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Background: Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder, resulting from thrombosis of the hepatic veins. Various treatments, including pharmacological, radiological and surgical interventions, have been used. Aim(s): To describe retrospectively our institution's experience with management of patients with BCS. Method(s): A retrospective study of all cases of primary Budd-Chiari syndrome presenting to our institution between January 2000 and August 2012 was performed. Patients with secondary Budd-Chiari syndrome due to malignancy or local mass compression were excluded. Result(s): Between 2000 and 2012, 27 patients with primary BCS presented with a median Rotterdam score of 1.16 (range: 0.07-2.11). A total of 24 patients (89%) had at least one risk factor, with the commonest being myeloproliferative neoplasm (MPN), detected in 17 of 24 (71%) of the tested patients, including four patients with normal blood counts at diagnosis. All patients were anticoagulated with warfarin or low-molecular-weight heparin (LMWH). A total of 25 (92.6%) patients also had primary radiological interventions, consisting of transjugular intrahepatic portosystemic shunt (TIPS) in 18 (67%) patients and/or angioplasty/stenting in 11 (40%). A total of 14 patients developed TIPS stenoses, requiring a median of 1.5 (range: 1-14) revisions. No patient developed TIPS failure requiring alternative therapy. Two patients were lost to follow-up. At a median follow up of 59 months (range: 2-248 months), the overall survival was 96% at 1 year and 81% at 5 years, much greater than predicted by the Rotterdam score. No patients required liver transplantation. Conclusion(s): There is a high incidence of MPN in patients with primary BCS, including patients with normal peripheral blood counts at the time of diagnosis. Our approach of anticoagulation, aggressive and early radiological intervention aimed at rapid decompression of the congested liver resulted in excellent medium-term outcom

Background: Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder, resulting from thrombosis of the hepatic veins. Various treatments, including pharmacological, radiological and surgical interventions, have been used. Aim(s): To describe retrospectively our institution's experience with management of patients with BCS. Method(s): A retrospective study of all cases of primary Budd-Chiari syndrome presenting to our institution between January 2000 and August 2012 was performed. Patients with secondary Budd-Chiari syndrome due to malignancy or local mass compression were excluded. Result(s): Between 2000 and 2012, 27 patients with primary BCS presented with a median Rotterdam score of 1.16 (range: 0.07-2.11). A total of 24 patients (89%) had at least one risk factor, with the commonest being myeloproliferative neoplasm (MPN), detected in 17 of 24 (71%) of the tested patients, including four patients with normal blood counts at diagnosis. All patients were anticoagulated with warfarin or low-molecular-weight heparin (LMWH). A total of 25 (92.6%) patients also had primary radiological interventions, consisting of transjugular intrahepatic portosystemic shunt (TIPS) in 18 (67%) patients and/or angioplasty/stenting in 11 (40%). A total of 14 patients developed TIPS stenoses, requiring a median of 1.5 (range: 1-14) revisions. No patient developed TIPS failure requiring alternative therapy. Two patients were lost to follow-up. At a median follow up of 59 months (range: 2-248 months), the overall survival was 96% at 1 year and 81% at 5 years, much greater than predicted by the Rotterdam score. No patients required liver transplantation. Conclusion(s): There is a high incidence of MPN in patients with primary BCS, including patients with normal peripheral blood counts at the time of diagnosis. Our approach of anticoagulation, aggressive and early radiological intervention aimed at rapid decompression of the congested liver resulted in excellent medium-term outcom

Background: While indomethacin and ibuprofen form the mainstay, paracetamol has recently been used for the closure of patent ductus arteriosus (PDA). The objective was to ascertain the efficacy of paracetamol in closing a symptomatic PDA (sPDA) when used as 'rescue' option, compare clinical and echocardiographic parameters and study the safety profile. Method(s): Retrospective appraisal of data fromApril 2014 to July 2015. Oral paracetamol was used for closure of a sPDA if ibuprofen either failed or was contraindicated. Pre-treatment testing for platelet count, serum creatinine and alanine transaminase (ALT) was performed and was repeated on day 3. A previously published echocardiographic scoring schema was used. The maximum composite score was 17. Partial closure was a priori defined as 'reduction in the composite score by >/= 50%of pre-treatment'. Result(s): Twenty infants with gestation age of 25.7 +/- 1.5 weeks and birthweight of 724.1 +/- 143 g were studied. Complete closure was noted in 10 (50%) infants with additional 4 (20%) infants showing partial closure. One infant needed PDA ligation. On univariate analysis, the pre-therapy composite score had a significant association with successful closure (higher echocardiographic score, lesser the closure). Furosemide therapy and late onset sepsis had a high likelihood ratio of unsuccessful closure (11.01 [2-tailed, P = 0.005] and 5.3 [2-tailed, P = 0.07]), respectively. Paired platelet count, creatinine and ALT data indicated safety of usage. Conclusion(s): Paracetamol was safe and effective in sPDA rescue closure. Sepsis and furosemide affected successful therapy. Future work should address clinical and echocardiographic assessment in study design.

The prevalence of cirrhosis in the Australian community is unknown. Moreover, as only 1-2% of patients with chronic hepatitis B (CHB) and C (CHC) are receiving active specialist assessment,(1,2) there is an urgent need to detect those in the community at high risk of liver related morbidity and mortality. Fibroscan liver stiffness measurement (LSM) is a simple safe and increasingly accepted non-invasive method of assessing liver disease severity. This study aims to determine the feasibility of community screening and estimate the prevalence of significant fibrosis and cirrhosis as assessed by non-invasive techniques in at-risk populations. Method(s): Participants were consecutively recruited from 14 primary care practices with high case-loads of CHB and CHC throughout Melbourne, Australia. Inclusion criteria included age 18-80 years, with evidence of infection duration >6 months, absence of prior or recent (<18 months) specialist input and no current or prior HCC, or pregnancy. Clinical assessment, transient elastography (Fibroscan 402) and blood analysis were performed at their place of primary care. AST to platelet ratio index (APRI) and Fibrosis-4 (FIB4) scores were calculated for comparison with LSM. Scans were considered reliable if the success rate was >60% and IQR/median stiffness < 0.3. A control primary hospital presentation cohort was recruited to assess for selection bias. LSMs of 8 kPa and 13 kPa were taken as cut-offs to represent a high probability of significant fibrosis (>F2) and cirrhosis (F4) respectively. Individuals meeting these cut-offs were referred to nearby tertiary centers for ongoing management. Result(s): A total of 238 patients were recruited during a pilot phase of 5 months. Of these, 160 were CHC and 78 were CHB. There was a 31.4% failure to attend rate in the community cohort. Fibroscan LSM was successfully obtained in all patients with four (1.6%) requiring use of the XL-probe. The community cohort was predominantly male (CHC 73.1% v

The prevalence of cirrhosis in the Australian community is unknown. Moreover, as only 1-2% of patients with chronic hepatitis B (CHB) and C (CHC) are receiving active specialist assessment,(1,2) there is an urgent need to detect those in the community at high risk of liver related morbidity and mortality. Fibroscan liver stiffness measurement (LSM) is a simple safe and increasingly accepted non-invasive method of assessing liver disease severity. This study aims to determine the feasibility of community screening and estimate the prevalence of significant fibrosis and cirrhosis as assessed by non-invasive techniques in at-risk populations. Method(s): Participants were consecutively recruited from 14 primary care practices with high case-loads of CHB and CHC throughout Melbourne, Australia. Inclusion criteria included age 18-80 years, with evidence of infection duration >6 months, absence of prior or recent (<18 months) specialist input and no current or prior HCC, or pregnancy. Clinical assessment, transient elastography (Fibroscan 402) and blood analysis were performed at their place of primary care. AST to platelet ratio index (APRI) and Fibrosis-4 (FIB4) scores were calculated for comparison with LSM. Scans were considered reliable if the success rate was >60% and IQR/median stiffness < 0.3. A control primary hospital presentation cohort was recruited to assess for selection bias. LSMs of 8 kPa and 13 kPa were taken as cut-offs to represent a high probability of significant fibrosis (>F2) and cirrhosis (F4) respectively. Individuals meeting these cut-offs were referred to nearby tertiary centers for ongoing management. Result(s): A total of 238 patients were recruited during a pilot phase of 5 months. Of these, 160 were CHC and 78 were CHB. There was a 31.4% failure to attend rate in the community cohort. Fibroscan LSM was successfully obtained in all patients with four (1.6%) requiring use of the XL-probe. The community cohort was predominantly male (CHC 73.1% v

Background Patients with hepatitis C virus (HCV) related bridging fibrosis and cirrhosis represent a population at increased risk of developing liver failure and hepatocellular carcinoma (HCC). Historically these patients tolerate and respond poorly to therapy. However, achieving sustained virological response (SVR) following antiviral treatment reduces the likelihood of clinical decompensation and improves morbidity and mortality. Our aim was to identify factors predicting treatment response in patients with biopsy proven bridging fibrosis and cirrhosis. Methods We retrospectively analysed the HCV treatment database in a large tertiary centre in Melbourne. We identified 104 patients with biopsy proven bridging fibrosis (Metavir F3) and Child Pugh A cirrhosis (Metavir F4) who received pegylated interferon and ribavirin between 2003-2010. Patient age, weight, ethnicity, gender, albumin, bilirubin, INR, platelet count, viral genotype, baseline viral load and liver ultrasound examination were obtained. Statistical analysis was performed using Fischer's exact test. Univariate and multivariate analyses were used to evaluate factors predicting treatment outcomes. Results 104 patients were included in this study with a total of 108 treatment episodes. The mean age of participants was 52 years (range 33-73 years), 60% were male, 52% were genotype 1 and 36% genotype 3. 62% had a viral load >=400,000 IU/ml. 55% were cirrhotic. Pre-treatment liver ultrasound was performed in 96 patients, 33 (34%) had ultrasound features of portal hypertension. SVR occurred in 41%, the mean age of these patients was 53 years, 64% were male, 27% were genotype 1, 48% were genotype 3, 47% had a viral load >=400,000 IU/ml, 50% were cirrhotic and 13% had ultrasound features of portal hypertension. Multivariate analysis identified genotype 3 (p = 0.002), the absence of ultrasound features of portal hypertension (p < 0.001) and a viral load <400,000 IU/ ml (p = 0.008) as independent predictors of SVR.

Background Patients with hepatitis C virus (HCV) related bridging fibrosis and cirrhosis represent a population at increased risk of developing liver failure and hepatocellular carcinoma (HCC). Historically these patients tolerate and respond poorly to therapy. However, achieving sustained virological response (SVR) following antiviral treatment reduces the likelihood of clinical decompensation and improves morbidity and mortality. Our aim was to identify factors predicting treatment response in patients with biopsy proven bridging fibrosis and cirrhosis. Methods We retrospectively analysed the HCV treatment database in a large tertiary centre in Melbourne. We identified 104 patients with biopsy proven bridging fibrosis (Metavir F3) and Child Pugh A cirrhosis (Metavir F4) who received pegylated interferon and ribavirin between 2003-2010. Patient age, weight, ethnicity, gender, albumin, bilirubin, INR, platelet count, viral genotype, baseline viral load and liver ultrasound examination were obtained. Statistical analysis was performed using Fischer's exact test. Univariate and multivariate analyses were used to evaluate factors predicting treatment outcomes. Results 104 patients were included in this study with a total of 108 treatment episodes. The mean age of participants was 52 years (range 33-73 years), 60% were male, 52% were genotype 1 and 36% genotype 3. 62% had a viral load >=400,000 IU/ml. 55% were cirrhotic. Pre-treatment liver ultrasound was performed in 96 patients, 33 (34%) had ultrasound features of portal hypertension. SVR occurred in 41%, the mean age of these patients was 53 years, 64% were male, 27% were genotype 1, 48% were genotype 3, 47% had a viral load >=400,000 IU/ml, 50% were cirrhotic and 13% had ultrasound features of portal hypertension. Multivariate analysis identified genotype 3 (p = 0.002), the absence of ultrasound features of portal hypertension (p < 0.001) and a viral load <400,000 IU/ ml (p = 0.008) as independent predictors of SVR.