title: Noninvasive methods, including transient elastography, for the detection of liver disease in adults with cystic fibrosis.
abstract: BACKGROUND
  Liver disease is the third leading cause of mortality in patients with cystic fibrosis (CF). However, detection of CF-associated liver disease (CFLD) is challenging.
  
  
  OBJECTIVE
  To evaluate the diagnostic performance of noninvasive methods for the detection of CFLD with a focus on transient elastography (TE).
  
  
  METHODS
  Patients at the Adult CF Clinic of Calgary and Southern Alberta (n=127) underwent liver stiffness measurement (LSM) by TE using the FibroScan (FS, Ecosens, France) M probe; aspartate aminotransferase to platelet ratio index (APRI) and FibroTest (FT) scores were also calculated. The diagnostic performance of these tools for the detection of CFLD (defined as two or more the following criteria: abnormal liver biochemistry, hepatomegaly or sonographic abnormalities other than steatosis) were compared using the area under ROC curves.
  
  
  RESULTS
  Forty-seven percent of the cohort was male. The median age was 27 years (interquartile range [IQR] 22 to 37 years) and body mass index 21 kg⁄m(2) (IQR 19 kg⁄m(2) to 23 kg⁄m(2)); 25% of patients were on ursodeoxycholic acid and 12% had undergone lung transplantation. The prevalence of CFLD was 14% (n=18). FS was successful in all patients; one (0.8%) patient had poorly reliable results (IQR⁄M >30% and LSM ≥7.1 kPa). Compared with patients without CFLD (n=109), individuals with CFLD had higher median LSM according to FS (3.9 kPa [IQR 3.4 to 4.9 kPa] versus 6.4 kPa [IQR 4.4 to 8.0 kPa]), APRI (0.24 [IQR 0.17 to 0.31] versus 0.50 [IQR 0.22 to 1.18]) and FT scores (0.08 [IQR 0.05 to 1.5] versus 0.18 [IQR 0.11 to 0.35]; all P<0.05). Area under ROC curve for FS, APRI and FT for the detection of CFLD were 0.78 (95% CI 0.65 to 0.92), 0.72 (95% CI 0.56 to 0.87) and 0.76 (95% CI 0.62 to 0.90) (P not significant). At a threshold of >5.2 kPa, the sensitivity, specificity, positive and negative predictive values of LSM according to FS for detecting CFLD were 67%, 83%, 40% and 94%, respectively.
  
  
  CONCLUSIONS
  FS, APRI and FT were useful noninvasive methods for detecting CFLD in adults.
authors: Matthew D
authors: MD
authors: Sadler
authors: Pam
authors: P
authors: Crotty
authors: Linda
authors: L
authors: Fatovich
authors: Stephanie
authors: S
authors: Wilson
authors: Harvey R
authors: HR
authors: Rabin
authors: Robert P
authors: RP
authors: Myers
mesh_terms: D000328:Adult; D000416:Alberta / Q000453:epidemiology; D001219:Aspartate Aminotransferases / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000150:complications*; D054459:Elasticity Imaging Techniques / Q000379:methods; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000097:blood / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology; D008297:Male; D010976:Platelet Count; D011237:Predictive Value of Tests; D015995:Prevalence; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index
chemical_list: D015415:Biomarkers; D001219:Aspartate Aminotransferases