Portopulmonary Hypertension
PoPH is a well-recognized complication of portal hypertension with or without cirrhosis and is classified as a subset of PAH. Identification of PoPH is crucial as it has a major impact on prognosis and liver transplant candidacy. Echocardiogram is the initial screening tool of choice and the patient should proceed to RHC for confirmation. PAH-directed therapy is the treatment of choice, allowing the patient to achieve a hemodynamic threshold to undergo a liver transplant safely. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - October 18, 2022 Category: Gastroenterology Authors: Yu Kuang Lai, Paul Y. Kwo Source Type: research

Hepatocellular Carcinoma
This is a review of current practices and upcoming developments regarding hepatocellular carcinoma (HCC). This includes a contemporary review of the diagnosis, staging, and treatment of HCC. Furthermore, the authors provide a review of certain ongoing trials and future directions of various treatment modalities for HCC. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - October 18, 2022 Category: Gastroenterology Authors: Previn Ganesan, Laura M. Kulik Source Type: research

Alcohol-Related Liver Disease Including New Developments
The prevalence of alcohol consumption, alcohol use disorder (AUD), and alcohol-related liver disease (ALD) has exponentially increased over the last several years and rates continue to increase. Significant alcohol use can cause progression from steatosis in the liver to inflammation, fibrosis, and eventually cirrhosis. Additional risk factors for the progression of ALD disease include gender, race, and genetic predisposition. As such, it is essential for clinicians to understand and implement screening tools for early diagnosis of both AUD and ALD and be aware of emerging novel treatment options. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - October 17, 2022 Category: Gastroenterology Authors: Parita Virendra Patel, Steven L. Flamm Source Type: research

Evaluation of an Abnormal Liver Panel After Liver Transplantation
Abnormal liver tests are common after liver transplantation. The differential diagnosis depends on the clinical context, particularly the time course, pattern and degree of elevation, and donor and recipient factors. The perioperative period has distinct causes compared with months and years after transplant, including ischemia-reperfusion injury, vascular thrombosis, and primary graft nonfunction. Etiologies seen beyond the perioperative period include biliary complications, rejection, infection, recurrent disease, and non-transplant-specific causes. The evaluation begins with a liver ultrasound with Doppler as well as ap...
Source: Clinics in Liver Disease - October 17, 2022 Category: Gastroenterology Authors: Jacqueline B. Henson, Andrew J. Muir Source Type: research

Evaluation of the Patient with Markedly Abnormal Liver Enzymes
Liver enzyme tests are very commonly ordered by physicians, and when they return as abnormal, they can pose a clinical challenge to the provider. Markedly abnormal liver enzymes indicate severe hepatic injury and require immediate evaluation. There are various causes for abnormal liver tests, including infectious, autoimmune, genetic, metabolic, drug, and vascular causes. An understanding of the patterns of aminotransferase and alkaline phosphatase elevations is useful in narrowing the differential diagnosis. A thorough history and physical examination, appropriate blood testing, and imaging are typically key to evaluating...
Source: Clinics in Liver Disease - October 17, 2022 Category: Gastroenterology Authors: Bethany Reutemann, Fredric D. Gordon Source Type: research

Noninvasive Fibrosis Testing in Chronic Liver Disease Including Caveats
Assessment of liver fibrosis is important as the range of liver disease management has expanded, rendering biopsy both imperfect and impractical in many situations. Noninvasive tests of fibrosis leverage laboratory, imaging and elastography techniques to estimate disease extent, often with the goal of identifying advanced fibrosis. This review attempts to summarize their utility across a broad range of possible clinical scenarios while considering the central tenets of health care quality: access, quality, and cost. For each test, it also discusses the caveats whereby each test may have reduced effectiveness and how to con...
Source: Clinics in Liver Disease - October 17, 2022 Category: Gastroenterology Authors: Adam P. Buckholz, Robert S. Brown Source Type: research

Evaluation of Liver Disease in Pregnancy
Liver disease in pregnancy often requires diagnostic and therapeutic considerations that are unique to pregnancy. Liver disease in pregnancy is commonly thought of as either liver disease unique to pregnancy, chronic liver disease, or liver disease coincidental to pregnancy. This review summarizes the approach to evaluation of liver disease in pregnancy. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - October 17, 2022 Category: Gastroenterology Authors: Gres Karim, Dewan Giri, Tatyana Kushner, Nancy Reau Source Type: research

Renal Insufficiency in Patients with Cirrhosis
Renal failure is one of the most prevalent complications in patients with cirrhosis and is of the utmost prognostic relevance. Acute kidney injury (AKI) in cirrhosis results from a spectrum of etiologies, of which hepatorenal syndrome (HRS) carries the worst prognosis. Correct differentiation of the etiology of AKI in cirrhosis is imperative, as treatment defers substantially. This review summarizes the current diagnostic criteria, pathophysiology, diagnosis, and therapeutic concepts for AKI and HRS –AKI in cirrhosis. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - October 17, 2022 Category: Gastroenterology Authors: Caroline L. Matchett, Douglas A. Simonetto, Patrick S. Kamath Source Type: research

Pruritus in Chronic Liver Disease
Pruritus can be associated with chronic liver disease, particularly cholestatic liver disease. Although the pathophysiology is uncertain, there are a few proposed mechanisms and much is still being discovered. Workup involves an assessment to rule out a dermatologic, neurologic, psychogenic, or other underlying systemic disorder. First-line therapy is cholestyramine, which is generally well tolerated and effective. In those who fail cholestyramine, alternative drugs including rifampicin and μ-opioid receptor antagonists can be considered. If medical therapy is ineffective and pruritus is significant, alternative experimen...
Source: Clinics in Liver Disease - October 17, 2022 Category: Gastroenterology Authors: Ranya Selim, Joseph Ahn Source Type: research

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Inpatient and outpatient consultation for patients with complex acute or chronic liver problems for Gastroenterology community practitioners is common. These encounters require detailed assessment of complicated issues and knowledge of current recommendations for optimal care. This issue of Clinics in Liver Disease entitled “Consultations in Liver Disease” is the fifth in a series dedicated to providing updated information to community gastroenterologists for consultations commonly encountered in practice. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - October 17, 2022 Category: Gastroenterology Authors: Steven L. Flamm Tags: Preface Source Type: research

Immunologic Responses and the Pathophysiology of Primary Biliary Cholangitis
Primary biliary cholangitis (PBC) is an autoimmune liver disease with a female predisposition and selective destruction of intrahepatic small bile ducts leading to nonsuppurative destructive cholangitis. It is characterized by seropositivity of antimitochondrial antibodies or PBC-specific antinuclear antibodies, progressive cholestasis, and typical liver histologic manifestations. Destruction of the protective bicarbonate-rich umbrella is attributed to the decreased expression of membrane transporters in biliary epithelial cells (BECs), leading to the accumulation of hydrophobic bile acids and sensitizing BECs to apoptosis...
Source: Clinics in Liver Disease - September 13, 2022 Category: Gastroenterology Authors: Ruiling Chen, Ruqi Tang, Xiong Ma, Gershwin M. Eric Source Type: research

Prognostic Scoring Systems in Primary Biliary Cholangitis
Primary biliary cholangitis (PBC) is a complex, chronic disease with a heterogeneous presentation, disease progression, and response to therapy. Several prognostic models based on disease stage and/or treatment response enhance risk stratification and therapeutic management. Recent work on disease modeling proposed early prediction of outcomes at PBC onset, yet this has not been implemented in clinical practice. Although early stratification of patients based on their individual risk of developing end-stage liver disease may prove cost-effective and actually become matter of medical deontology to timely offer the best ther...
Source: Clinics in Liver Disease - September 13, 2022 Category: Gastroenterology Authors: Miki Scaravaglio, Marco Carbone Source Type: research

The Evolving Landscape of Primary Biliary Cholangitis
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by the destruction of intrahepatic bile ductules, resulting in chronic cholestasis, that can progress to advanced fibrosis, cirrhosis, portal hypertension, and hepatocellular carcinoma (HCC), and if untreated, requires liver transplantation. Ursodeoxycholic acid (UDCA) has been an effective first-line agent for this condition for approximately three decades; however, approximately one-third of patients are not complete responders to UDCA. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - September 13, 2022 Category: Gastroenterology Authors: Binu V. John Tags: Preface Source Type: research

The Inconvenient Truth of Primary Biliary Cholangitis/Autoimmune Hepatitis Overlap Syndrome
The term ‘PBC/AIH-overlap’ has been applied when features of autoimmune hepatitis (AIH), be they biochemical, serological or histological, coexist with primary biliary cholangitis (PBC), either at first presentation or sequentially during disease course. Several treatment paradigms have been proposed, ex trapolated from those of the primary conditions. However, there are no randomised studies showing improved survival with combination therapy compared to bile acid monotherapy. In the absence of high-quality evidence, multidisciplinary patient-specific approaches must be used to individualise treatme nt pathways, with a...
Source: Clinics in Liver Disease - September 13, 2022 Category: Gastroenterology Authors: Nasir Hussain, Palak J. Trivedi Source Type: research

Primary Biliary Cholangitis in Males
Primary biliary cholangitis (PBC) is an immune-mediated chronic liver disease characterized by progressive cholestasis, bile duct destruction, biliary fibrosis, and cirrhosis. Patients who respond to ursodeoxycholic acid have an expected survival similar to the general population. Although PBC primarily affects females, the prevalence in males is higher than was previously believed, with contemporary studies suggesting a female-to-male ratio of 4 –6:1. A diagnosis of PBC is often delayed among males because of the myth that PBC is rare in males. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - September 13, 2022 Category: Gastroenterology Authors: Mina Shaker, Natalie Mansour, Binu V. John Source Type: research