Advanced strategies for intensive care management of acute liver failure.

Acute liver failure (ALF) is defined as the loss of hepatic function in conjunction with hepatic encephalopathy and coagulopathy. There is histological evidence of profound hepatocyte damage. If it is not aggressively managed, ALF can be fatal within a few days.

Mildly Elevated Liver Transaminase Levels: Causes and Evaluation.

Approximately 10% to 20% of the general population has elevated liver chemistry levels, including aspartate and alanine transaminases. Elevated transaminase levels may be associated with significant underlying liver disease and increased risk of liver-related and all-cause mortality. The most common causes of mildly elevated transaminase levels (two to five times the upper limit of normal) are metabolic dysfunction-associated steatotic liver disease (MASLD) and alcoholic liver disease.

Rheumatoid factor levels indicate cryoglobulinemia severity in hepatitis B e antigen-negative hepatitis B virus carriers: a 7-year prospective cohort study.

Background: The phenotype of cryoglobulinemia in hepatitis B virus (HBV) carriers remains elusive.

Methods: A 7-year prospective cohort of 648 hepatitis B e antigen (HBeAg)-negative Taiwanese HBV carriers [males: 344 (53%)] was conducted.

Results: Among 648, 189 (29.

A Novel Compound Heterozygous Mutation in Progressive Familial Intrahepatic Cholestasis (PFIC) 4: A Rare Case Report With Literature Review.

A 12-year-old female, resident of western India, presented with a history of pruritus associated with jaundice for two months. On presentation, she had icterus with mild palpable hepatomegaly. Investigations revealed direct hyperbilirubinemia and elevated transaminases, while gamma-glutamyl transferase levels were normal.

Sequential Use of Prednisolone and Cyclosporine Is Effective in the Management of Immunotherapy-Related Hemolytic Anemia.

Immune checkpoint inhibitors (CPIs) can cause immune-related organ dysfunctions, including nephritis, pneumonitis, thyroiditis, hepatitis, colitis and more rarely hematological toxicities like immune-related autoimmune hemolytic anemia (irAIHA). Very few cases of irAIHA associated with immunotherapy have been reported, and treatment protocols remain unclear. This is partly because not all irAIHA cases are Coomb's test positive.

Liver Transplantation for Autoimmune Hepatitis: 20 Years of Tertiary Centre Experience.

We analyzed the frequency of complications and survival rates in patients with autoimmune hepatitis (AIH) who underwent liver transplantation at a high-volume transplant center. Patients who underwent transplantation for AIH at the xxx University Liver Transplantation Institute between January 2002 and December 2021 were included. Patients with a confirmed diagnosis of AIH, without concomitant chronic liver disease, were included in the study.

Development of Behçet's disease on maintenance therapy for autoimmune hepatitis.

Behçet's disease is a clinical diagnosis with variable presentations. Liver involvement is rare in the absence of vascular complications. We describe a patient diagnosed with Hashimoto's thyroiditis and autoimmune hepatitis on azathioprine who developed extensive aphthous ulcers approximately 10 years later.

Natural History and Predictors of Clinical Outcomes in Autoimmune Liver Diseases: A Multicenter Study.

Background And Aim: Prognosis in autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) has historically been poor. This multicenter retrospective cohort study investigated the natural history and assessed the predictors of outcomes in patients with AIH, PBC, and PSC.

Methods: AIH, PBC, and PSC patients were identified from the state-wide Hepascore and Clinical Outcome cohort.

Unexplained Chronically Elevated Aminotransferases: Liver Biopsy Gives Major Information with Therapeutic Implication in One Patient Out of Seven.

Background & Aims: Liver biopsy contribution in patients with unexplained elevation of transaminases is not clearly established. The aim was to study liver biopsy contribution in patients with unexplained elevated transaminases strictly defined according to the current guidelines, reflecting the present clinical practice.

Methods: In a retrospective study, we identified all the liver biopsies performed in patients with elevated transaminases for at least six months.

Liver Failure in Autoimmune Hepatitis Overlap Syndrome With Primary Biliary Cholangitis: A Case Report.

Autoimmune hepatitis (AIH) is a complex and long-term liver condition, primarily affecting women, and is marked by high levels of serum gamma globulins, the presence of circulating autoantibodies, and a genetic association. The disease can manifest in a variety of ways, ranging from mild or no symptoms to severe acute liver inflammation. AIH is often associated with other autoimmune disorders, such as primary biliary cholangitis (PBC) and autoimmune thyroiditis.

Multiorgan Involvement: Is There a Relevant Pathogenetic Link?

Multisystem autoimmune disorders have different presenting symptoms with organ involvement phased over numerous years. We have a 56-year-old homemaker who is a known case of Graves' disease-post-thyroidectomy performed 20 years ago-and developed a volume overload state with exertional dyspnea for a period of 1.5 years.

An exploratory machine learning model for predicting advanced liver fibrosis in autoimmune hepatitis patients: A preliminary study.

Introduction And Objectives: Advanced fibrosis is a crucial stage in the progression of autoimmune hepatitis (AIH), where fibrosis can either regress or advance. This study aims to leverage machine learning (ML) models for the assessment of advanced liver fibrosis in AIH patients using routine clinical features.

Patients And Methods: A total of 233 patients diagnosed with AIH and underwent liver biopsy were included in the discovery cohort.

Sulforaphane regulation autophagy-mediated pyroptosis in autoimmune hepatitis via AMPK/mTOR pathway.

Autoimmune hepatitis (AIH) is a liver disease marked by inflammation of unknown origin. If untreated, it can progress to cirrhosis or liver failure, posing a significant health risk. Currently, effective drug therapies are lacking in clinical practice.

Autoimmune-Like Hepatitis Related to SARS-CoV-2 Vaccination: Towards a Clearer Definition.

Vaccines are the most effective tool against COVID-19 and are generally safe. Very rare and heterogeneous cases of acute liver injury associated to all types of SARS-CoV-2 vaccines have been reported, mostly with autoimmune features. Epidemiological studies used heterogeneous diagnostic criteria and included different populations.

Quantitative fibrosis identifies biliary tract involvement and is associated with outcomes in pediatric autoimmune liver disease.

Background: Children with autoimmune liver disease (AILD) may develop fibrosis-related complications necessitating a liver transplant. We hypothesize that tissue-based analysis of liver fibrosis by second harmonic generation (SHG) microscopy with artificial intelligence analysis can yield prognostic biomarkers in AILD.

Methods: Patients from single-center studies with unstained slides from clinically obtained liver biopsies at AILD diagnosis were identified.

Immune-mediated liver injury caused by immune checkpoint inhibitors exhibits distinct clinical features that differ from autoimmune hepatitis.

Background: Immune-mediated liver injury caused by immune checkpoint inhibitors (ILICI) and autoimmune hepatitis (AIH) are both related to the distorted immune system. However, ILICI differs from AIH in several distinct ways. We aimed to study the differences between ILICI and AIH.

Immunization-related complex regional pain syndrome: A systematic review of case reports.

Aim: Vaccines have been shown to have the highest efficacy in preventing infectious diseases through their ability to induce immunological memory against pathogens. An adverse reaction to a vaccine is an unexpected medical occurrence following immunization. Complex regional pain syndrome (CRPS) is a disease that has undergone much controversy regarding its onset post-vaccination.

Autoimmune Hepatitis and Vitamin D Deficiency: A Nationwide Perspective.

Background: Vitamin D deficiency is linked to worse outcomes in patients with chronic liver diseases (CLD). However, data in patients with autoimmune hepatitis (AIH) remain limited.

Aims: We aimed to assess the impact of vitamin D deficiency on the outcomes of individuals with AIH.

Cefepime-Induced Mixed Hepatocellular and Cholestatic Liver Injury: A Case Report.

Drug-induced liver injury (DILI) presents significant diagnostic challenges, particularly in patients with multiple comorbidities. We report a case involving a 72-year-old female treated with cefepime for urosepsis, who developed markedly elevated liver enzymes after two weeks of therapy. After excluding other potential causes, including viral hepatitis, ischemia, and autoimmune hepatitis, cefepime-induced mixed pattern liver injury was determined to be the likely etiology of the elevated liver enzymes.

Mogamulizumab-Associated Autoimmune Diseases: Insights From FAERS Database Analysis.

Background: Mogamulizumab is a monoclonal antibody targeting the C-C chemokine receptor 4, used to treat T-cell malignancies such as cutaneous T-cell lymphoma, adult T-cell leukemia/lymphoma, and peripheral T-cell lymphoma. However, real-world studies on mogamulizumab-associated adverse events (AEs) are limited.

Methods: Disproportionality analyses were performed to assess the safety profile of mogamulizumab based on data from the US Food and Drug Administration Adverse Event Reporting System (FAERS) database for the period spanning from October 2018 to December 2023.

Utility of Mac-2 binding protein glycosylation isomer as an excellent biomarker for the prediction of liver fibrosis, activity, and hepatocellular carcinoma onset: an expert review.

Mac-2 binding protein glycosylation isomer (M2BPGi) is a liver fibrosis biomarker that originated in Japan and has been covered by health insurance for 10 years. M2BPGi is useful not only for liver fibrosis stage prediction but also for assessment of the degree of liver inflammation and prediction of hepatocellular carcinoma development. The usefulness of M2BPGi for assessing disease progression in patients with various chronic liver diseases has been demonstrated over the past decade in a large number of patients.

Lysosomal Acid Lipase Deficiency: A Report of Two Cases and a Review of the Literature.

Lysosomal acid lipase deficiency (LAL-D) is an autosomal recessive genetic disease arising from mutations in the lipase A, lysosomal acid type (LIPA) gene, characterised by the formation of cholesterol esters and triglyceride storages, primarily in the liver and spleen. By analysing the level of lysosomal acid lipase (LAL), two forms were described in the literature: Wolman disease and cholesteryl-ester storage disease (CESD). Wolman disease usually manifests with rapidly progressive symptoms within the first year of life, while CESD is a latent condition, with significant features appearing later in life.

Acute Viral Hepatitis E Presenting With Bell's Palsy and Acute Pancreatitis: A Case Report.

Hepatitis E is a hepatotropic virus and the most common cause of acute viral hepatitis among adults in India. It has four genotypes, and genotype 1 is mostly associated with sporadic cases. It typically causes self-limiting acute hepatitis following a prodromal course.

A case presentation of widespread macular amyloidosis associated with dual hepatitis B and hepatitis C infection.

Macular amyloidosis is a variant of primary localized cutaneous amyloidosis in which amyloid protein is believed to be derived from keratinocytes. The care of this variant generally focuses on addressing the associated symptoms without the need to assess for underlying disease. However, an increasing number of cases of primary localized cutaneous amyloidosis have been reported in association with systemic diseases, particularly autoimmune diseases.