5(3.5-76.6) vs. 6.8%(0.05-48.5), p<0.0001. Three patients with genetically confirmed BSEP defect (PFIC-2), have low median THBA

level of 1.21 nmole/mmole Cr (0.96-2.28). Using receiver operating characteristic analysis, we found urine THBA ratio > 7.5% predicted good prognosis find more in infantile intrahepatic cholestasis (sensitivity 95.2%, specificity 85.2%), area under curve 0.90, p<0.0001. Conclusions: Elevation of THBA level was found to be associated with good prognosis in infantile intrahepatic cholestasis. Human PFIC patients have no increased levels of urinary THBA, unlike the mice model. The potential beneficial roles of THBA in cholestatic patients deserve further investigations. Disclosures: Mei-Hwei Chang - Grant/Research Support: Gilead, BMS The following people have nothing to disclose: Chee-Seng Lee, Kimura

Akihiko, Jia-Feng Wu, Yen-Hsuan Ni, Hong-Yuan Hsu, Huey-Ling Chen Objectives: Gastroesophageal(GE) varices are a manifestation of portal hypertension(PHT) due to advanced liver disease or portal vein thrombosis(PVT).We present here the prognostic management and evolution of our PHT program. Methods: All patients presenting in our centre with hypersplenism, suspected PHT or gastrointestinal (GI) bleeding and undergoing a first oesophagogastroduodenoscopy (OGD) between 200512 were included and data were collected. Results: 170 patients(91M), mean age 8.8y, were included.126 were diagnosed with liver disease-group A[biliary atresia(62) check details AIH, CF, CHF and other] and 44(PVT)-group B.46 patients presented with bleeding and were enrolled in an endoscopy program. In group A, 17 patients

underwent liver transplantation(LT), 2 died during follow-up(sepsis) and 1(group B) had a meso-Rex shunt. In group A at first OGD there were no oesophageal varices, grade 1, 2 or 3 in 39(31%), 15(12%), 23(18%) and 49(39%) with gastric varices present in 1, 2, 4, and 20, respectively. Mean values for haemoglobin(Hb), learn more platelet count(PLT), white cell count(WCC), INR, serum albumin(Alb), bilirubin, aspartate aminostransferase(AST), spleen size in cm and z-score, Clinical Prediction Rule(CPR), AST/platelet ratio index(APRI) were 11g/ dL, 131×10^9/l, 6.2×10^9/l, 1.19, 38g/L, 44mmol/L,114 IU/L,16cm, 8.35, 103.76, 2.56, respectively. In group B mean values for same variables were 10.8g/dL, 101x10A9/l, 4.3x10A9/l, 1.29, 41g/L, 15mmol/L, 55IU/L, 15.6, 7.35, 107.37, 1.54, respectively. No oesophageal varices, grade 1, 2 or 3 were recorded in 9(20%), 9(20%), 5(11%) and 21(48%) with gastric varices in 4, 2, 4, 7 and 11, respectively. Likelihood of (grade >2) varices PLT, WCC, INR, bilirubin, Alb, spleen size, CPR, APRI showed significance (p<0.05 for all) in group A and Hb(p<0.05) in group B.CPR delimited an AUROC of 0.722 and 0.662 for significant varices in group A and B, respectively.