Inborn errors of metabolism (IEM) encompasses a diverse group of genetic disorders with profound implications for both pediatric and adult hepatology. Traditionally associated with pediatric populations, these conditions...Inborn errors of metabolism (IEM) encompasses a diverse group of genetic disorders with profound implications for both pediatric and adult hepatology. Traditionally associated with pediatric populations, these conditions are increasingly recognized in adults, thanks to advancements in diagnostic tools such as whole exome sequencing. This review delves into the pathophysiology, clinical spectrum, diagnostic innovations, and therapeutic strategies for IEM-related liver diseases, offering a comprehensive reference for hepatology practitioners across the lifespan. Through illustrative case studies and actionable clinical frameworks, this article underscores the transformative potential of genomic medicine in shaping the diagnosis, treatment, and understanding of IEM in liver disease.
The number of patients awaiting liver transplant exceeds the number of liver grafts available. However, emerging technologies offer hope. Machine perfusion enhances the preservation, graft quality, and utilization of mar...The number of patients awaiting liver transplant exceeds the number of liver grafts available. However, emerging technologies offer hope. Machine perfusion enhances the preservation, graft quality, and utilization of marginal livers, thereby reducing unnecessary graft discards. Xenotransplantation provides an alternative organ source, augmenting the donor supply or serving as a bridge for critically ill patients. These innovations are described in this review, as the recent clinical applications of these technologies promise to alleviate organ scarcity, improve transplant outcomes, and save lives.
Disease recurrence after liver transplantation (LT) is common. Certain liver diseases such as autoimmune hepatitis and steatotic liver disease may appear de novo after LT. This review discusses post LT alcohol-associated...Disease recurrence after liver transplantation (LT) is common. Certain liver diseases such as autoimmune hepatitis and steatotic liver disease may appear de novo after LT. This review discusses post LT alcohol-associated liver disease, metabolic dysfunction-associated liver disease, autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis. Both recurrent and de novo diseases are important causes of allograft failure.
Reflecting continued enhancements of surgical techniques and immunosuppression, liver transplant recipients are experiencing increased longevity. Once patients have successfully navigated the perioperative and early post...Reflecting continued enhancements of surgical techniques and immunosuppression, liver transplant recipients are experiencing increased longevity. Once patients have successfully navigated the perioperative and early posttransplant period, their survival depends on management of immunosuppression to minimize systemic side effects that will contribute to the development of medical comorbidities while avoiding rejection. This includes mitigating cardiovascular risk factors and other components of the metabolic syndrome. The concept of transplant survivorship emphasizes a holistic approach to improving a patient's quality of life. Highlighting not only the medical management, transplant survivorship also stresses the psychological, mental, and spiritual well-being to further improve long-term outcomes.
The introduction of calcineurin inhibitors (CNIs) as the primary form of immunosuppression (IS) for liver transplantation (LT) in the late 1970s was a key in increasingly successful outcomes for transplantation over the...The introduction of calcineurin inhibitors (CNIs) as the primary form of immunosuppression (IS) for liver transplantation (LT) in the late 1970s was a key in increasingly successful outcomes for transplantation over the past 3 decades. Despite the side effects of CNI which directly contribute to the long-term morbidity and mortality post-LT, they will remain the cornerstone of IS in the near future. Efforts to minimize exposure to CNI will require the application of blood and tissue biomarkers that accurately identify the extent of IS and risk of rejection for individual patients.
Renal dysfunction is common in patients undergoing liver transplant (LT) evaluation, thereby making it imperative for hepatologists to know how to diagnose, manage, and optimize treatment of acute kidney injury (AKI) and...Renal dysfunction is common in patients undergoing liver transplant (LT) evaluation, thereby making it imperative for hepatologists to know how to diagnose, manage, and optimize treatment of acute kidney injury (AKI) and chronic kidney disease. This article reviews pre-transplant, peri-transplant, and post-transplant AKI diagnosis and management, and the role of renal replacement therapy in LT candidates.
The term "endohepatology" was coined over the last 15 years to describe the integration of interventional endoscopy and hepatology for the management of patients with chronic liver disease. In this article, we will revie...The term "endohepatology" was coined over the last 15 years to describe the integration of interventional endoscopy and hepatology for the management of patients with chronic liver disease. In this article, we will review the diagnostic and therapeutic applications of endoscopic ultrasound and endoscopic retrograde cholangiopancreatography among liver transplantation candidates and recipients.
Liver transplantation remains a life-saving therapy for a growing list of indications. Although 10,660 adult liver transplants were performed in the United States in 2023, a 50% increase over the preceding decade, the de...Liver transplantation remains a life-saving therapy for a growing list of indications. Although 10,660 adult liver transplants were performed in the United States in 2023, a 50% increase over the preceding decade, the demand continues to far exceed the supply. Efforts to expand the liver donor pool by using donors that were previously considered unsuitable have remained an important strategy to help overcome shortages. We discuss the progress that has been made over the past decade, as well as potential future barriers that will need to be overcome to help successfully expand the liver donor pool.
Hepatocellular carcinoma (HCC) and cholangiocarcinoma are the primary hepatic malignancies with established pathways to transplantation and model for end-stage liver disease (MELD) exception points. Other tumors managed...Hepatocellular carcinoma (HCC) and cholangiocarcinoma are the primary hepatic malignancies with established pathways to transplantation and model for end-stage liver disease (MELD) exception points. Other tumors managed with liver transplantation (LT) include hepatic epithelioid hemangioendothelioma and fibrolamellar HCC. LT for metastatic neuroendocrine tumor has been established with patient selection criteria and a path to MELD exception points. Additionally, recent data on LT for patients with unresectable hepatic colorectal metastases demonstrate increasingly encouraging initial results.
Major advances in managing critically ill patients with liver disease have improved their prognosis and access to intensive care facilities. Acute-on-chronic liver failure (ACLF) is now a well-defined disease and these p...Major advances in managing critically ill patients with liver disease have improved their prognosis and access to intensive care facilities. Acute-on-chronic liver failure (ACLF) is now a well-defined disease and these patients can be fast-tracked for liver transplantation (LT) with good outcomes if there are no contraindications. In acute liver failure, plasma exchange has improved prognosis for patients not eligible for immediate transplant. Further advances in novel therapies and refinement of the criteria for early LT in ACLF and also clinical implementation of artificial intelligence tools will probably constitute the next major breakthroughs in critically ill patients with liver disease.
Substance use disorders (SUDs) are rising in the general population and also in the transplant population. Alcohol use disorder leading to alcohol-associated liver disease is the most common of all SUDs in the liver tran...Substance use disorders (SUDs) are rising in the general population and also in the transplant population. Alcohol use disorder leading to alcohol-associated liver disease is the most common of all SUDs in the liver transplantation setting. Uncontrolled SUDs and relapse after transplant can lead to graft loss and mortality, as well as reduce quality of life and patient functioning. SUDs can be treated effectively through integrated, interprofessional management by addiction and medical/surgical professional members of the transplant team. Teams should be aware of pitfalls in interprofessional teamwork and communication so that corrective steps could be implemented for improved efficiency.
Rady ED, Anouti A, Thimphitthaya C
… +1 more, Cotter TG
Clin Liver Dis
· 2025 May · PMID 40287265
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Full text
Alcohol-associated liver disease (ALD) is the foremost cause of liver-related mortality in the United States comprising a spectrum of conditions from simple hepatic steatosis to more severe alcohol-associated cirrhosis a...Alcohol-associated liver disease (ALD) is the foremost cause of liver-related mortality in the United States comprising a spectrum of conditions from simple hepatic steatosis to more severe alcohol-associated cirrhosis and alcohol-associated hepatitis. There has been growing acceptance and application of early liver transplantation (eLT) for ALD. There is robust evidence for excellent patient and graft survival rates for eLT for ALD. Nevertheless, recidivism remains a major concern. This article aims to explore the recent trends in liver transplantation for ALD, as well as the advances in practice and outcomes, with focus on eLT and emerging challenges in this field.
Indications for liver transplantation continue to evolve rapidly, with the number of transplant procedures and donor organs more than doubling in the last 20 years. Despite the increase in donors and transplantation, the...Indications for liver transplantation continue to evolve rapidly, with the number of transplant procedures and donor organs more than doubling in the last 20 years. Despite the increase in donors and transplantation, there is broad geographic disparity in access to liver transplantation. Effective therapies for hepatitis C virus (HCV) have resulted in deep, sustained declines in HCV-related cirrhosis as an indication, with simultaneous increases in the frequency of liver transplantation for metabolic dysfunction-associated steatohepatitis and alcohol-related liver disease, especially among patients with brief periods of sobriety. MELD score-based allocation policies do not serve all indications for liver transplantation equally.
Embolic and ablative locoregional therapies (LRTs) for hepatocellular carcinoma are widely used to cure, bridge, or downstage patients for more definitive therapies. Common ablative therapies include microwave ablation a...Embolic and ablative locoregional therapies (LRTs) for hepatocellular carcinoma are widely used to cure, bridge, or downstage patients for more definitive therapies. Common ablative therapies include microwave ablation and radiofrequency ablation, while embolic options include transarterial chemoembolization and 90Y transarterial radioembolization. While these therapies can be highly effective for the appropriate stage of disease, LRTs can suffer from a high rate of posttreatment recurrences. Considerations for administration of specific therapies include disease burden and underlying liver function. Recent data on concomitant or adjuvant systemic therapy, with LRT, have the potential to improve disease control and improve outcomes in this high-risk patient population.
Liver transplantation (LT) is the standard-of-care for early hepatocellular carcinoma (HCC). Current selection criteria depend primarily on measures of tumor burden and alpha-fetoprotein levels. Evolving strategies inclu...Liver transplantation (LT) is the standard-of-care for early hepatocellular carcinoma (HCC). Current selection criteria depend primarily on measures of tumor burden and alpha-fetoprotein levels. Evolving strategies include the application of prognostic scores and the development of specialized molecular markers to predict recurrence. New technologies such as machine perfusion of donor organs are expected to dramatically improve the availability and access to LT in HCC.
Hepatic resection has long been considered the preferred treatment for hepatocellular carcinoma (HCC) when feasible, but its role, as well as the outcomes is evolving rapidly. This article explores the impact of the chan...Hepatic resection has long been considered the preferred treatment for hepatocellular carcinoma (HCC) when feasible, but its role, as well as the outcomes is evolving rapidly. This article explores the impact of the changing demographics of HCC, reviews current criteria for resection, considers the roles of liver transplantation and nonsurgical locoregional therapies vis-a-vis resection, highlights the potential of new systemic therapies (particularly immune checkpoint inhibitors) to improve outcomes, details the common complications associated with resection, and discusses recurrence of HCC after resection and its management.
In the setting of hepatocellular carcinoma (HCC) surveillance, a range of observations can be identified. Often, imaging findings and biochemical results are consistent with the diagnosis of HCC. However, challenges in H...In the setting of hepatocellular carcinoma (HCC) surveillance, a range of observations can be identified. Often, imaging findings and biochemical results are consistent with the diagnosis of HCC. However, challenges in HCC surveillance can arise in different clinical contexts, particularly when imaging results and tumor biomarkers are discordant. In this article, the authors describe 5 clinical scenarios based on our experiences in which additional evaluation was necessary to determine whether HCC was present or not.
Promoting the early detection and diagnosis of hepatocellular carcinoma (HCC) is a critical strategy to improve patient outcomes as this can lead to greater access to curative treatments. This review highlights the diagn...Promoting the early detection and diagnosis of hepatocellular carcinoma (HCC) is a critical strategy to improve patient outcomes as this can lead to greater access to curative treatments. This review highlights the diagnostic tests for HCC, including the use of the Liver Imaging Reporting and Data System systems and histopathology. Staging is essential for informing prognosis and guiding treatment decisions; this review also covers a widely used and well-validated staging system called the Barcelona-Clinic Liver Cancer (BCLC) algorithm. The BCLC incorporates tumor status, liver function, and patient performance to stage patients with newly diagnosed HCC.