Contemporary Epidemiology of Primary Biliary Cholangitis
Primary biliary cholangitis (PBC) is a cholestatic liver disease with potential evolution to liver cirrhosis when left untreated. Despite being rare, PBC has a substantial impact on the quality of life and survival of affected patients. Women are the most diagnosed worldwide; however, male subjects seem to have more aggressive disease and worse prognosis. Changing epidemiologic trends are emerging in PBC, with increasing global prevalence and slight smoothing of sex differences. In this review we present available data on incidence rates and prevalence of PBC worldwide, highlighting geographic differences and factors impac...
Source: Clinics in Liver Disease - September 11, 2022 Category: Gastroenterology Authors: Francesca Colapietro, Arianna Bertazzoni, Ana Lleo Source Type: research
Genetics of Primary Biliary Cholangitis
Primary biliary cholangitis (PBC) is a rare disease of the liver characterized by an autoimmune attack on the small bile ducts. PBC is a complex trait, meaning that a large list of genetic factors interacts with environmental agents to determine its onset. Genome-wide association studies have had a huge impact in fostering research in PBC, but many steps need still to be done compared with other autoimmune diseases of similar prevalence. This review presents the state-of-the-art regarding the genetic architecture of PBC and provides some thoughtful reflections about possible future lines of research, which can be helpful t...
Source: Clinics in Liver Disease - September 11, 2022 Category: Gastroenterology Authors: Alessio Gerussi, Rosanna Asselta, Pietro Invernizzi Source Type: research
Coronavirus Disease-2019 and Implications on the Liver
The coronavirus disease-2019 (COVID-19) pandemic has had a large impact on patients with chronic liver disease (CLD) and liver transplantation (LT) recipients. Patients with advanced CLD are at a significantly increased risk of poor outcomes in the setting of severe acute respiratory syndrome coronavirus 2 infection. The pandemic has also considerably altered the management and care that is provided to patients with CLD, pre-LT patients, and LT recipients. Vaccination against COVID-19 protects patients with CLD and LT recipients from adverse outcomes and is safe in these patients; however, vaccine efficacy may be reduced i...
Source: Clinics in Liver Disease - August 17, 2022 Category: Gastroenterology Authors: Patrick T. Campbell, Oren K. Fix Source Type: research
COVID-19 and Implications on the Liver
The COVID-19 pandemic has had a large impact on patients with CLD and LT recipients. Patients with advanced CLD are at a significantly increased risk of poor outcomes in the setting of SARS-CoV-2 infection. The pandemic has also considerably altered the management and care that is provided to patients with CLD, pre-LT patients and LT recipients. Vaccination against COVID-19 protects patients with CLD and LT recipients from adverse outcomes and is safe in these patients; however, vaccine efficacy may be reduced in LT recipients and other immunosuppressed patients. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - August 17, 2022 Category: Gastroenterology Authors: Patrick T. Campbell, Oren K. Fix Source Type: research
Overview of Progressive Familial Intrahepatic Cholestasis
Bile acid transport is a complex physiologic process, of which disruption at any step can lead to progressive intrahepatic cholestasis (PFIC). The first described PFIC disorders were originally named as such before identification of a genetic cause. However, advances in clinical molecular genetics have led to the identification of additional disorders that can cause these monogenic inherited cholestasis syndromes, and they are now increasingly referred to by the affected protein causing disease. The list of PFIC disorders is expected to grow as more causative genes are discovered. Here forth, we present a comprehensive ove...
Source: Clinics in Liver Disease - July 20, 2022 Category: Gastroenterology Authors: Sara Hassan, Paula Hertel Source Type: research
Pediatric Liver Transplantation
Liver transplantation (LT) for children results in excellent short- and long-term patient and graft survival. LT is a lifesaving procedure in children with acute or chronic liver disease, hepatic tumors, and select genetic metabolic diseases in which it can significantly improve quality of life. In this article, the authors discuss the unique aspects of pediatric LT, including the indications, appropriate patient selection and evaluation, allocation of organs, transplant surgery including the use of variant grafts, posttransplant care including immunosuppression management, prognosis, and transition of care. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - July 20, 2022 Category: Gastroenterology Authors: Sara Kathryn Smith, Tamir Miloh Source Type: research
Nonalcoholic Steatohepatitis in Children
Nonalcoholic fatty liver disease (NAFLD) is the leading cause of chronic liver disease in children. Although environmental factors are major contributors to early onset, children have both shared and unique genetic risk alleles as compared with adults with NAFLD. Treatment relies on reducing environmental risk factors, but many children have persistent diseases. No medications are approved specifically for the treatment of NAFLD, but some anti-obesity or diabetes treatments may be beneficial. Pediatric NAFLD increases the risk of diabetes and other cardiovascular risk factors. Long-term prospective studies are needed to de...
Source: Clinics in Liver Disease - July 20, 2022 Category: Gastroenterology Authors: Stavra A. Xanthakos Source Type: research
Pediatric Liver Disease
CLINICS IN LIVER DISEASE (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - July 20, 2022 Category: Gastroenterology Authors: Philip Rosenthal Source Type: research
Copyright
Elsevier (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - July 20, 2022 Category: Gastroenterology Source Type: research
Contributors
NORMAN GITLIN, MD, FRCP (LONDON), FRCPE (EDINBURGH), FAASLD, FACP, FACG (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - July 20, 2022 Category: Gastroenterology Source Type: research
Contents
Philip Rosenthal (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - July 20, 2022 Category: Gastroenterology Source Type: research
Forthcoming Issues
Primary Biliary Cholangitis (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - July 20, 2022 Category: Gastroenterology Source Type: research
Biliary Atresia in Children
Biliary atresia is a rare disease but remains the most common indication for pediatric liver transplantation as there are no effective medical therapies to slow progression after diagnosis. Variable contribution of genetic, immune, and environmental factors contributes to disease heterogeneity among patients with biliary atresia. Developing a deeper understanding of the disease mechanism will help to develop targeted medical therapies and improve patient outcomes. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - June 24, 2022 Category: Gastroenterology Authors: Swati Antala, Sarah A. Taylor Source Type: research
Alagille Syndrome
Alagille syndrome (ALGS) is a complex heterogenous disease with a wide array of clinical manifestations in association with cholestatic liver disease. Major clinical and genetic advancements have taken place since its first description in 1969. However, clinicians continue to face considerable challenges in the management of ALGS, particularly in the absence of targeted molecular therapies. In this article, we provide an overview of the broad ALGS phenotype, current approaches to diagnosis and with particular focus on key clinical challenges encountered in the management of these patients. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - June 24, 2022 Category: Gastroenterology Authors: Mohammed D. Ayoub, Binita M. Kamath Source Type: research
Alpha-1 Antitrypsin Deficiency Liver Disease
Liver disease in homozygous ZZ alpha-1 antitrypsin (AAT) deficiency occurs due to the accumulation of large quantities of AAT mutant Z protein polymers in the liver. The mutant Z protein folds improperly during biogenesis and is retained within the hepatocytes rather than appropriately secreted. These intracellular polymers trigger an injury cascade, which leads to liver injury. However, the clinical liver disease is highly variable and not all patients with this same homozygous ZZ genotype develop liver disease. Evidence suggests that genetic determinants of intracellular protein processing, among other unidentified genet...
Source: Clinics in Liver Disease - June 24, 2022 Category: Gastroenterology Authors: Anandini Suri, Dhiren Patel, Jeffrey H. Teckman Source Type: research
