145 Budd-Chiari syndrome (BCS): The Edinburgh experience

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POSTERS

system. Hence, low levels of actin-free Gc-globulin (AfGc) may serve as a prognostic marker of survival in acute liver failure (ALF). A i m : To evaluate the prognostic value of AfGc in paediatric ALE Patients and Methods: Serum AfGc levels were measured using a rapid ELISA kit (Antibody Shop, Gentofte, Denmark) at admission in 115 children (60 male, median age 5.05 years, range 0 17.4) with ALF ( I N R > 2 unresponsive to vitamin K and abnormal liver enzymes). Causes of ALF were: autoimmune hepatitis (10), drug-induced (16), haemophagocytic lymphohistiocytosis (5), hypoxia (4), viral hepatitis (10), metabolic (14), neonatal haemochromatosis (7), veno-occlusive disease (2), Wilson disease (6), idiopathic (41). Fifty-eight patients survived with medical treatment only (Group 1); 20 died (Group 2); 37 were transplanted, of whom 26 survived (Group 3) and 11 died (Group 4). Results: AfGc levels (gg/ml) were: Group 1: median 255 (range 0.2 525); Group 2:105 (0.1 365); Group 3:95 (0.1 355); Group 4:85 (0.1~70), values being significantly higher in Group 1 when compared to Group 2 (p 0.002) and to all the children who died or required transplantation taken together (p 0.00001). At a cut-off level of 190 gg/ml (obtained by plotting a receiver operative characteristic curve; the value of the area under the curve being 0.79), sensitivity and specificity for predicting survival with native liver were 85% and 62% respectively. Conclusions: These results show that AfGc levels on admission are of prognostic importance in children with ALE Prospective studies with serial measurements are necessary to further elucidate the accuracy and practical value of this test.

~ " ~ DOES THE BANFF SCORE OR ONE ITS SUBSCORES PREDICT OUTCOME IN PATIENTS WITH EARLY ACUTE CELLULAR REJECTION FOLLOWING LIVER TRANSPLANTATION? B.S. H6roldtl, M. Burattin 1, S. Bramhall 1, B. Gunson 1, P. Nightingale 2, J.M. Neuberger1. 1Liver Unit, 2Welcome Trust Clinical Research Facility,

Queen Elizabeth Hospital, Birmingham, UK Introduction: The Banff Scheme is a semi-quantitative score for the definition and grading of acute cellular rejection (ACR) of the liver allograft and provides an accepted and validated definition of ACR. The Banff score comprises three components: Endothelial inflammation (E), Bile duct damage (B) and Portal inflammation (P), each of which is scored from 0 to 3, these are combined to an aggregate score (RAI). A i m : To determine the prognostic value of the Banff score in predicting the response to increased immunosuppression and the long-term function of the graft. Methods: We undertook a retrospective study of consecutive patients who underwent a primary liver transplant between January 2000 and October 2004 with tacrolimus-based immunosuppression and survived >5 days. 495 patients were eligible for inclusion in the study of whom 231 had histologically confirmed ACR. This responded to one cycle of high dose steroids in 193. Results: There was no correlation between the RAI and response to steroids, the need for multiple cycles of steroids ("resistant rejection"), the development of chronic rejection or graft survival. There was a trend of patients with a lower RAI score having an increased risk of death (p 0225, hazard ratio for RAI score lower by 1 1.1.09, 95% confidence interval 0.939 1.309). Of the sub-scores, the E score was related to patient survival, with a lower score being associated with a worse outcome (p 0.048, Tarone Ware test). In a multivariable analysis serum bilirubin, AST and E score were significant predictors of death (p 0.010, hazard ratio for E ~< 1 versus E 2 was 2.564 (1.256 5.208), for E~< 1 versus E 3 was 2.755 (1.196 to 6.329). In univariable analysis B score and Bilirubin were significantly related to "resistant rejection" (p 0.018 and 0.002 respectively), but only Bilirubin was significant in multivariate analysis (logistic regression).

Conclusions: Although the Banff score is a useful marker of the severity of rejection, neither the total score nor the extent of its subscores is correlated with response to steroids: a low E score is associated with a poor graft outcome.

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MODEL TO PREDICT ONE YEAR GRAFT FAILURE IN PATIENTS TRANSPLANTED FOR HCV CIRRHOSIS

S. Iacob 1, S. Beckebaum 2, R. Iacob 1, V. Cicinnati 2, C. Klein 2, L. Gheorghe 1, C. Gheorghe 1, I. Popescu 3, A. Frilling 4, M. Malago 4, G. Gerken2, C. Broelsch4. gCenter of Gastroenterology and Hepatology,

Fundeni Clinical Institute, Bucharest, Romania," 2Department of Gastroenterology and Hepatology, University Hospital Essen, Germany," 3Center of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Bucharest, Romania," 4Department of General, Visceral and Transplantation Surgery, University Hospital Essen, Germany B a c k g r o u n d and A i m : Hepatitis C virus (HCV) associated liver disease represents the leading indication for liver transplantation (LT) in both Europe and the USA, accounting for approximately 50% of cases. HCV associated allograft injury is incriminated as the most common cause of both death and graft failure among HCV infected recipients. The aim of the study was to create a model which can be used to predict graft failure at 1 year post LT. Methods: We reviewed the records of 168 patients who underwent LT for HCV liver cirrhosis between January 1989 and October 2004. To identify potential predictors of graft failure, univariate and multivariate logistic regression were used. The parameters identified as independent predictors of graft loss at 1 year were used in the logistic regression equation to form a new model. Results: There were 50 graft failures (29.8%) 1 year after LT. In the univariate analysis the following parameters were identified as predictors of graft failure: recipient age at LT over 40 years (p 0.03), presence of inflammation on the liver biopsy after reperfusion of the liver (p 0.01), presence of early (within the first 6 months) biliary complications (p 0.005), administration of prednisone <180 days (p < 0.0001) and mycophenolate mofetil (MMF) <180 days (p <0.0001), induction without azathioprine (p 0.003) and without MMF (p 0.04), absence of post-LT antiviral therapy (p 0.008), presence of steatosis on the first liver biopsy with evidence of recurrent hepatitis (p 0.05). The multivariate analysis has identified: presence of early biliary complications (p 0.01), administration of prednisone <180 days (p 0.01) and MMF <180 days (p 0.001) as independent predictors of graft loss. A model incorporating these independent variables was developed to identify recipients at greatest risk for graft loss at 1 year (area under the receiver-operating characteristic curve 0.81). Conclusions: There are several early post-transplant variables such as early biliary complications and certain immunosuppressive regimens that can identify HCV recipients with a high risk of graft failure within 1 year.

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BUDD-CHIARI SYNDROME (BCS): THE EDINBURGH EXPERIENCE

N. Kocharl., S.P. Griffin 1, J.W. Ferguson1, D.N. Redhead 2, N.D.C. Finlayson1, RC. Hayes 1. 1Liver Unit, 2Department of Radiology,

Royal Infirmary of Edinburgh, Edinbu@, UK B a c k g r o u n d : BCS may present with fulminant hepatic failure (FHF) or chronic liver disease. Accepted algorithms for management have yet to be determined. We report on the experience with patients admitted with BCS to the Royal Infirmary of Edinburgh since 1977. Methods: Retrospective review of case notes, using dedicated patient data bases. Results: 25 patients (20 females) with BCS were identified up to 2005. Mean age at diagnosis: 32• years. 14 (56%) patients had underlying

O1B. Liver transplantation~surgery~acute liver failure prothrombotic condition. Diagnosis was made by ultrasound examination in all but one patient and confirmed by CT scan and/or venography. Liver biopsy, when performed, supported the diagnosis in 10 of the 14 patients. Presentation was most commonly as decompensated chronic liver disease (20). A minority presented with FHF (3) or subacute liver failure (2). Most common clinical features were upper abdominal discomfort with abdominal • ankle swelling (68%) and ascites (76%). Hepatic encephalopathy was uncommon at presentation (20%) but predicted poor prognosis (4 transplants and 1 death due to FHF). A TIPSS was attempted for 10, initially successful in six, but in 5 thrombosed within 12 months. A surgical shunt was performed in six, 3 of whom had prior attempts at TIPSS insertion. This was successful in two patients. The other four patients either died due to liver failure (2) or have required transplant (2). Overall, 10 (40%) patients have died (8 liver related) with most within 20 months of symptom onset. 7 patients had a liver transplant (OLT). 4 have died after the transplant. Warfarin was started in 17, of whom 6 have died and 7 have survived without transplantation. Of the OLT patients who died, only one had been on warfarin post liver transplantation compared with the survivors, all of whom remain on long term warfarin. Comment: Budd~Chiari in Scotland is a rare disorder that primarily affects young women. Underlying prothrombotic conditions are common. It is associated with a poor prognosis despite aggressive intervention. TIPSS may be initially helpful but maintaining long-term patency of TIPSS may be difficult. Long-term anticoagulation with warfarin may improve survival with or without transplantation.

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USE OF A 13C METHACETIN BREATH TEST (MBT) FOR FOLLOW-UP OF PATIENTS WITH ACUTE LIVER DISEASE

G. Lalazar 1, M. Shuvy 1, O. Pappo 2, T.R. Hajaj 1, M. Margalit 1. 1Liver

Unit, Department of Medicine, 2Department of Pathology, Hadassah Hebrew University Medical Centeg .Jerusalem, Israel Background: Frequent evaluation of liver function is of importance in the follow-up of patients with acute liver disease. Currently used parameters, including clinical assessment, serum liver enzymes, synthetic function and ammonia levels, are often inadequate for accurate assessment of disease status in patients with severe acute liver disorders. The BreathID| continuous online 13C methacetin breath test MBT (Oridion Ltd.) evaluates liver microsomal function, which is not assessed by current diagnostic tests. Aim: To assess the role of a unique continuous 13C methacetin breath test for follow-up of patients with acute severe liver disease. Methods: Four patients with acute severe liver disease (acute hepatitis A, sub-acute hepatitis B, autoimmune hepatitis, paracetamol intoxication) were followed with a non-invasive 13C MBT (75 mg PO) during various phases of their illness. We assessed the correlation between standard follow-up parameters, including clinical assessment, serum liver enzymes, synthetic function and serum ammonia level and breath test scores including PDR peak (percentage 13C dose recovered per-hour), CPDR30 and CPDR60 (cumulative PDR at 30 and 60 minutes, respectively). Results: In all patients, clinical improvement and normalization of biochemical parameters were accompanied by progressive improvement of BreathID| 13C MBT scores. In the patient with sub-acute hepatitis B, improvement of the breath test scores lagged behind clinical and biochemical improvement by several weeks (Figure 1). This may indicate that liver microsomal function takes longer to recover than standard measured parameters in some acute liver disorders. Conclusions: Assessment of liver microsomal function by the BreathID| 13C MBT is a rapid, noninvasive follow-up tool in acute liver disease of diverse etiologies. Our data suggests that, while correlating with standard

(b) Clinical

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parameters, the breath test can reveal lingering microsomal hepatic dysfunction, which may have additional clinical and therapeutic implications. Day

ALT

AST

GGT

Bil.

INR

PDR peak

1

3761

2663

188

221

124

16.45

2

3195

2138

144

232

2.39

9.22

6

2914

1799

114

319

2.16

6.55

20

600

454

85

71

1.36

12.36

48

33

49

39

19

1.12

23.76

~ - 7 ] TERLIPRESSIN A N D N O R A D R E N A L I N E INCREASES CEREBRAL PERFUSION W I T H O U T AFFECTING INTRACRANIAL PRESSURE IN PATIENTS WITH FULMINANT HEPATIC FAILURE T. Dethlott; M. Eefsen, B.A. Hansen, F.S. Larsen. Dept. of Hepatology,

Rigshospitalet, Copenhagen, Denmark Objective: Inotropic support is often necessary to secure sufficient circulation in patients with fulminant hepatic failure (FHF). In severe cases with manifest cardiovascular shock the responds to volume expansion and infusion of noradrenaline (NA) is often inadequate. In such patients administration of terlipressin may be valuable to secure sufficient systemic and cerebral circulation. The aim of this study was to compare the effect of NA and terlipressin on cerebral perfusion pressure (CPP), cerebral blood flow (CBF) and intracranial pressure (ICP). Patients and Methods: Seven patients with FHF (median age, 19yrs; range, 15 61; 4 women) maintained on mechanical ventilation (median PaCO2, 4.40kPa; range 3.47 4.35) after development of hepatic encephalopathy, stages 3 to 4, had mean arterial pressure increased ~20 mmHg first by NA and later by terlipressin. Cerebral perfusion was measured by transcranial Doppler sonography, which was used to measure mean flow velocity (Vmean). In each patient concomitant measurement o f Vmean, mean arterial pressure and ICP was first recorded during norepinephrine infusion. 30 min after stopping NA infusion each patient then received a bolus injection of terlipressin (1 mg i.v.) with recording o f Vmean , arterial pressure and ICR CPP was calculated as mean arterial pressure minus ICE Results: During NA infusion CPP increased from 61 (range 40 88) to 80 (58 110) mmHg, Vmean from a median of 60 (21 113) to 80 (40 194) crn/s (P <0.05). ICP remained unchanged, i.e. 10 (4 16) vs. 10 (5 21) mmHg (NS). After injection of terlipressin CPP increased from 55 (40 113) to 76 (59 151) mmHg (p <0.05), Vmean from 70 (23 113) to 80 (17 109) crrds (P <0.01). ICP remained unchanged (7 (5 16) vs. 9 (5 20) mmHg (NS). The slopes between CPP and Vmean were similar in the NE study compared to the terlipressin study (1.07 vs. 1.09) (NS). Conclusion: This study shows that administration of NA and terlipressin increases cerebral perfusion pressure and blood flow to the same degree in patients with FHF without influencing ICR This finding indicates that terlipressin may be valuable as rescue treatment of arterial hypotension with imminent cerebral hypoxia in patients with FHF not responding to standard therapy with volume expansion and NA infusion.

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CLINICAL FEATURES A N D O U T C O M E OF HEPATIC FAILURE ASSOCIATED WITH E P S T E I N - B A R R VIRUS INFECTION

Y.S. Lim, D.K. Kim, K.M. Kim, H.C. Lee, Y.H. Chung, Y.S. Lee, D.J. Suh. Department of Gastroenterology/Asan Medical Cente~ Seoul,

South Korea Background and Aims: Although Epstein Barr virus (EBV) infection is usually self-limited with complete recovery, acute hepatic failure may be