Symptom patterns in the daily life of PSC patients

This study examines the impact of donor-recipient sex mismatch on outcomes in liver transplantation. The researchers analyzed data from 68,950 adult liver transplant recipients in the United States between 2002 and 2019. They found that female recipients had a higher risk of graft failure and mortality compared to male recipients, regardless of donor sex. However, the risk was even higher for female recipients who received livers from male donors. Interestingly, male recipients who received livers from female donors also had an increased risk of graft failure and mortality compared to those who received male donor livers. The study suggests that sex mismatch, particularly female-to-male and male-to-female transplants, may negatively affect liver transplant outcomes. These findings highlight the importance of considering donor-recipient sex matching in liver transplantation to potentially improve long-term outcomes.

Liver complications in celiac disease

This review article discusses the association between celiac disease (CD) and various liver disorders. The authors explain that liver abnormalities are common extraintestinal manifestations of CD, ranging from mild liver enzyme elevations to severe liver diseases. The article categorizes liver complications in CD into two main types: cryptogenic liver disorders (including mild and severe liver damage) and autoimmune liver diseases. Cryptogenic liver disorders often improve with a gluten-free diet (GFD), while autoimmune liver conditions may or may not respond to GFD. The review covers specific liver conditions associated with CD, including primary biliary cirrhosis, primary sclerosing cholangitis, autoimmune hepatitis, and autoimmune cholangitis. The prevalence of CD in patients with these liver diseases is generally higher than in the general population. The authors emphasize the importance of screening for CD in patients with unexplained liver enzyme elevations or liver diseases, as early detection and treatment with a GFD can potentially improve liver function and overall health outcomes in some cases.

Donor gender does not affect liver transplantation outcome in children

The article titled “Donor gender does not affect liver transplantation outcome in children” investigates whether the gender of liver donors influences the success of liver transplants in pediatric patients. The study analyzed 335 primary liver transplants performed on children at the University of Pittsburgh Medical Center. Contrary to findings in adult liver transplants, where female-to-male donor-recipient pairings have shown higher failure rates, the study found no significant difference in transplant outcomes based on donor and recipient gender pairings in children. This conclusion held true even when considering variables such as age, etiology of liver disease, and blood group of the recipient. The researchers suggest that the lack of gender influence in pediatric liver transplants may be due to differences in hepatocyte phenotypic expression induced by puberty, which are not present in children. Thus, donor gender should not be a criterion for donor selection in pediatric liver transplantation.

“Small for size syndrome” following living donor and split liver transplantation

The article explores the complications that arise when using partial liver grafts from living donors or split cadaveric grafts. SFSS is characterized by primary dysfunction within the first post-operative week, marked by coagulopathy, ascites, and hyperbilirubinemia, primarily due to portal hyperperfusion, venous congestion, and arterial hypoperfusion. The article identifies risk factors, such as a graft to recipient weight ratio of less than 0.8 and significant steatosis, and discusses strategies to prevent SFSS, including modulating portal inflow. It also addresses the debate over the middle hepatic vein’s role in transplantation and examines the paradoxical relationship between graft size, portal flow, and liver regeneration.

Two Decades of Liver Transplants for Primary Biliary Cholangitis: A Comparative Study of Living Donors vs. Deceased Donor Liver Transplantations

This study compares outcomes of living donor liver transplantation (LDLT) and deceased donor liver transplantation (DDLT) in patients with primary biliary cholangitis (PBC) over a 20-year period (2002-2021). Analyzing data from the UNOS database, the researchers examined 3,950 liver transplants, including 3,482 DDLTs and 468 LDLTs. The results showed that LDLT recipients had superior patient survival rates compared to DDLT recipients at 1, 2, 3, 5, and 10 years post-transplant. There was no significant difference in graft survival between the two groups. LDLT also demonstrated better patient and graft survival compared to donation after circulatory death (DCD) transplants. The study identified several factors associated with increased mortality and graft failure risk, including younger age, male gender, high BMI, diabetes, and history of dialysis. Overall, the findings suggest that LDLT is a viable and potentially superior option for PBC patients requiring liver transplantation, offering improved long-term survival outcomes compared to DDLT.

Persistent Gender Disparities in Liver Transplantation

The article highlights ongoing challenges women face in accessing liver transplants despite similar mortality rates from cirrhosis compared to men. It points out that women are less likely to be wait-listed and receive transplants, partly due to the Model for End-Stage Liver Disease (MELD) score, which may underestimate women’s mortality risk. The disparities are influenced by both biological and social factors, including geographic location, healthcare access, and the MELD score’s limitations in accurately reflecting disease severity in women. The article calls for increased awareness, innovative solutions, public health campaigns, and sustained research to ensure equitable access to liver transplants for all patients, emphasizing the need for policy changes and legal reforms to address these gender disparities.

AASLD Practice Guidance on Primary Sclerosing Cholangitis and Cholangiocarcinoma

This article presents the American Association for the Study of Liver Diseases (AASLD) 2022 practice guidance on primary sclerosing cholangitis (PSC) and cholangiocarcinoma. It provides comprehensive recommendations for the diagnosis, management, and treatment of PSC, a chronic liver disease characterized by inflammation and fibrosis of the bile ducts. The guidance covers various aspects of PSC, including its epidemiology, clinical presentation, diagnostic approaches, and treatment options. It also addresses the increased risk of cholangiocarcinoma in PSC patients and provides guidelines for surveillance and management of this complication. The document emphasizes the importance of a multidisciplinary approach to PSC care, involving hepatologists, gastroenterologists, and other specialists. It offers evidence-based recommendations on topics such as the use of ursodeoxycholic acid, management of dominant strictures, and liver transplantation in PSC patients. Overall, this practice guidance serves as a comprehensive resource for healthcare providers managing patients with PSC and associated cholangiocarcinoma.

Liver enzyme alteration: a guide for clinicians

Isolated alterations of biochemical markers of liver damage in a seemingly healthy patient can present a challenge for the clinician. In this review we provide a guide to interpreting alterations to liver enzyme levels. The functional anatomy of the liver and pathophysiology of liver enzyme alteration are briefly reviewed. Using a schematic approach that classifies enzyme alterations as predominantly hepatocellular or predominantly cholestatic, we review abnormal enzymatic activity within the 2 subgroups, the most common causes of enzyme alteration and suggested initial investigations.

Surgical Technique of Orthotopic Liver Transplantation

The results of orthotopic liver transplantation (OLTx) have improved dramatically over the last few years. One of the most important contributing factors to the overall success of this operation is the refinement and standardization of the surgical procedure. Although still a complex undertaking, these technical advances have transformed the operation into one that is within the capability of many competent general and vascular surgeons and one that is practical on a large-scale basis. This article will highlight various aspects of the surgical technique of orthotopic liver transplantation (OLTx) that have evolved over a 25-year period initially at the University of Colorado in Denver, and then it will cover from 1981 onward, as practiced at the University of Pittsburgh. These techniques, or modifications thereof, have been disseminated to and adopted throughout the majority of liver transplant centers across the world. The complete transplant procedure is composed of four main stages: the donor hepatectomy, the recipient hepatectomy, the implantation of the graft (4 vascular anastomoses), followed by
hemostasis and the bile duct reconstruction.

Biliary reconstruction in liver transplant patients with primary sclerosing cholangitis, duct-to-duct or Roux-en-Y?

This article discusses the use of extracorporeal photopheresis (ECP) as a treatment for chronic graft-versus-host disease (cGVHD) in pediatric patients who have undergone allogeneic hematopoietic stem cell transplantation (HSCT). The study, conducted at the University of Ulm in Germany, involved 15 children with steroid-refractory cGVHD who received ECP treatment. The results showed that 80% of the patients responded positively to ECP, with 40% achieving complete remission and 40% partial remission. The treatment was well-tolerated, with no severe side effects reported. The study also found that ECP allowed for a significant reduction in immunosuppressive medications, particularly corticosteroids. The authors conclude that ECP is a safe and effective second-line therapy for pediatric patients with steroid-refractory cGVHD, potentially improving their quality of life and reducing the risk of long-term complications associated with prolonged immunosuppression[1].

Recurrence of disease following organ transplantation in autoimmune liver disease and systemic lupus erythematosus

This study investigates the effectiveness of extracorporeal photopheresis (ECP) in treating chronic graft-versus-host disease (cGVHD) in pediatric patients. The researchers conducted a retrospective analysis of 30 children who received ECP for steroid-refractory cGVHD following allogeneic hematopoietic stem cell transplantation. The results showed that ECP was well-tolerated and led to significant improvements in cGVHD symptoms across various organ systems. Overall response rates were high, with 73% of patients experiencing either partial or complete response. Notably, ECP treatment allowed for a substantial reduction in steroid use, with 43% of patients able to discontinue steroids entirely. The study concludes that ECP is a safe and effective second-line therapy for pediatric cGVHD, offering the potential to improve quality of life and reduce long-term complications associated with prolonged immunosuppression.

Living-Donor Liver Transplant and Improved Post-Transplant Survival in Patients with Primary Sclerosing Cholangitis

This study evaluates the outcomes of living donor liver transplantation (LDLT) versus deceased donor liver transplantation (DDLT) in patients with primary sclerosing cholangitis (PSC) using data from the United Network for Organ Sharing (UNOS) database. The analysis included 4,679 DDLTs and 805 LDLTs performed between 2002 and 2019. The findings indicate that LDLT recipients had significantly better patient and graft survival rates at 1, 3, 5, and 10 years post-transplant compared to DDLT recipients. Specifically, LDLT showed superior patient survival rates (95.2%, 92.6%, 90.1%, and 81.9%) compared to DDLT (93.2%, 87.6%, 83.3%, and 72.7%) and better graft survival rates (94.1%, 91.1%, 88.5%, and 80.5%) compared to DDLT (92.1%, 86.5%, 82.1%, and 70.9%). Factors such as younger recipient age, male gender, higher MELD score, diabetes, hepatocellular carcinoma, and cholangiocarcinoma were associated with increased mortality and graft failure. The study concludes that LDLT offers a survival benefit over DDLT for PSC patients, suggesting it as a viable option to improve long-term outcomes.

Biliary complications after adult-to-adult living-donor liver transplantation: An international multicenter study of 3633 cases

This study looked at bile-related problems in liver transplants from living donors. It involved 3,633 adult patients from 18 centers between 2016 and 2020. The main issues were bile leaks (11.4% of cases) and narrowing of bile ducts (20.6% of cases). Risk factors for bile leaks included having multiple bile duct connections, a specific surgical technique called Roux-en-Y, and previous major abdominal surgery. For bile duct narrowing, risks included blood type incompatibility, high blood loss during surgery, and previous abdominal surgery. Patients with these complications stayed in the hospital longer and had more serious issues overall. The study found that these bile problems significantly reduced the survival of the transplanted liver.

The researchers conclude that careful selection of donors and better planning before surgery are crucial to reduce these risks and improve transplant success.

Physical Function, Physical Activity, and Quality of Life After Liver Transplantation (May 2020)

This article presents a comprehensive review of extracorporeal photopheresis (ECP) as a treatment for chronic graft-versus-host disease (cGVHD) in both adult and pediatric patients. ECP is described as an immunomodulatory therapy that involves collecting peripheral blood mononuclear cells, exposing them to ultraviolet A light in the presence of 8-methoxypsoralen, and then reinfusing them into the patient. The review highlights ECP’s efficacy in treating steroid-refractory cGVHD, with response rates ranging from 61% to 91% across various studies. The treatment is noted for its excellent safety profile, with minimal side effects and the ability to reduce steroid use in responding patients. The authors discuss the challenges in standardizing ECP protocols and outcome measures, as well as the need for prospective randomized trials to further establish its role in cGVHD management. They also explore potential biomarkers for predicting treatment response and emphasize the importance of early intervention with ECP to improve outcomes.

Physical Fitness, Fatigue, and Quality of Life after Liver transplantation (Feb. 2007)

This article discusses the use of extracorporeal photopheresis (ECP) as a treatment for chronic graft-versus-host disease (cGVHD) in pediatric patients following allogeneic hematopoietic stem cell transplantation (HSCT). The study, conducted at the University of Ulm in Germany, involved 15 children with steroid-refractory cGVHD who received ECP treatment. The results showed that 80% of patients responded positively to ECP, with 40% achieving complete remission and 40% partial remission[1]. The treatment was well-tolerated, with no severe side effects reported. Importantly, ECP allowed for a significant reduction in immunosuppressive medications, particularly corticosteroids. The authors conclude that ECP is a safe and effective second-line therapy for pediatric patients with steroid-refractory cGVHD, potentially improving their quality of life and reducing the risk of long-term complications associated with prolonged immunosuppression.

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National Living Donor Assistance Center

The National Living Donor Assistance Center (NLDAC) website provides information and support for individuals considering living organ donation. The organization aims to remove financial barriers to donation by offering assistance with travel expenses, lost wages, and dependent care costs for eligible donors[1]. The site allows potential donors to check their eligibility for assistance and begin the application process. Additionally, NLDAC encourages visitors to participate in a survey about living organ donation and offers the opportunity to contribute financially to their mission of supporting living donors[1].