title: Longitudinal Transient Elastography Measurements Used in Follow-up for Patients with Cystic Fibrosis.
abstract: Cystic fibrosis-related liver disease (CFLD) is diagnosed using a combination of criteria. Transient elastography (TE), an ultrasonographic method to evaluate liver stiffness, can differentiate patients with and without liver disease. This retrospective study (2007-2013) aimed to detect developing CFLD using consequent TE measurements. All cystic fibrosis patients with TE measurements between 2007 and 2013 (n = 150, median age 17 (9-24) y) were included, of which 118 had a median of three (range, 2-4) measurements with an interval of 1 (1-2) y. Twenty (14%) had CFLD at the first TE measurement; five (3%) developed CFLD during follow-up. The median TE value in CFLD was 14 kPa (8.7-32.2) compared with 5.3 (4.9-5.7) in cystic fibrosis patients without liver disease (CFnoLD; p = 0.0001). In CFnoLD, TE was correlated with age (p = 0.031). A TE result >6.8 kPa had a sensitivity of 91.5% and a specificity of 91.7% in predicting CFLD, according to the receiver operating characteristics analysis. It also has a positive predictive value of 88.6% and a negative predictive value of 86.9%, increasing to 91.7% and 98%, respectively, in patients at risk (<14 y) for developing CFLD. Patients with developing CFLD had progressively increasing consecutive TE measurements.
authors.affiliation: Cystic fibrosis Centre Ghent, Ghent University Hospital, Ghent, Belgium; Department of Pediatric Gastroenterology and Hepatology, Ghent University Hospital, Ghent, Belgium. Electronic address: stephanie.vanbiervliet@ugent.be.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Van Biervliet
authors.affiliation: Department of Adult Hepatology, Ghent University Hospital, Ghent, Belgium.
authors.forename: Hugo
authors.initials: H
authors.lastname: Verdievel
authors.affiliation: Department of Pediatric Gastroenterology and Hepatology, Ghent University Hospital, Ghent, Belgium.
authors.forename: Saskia
authors.initials: S
authors.lastname: Vande Velde
authors.affiliation: Department of Pediatric Gastroenterology and Hepatology, Ghent University Hospital, Ghent, Belgium.
authors.forename: Ruth
authors.initials: R
authors.lastname: De Bruyne
authors.affiliation: Cystic fibrosis Centre Ghent, Ghent University Hospital, Ghent, Belgium.
authors.forename: Danny
authors.initials: D
authors.lastname: De Looze
authors.affiliation: Department of Adult Hepatology, Ghent University Hospital, Ghent, Belgium.
authors.forename: Xavier
authors.initials: X
authors.lastname: Verhelst
authors.affiliation: Department of Adult Hepatology, Ghent University Hospital, Ghent, Belgium.
authors.forename: Anja
authors.initials: A
authors.lastname: Geerts
authors.affiliation: Cystic fibrosis Centre Ghent, Ghent University Hospital, Ghent, Belgium.
authors.forename: Eddy
authors.initials: E
authors.lastname: Robberecht
authors.affiliation: Department of Adult Hepatology, Ghent University Hospital, Ghent, Belgium.
authors.forename: Hans
authors.initials: H
authors.lastname: Van Vlierberghe
mesh_terms: D000293:Adolescent; D000375:Aging; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D055119:Elastic Modulus; D054459:Elasticity Imaging Techniques / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008107:Liver Diseases / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D008137:Longitudinal Studies; D008297:Male; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D055815:Young Adult
keywords: Cystic fibrosis; Fibroscan; Liver disease; Portal ultrasound; Transient elastography