Pictured left to right: J Squires, R Squires
James E Squires, David A Rudnick, Regina M Hardison, Simon Horslen, Vicky L Ng, Estella M Alonso, Steven H Belle, Robert H Squires, The Pediatric Acute Liver Failure Study Group. Liver Transplant Listing in Pediatric Acute Liver Failure (PALF): Practices and Participant Characteristics. Hepatology. 2018 August 2.
ABSTRACT
Liver transplant (LT) decisions in pediatric acute liver failure (PALF) are complex. Three phases of the PALF registry, containing data on 1144 participants over 15 years, were interrogated to characterize clinical features associated with listing status. A decrease in the cumulative incidence (CI) of listing (p<0.005) and receiving (p<0.05) LT occurred without an increase in the CI of death (p=0.67). Time-to listing was constant, and early (1 day; Q1-Q3=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; higher INR, bilirubin, lactate, and venous ammonia; and had lower peripheral lymphocytes and transaminase levels compared to those deemed “not sick enough”. Participants listed vs those deemed “medically unsuitable” were older, with higher serum aminotransferase levels, bilirubin, platelets, and albumin; with 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 those who were not “medically suitable”. Removal from the LT list was a rare event.
CONCLUSIONS: The CI of listing for and receiving LT decreased throughout the PALF study without an increase in the CI of death. While all participants fulfilled entry criteria for PALF, significant differences were noted between participants listed for LT versus 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.
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