Search

Objectives: This study describes the natural history of patients with polycystic liver disease, a rare disorder characterized by multiple hepatic cysts., Methods: Cases were identified through review of charts from a hepatology practice, a hepatobiliary surgery practice and a retrospective chart review of inpatient charts from 1990 to 2002. All patients had greater than four simple liver cysts without infectious etiology. Medical records were reviewed for history, physical examination, imaging, and laboratory data. Patients' family practitioners provided follow-up., Results: Fifty-three cases (62.3% female, 37.7% male) were identified. The mean age at diagnosis was 56.4 yr. Thirty-eight cases (71.7%) had associated polycystic kidney disease. The minority of patients were symptomatic at diagnosis (pain in 19 (36.5%), dyspnea in 5 (9.6%), and restricted mobility in 5 (9.6%) with hepatomegaly in 23 (45.1%). Follow-up information was attainable for 40 patients with a mean follow-up duration of 4.69 yr (range 0.1-15 yr). Within this subgroup, 9 patients (22.5%) had cyst bleeding, 5 (12.5%) had cyst rupture, 5 (12.5%) had cyst infection, 12 (30%) required an intervention. One patient (2.5%) developed portal hypertension, and two (5%) received a liver transplant. One patient (2.5%) died due to complications from liver cysts., Conclusions: Most patients in this highly selected cohort were asymptomatic with normal hepatic function. Pain was the most common symptom. The natural history is variable however, with some patients developing complications including portal hypertension. Minimally invasive interventions are appropriate initially, with hepatic resection and liver transplantation reserved for those with severe symptoms or life-threatening complications.

Prior to the introduction of virally inactivated clotting factor concentrates, the majority of individuals with congenital bleeding disorders became infected with the hepatitis C virus. Although liver biopsy is valuable in prognosis and guiding antiviral therapy, there is a reluctance to perform biopsies in this population because of the risk of hemorrhage. The purpose of this study was to evaluate the safety of transjugular liver biopsy, and the usefulness of evaluating liver histology in this patient population. Liver histopathology was assessed by the METAVIR index and compared with corrected sinusoidal pressures, platelet counts, and abdominal ultrasonography. Liver biopsy was performed at seven Canadian centers in 65 patients with hemophilia or von Willebrand's disease. Biopsies were done on an outpatient basis, followed by a 4-hr observation period in hospital. Normal hemostasis was maintained during the peribiopsy period, with follow-up doses of factor concentrate self administered by the patient at home. One patient (1.4%) had significant bleeding leading to readmission and red cell transfusion. Liver histology showed 14 patients (22%) had cirrhosis. Ten patients had elevated corrected sinusoidal pressures; 7 of these (70%) had cirrhosis on biopsy, and the other 3 (30%) likely had cirrhosis although histology showed stage 3 fibrosis. This series represents the largest reported experience of transjugular biopsy in individuals with congenital bleeding disorders. We conclude that this procedure can be safely performed on an outpatient basis. The diagnosis of cirrhosis and/or portal hypertension was made in a substantial proportion of individuals (26%), all of whom had asymptomatic liver disease., (Copyright 2005 Wiley-Liss, Inc.)

OBJECTIVES:Liver transplantation for alcoholic liver disease (ALD) can be complicated by abusive or “problem” drinking (PD) after transplant. There are limited data for evaluating the effect of pre-transplant abstinence on post-transplant PD. Few existing studies have included a substantial number of patients with co-existing causes of hepatic dysfunction, and the effect of PD on survival in recent European studies has been controversial. We hypothesized that a longer duration of pre-transplant abstinence would lead to less PD after transplantation. Accordingly, the objectives of this study are to analyze a North American cohort of patients with ALD with or without a secondary diagnosis of liver disease to estimate (i) the incidence of PD and its predictors, as well as (ii) the effect of PD on patient survival.METHODS:We conducted a retrospective review of all patients transplanted for ALD surviving for more than 3 months after transplant. PD was defined as either any drinking (AD) to the point of intoxication or drinking above the toxic threshold (>20 g/day in women and >40 g/day in men) on at least two separate occasions. We used Cox's proportional hazards regression to estimate risk ratios and Kaplan–Meier curves with log-rank analysis to compare survival.RESULTS:Of 213 eligible transplant patients, 42 were excluded. Of the 171 remaining patients, 78% were male; mean age was 52 years. Overall 53% of patients had co-existing causes of liver dysfunction. The mean follow-up was 64.8 months. The median pre-transplant abstinence was 19 months. In all patients, the risk of AD was 24% and PD 13%. Pre-transplant abstinence duration was the only independent predictor of PD after transplant. For every 1-month increment in pre-transplant abstinence, there was a 5% decrease in the adjusted relapse rate. There was no survival difference noted between problem drinkers and non-drinkers.CONCLUSIONS:The risk of PD decreased with increasing pre-transplant abstinence. Our data support pre-transplant abstinence as an important predictor of post-transplant recidivism; however, the optimal period of abstinence remains unclear. Patients with <18 months of abstinence may benefit from more intensive follow-up and rehabilitation after transplant.Am J Gastroenterol 2009; 104:1700–1706; doi:10.1038/ajg.2009.226; published online 26 May 2009 [ABSTRACT FROM AUTHOR]

Background: The pathogenesis of refractory ascites (RA) is linked to splanchnic vasodilation. We hypothesized that a combination of midodrine, octreotide long-acting release (LAR) and albumin would result in increased natriuresis, better control of ascites and an improvement in renal function in patients with RA±Type 2 hepatorenal syndrome. Methods: A prospective pilot study in patients with RA as defined by the International Ascites Club. Consecutive patients received an intramuscular injection of octreotide-LAR, 50 g of albumin three times per week and midodrine titrated to increase the systolic blood pressure for 1 month. Results: Ten patients with RA were enrolled and eight with complete data to 1 month post-treatment were included in the analysis. There was no change in renal function but there was a trend towards a reduction in the volume of ascites removed by paracentesis ( P=0.08) and a significant reduction in the plasma renin ( P=0.01) and aldosterone concentrations ( P=0.01). Interestingly, there was a transient worsening in the model for end-stage liver disease (MELD) score ( P=0.01). The deterioration in MELD was completely reversible after discontinuation of therapy. Conclusions: To our knowledge, this is the first study of prolonged midodrine, octreotide and albumin therapy in RA. We observed a significant reduction in the plasma renin and aldosterone concentrations and a trend towards a reduction in the volume of ascites removed by paracentesis without an effect on renal function. The beneficial effects are at the expense of a reversible deterioration in the MELD score. Large controlled trials are needed before this therapy can be routinely recommended. [ABSTRACT FROM AUTHOR]

Goals: To assess the current practice patterns of liver transplant centres in Canada and the USA regarding transplant eligibility. Background: Liver transplantation is an evolving field and today remains the only life-sustaining treatment for end-stage liver disease. Issues of allocation and transplant eligibility are important factors in the ethical practice of medicine. Study: Questionnaires were mailed to liver transplant programme directors in Canada and the USA inquiring about current practices regarding recipient eligibility. Results: This study demonstrates that there is consensus in the use of other eligibility criteria, including non-compliance, social status, abstinence from alcohol and methadone and cocaine use. Interestingly, literature is lacking to support the use of these parameters as eligibility criteria with the exception of alcohol. There is a lack in consensus regarding marijuana use, human immunodeficiency virus status, ability to accept blood transfusions and prisoner status. The literature suggests that liver transplantation in select patients who refuse blood transfusions results in good outcomes. Conclusions: Important decisions regarding transplant eligibility still have to be made empirically in the absence of scientific literature about various social issues. While consensus among transplant programmes is useful, it is important that we continue to use the evidence in the literature to revise these eligibility criteria, keeping in mind ethical principles applied to the access and allocation of a scarce resource. [ABSTRACT FROM AUTHOR]

Chatur N, Ramji A, Bain VG, Ma MM, Marotta PJ, Ghent CN, Lilly LB, Heathcote EJ, Deschenes M, Lee SS, Steinbrecher UP, Yoshida EM. Transplant immunosuppressive agents in non-transplant chronic autoimmune hepatitis: the Canadian association for the study of liver (CASL) experience with mycophenolate mofetil and tacrolimus. Liver International 2005: DOI: 10.1111/j.1478-3231.2005.01107.x © Blackwell Munksgaard 2005 Conventional treatment of autoimmune hepatitis consists of either prednisone alone or in combination with azathioprine. Ten to 20% of patients do not respond to or are intolerant of this treatment. Novel drug treatments include immunosuppressive drugs such as tacrolimus (TAC), mycophenolate mofetil (MMF), methotrexate and cyclosporine. We describe a multi-centre Canadian experience with MMF and TAC. To study a multi-centre patient population who had failed conventional therapy and were treated with non-conventional medical therapy for autoimmune hepatitis and document response. Members of the Canadian Association for the Study of Liver (CASL) obtained MMF from Hoffmann-La Roche Ltd, as part of a compassionate release program, were contacted for standardized data on patients with AIH who received MMF or TAC. Response definitions based on aminotransferase changes were: Complete response (CR)-sustained normalization, partial response (PR)-improvement by greater than 50%, non-response (NR)-less than 50% improvement and relapse (RP)-initial CR or PR followed by an increase in aminotransferases. A total of 16 patients were identified: six in Ontario, one in Quebec, five in Alberta and four in British Columbia. Three were treated with TAC, eleven with MMF and two with combination MMF and TAC. CR was observed in 50%, PR in 12.5%, RP in 25% and NR occurred in 12.5%. The CR for MMF without TAC was approximately 64%. MMF is effective and well tolerated by patients with autoimmune hepatitis who do not respond to, or are intolerant of, conventional immunosuppressive agents. [ABSTRACT FROM AUTHOR]

Fulminant hepatic failure (FHF) is defined as acute liver failure with hepatic encephalopathy in patients with no history or stigmata of chronic liver disease. Historically, its prognosis was extremely poor. However, the emergence of orthotopic liver transplantation as a viable form of therapy for liver failure and advances in intensive care medicine have improved patient survival dramatically. Treatment of this condition revolves around supportive medical care and timely referral for orthoepic liver transplantation as necessary. Treatment needs to be individualized for each patient, because prognosis varies, depending on the etiology of the condition. The clinical manifestations of FHF and the management of (his condition are discussed. Future prospects for the management of FHF are also reviewed. [ABSTRACT FROM AUTHOR]