Wilson Disease and Alpha1-Antitrypsin Deficiency: A Review of Non-Invasive Diagnostic Tests

被引:1
作者
Guillaud, Olivier [1 ,2 ,3 ]
Dumortier, Jerome [1 ,3 ,4 ]
Couchonnal-Bedoya, Eduardo [1 ,5 ]
Ruiz, Mathias [5 ,6 ]
机构
[1] Hosp Civils Lyon, Hop Femme Mere Enfant, Ctr Natl Reference Malad Wilson, F-69500 Bron, France
[2] Clin Sauvegarde, Ramsay Gen Sante, F-69009 Lyon, France
[3] Hosp Civils Lyon, Hop Edouard Herriot, Federat Special Digest, F-69003 Lyon, France
[4] Univ Claude Bernard Lyon 1, Fac Med Lyon Est, F-69003 Lyon, France
[5] Hop Femme Mere Enfant, Serv Hepatogastroenterol & Nutr Pediat, Hosp Civils Lyon, F-69500 Bron, France
[6] Hop Femme Mere Enfant, Ctr Natl Reference Atresie Voies Biliaires & Chole, Hosp Civils Lyon, F-69500 Bron, France
关键词
Wilson disease; alpha1-antitrypsin deficiency; non-invasive tests; diagnosis; URINARY COPPER EXCRETION; RELATIVE EXCHANGEABLE COPPER; KAYSER-FLEISCHER RING; ALPHA-1-ANTITRYPSIN DEFICIENCY; LIVER-DISEASE; PENICILLAMINE CHALLENGE; TRANSIENT ELASTOGRAPHY; POSITION PAPER; CHILDREN; FIBROSIS;
D O I
10.3390/diagnostics13020256
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
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页数:18
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