Assessment of liver fibrosis by transient elastography (Fibroscan ® ) in patients with A1AT deficiency.

Autor: Guillaud O; Service d'hépato-gastro-entérologie, hôpital Édouard-Herriot, hospices civils de Lyon, 69437 Lyon, France. Electronic address: olivier.guillaud@chu-lyon.fr., Dumortier J; Service d'hépato-gastro-entérologie, hôpital Édouard-Herriot, hospices civils de Lyon, 69437 Lyon, France; Université Claude-Bernard Lyon 1, 69100 Villeurbanne, France. Electronic address: jerome.dumortier@chu-lyon.fr., Traclet J; Service de pneumologie, groupement hospitalier Est, hospices civils de Lyon, 69677 Bron, France. Electronic address: julie.traclet@chu-lyon.fr., Restier L; Service de gastro-entérologie, hépatologie et nutrition pédiatriques, hôpital Femme-Mère-Enfant, hospices civils de Lyon, 69677 Bron, France. Electronic address: lioara.restier@chu-lyon.fr., Joly P; Unité de pathologie moléculaire du globule rouge, laboratoire de biochimie et de biologie moléculaire, hôpital Édouard-Herriot, hospices civils de Lyon, 69437 Lyon, France. Electronic address: philippe.joly@chu-lyon.fr., Chapuis-Cellier C; Université Claude-Bernard Lyon 1, 69100 Villeurbanne, France; Laboratoire d'immunologie, centre de biologie Sud, centre hospitalier Lyon-Sud, hospices civils, 69310 Pierre-Bénite, France. Electronic address: colette.cellier@chu-lyon.fr., Lachaux A; Université Claude-Bernard Lyon 1, 69100 Villeurbanne, France; Service de gastro-entérologie, hépatologie et nutrition pédiatriques, hôpital Femme-Mère-Enfant, hospices civils de Lyon, 69677 Bron, France. Electronic address: alain.lachaux@chu-lyon.fr., Mornex JF; Service de pneumologie, groupement hospitalier Est, hospices civils de Lyon, 69677 Bron, France; Université de Lyon, Inra, UMR754, 69007, Lyon, France. Electronic address: jean-francois.mornex@chu-lyon.fr.
Jazyk: angličtina
Zdroj: Clinics and research in hepatology and gastroenterology [Clin Res Hepatol Gastroenterol] 2019 Feb; Vol. 43 (1), pp. 77-81. Date of Electronic Publication: 2019 Jan 03.
DOI: 10.1016/j.clinre.2018.08.016
Abstrakt: Background: Alpha-1-antitrypsin deficiency (A1ATD) is a common genetic condition which predisposes to emphysema and liver disorders. It is estimated that 10-15% of homozygous individuals for the Z allele (PiZZ) may develop liver fibrosis. The optimal modalities to detect liver disease in PiZZ adult patients need to be defined. The aim of this prospective study was to perform a systematic non-invasive evaluation of the liver fibrosis by elastometry using Fibroscan ® in a cohort of A1ATD patients with emphysema.
Methods: Patients followed in our respiratory unit were enrolled in this prospective study and underwent on the same day a physical examination, a biochemical profiling, an abdominal ultrasound (US) and a Fibroscan ® .
Results: Twenty-nine PiZZ adults (19 male) were included. Median age was 50.4 yrs (21.5-67.2). Median serum A1AT level was 0.20 g/L (0.15-0.33). Liver Function Tests (LFT) were not normal in 2 patients and US was abnormal in 6 patients. Two patients had both abdnormal LFT and US. Fibroscan ® was technically feasible in 28/29 (97%) patients. Median liver stiffness was 4.5 kPa (2.8-32.8), and was > 7.2 kPa in 5/28 (18%) and > 14 kPa in 2/28 (7%) patients. Liver stiffness was increased in 2/2 (100%) patients with abnormal LFT and US, in 1/4 (25%) with abnormal LFT or US and in 2/22 (10%) patients with normal LFT and US.
Conclusions: Fibroscan ® is an easy and repeatable tool which can be used in PiZZ patients to screen for the presence of significant liver fibrosis and to identify patients at higher risk to develop liver complications in the future and who may benefit from a closer surveillance.
(Copyright © 2018 Elsevier Masson SAS. All rights reserved.)
Databáze: MEDLINE
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