Liver Transplant Listing in Pediatric Acute Liver Failure: Practices and Participant Characteristics

Abstract

Liver transplant (LT) decisions in pediatric acute liver failure (PALF) are complex. Three phases of the PALF registry, containing data on 1,144 participants over 15 years, were interrogated to characterize clinical features associated with listing status. A decrease in the cumulative incidence of listing (P < 0.005) and receiving (P < 0.05) LT occurred without an increase in the cumulative incidence of death (P = 0.67). Time to listing was constant and early (1 day; quartiles 1-3 = 0-2; P = 0.88). The most frequent reasons for not listing were "not sick enough" and "medically unsuitable." Participants listed for LT were more likely male, with coma grade scores >0; had higher international normalized ratio, bilirubin, lactate, and venous ammonia; and had lower peripheral lymphocytes and transaminase levels compared to those deemed "not sick enough." Participants listed versus those deemed "medically unsuitable" were older; had higher serum aminotransferase levels, bilirubin, platelets, and albumin; and had lower lactate, venous ammonia, and lymphocyte count. An indeterminate diagnosis was more prevalent in listed participants. Ventilator (23.8%) and vasopressor (9.2%) support occurred in a significant portion of listed participants but less frequently than in those who were not "medically suitable." Removal from the LT list was a rare event. Conclusion: The cumulative incidence of listing for and receiving LT decreased throughout the PALF study without an increase in the cumulative incidence of death. While all participants fulfilled entry criteria for PALF, significant differences were noted between participants listed for LT and those deemed "not sick enough" as well as those who were "medically unsuitable." Having an indeterminate diagnosis and a requirement for cardiopulmonary support appeared to influence decisions toward listing; optimizing listing decisions in PALF may reduce the frequency of LT without increasing the frequency of death.

Improved Survival Following Living Donor Liver Transplantation for Pediatric Acute Liver Failure: Analysis of 20 Years of US National Registry Data

“…Over the study period, candidate age and sex distributions were not statistically different. The incidence of viral and drug‐induced PALF has decreased, but the etiology of PALF was mostly indeterminate, which is consistent with other reports . Since the inception of the PELD/MELD score in 2002, median calculated PELD/MELD score of candidates has increased.…”

“…The incidence of viral and drug-induced PALF has decreased, but the etiology of PALF was mostly indeterminate, which is consistent with other reports. (29) Since the inception of the PELD/MELD score in 2002, median calculated PELD/MELD score of candidates has increased. Figure 1A demonstrates a significant decrease in the number of listed candidates over the past 2 decades (P < 0.0001).…”

Pediatric liver transplant following near catastrophic head bleed: Lessons learned

“…encephalopathy. 2,3 As might be expected, when these patients present, they are extremely ill and may be beyond the window to tolerate a liver transplant.…”

“…The etiology of liver failure during the neonatal period and early infancy in this patient population includes metabolic disorders (tyrosinemia etc), biliary atresia, GALD, infection, and hypoxia‐ischemia‐related hepatopathy . The most common presentation is jaundice and hypoglycemia followed by renal failure, bleeding, and encephalopathy . As might be expected, when these patients present, they are extremely ill and may be beyond the window to tolerate a liver transplant.…”

Timing of liver transplantation for pediatric acute liver failure due to mushroom poisoning: a case report and literature review

“…Without LT, PALF is a devastating process, with high mortality rate. With the advent and advancement of pediatric liver transplantation (PLT), patients are now likely to survive [5]. However, considering the spontaneous recovery potential and complications of PLT, the timing of PLT for PALF is very important.…”