Acute Intermittent Porphyria in an Adolescent Patient: Diagnostic and Treatment Challenges.

Acute intermittent porphyria (AIP) is a rare inherited metabolic disorder caused by decreased activity of the enzyme porphobilinogen deaminase in the heme synthesis pathway. This leads to the accumulation of toxic porphyrin precursors, such as porphobilinogen and δ-aminolevulinic acid. Clinical manifestations typically include episodic bouts of severe neurovisceral pain and autonomic dysfunction.

False-Positive Phosphatidylethanol Results Due to Blood Transfusion and Implications in the Process of Liver Transplantation Selection.

Phosphatidylethanol (PEth) testing is becoming increasingly common as a tool to assess for alcohol consumption in the practice of addiction medicine. Its potential to be an objective measure of ethanol exposure is appealing; however, the field has yet to develop a complete understanding of the factors that can influence a PEth level. Here we describe 3 patient cases in which blood transfusion within the preceding 28 days was the reason that PEth studies were positive in patients undergoing liver transplant evaluation.

Review of Current and Upcoming Second-Line Treatments for Primary Biliary Cholangitis.

Background: Treatment for primary biliary cholangitis (PBC) was defined by its singular relationship with ursodeoxycholic acid (UDCA) for decades. However, nearly 40% of patients fail to achieve adequate biochemical response with UDCA, necessitating second-line therapies.

Aims: The aim of our review was to assess the efficacy and safety of second-line therapies for PBC from phase three trials.

Alcohol-Associated Hepatitis: Short- and Long-Term Management.

Alcohol-associated hepatitis, considered a severe form of alcohol-associated liver disease, carries with it multiple negative health outcomes ranging not only to increased hospitalizations but also increased rates of mortality. While the inpatient management remains critical in optimizing clinical outcomes, a shift in focus to the outpatient management of alcohol-associated hepatitis is warranted as a long-term solution to this emerging health pandemic. Here, we review the clinical presentation, diagnosis, and current prognostication scoring systems for alcohol-associated hepatitis.

Safety of Tenofovir Disoproxil Fumarate Among Breastfeeding Infants of Patients With Chronic Hepatitis B: A Systematic Review.

An integral component to achieving worldwide chronic hepatitis B (CHB) elimination is addressing vertical transmission. Guidelines differ in their recommendations for breastfeeding while on tenofovir disoproxil fumarate (TDF). To conduct a systematic review of published studies analysing the concentration of tenofovir (TFV) in the breast milk of mothers receiving TDF and determining infant exposure from breastfeeding.

Reducing diagnostic delays in acute hepatic porphyria using health records data and machine learning.

Background: Acute hepatic porphyria (AHP) is a group of rare but treatable conditions associated with diagnostic delays of 15 years on average. The advent of electronic health records (EHR) data and machine learning (ML) may improve the timely recognition of rare diseases like AHP. However, prediction models can be difficult to train given the limited case numbers, unstructured EHR data, and selection biases intrinsic to healthcare delivery.

Hepatitis C Treatment Among Primary Care and Specialty Providers: A Single Center Study, 2015 to 2022.

Introduction: Despite national goals to eliminate Hepatitis C (HCV) and the advancement of curative, well-tolerated direct-acting antiviral (DAAs) regimens, rates of HCV treatment have declined nationally since 2015. Current HCV guidelines encourage treatment of HCV by primary care providers (PCPs). Payors have reduced restrictions to access DAAs nationally and in California however it remains unclear if the removal of these restrictions has impacted the proportion of PCPs prescribing DAAs at a health system level.

Lower FIB-4 threshold in patients with diabetes improves diagnostic accuracy of the test in a Hispanic population.

Background: Non-invasive tests (NITs) can be used to estimate the severity of fibrosis in patients with nonalcoholic fatty liver disease (NAFLD) but their diagnostic accuracy is variable. Hispanic patients are at increased risk of NAFLD and diabetes. We evaluate the diagnostic performance of the fibrosis index based on 4 factors (FIB-4) in a population of Hispanic patients who underwent vibration-controlled transient elastography (VCTE).

Natural History of Indeterminate Liver Nodules in Patients With Advanced Liver Disease: A Multicenter Retrospective Cohort Study.

Introduction: Indeterminate liver nodules (ILNs) are frequently encountered on diagnostic imaging after positive hepatocellular carcinoma (HCC) surveillance results, but their natural history remains unclear.

Methods: We conducted a multicenter retrospective cohort study among patients with ≥1 newly detected LI-RADS 3 (LR-3) lesion ≥1 cm or LI-RADS 4 (LR-4) lesion of any size (per LI-RADS v2018) between January 2018 and December 2019. Patients were followed with repeat imaging at each site per institutional standard of care.

Long-term open-label vebicorvir for chronic HBV infection: Safety and off-treatment responses.

Background & Aims: The investigational first-generation core inhibitor vebicorvir (VBR) demonstrated safety and antiviral activity over 24 weeks in two phase IIa studies in patients with chronic HBV infection. In this long-term extension study, patients received open-label VBR with nucleos(t)ide reverse transcriptase inhibitors (NrtIs).

Methods: Patients in this study (NCT03780543) previously received VBR + NrtI or placebo + NrtI in parent studies 201 (NCT03576066) or 202 (NCT03577171).

Bulevirtide and emerging drugs for the treatment of hepatitis D.

Introduction: Hepatitis delta virus (HDV) causes acute and chronic liver disease that requires the co-infection of the Hepatitis B virus and can lead to significant morbidity and mortality. Bulevirtide is a recently introduced entry inhibitor drug that acts on the sodium taurocholate cotransporting peptide, thereby preventing viral entry to target cells in chronic HDV infection. The mainstay of chronic HDV therapy prior to bulevirtide was interferon alpha, which has an undesirable side effect profile.

Epidemiology and outcomes of non-alcoholic fatty liver disease in African Americans.

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Reducing diagnostic delays in Acute Hepatic Porphyria using electronic health records data and machine learning: a multicenter development and validation study.

Importance: Acute Hepatic Porphyria (AHP) is a group of rare but treatable conditions associated with diagnostic delays of fifteen years on average. The advent of electronic health records (EHR) data and machine learning (ML) may improve the timely recognition of rare diseases like AHP. However, prediction models can be difficult to train given the limited case numbers, unstructured EHR data, and selection biases intrinsic to healthcare delivery.

Barriers to Lactulose Adherence in Patients with Cirrhosis and Hepatic Encephalopathy.

Background: Hepatic encephalopathy (HE) is a major cause of mortality and morbidity in patients with cirrhosis. Lactulose non-adherence is one of the most frequently reported precipitants of hospital admission for HE.

Aims: We aimed to identify which factors contribute most to lactulose non-adherence and propose strategies to promote greater adherence and utilization of lactulose.

MR imaging biomarkers in HCC: outcomes correlation in liver transplant listed patients.

Purpose: To determine hepatocellular carcinoma (HCC) magnetic resonance imaging (MRI) biomarkers that enable the prediction of delisting from tumor progression versus successful transplantation in patients listed for orthotopic liver transplantation (OLT).

Methods: With IRB approval and HIPPA compliance, patients with HCC awaiting OLT who were delisted due to HCC progression from 2006 to 2015 were identified. Patients with adequate MR images for review were subsequently matched with a cohort of patients successfully bridged to OLT in the same time period.

Safety and efficacy of vebicorvir administered with entecavir in treatment-naïve patients with chronic hepatitis B virus infection.

Background & Aims: Nucleos(t)ide reverse transcriptase inhibitors do not completely suppress HBV DNA in chronic HBV infection (cHBV). Vebicorvir (VBR) is an investigational core inhibitor that interferes with multiple aspects of HBV replication. This phase II trial evaluated the safety and efficacy of VBR in combination with entecavir (ETV) in treatment-naïve patients with cHBV.

Autoimmune Hepatitis-Like Syndrome Following COVID-19 Vaccination: A Systematic Review of the Literature.

Objectives: During the summer of 2021, case reports began to emerge documenting a small number of individuals who developed autoimmune hepatitis (AIH) following COVID-19 vaccination. These cases are rare and novel, and very little is known. In our systematic review, we analyzed every published case of AIH and reviewed their characteristic findings, treatment, and outcomes.

Safety and efficacy of vebicorvir in virologically suppressed patients with chronic hepatitis B virus infection.

Background & Aims: HBV nucleos(t)ide reverse transcriptase inhibitors (NrtIs) do not completely suppress HBV replication. Previous reports indicate persistent viremia during NrtI treatment despite HBV DNA being undetectable. HBV core inhibitors may enhance viral suppression when combined with NrtIs.