http://www.ncbi.nlm.nih.gov/pubmed/27367788

Ng VL, Li R, Loomes KM, Leonis MA, Rudnick DA, Belle SH, Squires RH; Pediatric Acute Liver Failure Study Group (PALFSG). J Pediatr Gastroenterol Nutr. 2016 Jun 30. [Epub ahead of print]

Abstract

OBJECTIVE:
Hepatic encephalopathy (HE) is challenging to identify in children with acute liver failure (ALF), and was not a requirement for enrollment into the Pediatric ALF Study Group (PALFSG). The outcomes of PALFSG participants presenting with and without HE are presented.

METHODS:
PALFSG participants were classified based on daily assessment of HE during the first 7 days following study enrollment: Group1 - never developed HE; Group2 - no HE at enrollment with subsequent HE development; and Group 3 - HE at study enrollment. Clinical and biochemical parameters and outcomes of death, spontaneous recovery (SR), or liver transplantation (LT) were compared between groups.

RESULTS:
Data from 769PALFSG (54% male; median age 4.2 years; range 0-17.9 years) participants were analyzed, with 277 in Group 1 (36%), 83 in Group 2 (11%) and 409 in Group 3 (53%). Mortality occurred in 11% of all participants and was highest among Group 3 participants who demonstrated persistent grade III-IV HE (55%) or showed progression of HE (26%). Eleven (4%) Group 1 participants died within 21 days of enrollment. SR was highest in Group 1 (79%) and lowest in Group 2 (25%; p < .001).

CONCLUSION:
Mortality21 days following enrollment was highest in participants enrolled with severe HE (grades III or IV) or demonstrating HE progression. However, 4% of participants without recorded clinical HE in the 7 days following enrollment died within 21 days. Improved assessment of neurological injury and PALF prognostication schema are needed.