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. 2022 Oct;10(8):795-804.
doi: 10.1002/ueg2.12269. Epub 2022 Jun 30.

Indeterminate pediatric acute liver failure: Clinical characteristics of a temporal cluster of five children in the Netherlands in the spring of 2022

Willem S Lexmond  1 , Vincent E de Meijer  2 , René Scheenstra  1 , Sander T H Bontemps  3 , Evelien W Duiker  4 , Elisabeth H Schölvinck  5 , Xuewei Zhou  6 , Karin J von Eije  6 , Koen M E M Reyntjens  7 , Henkjan J Verkade  1 , Robert J Porte  2 , Ruben H de Kleine  2
Affiliations

Indeterminate pediatric acute liver failure: Clinical characteristics of a temporal cluster of five children in the Netherlands in the spring of 2022

Willem S Lexmond et al. United European Gastroenterol J. 2022 Oct.

Abstract

There is increasing global concern of severe acute hepatitis of unknown etiology in young children. In early 2022, our center for liver transplantation in the Netherlands treated five children who presented in short succession with indeterminate acute liver failure. Four children underwent liver transplantation, one spontaneously recovered. Here we delineate the clinical course and comprehensive diagnostic workup of these patients. Three of five patients showed a gradual decline of liver synthetic function and had mild neurological symptoms. Their clinical and histological findings were consistent with hepatitis. These three patients all had a past SARS-CoV-2 infection and two of them were positive for adenovirus DNA. The other two patients presented with advanced liver failure and encephalopathy and underwent dialysis as a bridge to transplantation. One of these children spontaneously recovered. We discuss this cluster of patients in the context of the currently elevated incidence of severe acute hepatitis in children.

Keywords: acute liver failure; adenovirus; hepatitis; liver transplantation.

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Conflict of interest statement

The Authors declare that there is no conflict of interest.

Figures

FIGURE 1
FIGURE 1
Incidence of indeterminate Pediatric acute liver failure (PALF) in our national transplant center. Blue bars represent the total number of cases, red bars represent the proportion of cases undergoing liver transplantation
FIGURE 2
FIGURE 2
Biochemical parameters. Evolution of alanine aminotransferase (ALT), bilirubin, International Normalized Ratio (INR) and ammonia levels from time of first admission to the transplant center until transplantation (case 1, 2, 3 and 5) or discharge (case 4)
FIGURE 3
FIGURE 3
Explant histology of representative cases. Case 1. Hematoxylin eosin (HE) stain shows massive acute necrosis of the hepatocytes with a retained architecture with minimal portal inflammation. HepPar immunohistochemistry stains differentiate viable and necrotic hepatocytes. Reticulin stain shows a preserved architecture without collapse. Case 5. HE stain shows portal and lobular inflammation with venulitis of central veins and perivenular congestion and massive ductular reaction. HepPar shows extensive loss of hepatocytes. In the reticulin collapse of fibers between the ductular reaction. All images were captured via digital pathology
See this image and copyright information in PMC

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