https://pubmed.ncbi.nlm.nih.gov/33908738/ Wilson disease
J Pediatr Gastroenterol Nutr. 2021 Mar 17.
doi: 10.1097/MPG.0000000000003128.Online ahead of print.

Fatty Acid-Binding Protein 1 as a Potential New Serological Marker of Liver Status in Children with Wilson Disease

Joanna Beata Bierła 1, Wojciech Jańczyk, Ewa Konopka, Aldona Wierzbicka-Rucińska, Sebastian Więckowski, Łukasz Obrycki, Jędrzej Sarnecki, Ewelina Kanarek, Bożena Cukrowska, Piotr Socha

Abstract

Objectives: Wilson's disease (WD) is a copper metabolism disorder with toxic copper accumulation in the liver leading to liver steatosis or fibrosis. In vitro studies suggest that fatty acid-binding protein 1 (L-FABP) and lipid droplet-associated protein 5 (PLIN5) may have an impact on both processes, but knowledge about these potential biomarkers is insufficient in the case of WD. Thus, the aim of this study was to determine L-FABP and PLIN5 levels in sera of WD patients in relation to liver steatosis/fibrosis.

Methods: The final study involved 74 WD children in whom liver steatosis (WD1 subgroup, n = 28) and fibrosis (WD2 subgroup, n = 13) were assessed with the use of transient elastography. Control groups included WD children without steatosis and fibrosis (WD0 subgroup, n = 33) and healthy children (n = 75). L-FABP and PLIN5 measurements were performed in sera with the use of the immunoenzymatic method.

Results: L-FABP was significantly higher in the WD2 subgroup, and the correlation between L-FABP concentration and liver fibrosis was confirmed statistically by regression analysis (p = 0.04) with Pearson's coefficient r = 0.24. L-FABP was significantly correlated with aminotransferase ALT (r = 0.42) and AST (r = 0.37) activity. PLIN5 concentration was similar in all groups and was not related to steatosis and fibrosis.

Conclusions: Our results suggest that serum L-FABP could be a novel biomarker of liver fibrosis in WD children.