IgG4 plasma cell neoplasm in liver transplant biopsy masquerading as rejection.

IgG4 plasma cell neoplasm and myeloma are rare disease entities, not associated with systemic fibroinflammatory IgG4 related disease. We herein present a case of IgG4 plasma cell neoplasm in a liver transplant biopsy. A 55 year old female was treated with living donor transplant and had a complicated post-operative course.

Vibration-controlled Transient Elastography for Assessment of Liver Fibrosis at a USA Academic Medical Center.

Background And Aims: Vibration-controlled transient elastography (VCTE) is a noninvasive tool that uses liver stiffness measurement (LSM) to assess fibrosis. Since real-life data during everyday clinical practice in the USA are lacking, we describe the patterns of use and diagnostic performance of VCTE in patients at an academic medical center in New York City.

Methods: Patients who received VCTE scans were included if liver biopsy was performed within 1 year.

Student-Authored Autopsy Reports of Anatomical Donors: Their First Patients.

The preparation of student-authored autopsy reports of anatomical donors was added to the Gross Anatomy course to integrate the basic and clinical sciences and determine whether students considered this early clinical exposure to be a valuable experience. All donors were scanned using computerized tomography (CT) and student groups received the scan of their donor and a report written by a radiologist. As students dissected, they took photographs and biopsies of pathological findings that were processed for microscopic evaluation.

A molecular single-cell lung atlas of lethal COVID-19.

Respiratory failure is the leading cause of death in patients with severe SARS-CoV-2 infection, but the host response at the lung tissue level is poorly understood. Here we performed single-nucleus RNA sequencing of about 116,000 nuclei from the lungs of nineteen individuals who died of COVID-19 and underwent rapid autopsy and seven control individuals. Integrated analyses identified substantial alterations in cellular composition, transcriptional cell states, and cell-to-cell interactions, thereby providing insight into the biology of lethal COVID-19.

Hepatic pathology in patients dying of COVID-19: a series of 40 cases including clinical, histologic, and virologic data.

The novel coronavirus SARS-CoV-2 (coronavirus disease 19, or COVID-19) primarily causes pulmonary injury, but has been implicated to cause hepatic injury, both by serum markers and histologic evaluation. The histologic pattern of injury has not been completely described. Studies quantifying viral load in the liver are lacking.

Transient-mixed Chimerism With Nonmyeloablative Conditioning Does Not Induce Liver Allograft Tolerance in Nonhuman Primates.

Background: Although short-term outcomes for liver transplantation have improved, patient and graft survival are limited by infection, cancer, and other complications of immunosuppression. Rapid induction of tolerance after liver transplantation would decrease these complications, improving survival and quality of life. Tolerance to kidneys, but not thoracic organs or islets, has been achieved in nonhuman primates and humans through the induction of transient donor chimerism.

COVID-19 Associated Hepatitis Complicating Recent Living Donor Liver Transplantation.

We present a case of COVID-19 hepatitis in a living donor liver allograft recipient whose donor subsequently tested positive for COVID-19. The patient is a female infant with biliary atresia (failed Kasai procedure). She recovered well, with improving liver function tests for 4 days.

Utility of 3D Printed Cardiac Models for Medical Student Education in Congenital Heart Disease: Across a Spectrum of Disease Severity.

The most common modes of medical education for congenital heart disease (CHD) rely heavily on 2-dimensional imaging. Three-dimensional (3D) printing technology allows for the creation of physical cardiac models that can be used for teaching trainees. 3D printed cardiac models were created for the following lesions: pulmonic stenosis, atrial septal defect, tetralogy of Fallot, d-transposition of the great arteries, coarctation of the aorta, and hypoplastic left heart syndrome.

Pediatric Nonalcoholic Fatty Liver Disease in New York City: An Autopsy Study.

Objective: To assess the prevalence and severity of nonalcoholic liver disease (NAFLD) in children in a diverse population sample in New York City.

Study Design: Liver specimens were examined from children 2-19 years old who died of unexpected causes within 48 hours of medical presentation and underwent autopsy in New York City from 2005 to 2010. Records were reviewed for age, sex, weight, height, and race.

Novel H-shunt Venovenous Bypass for Liver Transplantation in Cynomolgus Macaques.

Cynomolgus monkeys are often used in preclinical transplantation research. Performing liver transplantation in cynomolgus monkeys is challenging because they poorly tolerate portal vein clamping during the anhepatic phase. Finding an alternative to portal vein clamping is necessary before preclinical liver transplant models can be performed with reliable outcomes.

Heart Disease and the Liver: Pathologic Evaluation.

Liver injury due to acute and chronic heart failure has long been recognized. This article discusses the concepts of acute cardiogenic liver injury (ACLI) and cardiac or congestive hepatopathy (CH) along with their clinical manifestations and sequelae. Histologically, ACLI manifests as centrilobular hepatocellular necrosis, whereas CH is associated with centrilobular hepatocyte atrophy, dilated sinusoids, and perisinusoidal fibrosis, progressing to bridging fibrosis and ultimately cirrhosis.

Current Concepts in Pediatric Nonalcoholic Fatty Liver Disease.

Nonalcoholic fatty liver disease (NAFLD) represents a spectrum of disease. Its increasing prevalence is a direct result of historically high rates of obesity. Hepatocyte lipid accumulation is the first step in a cascade of metabolic and inflammatory events thought to precipitate NAFLD.

Hepatocyte TAZ/WWTR1 Promotes Inflammation and Fibrosis in Nonalcoholic Steatohepatitis.

Nonalcoholic steatohepatitis (NASH) is a leading cause of liver disease worldwide. However, the molecular basis of how benign steatosis progresses to NASH is incompletely understood, which has limited the identification of therapeutic targets. Here we show that the transcription regulator TAZ (WWTR1) is markedly higher in hepatocytes in human and murine NASH liver than in normal or steatotic liver.

TTC39B deficiency stabilizes LXR reducing both atherosclerosis and steatohepatitis.

Cellular mechanisms that mediate steatohepatitis, an increasingly prevalent condition in the Western world for which no therapies are available, are poorly understood. Despite the fact that its synthetic agonists induce fatty liver, the liver X receptor (LXR) transcription factor remains a target of interest because of its anti-atherogenic, cholesterol removal, and anti-inflammatory activities. Here we show that tetratricopeptide repeat domain protein 39B (Ttc39b, C9orf52) (T39), a high-density lipoprotein gene discovered in human genome-wide association studies, promotes the ubiquitination and degradation of LXR.

The Pathology of Acute Liver Failure.

Acute liver failure (ALF) is a rare and severe liver disease that usually develops in 8 weeks or less in individuals without preexisting liver disease. Its chief causes worldwide are hepatitis virus infections (hepatitis A, B, and E) and drug hepatotoxicity (particularly intentional or unintentional acetaminophen toxicity). Massive hepatic necrosis is often seen in liver specimens in ALF and features marked loss of hepatocytes, variable degrees of inflammation, and a stereotypic proliferation of bile ductular structures (neocholangioles) derived from activated periportal hepatic progenitor cells.

Hepatocellular adenoma classification: a comparative evaluation of immunohistochemistry and targeted mutational analysis.

Background: Four subtypes of hepatocellular adenomas (HCA) are recognized: hepatocyte-nuclear-factor-1α mutated (H-HCA), β-catenin-mutated type with upregulation of glutamine synthetase (b-HCA), inflammatory type (IHCA) with serum-amyloid-A overexpression, and unclassified type. Subtyping may be useful since b-HCA appear to have higher risk of malignant transformation. We sought to apply subtype analysis and assess histological atypia, correlating these with next-generation sequencing analysis.

Genome-wide association study in mice identifies loci affecting liver-related phenotypes including Sel1l influencing serum bile acids.

Unlabelled: Using publicly available data from inbred mouse strains, we conducted a genome-wide association study to identify loci that accounted for liver-related phenotypes between C57BL/6J and A/J mice fed a Paigen diet. We confirmed genome-wide significant associations for hepatic cholesterol (chromosome 10A2) and serum total bile acid concentration (chromosome 12E) and identified a new locus for liver inflammation (chromosome 7C). Analysis of consomic mice confirmed that chromosome 12 A/J alleles accounted for the variance in serum total bile acid concentrations and had pleiotropic effects on liver mass, serum cholesterol, and serum alanine aminotransferase activity.

Hepatocellular Carcinoma in Noncirrhotic Liver with Glycogenotic Foci: Basic Science Meets Genomic Medicine.

During the past decade, the application of genomic analysis to liver tumors has provided extensive data concerning tumor phenotypes, signatures, outcomes, and prognosis. In this report the authors describe a middle-aged man without known risk factors for liver disease or hepatocellular carcinoma (HCC) who developed a 19-cm HCC in his right lobe. The underlying liver was normal histologically except for multifocal glycogenotic foci similar to those found in experimental chemical carcinogenesis.

Longterm outcomes of auxiliary partial orthotopic liver transplantation in preadolescent children with fulminant hepatic failure.

By preserving part of the native liver, auxiliary partial orthotopic liver transplantation (APOLT) provides the advantage of potential immunosuppression (ISP) withdrawal if the native liver recovers but has had limited acceptance, especially in the United States, due to technical complications and low rates of native liver regeneration. No previous study has evaluated APOLT specifically for preadolescent children with fulminant hepatic failure (FHF). This population might benefit especially based on greater capacity for liver regeneration.

The Evolving Role of Nonalcoholic Fatty Liver Disease in Hepatic Neoplasia: Inflammatory Hepatocellular Adenoma in a Man with Metabolic Syndrome.

Growing awareness of the spectrum of liver diseases related to nonalcoholic fatty liver disease (NAFLD) has drawn attention to the complex pathogenetic pathways that are operative in livers with macrovesicular steatosis and to the potential development of hepatocellular adenoma and hepatocellular carcinoma in unusual clinical settings. This report describes an older man with metabolic syndrome who developed a 3.9 cm.

Discovery of a Novel Hepatovirus (Phopivirus of Seals) Related to Human Hepatitis A Virus.

Unlabelled: Describing the viral diversity of wildlife can provide interesting and useful insights into the natural history of established human pathogens. In this study, we describe a previously unknown picornavirus in harbor seals (tentatively named phopivirus) that is related to human hepatitis A virus (HAV). We show that phopivirus shares several genetic and phenotypic characteristics with HAV, including phylogenetic relatedness across the genome, a specific and seemingly quiescent tropism for hepatocytes, structural conservation in a key functional region of the type III internal ribosomal entry site (IRES), and a codon usage bias consistent with that of HAV.