Chronic Liver Disease in Patients with Prolidase Deficiency: A Case Series.

Introduction: Prolidase deficiency is a rare autosomal recessive disorder caused by variants in the gene. Patients usually have multi-organ involvement and a wide range of clinical features including recurrent skin ulcers, dysmorphic facial features, recurrent infections, intellectual disability, and splenomegaly. Studies have shown that patients with prolidase deficiency may have hepatic manifestations including hepatomegaly and abnormal liver enzymes.

Metreleptin therapy for nonalcoholic steatohepatitis: Open-label therapy interventions in two different clinical settings.

Background: Recombinant leptin therapy reverses nonalcoholic steatohepatitis (NASH) in leptin-deficient lipodystrophy. We inquired if leptin therapy would improve nonalcoholic steatohepatitis in more common forms of this heterogeneous condition.

Methods: Nine male patients with relative leptin deficiency (level < 25th percentile of body mass index- and gender-matched United States population) and biopsy-proven NASH and 23 patients with partial lipodystrophy and NASH were recruited for two distinctive open-label trials.

Non-alcoholic fatty liver disease in diabetes: When to refer to the hepatologist?

Non-alcoholic fatty liver disease (NAFLD) has become one of the most common chronic liver diseases worldwide. A strong relationship exists between NAFLD and diabetes mellitus. There is growing evidence of a mechanistically complex and strong association between the two diseases.

A Deeper Look at the Small Bowel: Training Pathways in Video Capsule Endoscopy and Device-Assisted Enteroscopy.

Background: Although there are guidelines for video capsule endoscopy (VCE) and device-assisted enteroscopy (DAE), little is known about fellowship training in these technologies.

Aims: The aims were to better characterize current small bowel endoscopy training in 3-year GI fellowship programs and 4th-year advanced endoscopy programs in the U.S.

Effect of lifetime alcohol consumption on the histological severity of non-alcoholic fatty liver disease.

Background & Aims: Non-alcoholic fatty liver disease (NAFLD) is defined based on recent alcohol consumption; however, remote or lifetime alcohol consumption is not taken into account. It is not known whether lifetime alcohol consumption contributes to the severity of disease in patients with NAFLD. To determine the effect of lifetime alcohol consumption on the histological severity in patients with NAFLD.

CD73 (ecto-5'-nucleotidase) hepatocyte levels differ across mouse strains and contribute to mallory-denk body formation.

Unlabelled: Formation of hepatocyte Mallory-Denk bodies (MDBs), which are aggregates of keratins 8 and 18 (K8/K18), ubiquitin, and the ubiquitin-binding protein, p62, has a genetic predisposition component in humans and mice. We tested the hypothesis that metabolomic profiling of MDB-susceptible C57BL and MDB-resistant C3H mouse strains can illuminate MDB-associated pathways. Using both targeted and unbiased metabolomic analyses, we demonstrated significant differences in intermediates of purine metabolism.

Clinical and histological features of idiosyncratic acute liver injury caused by temozolomide.

Objective: To describe the clinical, biochemical and histological features of 4 patients with apparent hepatotoxicity due to temozolomide and to summarize the available literature of hepatotoxicity associated with this agent.

Design: Case series

Setting: Patients were participants in the Drug-Induced Liver Injury Network a United States multicenter cooperative study.

Patients: Four patients (ages 47 to 70 years; 3 men, 1 woman) developed liver injury 1 to 7 months after starting temozolomide chemotherapy.

Effectiveness of hepatocellular carcinoma surveillance in patients with cirrhosis.

Background: Surveillance for hepatocellular carcinoma (HCC) is recommended in patients with cirrhosis, but the effectiveness of a surveillance program in clinical practice has yet to be established.

Aims: To evaluate the effectiveness of a surveillance program with ultrasound and alpha-fetoprotein (AFP) to detect early HCCs.

Methods: Four hundred and forty-six patients with Child A/B cirrhosis were prospectively enrolled between January 2004 and September 2006 and followed until July 2010.

Editorial: Exercise for NAFLD: does intensity matter?

Currently, there are no established means for the prevention or treatment of non-alcoholic steatohepatitis (NASH) despite our increasing understanding of nonalcoholic fatty liver disease (NAFLD) pathogenesis implicating insulin resistance (IR) as a key factor and highlighting the central role of lipotoxic liver injury in the development of NASH. Lifestyle interventions aiming at decreasing IR and preventing lipotoxicity, including weight loss, diet, and physical exercise, are in the frontline for NASH patient management. Physical activity may ameliorate IR, maintain weight loss, and improve liver histology in NASH patients.

Changes in insulin sensitivity and body weight during and after peginterferon and ribavirin therapy for hepatitis C.

Background & Aims: Chronic hepatitis C is associated with an increased prevalence of insulin resistance, which might result from liver disease, metabolic factors, or the hepatitis C virus (HCV) itself. The effect of antiviral treatment on insulin sensitivity is not well-known. We evaluated changes in insulin resistance and weight in patients with hepatitis C during and after peginterferon and ribavirin therapy.

Associations between serum lipids and hepatitis C antiviral treatment efficacy.

Unlabelled: Approximately one half of patients who undergo antiviral therapy for chronic hepatitis C virus (HCV) genotype 1 infection do not respond to treatment. African Americans (AAs) are less responsive to treatment than Caucasian Americans (CAs), but the reasons for this disparity are largely unknown. Recent studies suggest that serum lipids may be associated with treatment response.

Host genetics, steatosis and insulin resistance among African Americans and Caucasian Americans with hepatitis C virus genotype-1 infection.

Hepatic steatosis is the accumulation of fat in liver cells. Insulin resistance (IR) occurs when normal amounts of insulin do not stimulate insulin activity in cells. Both conditions have been described in hepatitis C virus (HCV) infection and are thought to be biologically related.

Assessing the validity of self-reported medication adherence in hepatitis C treatment.

Objective: To assess the validity of self-reported medication adherence provided by individuals in treatment for hepatitis C virus (HCV) infection with a regimen of peginterferon and ribavirin.

Methods: Adherence was evaluated prospectively among 196 African American and 205 white subjects enrolled in Virahep-C (Viral Resistance to Antiviral Therapy of Chronic Hepatitis C), a treatment study for genotype 1 HCV infection. Adherence to the prescribed dose was measured by 2 methods: self-report questions administered during multiple clinic visits, using a touch screen computer; and recordings of bottle openings, using an electronic monitor placed inside the cap of prescription containers.

Race, insulin resistance and hepatic steatosis in chronic hepatitis C.

Unlabelled: Hepatic steatosis is common in chronic hepatitis C and has been linked to concurrent obesity, insulin resistance, diabetes, disease severity, and poor response to therapy. Racial differences in rates of obesity and diabetes may contribute to racial differences in hepatic steatosis and treatment response. The aim of the present study was to compare hepatic steatosis and its associations between African American (AA) and Caucasian American (CA) patients with chronic hepatitis C, genotype 1, participating in a prospective study of peginterferon and ribavirin therapy.

Metabolic syndrome is associated with greater histologic severity, higher carbohydrate, and lower fat diet in patients with NAFLD.

Objectives: Nonalcoholic fatty liver disease (NAFLD) is considered as the hepatic manifestation of metabolic syndrome. Insulin resistance (IR) is a key component of metabolic syndrome. The aim was to determine the dietary composition, physical activity, and histologic severity between NAFLD patients with and without metabolic syndrome.

Peginterferon and ribavirin treatment in African American and Caucasian American patients with hepatitis C genotype 1.

Background & Aims: Compared with Caucasian Americans (CA), African Americans (AA) with chronic hepatitis C are less likely to respond to interferon-based antiviral therapy.

Methods: In a multicenter treatment trial, 196 AA and 205 CA treatment-naive patients with hepatitis C virus (HCV) genotype 1 infection were treated with peginterferon alfa-2a (180 microg/wk) and ribavirin (1000-1200 mg/day) for up to 48 weeks. The primary end point was sustained virologic response (SVR).

Hepatic fat fraction: MR imaging for quantitative measurement and display--early experience.

Unlabelled: The institutional review board approved this HIPAA-compliant study. After all five patients with nonalcoholic fatty liver disease signed a consent, they underwent magnetic resonance (MR) imaging for hepatic fat quantification. The purpose of this study was to develop a fast and accurate method to acquire and display quantitative maps of the percentage of hepatic fat.

One-year intense nutritional counseling results in histological improvement in patients with non-alcoholic steatohepatitis: a pilot study.

Background And Aim: In individuals with biopsy-proven non-alcoholic steatohepatitis (NASH), short-term weight loss has been shown to improve biochemical abnormalities; however, its effect on liver histology is largely unknown. The aim of the article is to determine if dietary intervention is effective in improving histological features of steatohepatitis in patients with biopsy-proven NASH.

Methods: Twenty-three patients (11M/12F) with BMI >25 kg/m(2) and biopsy-proven NASH received standardized nutritional counseling aimed at reducing insulin resistance (IR) and weight.

Prognosis of hepatocellular carcinoma: comparison of 7 staging systems in an American cohort.

Currently there is no consensus as to which staging system is best in predicting the survival of patients with hepatocellular carcinoma (HCC). The aims of this study were to identify independent predictors of survival and to compare 7 available prognostic staging systems in patients with HCC. A total of 239 consecutive patients with cirrhosis and HCC seen between January 1, 2000, and December 31, 2003, were included.

Alcohol, tobacco and obesity are synergistic risk factors for hepatocellular carcinoma.

Background/aims: Alcohol has been shown to be an important risk factor for hepatocellular carcinoma (HCC). The role of tobacco as a risk factor for HCC is controversial. Recently, obesity has been reported to be a risk factor for HCC.

A simple noninvasive index can predict both significant fibrosis and cirrhosis in patients with chronic hepatitis C.

Information on the stage of liver fibrosis is essential in managing chronic hepatitis C (CHC) patients. However, most models for predicting liver fibrosis are complicated and separate formulas are needed to predict significant fibrosis and cirrhosis. The aim of our study was to construct one simple model consisting of routine laboratory data to predict both significant fibrosis and cirrhosis among patients with CHC.

Des-gamma carboxyprothrombin can differentiate hepatocellular carcinoma from nonmalignant chronic liver disease in american patients.

Mortality due to hepatocellular carcinoma (HCC) has not improved over the last 20 years. This is in part due to the poor performance of available tumor markers leading to delays in diagnosis. Des-gamma carboxy-prothrombin (DCP) has been reported to be more sensitive and specific for the diagnosis of HCC in Japanese patients compared with alpha-fetoprotein (AFP).