Liver and skin histopathology in adults with acid sphingomyelinase deficiency (Niemann-Pick disease type B).

Acid sphingomyelinase deficiency (ASMD) is a lysosomal storage disorder characterized by the pathologic accumulation of sphingomyelin (SM) in multiple cell types, and occurs most prominently within the liver, spleen, and lungs, leading to significant clinical disease. Seventeen ASMD patients underwent a liver biopsy during baseline screening for a phase 1 trial of recombinant human acid sphingomyelinase (rhASM) in adults with Niemann-Pick disease type B. Eleven of the 17 were enrolled in the trial and each received a single dose of rhASM and underwent a repeat liver biopsy on day 14.

Long-term outcome after liver transplantation.

Liver transplantation is a life-saving therapy for patients with end-stage liver disease, acute liver failure, and liver tumors. Over the past 4 decades, improvements in surgical techniques, peritransplant intensive care, and immunosuppressive regimens have resulted in significant improvements in short-term survival. Focus has now shifted to addressing long-term complications and improving quality of life in liver recipients.

Plasma cell hepatitis (de-novo autoimmune hepatitis) developing post liver transplantation.

Purpose Of Review: Cases of de-novo autoimmune hepatitis/plasma cell hepatitis (PCH) are increasingly being diagnosed by liver transplant centers. Its pathogenesis is poorly understood but this entity appears to be a variant of rejection. Herein, we review recent clinical reports of patients developing PCH.

Mortality associated with carbapenem-resistant Klebsiella pneumoniae infections in liver transplant recipients.

Resistant bacterial infections are important causes of morbidity and mortality after liver transplantation (LT). This was a retrospective cohort study evaluating the outcomes associated with carbapenem-resistant Klebsiella pneumoniae (CRKP) infections after LT. In a 2005-2006 cohort of 175 consecutive LT recipients, 91 infection episodes were observed in 61 patients (35%).

Is there a better way to monitor abstinence among substance abusers awaiting transplantation?

Purpose Of Review: Conventional biomarkers are poor measures of ongoing abstinence. Due to short and variable windows of detection, the chances that an active substance user will be detected during the 6-month waiting period depend on the testing method and the drug. Herein we discuss hair testing as an alternative matrix for monitoring abstinence.

Outcomes of liver transplant recipients with hepatitis C and human immunodeficiency virus coinfection.

Hepatitis C virus (HCV) is a controversial indication for liver transplantation (LT) in human immunodeficiency virus (HIV)-infected patients because of reportedly poor outcomes. This prospective, multicenter US cohort study compared patient and graft survival for 89 HCV/HIV-coinfected patients and 2 control groups: 235 HCV-monoinfected LT controls and all US transplant recipients who were 65 years old or older. The 3-year patient and graft survival rates were 60% [95% confidence interval (CI) = 47%-71%] and 53% (95% CI = 40%-64%) for the HCV/HIV patients and 79% (95% CI = 72%-84%) and 74% (95% CI = 66%-79%) for the HCV-infected recipients (P < 0.

Risk for immune-mediated graft dysfunction in liver transplant recipients with recurrent HCV infection treated with pegylated interferon.

Background & Aims: Patients with recurrent hepatitis C virus infection treated with pegylated interferon (PEG) after liver transplantation can develop severe immune-mediated graft dysfunction (IGD) characterized by plasma cell hepatitis or rejection.

Methods: We conducted a multicenter case-control study of 52 liver transplant recipients with hepatitis C to assess the incidence of, risk factors for, and outcomes of PEG-IGD. Data from each patient were compared with those from 2 matched patients who did not develop PEG-IGD (n = 104).

Hepatoportal sclerosis: CT and MRI appearance with histopathologic correlation.

Objective: The purposes of this study were to describe the spectrum of cross-sectional imaging findings of pathologically proven hepatoportal sclerosis and to compare the features of advanced and nonadvanced hepatoportal sclerosis.

Materials And Methods: Eighteen patients with a histopathologic diagnosis of hepatoportal sclerosis who had concurrent MRI or CT images participated in the study. The following imaging features were assessed: presence of liver nodularity and liver lesions, portal vein patency, presence and degree of portal hypertension, liver volume, and caudate-to-right lobe ratio.

Orthotopic liver transplantation for adults with Alagille syndrome.

Introduction: Alagille syndrome (AGS) is an inherited multisystem disorder, and liver transplantation (LT) may be required in pediatric patients with AGS (P-AGS). There are limited data regarding the outcomes of LT in adults with AGS (A-AGS).

Aim: To determine and compare the outcomes of LT in A-AGS vs.

Acute cellular rejection resulting in sinusoidal obstruction syndrome and ascites postliver transplantation.

Background: The cause of ascites formation postliver transplantation (LT) is multifactorial. Sinusoidal obstruction syndrome (SOS) is a rare cause of ascites post-LT and has been reported to occur as a sequela of acute cellular rejection (ACR). We sought to examine the histologic features of patients developing ascites in the setting of ACR.

Restoration of distorted colour microscopic images from transverse chromatic aberration of imperfect lenses.

An algorithm is presented for restoration of colour microscopic images with distortions from imperfect microscope lenses having transverse chromatic aberrations, resulting in a magnification that slightly varies with wavelengths or colours. The differential of each colour component image is computed as the difference between the component image and its slightly magnified version. The absolute values in the differential component images are generally higher at the edges where greater discontinuities occur.

Liver transplantation is possible in some patients with liver metastasis of colon cancer.

Liver metastases from colorectal cancer are an absolute contraindication for liver transplantation. Aggressive therapy with liver resection and local chemotherapy in selected patients may be able to provide long-term cure. Given the risks of tumor recurrence, whether patients with post chemotherapy complications leading to liver failure should be offered transplantation is a challenging question in an era of limited organ availability.

Hepatocellular carcinoma arising in a pigmented telangiectatic adenoma with nuclear β-catenin and glutamine synthetase positivity: case report and review of the literature.

Telangiectatic hepatocellular adenoma is a rare, recently recognized subtype of hepatocellular adenoma that is often underrecognized by pathologists. We report a case of hepatocellular carcinoma arising within a pigmented telangiectatic hepatocellular adenoma in a noncirrhotic man with diffuse glutamine synthetase and nuclear β-catenin positivity. This case highlights malignant transformation of telangiectatic adenomas, and describes a previously unreported association between pigment deposition and telangiectatic adenoma.

Acute liver failure caused by 'fat burners' and dietary supplements: a case report and literature review.

Globally, people are struggling with obesity. Many effective, nonconventional methods of weight reduction, such as herbal and natural dietary supplements, are increasingly being sought. Fat burners are believed to raise metabolism, burn more calories and hasten fat loss.

Serum and ascites chromogranin-A in patients with metastatic neuroendocrine tumors.

Objectives: Ascites secondary to neuroendocrine tumor metastases may arise from a variety of mechanisms. Our aim was to measure serum and ascitic chromogranin-A (CgA) to help determine whether ascites resulted from intraperitoneal/retroperitoneal disease burden or from other carcinoid complications such as congestive heart failure or portal hypertension.

Methods: Patients with metastatic neuroendocrine tumors and ascites were identified.

Clinical characterization of patients developing histologically-proven fibrosing cholestatic hepatitis C post-liver transplantation.

Aim:   Fibrosing cholestatic hepatitis C (FCH) post-liver transplantation (LT) is an uncommon disorder with extremely poor outcome. Using stringent histological criteria, we sought to identify cases of FCH to better characterize its incidence, clinical features and outcomes.

Methods:   From January 1991 to December 2007, 973 LT for hepatitis C virus (HCV) were performed at our center.

The management of hospitalized patients with cirrhosis: the Mount Sinai experience and a guide for hospitalists.

Background: Cirrhosis and chronic liver disease carry appreciable morbidity and mortality. Cirrhotic patients frequently require hospitalization and their care is both extremely complex and labor-intensive.

Aim: We seek to provide a review for gastroenterologists, hepatologists, internists, and hospitalists on the approach to care in patients hospitalized for complications related to end-stage liver disease.

Increasing hepatic arteriole wall thickness and decreased luminal diameter occur with increasing age in normal livers.

Background & Aims: There is no data to suggest that the size of bile ducts, portal venules, and hepatic arterioles varies according to age in the normal human liver. We sought to examine whether hepatic arteriolar size, wall thickness, and luminal diameter change with increasing age.

Methods: Histologically normal liver specimens from 90 live and deceased donors were separated into three groups of thirty: donor age<30, 31-60, and>60years old.

Pneumatosis intestinalis and mesenteric venous gas - a manifestation of bacterascites in a patient with cirrhosis.

We herein report a patient with decompensated cirrhosis secondary to autoimmune hepatitis, who presented with pneumatosis intestinalis (PI) and portal venous gas. Mesenteric ischemia has been recognized as a common and life-threatening cause of PI which portends a grave prognosis. The patient was found to have bacterascites and recovered after appropriate antibiotic therapy.

The development of hepatoportal sclerosis and portal hypertension due to didanosine use in HIV.

Hepatoportal sclerosis (HPS) is one of several entities known to cause noncirrhotic portal hypertension. To date, its etiology is unknown. There have been increasing reports of HPS occurring in patients with human immunodeficiency virus (HIV), and the US Food and Drug Administration (FDA) recently issued an advisory regarding the development of noncirrhotic portal hypertension in association with didanosine (ddI) use.

Cross-genotypic polyclonal anti-HCV antibodies from human ascitic fluid.

Many anti-HCV antibodies are available, but more are needed for research and clinical applications. This study examines whether ascitic fluid from cirrhotic patients could be a source of reagent-grade antibodies. Ascitic fluid from 29 HCV patients was screened by ELISA for anti-HCV antibodies against three viral proteins: core, NS4B, and NS5A.