Status 1B designation does not adequately prioritize children with acute-on-chronic liver failure for liver transplantation.

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. ALF [95% confidence interval, CI, 1.02-2.19; p = 0.04] and HR, 1.64 vs. non-ACLF [95% CI, 1.15-2.34; p = 0.01]). ACLF has the least favorable waitlist and post-LT outcomes of all patients who are status 1A/1B. Increased prioritization on the LT waiting list may offer children with ACLF an opportunity for enhanced outcomes.

Pediatric Intensivist and Pediatric Neurologist Perspectives and Practices on Death by Neurologic Criteria.

Controversies surrounding the determination of death by neurologic criteria (DNC), also known as brain death, have become increasingly common over the last decade, occasionally leading to parental refusal of all or part of an evaluation or declaration of DNC. We performed a prospective, cross-sectional study of pediatric neurologists and intensivists who participate in professional listservs to ascertain perspectives and practices concerning the evaluation of DNC, specifically on obtaining permission for evaluations and managing refusals. Of the 334 respondents who had performed an evaluation for DNC, 35 percent reported they had experienced at least one parental refusal, and 64.4 percent reported that they did not seek permission to perform an evaluation. Pediatric neurologists, careproviders who had less experience doing evaluations, and careproviders who had experienced parental refusal of an evaluation were more likely to obtain permission from parents. Most (80.8 percent) of respondents reported that their institution had a DNC policy. We found variability in many aspects of DNC evaluations and declarations, as well as the handling of refusals. Lack of consistency may make it more difficult for careproviders and families. Greater understanding of parental refusal of DNC evaluation is essential to inform efforts to increase consistency.

Decision making in liver transplantation--limited application of the liver donor risk index.

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 through the determination of the relative risk of graft failure after transplantation. Online and paper surveys about attitudes and practices regarding decision making in liver transplantation and the role of the LDRI were sent to liver transplant physicians. One hundred forty-seven of 401 eligible respondents (37%) 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 the programs. Eighty-eight of 143 respondents (62%) reported that they were very familiar with the LDRI, but the vast majority (114/137 or 83%) rarely or never discussed the concept of the LDRI with their patients. A majority of the respondents (96/132 or 73%) believed that the LDRI does not adequately describe a liver's relative risk of graft failure and that there are factors making the LDRI potentially misleading (122/138 or 88%). Nevertheless, 60 of 130 respondents (46%) believed that the LDRI would increase/improve shared decision making. The LDRI has not been widely adopted because of concerns that (1) it does not accurately reflect posttransplant 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 in how transplant programs address a candidate's refusal. Research is needed to determine evidence-based best practice.

Pediatric brain death certification: a narrative review.

In the five decades since its inception, brain death has become an accepted medical and legal concept throughout most of the world. There was initial reluctance to apply brain death criteria to children as they are believed more likely to regain neurologic function following injury. In spite of early trepidation, criteria for pediatric brain death certification were first proposed in 1987 by a multidisciplinary committee comprised of experts in the medical and legal communities. Protocols have since been developed to standardize brain death determination, but there remains substantial variability in practice throughout the world. In addition, brain death remains a topic of considerable ethical, philosophical, and legal controversy, and is often misrepresented in the media. In the present article, we discuss the history of brain death and the guidelines for its determination. We provide an overview of past and present challenges to its concept and diagnosis from biophilosophical, ethical and legal perspectives, and highlight differences between adult and pediatric brain death determination. We conclude by anticipating future directions for brain death as related to the emergence of new technologies. It is our position that providers should endorse the criteria for brain death diagnosis in children as proposed by the Society of Critical Care Medicine (SCCM), American Academy of Pediatrics (AAP), and Child Neurology Society (CNS), in order to prevent controversy and subjectivity surrounding what constitutes life versus death.

Multiple organ involvement and ICU considerations for the care of acute liver failure (ALF) and acute on chronic liver failure (ACLF) in children.

Liver disease results in approximately 15,000 pediatric hospitalizations per year in the United States and is a significant burden to child health. Major etiologies of liver failure and indications for transplantation in children include: biliary atresia, metabolic/genetic conditions, toxins, infections, tumors, and immune-mediated liver/biliary injury. Children requiring transplantation are placed on the United Network of Organ Sharing waitlist including those with acute liver failure (ALF) and acute on chronic liver failure (ACLF). ALF is a clinical syndrome in which a previously healthy child develops rapid-onset hepatic dysfunction, and becomes critically ill with multiple organ dysfunction within days. ACLF, by contrast, is generally described as an acute decompensation of pre-existing chronic liver disease (CLD) brought on by a precipitating event, with higher risk of mortality. Children with ALF and ACLF receive multidisciplinary care in pediatric intensive care units (ICUs) due to multiple organ system involvement and high risk of decompensation in these patients. The care of these patients requires a holistic approach that addresses the complex interplay between hepatic and extra-hepatic organ systems. This review will define and describe ALF and ACLF in the pediatric population, and outline the effects of ALF and ACLF on individual organ systems with diagnostic and management considerations in the ICU while awaiting liver transplantation.

Autoimmune Liver Diseases in Children.

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.].

Disclosing Health and Health Behavior Information between Living Donors and Their Recipients.

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.