title: Liver Involvement in Patients With PiZZ-Emphysema, Candidates for Lung Transplantation.
abstract: Information about the prevalence and nature of liver disorders in adults with alpha1-antitrypsin deficiency is scarce. At our center, systematic liver biopsy screening is part of the evaluation before lung transplantation (LT) in the emphysema patients with the PiZZ phenotype. Our aim was to report our experience with this prospective screening. Clinical, liver function, and imaging parameters as well as liver histology data were analyzed for 23 consecutive adult patients with PiZZ severe emphysema referred to our center for consideration of LT from 2006 to 2014. Overall 20 (87%) featured chronic liver disease characterized by a chronic inflammation and/or a significant portal fibrosis on histology. Two of the 23 patients (8.7%) had septal fibrosis according to the Metavir and Ishak scores and met our definition of severe chronic liver disease. They were both clinically asymptomatic with normal liver function tests. On abdominal ultrasonography, the liver appeared normal in one patient and with abnormal contours in the other. Our data indicate that in adults with PiZZ-related emphysema being evaluated for LT, most patients had some histologic involvement. The prevalence of severe liver dysfunction is <10%.
authors.affiliation: Service de pneumologie B, Hôpital Bichat, Université Paris 7, Inserm UMR1152, Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Morer
authors.affiliation: Service d'anatomie pathologique, Hôpital Bichat, Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Choudat
authors.affiliation: Service de pneumologie B, Hôpital Bichat, Université Paris 7, Inserm UMR1152, Paris, France.
authors.forename: G
authors.initials: G
authors.lastname: Dauriat
authors.affiliation: Service d'hépatologie, Hôpital Beaujon, Clichy, France.
authors.forename: F
authors.initials: F
authors.lastname: Durand
authors.affiliation: Service d'anatomie pathologique, Hôpital Beaujon, Clichy, France.
authors.forename: D
authors.initials: D
authors.lastname: Cazals-Hatem
authors.affiliation: Service de pneumologie B, Hôpital Bichat, Université Paris 7, Inserm UMR1152, Paris, France.
authors.forename: G
authors.initials: G
authors.lastname: Thabut
authors.affiliation: Service de pneumologie B, Hôpital Bichat, Université Paris 7, Inserm UMR1152, Paris, France.
authors.forename: O
authors.initials: O
authors.lastname: Brugière
authors.affiliation: Service de chirurgie thoracique et vasculaire, Hôpital Bichat, Paris, France.
authors.forename: Y
authors.initials: Y
authors.lastname: Castier
authors.affiliation: Service de pneumologie B, Hôpital Bichat, Université Paris 7, Inserm UMR1152, Paris, France.
authors.forename: H
authors.initials: H
authors.lastname: Mal
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008099:Liver / Q000503:physiopathology*; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D011379:Prognosis; D011446:Prospective Studies; D011656:Pulmonary Emphysema / Q000209:etiology / Q000601:surgery*; D012189:Retrospective Studies; D012307:Risk Factors; D055815:Young Adult; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications*
keywords: clinical research/practice; liver disease; lung disease; lung transplantation/pulmonology