsin Deficiency: A Review of Non-Invasive Diagnostic Tests |
title | Wilson Disease and Alpha1-Antitrypsin Deficiency: A Review of Non-Invasive Diagnostic Tests |
title_full | Wilson Disease and Alpha1-Antitrypsin Deficiency: A Review of Non-Invasive Diagnostic Tests |
title_fullStr | Wilson Disease and Alpha1-Antitrypsin Deficiency: A Review of Non-Invasive Diagnostic Tests |
title_full_unstemmed | Wilson Disease and Alpha1-Antitrypsin Deficiency: A Review of Non-Invasive Diagnostic Tests |
title_short | Wilson Disease and Alpha1-Antitrypsin Deficiency: A Review of Non-Invasive Diagnostic Tests |
title_sort | wilson disease and alpha1-antitrypsin deficiency: a review of non-invasive diagnostic tests |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9857715/ https://www.ncbi.nlm.nih.gov/pubmed/36673066 http://dx.doi.org/10.3390/diagnostics13020256 |
work_keys_str_mv | AT guillaudolivier wilsondiseaseandalpha1antitrypsindeficiencyareviewofnoninvasivediagnostictests AT dumortierjerome wilsondiseaseandalpha1antitrypsindeficiencyareviewofnoninvasivediagnostictests AT couchonnalbedoyaeduardo wilsondiseaseandalpha1antitrypsindeficiencyareviewofnoninvasivediagnostictests AT ruizmathias wilsondiseaseandalpha1antitrypsindeficiencyareviewofnoninvasivediagnostictests |