Acute-on-chronic liver failure (ACLF) is an acute decompensation of chronic liver disease leading to multiorgan failure and mortality. The objective of this study was to evaluate characteristics and outcomes of children with ACLF who are at the highest priority for liver transplantation (LT) on the United Network for Organ Sharing (UNOS) database-listed as status 1B. The characteristics and outcomes of 478 children with ACLF listed as status 1B on the UNOS LT waiting list from 2007-2019 were compared with children with similar or higher priority listing for transplant: 929 with acute liver failure (ALF) listed as status 1A and 808 with metabolic diseases and malignancies listed as status 1B (termed "non-ACLF"). Children with ACLF had comparable rates of cumulative organ failures compared with ALF (45% vs. 44%; p > 0.99) listings, but higher than non-ACLF (45% vs. 1%; p < 0.001). ACLF had the lowest LT rate (79%, 84%, 95%; p < 0.001), highest pre-LT mortality (20%, 11%, 1%; p < 0.001), and longest waitlist time (57, 3, 56 days; p < 0.001), and none recovered without LT (0%, 4%, 1%; p < 0.001). In survival analyses, ACLF was associated with an increased adjusted hazard ratio (HR) for post-LT mortality (HR, 1.50 vs.

Introduction The liver donor risk index (LDRI), originally developed in 2006 by Feng et. al and since modified, is a method of evaluating liver grafts from deceased donors by determining the relative risk of graft failure post transplantation. Methods Online and paper surveys sent to liver transplant physicians asking about their attitudes and practices regarding decision-making in liver transplantation and the role of LDRI. Results 147 of 401 (37%) eligible respondents returned partial or complete surveys. The majority of the respondents were male (116/134 or 87%) and practiced in academic medical centers (128/138 or 93%). Transplant coordinators initially contacted the candidate with an offer in 81% of programs. Eighty-eight of 143 (62%) respondents reported that they were very familiar with LDRI, but the vast majority (114/137 or 83%) rarely or never discuss the concept of LDRI with their patients. A majority of respondents (96/132 or 73%) believe that LDRI does not adequately describe a liver’s relative risk of graft failure and that there are factors that make LDRI potentially misleading (122/138 or 88%). Nevertheless, 60 of 130 (46%) believe that LDRI would increase/improve shared decision making. Discussion The LDRI has not been widely adopted because of concerns that 1) it does not accurately reflect post-transplant survival; 2) it excludes relevant donor and recipient factors, and 3) it is too complicated for candidates to grasp. There is a need to improve it or to develop other decision making tools to help promote shared decision making. There is also great diversity in how liver offers are made to ambulatory candidates, and how transplant programs address a candidate’s refusal. Research is needed to determine evidence-based best practice.

Autoimmune liver disease remains difficult to diagnose, and distinguishing the various causes is difficult as well. In children, it can present with wide variation, including autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and the “overlap syndrome” of AIH/PSC, also known as autoimmune sclerosing cholangitis. These liver disorders are thought to be immune-mediated, but their etiology remains unclear. They are not secondary to inherited or acquired diseases and they are not associated with any drugs, so they can only be diagnosed if these other diseases or conditions are excluded. Because there is considerable commonality in the clinical presentation of these diseases but differences in their management, appropriate treatment may be delayed, increasing the risk for liver transplantation. Further education for general pediatricians and trainees is needed. This article reviews the differences between AIH and PSC, as well as the newly recognized overlap syndrome of both of these diseases. [ Pediatr Ann. 2018;47(11):e452–e457.]

Background and objectives Living donor guidelines-both national and international-either do not address or are vague about what information can be shared between prospective living donors and transplant candidates, as well as when to make such disclosures and who should make them. This study explored the attitudes of donors and recipients regarding how much information they believe should be shared.Design, setting, participants, & measurements Two Email invitations were sent by the National Kidney Foundation (national headquarters) to its Email listservs, inviting members to participate in an online survey to assess the attitudes of kidney transplant stakeholders regarding the disclosure of health and health risk behavior information.Results From approximately 4200 unique Email addresses, 392 (9.3%) respondents completed part or all of the survey. The analyses were limited to the 236 respondents who self-identified as either donors (potential and actual, n=160) or recipients (candidates and actual, n=76). Overall, 79% (186 of 234) of respondents supported disclosure of general recipient health information that would affect post-transplant outcome to donors, and 88% (207 of 235) supported disclosure of general donor health information to recipients. Recipients and donors were also supportive of sharing donor and recipient information, particularly information relevant to graft and patient survival. There is some reticence, however, about sharing social information. The closer the relationship, the more information they are willing to share. Both donors and recipients wanted the transplant team involved in the information disclosure. Over three quarters of donors (79%) and recipients (78%) did not think the recipient had a right to know why a donor was excluded from donating.Conclusions Both donors and recipients want a significant amount of health information to be disclosed. The opinions of other stakeholders need to be surveyed to determine whether a revision of current policies and practices is warranted.

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