Cystic Fibrosis-related liver disease: clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies Authors

Jérémy Dana (1, 2, 3) , Dominique Debray (4) , Aurélie Beaufrère (5) , Sophie Hillaire , Monique Fabre (6) , Caroline Reinhold (7, 8) , Thomas Baumert (2, 3) , Laureline Berteloot (9) , Valérie Vilgrain (1)

1 Department of Radiology, AP-HP, Beaujon University Hospital Paris Nord, F-92110 Clichy
2 IVH - Institut de Recherche sur les Maladies Virales et Hépatiques
3 IHU Strasbourg - L'Institut hospitalo-universitaire de Strasbourg
4 ICAN - Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases
5 Département de Pathologie [Hôpital Beaujon]
6 Service de pathologie [CHU Necker]
7 McGill University = Université McGill [Montréal, Canada]
8 RI-MUHC - Research Institute of the McGill University Health Centre
9 Service de radiologie pédiatrique [CHU Necker]

Cystic Fibrosis-related Liver Disease, related to the underlying CFTR defect, includes two main, sometimes coexisting, manifestations: focal biliary fibrosis and porto-sinusoidal vascular disease. - Portal hypertension, either as a direct complication of biliary cirrhosis or secondary to porto-sinusoidal vascular disease, should be carefully assessed because it may develop in the absence of clinically significant portal hypertension and, 3) a coarse macronodular pattern suggesting regenerative nodular hyperplasia.

Mots clés: Cystic Fibrosis Liver Disease Focal Biliary Fibrosis Porto-sinusoidal Vascular Disease Noninvasive Diagnostic Radio-pathologic correlations; Cystic Fibrosis Liver Disease; Focal Biliary Fibrosis; Porto-sinusoidal Vascular Disease; Noninvasive Diagnostic; Radio-pathologic correlations

Domaines: Hépatologie et Gastroentérologie; Imagerie médicale

Jérémy Dana, Dominique Debray, Aurélie Beaufrère, Sophie Hillaire, Monique Fabre, et al.. Cystic Fibrosis-related liver disease: clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies Authors. Journal of Hepatology, 2022, 76 (2), pp.420-434. ⟨10.1016/j.jhep.2021.09.042⟩. ⟨hal-04044123⟩