K D Lindor

..Liver transplantation is a good option for patients with advanced PSC, although the disease can recur after successful transplantation...

..In this article we discuss the etiology, pathogenesis and diagnosis of nonalcoholic fatty liver disease as well as approaches to its management...

..Liver-related death is a leading cause of mortality, although the absolute risk is low...

..Effective therapy reduces the need for transplantation and improves life expectancy. For advanced liver disease or incomplete response to UDCA, new therapies to cure or retard the progression of disease in PBC are needed...

..In conclusion, 2 years of therapy with UDCA at a dose of 13 to 15 mg/kg/d, although safe and well tolerated, is not better than placebo for patients with NASH...

..Currently, there are multiple potential therapeutic avenues to explore for patients with PSC, and it is hoped that one of these will lead to identification of a proven therapy for this disease...

..Although there is no known beneficial therapy for these patients, research aimed toward better understanding the pathogenetic mechanisms and clinical trials with promising new agents are ongoing...

..01]). Conclusion: Long-term, high-dose UDCA therapy is associated with improvement in serum liver tests in PSC but does not improve survival and was associated with higher rates of serious adverse events...

..This review will provide an overview of the role of nuclear receptors in bile acid homeostasis with a focus on the farnesoid X receptor (FXR) and its potential therapeutic use in cholestatic liver diseases...

..Short-term therapy, however, does not facilitate reduction in the dose of corticosteroids or its withdrawal, affect clinical outcome, or reduce histological activity...

..No significant risk reduction in developing pruritus with UDCA therapy was observed compared to placebo-treated patients. The long-term administration of rifampin for refractory pruritus is associated with occasional hepatotoxicity...

..In conclusion, MMF does not appear to have clinically important benefits for PSC despite being tolerated by most patients. The results of this pilot study do not support further study of MMF as a single agent in the treatment of PSC...

..A subset, however, have no such features but go on to develop a 'sequential' AIH overlap syndrome. Objectives: Describe our experience with eight patients who developed AIH after the diagnosis of PBC was made...

..A PSC-like variant attracting attention is cholangitis marked by raised levels of the immunoglobulin G4 subclass, prominence of plasma cells within the lesions, and steroid responsiveness...

..4). High-dose UDCA was well tolerated. CONCLUSIONS: UDCA at a dose of 25-30 mg/kg per day may be of benefit for patients with PSC, and this regimen deserves further evaluation in a long-term, randomized, placebo-controlled trial...

..2) was associated with the treatment. Fatigue and health-related quality of life scores during treatment demonstrated a trend toward improvement. Moexipril was not clinically beneficial to PBC patients responding suboptimally to UDCA...

..Our results suggest that eosinophils in patients with PBC are not only increased in number, but also release granule proteins, and that UDCA treatment inhibits this eosinophil activation/degranulation...

..We report the incidence and prevalence of this condition in the general population. We also examined the validity of the Mayo natural history model for PBC among these unselected patients from the community...

..While short- and medium-term outcomes remain favorable, especially if compared to patients transplanted for other indications, continued follow-up may identify reduced long-term graft and patient survival...

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..Testing PSC patients for IgG4 and treating those with elevated levels with corticosteroids in clinical trials should be considered...

..Changes of aminotransferases do not parallel changes in fibrosis stage. Diabetic patients with elevated BMI and low fibrosis stage are at risk for higher rates of fibrosis progression...

..In this paper, we review the literature pertaining to the current state of NAFLD management with an emphasis on pharmacotherapy...

..These data, combined with our previous study, demonstrate its applicability in a wide spectrum of disease severity, ranging from early, ambulatory-phase disease to decompensated cirrhosis necessitating liver transplantation...

..CONCLUSIONS: Betaine is a safe and well tolerated drug that leads to a significant biochemical and histological improvement in patients with NASH. This novel agent deserves further evaluation in a randomized, placebo-controlled trial...

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..However, these therapies are rarely applied to these patients because of the advanced nature of the disease at the time of diagnosis. Efforts should be directed at earlier identification of potential surgical candidates...

..This study was undertaken to determine which variables indicate the need for liver biopsy in patients with positive AMA and suspected PBC...

..Liver biopsy is required to rule out AIH in most NAFLD patients with positive autoantibodies...

..30 US dollars would be required. In conclusion, MRC has comparable accuracy to ERCP and results in cost savings when used as the initial test strategy for diagnosing PSC...

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..Despite promising results, no therapy has demonstrated a proven benefit...

..We aimed at determining the relation between leptin and liver fibrosis in human NAFLD...

..This is the first report of a successful living donor right liver graft in an adult with end-stage liver disease secondary to primary sclerosing cholangitis and situs inversus totalis...

..Three of the four patients had drug-related adverse events with fluoxetine. In this study, fluoxetine did not improve fatigue in PBC and was associated with adverse events...

..Mycophenolate mofetil (MMF) is an immunosuppressive medication that inhibits T and B lymphocyte proliferation. The aim of this investigation was to determine the safety and estimated efficacy of MMF in patients with PBC...

..Liver transplantation remains the only successful treatment option for patients with advanced liver disease from PSC. A diagnosis of PSC should be considered among individuals with IBD and elevated serum liver biochemical tests...

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..Thus, the identification and follow-up of these relatives may lead to earlier disease diagnosis and treatment. Furthermore, if AMA development is heritable, this trait will provide a basis to dissect the genetic predisposition to PBC...

..The authors aimed to compare the publication records, academic promotions, and leadership appointments of women and men physicians longitudinally throughout academic careers...

..Patients with NAFLD have a lower life expectancy compared to the general population, with coronary artery disease (CAD) as the leading cause of death...

..In PBC and PSC, adequate management of complications of chronic cholestasis is important. For both diseases, liver transplantation is the only curative option...

..Primary biliary cirrhosis is a cholestatic liver disease that at one time was the leading indication for liver transplantation. Treatment with ursodeoxycholic acid has clearly improved the natural history of primary biliary cirrhosis...

..The results of this pilot study warrant further evaluation of silymarin in patients with PSC in a large-scale, controlled trial...

..Further studies are needed for determining diagnosis, natural history and optimal therapeutic strategies of overlap syndromes of autoimmune liver disease...

..We sought to evaluate the effect of UDCA on colorectal neoplasia in a group of patients with UC and PSC enrolled in a randomized, placebo-controlled trial...

..In conclusion, PSC significantly decreases survival in this child population. Although pharmacologic therapy may improve symptoms and liver test results initially, it does not seem to impact the long-term outcome...

..Finally, hyperlipidemia in PBC does not seem to be associated with an increased risk of atherogenesis. New therapies in this patient population are currently under investigation...

..Emerging data suggest that oral antibiotics may have therapeutic effects in primary sclerosing cholangitis (PSC), but published studies are limited...

..Ursodeoxycholic acid (UDCA) in a dose of 28-30 mg/kg/day increases the likelihood of clinical deterioration of primary sclerosing cholangitis (PSC) patients...

..Further data are needed before more widespread use of troglitazone can be recommended for patients with nonalcoholic steatohepatitis...

..Our goals were to assess budesonide in patients with treatment-dependent autoimmune hepatitis and to determine if efficacy and safety justified a controlled trial...

..In this short period of treatment, UDCA was not associated with improved patient and graft survival compared to untreated patients...

..02 g/cm(2) (0.01-0.10) vs. 0.00 g/cm(2) (-0.120-0.040), P=0.06)]. In conclusion, raloxifene appears safe and of benefit in preventing bone loss in patients with PBC. Larger studies with longer follow-up are warranted...

..62) or albumin (4.0 +/- 0.2 vs 4.2 +/- 0.3, p= 0.41). CONCLUSIONS: Aminotransferase levels among patients with NASH improve with administration of pentoxifylline. Strategies to overcome side effects will be needed for future trials...

..Although UDCA therapy improves liver biochemistries, it may not delay disease progression during the short period of treatment...

..03). CONCLUSIONS: When the long-term outcome of SSC patients was compared to matched PSC controls, the SSC patients had a poorer outcome. The natural history of SSC is characterized by a shortened life expectancy...

..The risk of complications associated with this procedure is not well established in these patients. The aim of this retrospective study was to compare the risk of ERCP complications in PSC vs. non-PSC patients...

..The central and the peripheral mechanisms that have been suggested for the pathogenesis of fatigue in PBC are also discussed and treatment options are reviewed...

..Both an infectious etiology and molecular mimicry have been implicated. The aim is to study the prevalence of Chlamydial antigens and RNA in the liver tissue of patients with PBC...

..Primary biliary cirrhosis (PBC) is frequently associated with autoimmune diseases, including thyroid disease, although it is uncertain that this association is higher than in other liver diseases...

..Although a substantial percentage of patients will achieve biochemical normalization on UDCA alone, there is a continued need for therapeutic options for others who have less complete biochemical responses...

..The purpose of this study was to examine how often routine liver biopsies provide important information in patients with PSC...

..Patients with PBC should receive ursodeoxycholic acid (UDCA); the role of and response to additional immunosuppressive therapy are unknown when AIH overlaps PBC...

..In this review we highlight recent advances in PSC pathogenesis, diagnosis and management...

..The aim of this prospective study was to determine whether patients with solid food dysphagia and normal upper endoscopy have symptomatic benefit from empiric dilation using a through-the-scope balloon...

..These findings led to the introduction of targeting nuclear receptors as treatment strategies for various liver diseases...

..The importance of its recognition and the appropriate management modifications are discussed...

..PBC patients who are older, thinner and have more advanced liver disease may have the most benefit from bone density measurements and treatment for their osteoporosis...

..0. CONCLUSION: Other than deficiency of vitamin A, deficiency of fat-soluble vitamins occurs uncommonly in patients with PBC. A Mayo risk score > or = 5 helps in selecting patients with PBC for surveillance for vitamin A deficiency...

..Stenting was associated with more complications, and its role after dilation should be assessed in a randomized trial rather than being accepted as routinely indicated in this setting...

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..Our results suggest a clinically applicable and useful approach to identify patients with PSC who are more likely to benefit from endoscopic screening for esophageal varices...

..A healthy baby boy was delivered at 33.5 weeks. The mother required cholangiography and stent placement immediately after delivery, but her postpartum course was otherwise unremarkable...

..Pirfenidone did not benefit patients with PSC, and it was frequently associated with adverse events. The results of this pilot study discourage further trials of pirfenidone in patients with PSC...

..Lower platelet count and higher total bilirubin at 2 years were significantly associated with an increased risk of developing new varices in patients with PSC...

..Applicability of the revised IAHG scoring system, however, remains questionable, as nearly 20% of PBC patients will be classified with probable AIH overlap...

..Minocycline may also inhibit cell death pathways by reducing both proapoptotic and proinflammatory enzyme activation. We sought to investigate the safety and efficacy of minocycline among patients with PSC...

..Discussions with PBC patients beginning UDCA treatment should include the beneficial effects this medication has on disease outcome, but should also mention weight gain as a possible side effect...

..Liver transplantation is a life-extending therapeutic alternative for patients with end-stage NAFLD, but NAFLD may recur after liver transplantation...

..The aim of our study was to validate the need for annual surveillance for colon neoplasia in patients ≤45 of age with a combined diagnosis of PSC and IBD...

..Neither choline deficiency nor celiac sprue by anti-endomysial antibody testing was associated with NASH...

..Additionally, these models have also been employed for determining the optimal selection and timing for liver transplantation when advanced disease is imminent...

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..FISH testing should be used selectively in patients with other signs indicating CCA and not as a screening tool in all PSC patients undergoing endoscopic retrograde cholangiopancreatography (ERCP)...

..Some patients, however, progress to classic PSC and/or end-stage liver disease with the consequent necessity of liver transplantation...

..These two cases may indicate a possible association between SSC and AIH with CCA, thus, expanding the spectrum of risk factors of CCA...

..Despite advances in understanding of the disease, it remains one of the major indications for liver transplantation worldwide...

..Further studies are needed so that optimal HCC surveillance recommendations in this population can be determined and included in the practice guidelines for PBC...

..During the complex clinical management of these patients, liver biopsies may be obtained during evaluation of abnormal liver tests. The purpose of our study was to assess the safety and use of liver biopsy in this patient population...

..The benefits of ursodeoxycholic acid were again confirmed...

..Nevertheless, patients with PBC suffer significantly more than controls from a variety of symptoms that are beyond the immediate impact of liver failure and affect their lifestyle, personal relationships, and work activities...

..Survival was similar for patients with PBC with and without ANA. In conclusion, features of AIH in PBC may be transient and response to UDCA therapy similar to patients with PBC without features of AIH...

..This study aimed at evaluating the effect of ursodeoxycholic acid (UDCA) treatment on histologic progression in primary biliary cirrhosis (PBC)...

..Exogenous estrogens may also contribute to explain the female predominance of the disease...

..CONCLUSIONS: Based on current evidence, we cannot recommend the use of herbal supplements for the routine treatment of any chronic liver disease and further well-designed clinical trials are necessary...

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