Twenty-Year Comparative Analysis of Patients With Autoimmune Liver Diseases on Transplant Waitlists.

BACKGROUND & AIMS: The rarity of autoimmune liver disease poses challenges to epidemiology studies. However, waitlists for liver transplantation can be used to study patients with end-stage liver diseases. We used these waitlists to assess trends in numbers and demographics of patients awaiting liver transplant for primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), or autoimmune hepatitis (AIH). METHODS: We collected data from UK and US national registries for all adults on liver transplant waitlists, from January 1, 1995, through December 31, 2014. We analyzed data from patients with PBC (n = 1434 in the United Kingdom and n = 5598 in the United States), PSC (n = 1093 in the United Kingdom and n = 6820 in the United States), and AIH (n = 538 in the United Kingdom and n = 4949 in the United States). Numbers of listings per year were adjusted to the estimated populations during each year. Regression analyses were used to examine trends and comparative statistics were used to evaluate differences in individual characteristics among groups. RESULTS: Over the total study period, listings for PBC were 1.2 and 1.0 per million population per year in the United Kingdom and United States, respectively; for PSC, 0.9 and 1.2 per million population per year; and for AIH, 0.5 and 0.8 per million population per year. Over the period studied, numbers of listings for PBC decreased by 50% in both countries; changes in numbers of listings for PSC and AIH were smaller and not consistent between countries. By 2014, PSC had become the leading indication for liver transplantation among patients with autoimmune liver diseases in both countries. Median patient ages at time of listing were lower than those reported as median age of diagnosis for AIH and PBC. The ratio of women:men with PBC decreased by almost 50% from 1995 through 2014. Men with PSC were placed on the waitlist with higher disease severity scores than women in both countries. Among patients with PBC, those of black race were under-represented on waitlists from both countries. Among patients with PSC, Hispanics were under-represented on waitlists in the United States. Patients of non-white races were placed on waitlists at younger ages for all diseases; age differences in waitlist placement varied by up to 10 years, depending on race, among patients with PBC. CONCLUSIONS: In an analysis of data collected from UK and US national liver transplant registries over 20 years, we found that PSC has become the leading indication for liver transplantation among patients with autoimmune liver diseases. Numbers of patients with PBC placed on waitlists, and the ratio of women:men with PBC, each decreased by almost 50%, possibly due to increased treatment with ursodeoxycholic acid. Within groups of patients on the transplant waitlist for PBC, PSC, or AIH, we found differences in age, sex, disease severity scores, and ethnicity between diseases and countries that require further study.

Combined Lung-Liver and Delayed Kidney Transplantation for Cystic Fibrosis Clinical Approach and Outcome: A Case Report.

Reports on the long-term outcomes and immunosuppressive regimens of multiorgan transplant patients are limited. Here, we describe a patient with cystic fibrosis complicated by multiorgan failure who was successfully treated with combined liver lung transplant and delayed kidney transplant, resulting in excellent outcomes. Delayed kidney transplant was done to reduce the operative stress of a single procedure, giving time for adequate resuscitation and weaning from vasopressors. Our patient's postoperative course was complicated by post-transplant lymphoproliferative disease, which was successfully treated with rituximab and reduced dosages of immunosuppression.

Most pediatric transplant centers are low volume, adult-focused, and in proximity to higher volume pediatric centers.

BACKGROUND: Independent studies provide evidence that low volume pediatric solid organ transplant centers have inferior outcomes compared to high volume pediatric centers. The study assessed whether patients treated at low volume pediatric centers have access to higher volume pediatric centers, which offer potentially better outcomes. METHODS: We analyzed center specific data on 467 pediatric solid organ transplant centers in the U.S using the Organ Procurement and Transplantation Network database from 2002 to 2014. The proximities of low volume pediatric centers to high volume pediatric centers were determined using Maptive, a tool based on Google Maps. RESULTS: Most low volume pediatric transplant centers focused on transplantation of adults (84% heart, 83% liver, and 93% kidney programs). A majority of low volume pediatric centers (77% for heart, 53% for lung, 68% for liver and 90% for kidney) were within 150 miles of high volume centers. Among all children listed for transplantation, 30.7% were listed in low volume pediatric centers. Most low volume pediatric centers are adult focused and near high volume pediatric centers. CONCLUSION: We need greater scrutiny of outcomes, particularly waitlist outcomes, of low volume pediatric solid organ transplant centers located close to high volume pediatric solid organ transplant centers. TYPE OF STUDY AND LEVEL OF EVIDENCE: Retrospective Comparative Study, Level III.

Haemophagocytic lymphohistiocytosis presenting as liver failure following Epstein-Barr and prior hepatitis A infections.

Haemophagocytic lymphohistiocytosis (HLH) is associated with high mortality even after prompt diagnosis. We present a young man with HLH triggered by two common viral diseases, infectious mononucleosis and hepatitis A. This patient presented with fever, rapidly progressive liver failure, anasarca and cholestasis, followed by anaemia and neutropenia. His carbohydrate antigen 19-9 reached over 9000 U/mL. Initial bone marrow and liver biopsies did not show histological features of malignancy or HLH. The patient was finally diagnosed and treated almost 1 year after the initial symptoms started, and had an excellent response with etoposide and dexamethasone. This case is unusual because it was triggered following mononucleosis in a patient with positive total antibodies against hepatitis A, with rapidly developing liver failure, and also because the patient's response was excellent despite the delay in treatment. It underscores the importance of suspecting HLH when severe systemic illness develops after a viral infection, even in the absence of clear histological features.