Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population.

Background: Primary sclerosing cholangitis (PSC) is associated with a broad phenotypic spectrum in different populations from diverse ethnic and racial backgrounds. This study aimed to describe the clinical characteristics and outcomes of PSC in a multicenter cohort of patients from Brazil. Methods: Data from the Brazilian Cholestasis Study Group were retrospectively reviewed to assess demographic information and clinical characteristics of PSC, as well as the outcomes, such as transplantation-free survival. Results: This cohort included 210 patients. After excluding 33 (15.7%) patients with PSC and overlap syndrome of autoimmune hepatitis, 177 (97 males, median age 33 (21-42) years) with clear-cut PSC were eligible for this study. Most of the patients (n = 139, 78.5%) were symptomatic, and 104 (58.7%) had advanced PSC at the time of diagnosis. Concurrent inflammatory bowel disease was observed in 78 (58.6%) of the investigated patients (n = 133), and most of them had ulcerative colitis (n = 61, 78.2%). The 1- and 5-year survival free of liver transplantation or death were 92.3 ± 2.1% and 66.9 ± 4.2%, respectively, and baseline advanced PSC, pruritus, and elevated bilirubin levels were independent risk factors for the composite adverse outcome. Females were significantly older and had lower bilirubin levels than males at baseline, but survival was not associated with sex. Approximately 12.4% (n = 22) of patients with PSC died, and 32.8% (n = 58) underwent liver transplantation at a median follow-up time of 5.3 and 3.2 years. Conclusion: Multiethnic Brazilian PSC patients exhibited a less pronounced male predominance and a lower frequency of inflammatory bowel disease than Caucasians. Adverse outcomes were more frequent, probably due to advanced disease at baseline.

Liver hemodynamic patterns in nonalcoholic steatosis: Doppler ultrasonography and histological evaluation.

BACKGROUND: The aim of this study was to evaluate hepatic Doppler ultrasound (US) indices for steatosis diagnosis and grading, having biopsy as reference. METHODS: Doppler and conventional US were performed in 49 healthy volunteers, without risk factors for nonalcoholic fatty liver disease (NAFLD), and in 49 patients with NAFLD and at least one risk factor: obesity, dyslipidemia and/or diabetes mellitus. Significant alcohol intake and hepatitis B or C were exclusion criteria. NAFLD patients were biopsied, and steatosis severity graded histologically. Portal Venous Pulsatility Index (PVI), Hepatic Artery Resistance Index (HARI) and Pulsatility Index (HAPI) were analyzed in hilum. Hepatic vein waveform pattern (HVWP) was classified as triphasic, biphasic or monophasic. Two pathologists analyzed histological samples. ROC curve defined sensitivity and specificity and multivariate analysis defined an equation for classifying patients. RESULTS: In NAFLD group, 89.79% had histologic criteria for non-alcoholic steatohepatitis (NASH). Mild steatosis was present in 44.89%, moderate steatosis in 38.77% and severe steatosis in 16.32%. In NAFLD group, 65.29% were obese and body mass index (BMI) had significant correlation with steatosis grading (r=0.77; P<0.0001). PVI correlated with presence of steatosis (r=-0.69, P<0.0001) as HVWP (r=-0.61, P< 0.0001). PVI ideal cutoff for predicting steatosis was 0.26 (sensitivity, 91%; specificity, 79.6%). The equation 16.15PVI+1.96HVWP enables to differentiate the healthy and the steatosis patients. HARI and HAPI could not differentiate the healthy from the steatosis group. None of the indices correlated with steatosis grading. CONCLUSIONS: Portal and hepatic vein indices allow non-invasive steatosis diagnosis but are limited to quantify it. Histology remains important for steatohepatitis diagnosis and for steatosis grading.