Conclusion: About 9% of patients with HHT develop symptomatic liver disease. A simple scoring system using age, gender, hemoglobin and alkaline phosphatase can stratify patients into low, moderate and high risk for clinically significant liver disease. Estimated probability of clinically significant

liver disease in patients with HHT based on Simple Clinical Scoring Index. Cumulative Score using Simple Clinical Scoring Index Estimated Probability of Clinically Significant Hepatic Involvement (%) 0 0.4 1 1.2 2 3.2 3 8.2 4 19.5 5 39.7 6 64.1 7 82.9 8 93.0 Disclosures: The following people have nothing to disclose: Siddharth Singh, Karen L. Swanson, Matthew Hathcock, Walter K. Kremers, John Pallanch, Michael J. Krowka, Patrick S. Kamath Gut milieu alterations are associated with cirrhosis complications such Sirolimus concentration as hepatic encephalopathy(HE)and infections. An unfavorable learn more gut microbiome(dysbiosis) could modulate cirrhosis progression. Aim: Evaluate gut microbiota changes across the spectrum of cirrhosis. Methods: Cirrhotics and age-matched controls underwent a cross-sectional stool analysis using multitagged pyrosequencing. Microbiome abundance and cirrhosis dysbiosis ratio

(CDR); ratio of the beneficial autochthonous (Lachnospiraceae+Ruminococaceae+Veillonellaceae+Clostridiales Incertae Sedis XIV) and potentially pathogenic taxa abundance (Enterobacteriaceae+Bacteroidaceae) was compared between groups. Results: 250 cirrhotics [(206 outpatients (no HE: 139, HE: 67), infected inpatients: 44] &25 controls were included. Etiology was alcohol 20%, NASH 14%, rest HCV. MELD was highest in inpatients compared to HE & no HE pts check details (19 vs 14 vs

10, p<0.001), was negatively related to CDR & autochthonous taxa (all p<0.0001) and positively with pathogenic ones; (Staphylococcae, Enterococcaeae &Enterobacteriaceae, p<0.001). With worsening cirrhosis, there was further dysbiosis compared to controls due to autochthonous taxa reduction &pathogenic taxa overgrowth(Table). Dysbiosis (CDR 0.74 vs 0.15, p<0.001) was seen in inpatient vs outpatients. In outpatients HE pts had a significantly lower CDR compared to non-HE (p=0.04). Etiology analysis: Despite similar MELD (12 vs 13) alcoholics had a lower CDR (1.8 vs 3.9) due to lower authochthonous taxa (all p<0.001)compared to non-alcoholics. However NASH cirrhotics had similar CDR(3.8 vs 3.0) than the rest but higher Bacteroidaceae (43 vs 19%, p<0.001), PorphyromcnadaceaeM vs 1%, p=0.003), &lower Veillonellaceae (2 vs 0%, p<0.001). Conclusions: The Cirrhosis Dysbiosis Ratio quantifies the unfavorable gut microbiome that is ssociated with worsening disease severity in this large cirrhotic population. This dysbiosis could be play a role in pathogenesis and progression of cirrhosis. Significantly Different Microbiota Abundances Median % taxa abundance (all p<0.