title: Utility of transient elastography in the non-invasive evaluation of cystic fibrosis liver disease.
abstract: BACKGROUND
  Liver disease frequently complicates cystic fibrosis (CF), with CF liver disease (CFLD) a leading cause of death. Liver biopsy is rarely performed because of the patchy nature of the disease. Transient elastography can reliably stage liver fibrosis via liver stiffness measurement (LSM).
  
  
  OBJECTIVE
  To evaluate LSM as a diagnostic tool in adults with CFLD.
  
  
  METHODS
  Fifty adult patients with CF were prospectively studied: 25 with CFLD and 25 without CFLD. The presence of CFLD and portal hypertension (PHT) was assessed according to strict established criteria based on serial biochemistry and imaging. All patients underwent LSM; APRI, Hepascore(®) and Forns score were calculated.
  
  
  RESULTS
  Median LSM was higher in those with CFLD [8.1 kPa (IQR 6.8-9.5) vs. 5.0 kPa (IQR 4.1-5.6); P < 0.001]. On multivariate analysis, LSM was the only variable associated with CFLD (OR 2.74, 95% CI 1.53-4.89; P = 0.001). AUROC for LSM predicting CFLD was 0.87 (95% CI 0.77-0.98) and an LSM ≥ 6.8 kPa predicted CFLD with 76.0% sensitivity and 92.0% specificity. Median LSM was higher in those with PHT [15.7 kPa (IQR 9.2-17.2) vs. 5.4 kPa (IQR 4.3-6.8); P < 0.001]. The AUROC for LSM predicting the presence of PHT was 0.96 (95% CI 0.92-1.00). An LSM cut-off of ≥ 8.9 kPa predicted the presence of PHT with 87.5% sensitivity, 90.5% specificity, 63.6% positive predictive value and 92.9% negative predictive value.
  
  
  CONCLUSIONS
  LSM is an accurate and reliable non-invasive tool in assessing CFLD and PHT. An LSM ≥ 6.8 kPa is highly suggestive of CFLD and an LSM <8.9 kPa reliably excludes PHT.
authors: Department of Gastroenterology, Alfred Hospital, Melbourne, Australia.
authors: Matthew T
authors: MT
authors: Kitson
authors: William W
authors: WW
authors: Kemp
authors: David M
authors: DM
authors: Iser
authors: Eldho
authors: E
authors: Paul
authors: John W
authors: JW
authors: Wilson
authors: Stuart K
authors: SK
authors: Roberts
mesh_terms: D000328:Adult; D019540:Area Under Curve; D016022:Case-Control Studies; D003550:Cystic Fibrosis / Q000150:complications*; D054459:Elasticity Imaging Techniques / Q000379:methods*; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis*; D008107:Liver Diseases / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology*; D008297:Male; D015999:Multivariate Analysis; D011446:Prospective Studies; D012372:ROC Curve; D014739:Victoria