The serum concentration of fatty acid-binding protein 1 (L-FABP) is elevated in pediatric patients with Wilson disease (WD) and fibrosis, according to a study in the Journal of Pediatric Gastroenterology and Nutrition.

Researchers assessed serum concentrations of L-FABP and lipid droplet-associated protein 5 (PLIN5) in children with WD in relation to liver steatosis and fibrosis. The cross-sectional study included 74 patients (41 girls and 33 boys) from July 2019 to October 2020. Their mean age was 12.3 years (range, 2.5-17.9 years). A control group included 75 healthy children (mean age, 12.6 years; 42 girls and 33 boys) from October to November 2019. L-FABP and PLIN5 were determined with use of commercially available immunoenzymatic tests.

According to transient elastography results and use of cutoff values of controlled attenuation parameter (CAP) >250 dB/m for liver steatosis diagnosis and cutoff values of LSM >6 kPa for liver fibrosis diagnosis, the participants were categorized into 3 subgroups: WD0, children without fibrosis and steatosis (n=33; age range, 2.5-17.9 years); WD1, children with steatosis and without fibrosis (n=28; age range, 5.1-17.7 years); and WD2, children with fibrosis (n=13; age range, 11.2-17.9 years).


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Liver stiffness measurement (LSM) values related to liver fibrosis were significantly increased in the WD2 group (mean value [MV] = 9.6±3.2 kPa) vs the WD0 group (MV = 4.2±0.9 kPa) and WD1 group (MV = 4.5±0.8 kPa). CAP values related to liver steatosis were significantly higher among WD1 participants (MV = 280.6±27.6 dB/m) vs WD0 participants (MV = 206.0±37.6 dB/m).

L-FABP was significantly associated with aminotransferase activities (P <.0001), and Pearson coefficients demonstrated a moderate positive correlation between L-FABP and alanine aminotransferase or aspartate aminotransferase (r = 0.42 and 0.36, respectively).

The mean concentration of serum PLIN5 was similar in all groups: control group (MV = 7.72±4.60 ng/mL), WD0 (MV = 7.72±4.34 ng/mL), WD1 (MV = 6.05±4.05 ng/mL), and WD2 (MV = 8.15±4.25 ng/mL).

Among several study limitations, the investigators noted that the cutoff points for liver stiffness involve a risk of uncertainty and that transient elastography provides slightly poorer predictive values for intermittent stages of fibrosis. Additionally, the study did not assess serum samples of patients with WD and fulminant liver failure.

“[O]ur results may provide the basis for a novel, non-invasive serological diagnostic tool for WD children,” the researchers noted. “Even if PLIN5 serum concentration did not show its expected suitability, L-FABP was confirmed to be an effective indicator,” they concluded.

Reference

Beata Bierła J, Jańczyk W, Konopka E, et al. Fatty acid-binding protein 1 as a potential new serological marker of liver status in children with Wilson disease. J Pediatr Gastroenterol Nutr. 2021;73(4):455-462. doi: 10.1097/MPG.0000000000003128