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Objectives: In cystic fibrosis (CF), liver disease (LD) is the third leading cause of mortality. As liver biopsy was considered inconsistent in CFLD diagnosis, a combination of modalities were utilized in the conventional Debray criteria (DC). More recently, noninvasive liver fibrosis biomarkers were applied by Koh et al (New criteria-NC). In the current study, we aimed to evaluate noninvasive biomarkers for the CFLD diagnosis. Methods: Longitudinal data were collected from a cohort of genetically confirmed CF patients. CFLD was diagnosed by both DC and NC. Apart from transient elastography (TE)>6.8 kPa, biomarkers incorporated in the NC included AST/ALT-ratio (AAR)≥1, FIB-4 index ≥3.25 and APRI >0.50. Results: 62 patients with CF, [56.5% male, age at enrollment 25 (22-31) years], were prospectively followed- up for 33 (28-36) months. Sixteen (25.8%) and 27 (43.5%) patients met DC and NC, respectively. Twenty-four fulfilling NC had at least one positive biomarker (6 TE, 7 AAR, 6 both TE and AAR, 2 both APRI and AAR and 3 both APRI and TE). Thirteen (48.1%) had diffuse LD/cirrhosis by the NC and all had at least one additional parameter classifying them as CFLD. From the 14 (51.8%) with no-diffuse- LD, 64.3%, 14.3% and 21.4% had 2, 3 and 4 of the necessary modalities incorporated in NC, respectively, confirming their classification as CFLD. TE was 100% specific to rule in CFLD but had a moderate sensitivity. Conclusions: NC were able to identify 17.7% more CFLD patients compared to DC. The multiple biomarkers incorporated in NC may enhance the ability to detect CFLD. [ABSTRACT FROM AUTHOR]

Background and aims: Hepatopulmonary syndrome (HPS) has been associated with left atrial enlargement, but the presence of other markers of left and right diastolic and/or systolic cardiac dysfunction has not been clarified. We prospectively evaluated the possible associations between echocardiographical-Doppler findings and HPS. Methods: Seventy-nine cirrhotic patients without endogenous heart or pulmonary disease were included. HPS was diagnosed by the presence of increased arterial-alveolar-difference and intrapulmonary right-to-left shunt at contrast-enhanced transthoracic echocardiography. Echocardiographical systolic and diastolic indices, tissue Doppler imaging (TDI) of mitral and tricuspid annular motion and M-mode echocardiography measurements were recorded. Results: Hepatopulmonary syndrome was diagnosed in 12 (15.2%) patients. Patients with compared with those without HPS had significantly younger age, albumin levels and saturation of oxygen (SaO2) in an erect position, but higher left ventricular end diastolic diameter (LVEDD), ejection fraction, E-wave peak velocity of tricuspid valve, left atrial volume, TDI E wave (early diastolic period) at the right free wall/tricuspid annulus (cm/s) and TDI S wave (systolic) at the left lateral wall/mitral annulus (TDI Smv). In multivariate analysis, the presence of HPS was found to be independently associated with younger age ( P=0.027), lower SaO2 in an erect position ( P=0.023), higher LVEDD ( P=0.019) and higher TDI Smv ( P=0.026). LVEDD and TDI Smv offered good diagnostic accuracy for HPS diagnosis (AUROC/c-statistic: 0.724 and 0.736 respectively). Conclusions: We confirmed that in patients with cirrhosis, the development of HPS is associated with higher cardiac output and hyperdynamic circulation. Left ventricle enlargement and higher systolic velocity in the mitral valve represent satisfactory indirect markers of HPS. [ABSTRACT FROM AUTHOR]