Laboratory criteria for improving the diagnosis of autoimmune hepatitis and Wilson’s disease in children

Specification of laboratory criteria for improving differential diagnostics of children with autoimmune hepatitis (AIH) and Wilson’s disease (WD) is a relevant task in pediatrics. Increased titers of antinuclear factor (ANF) in blood serum is among laboratory criteria for diagnostics of AIH. Some WD cases have been described which can manifest as autoimmune hepatitis (AIH) with detection of non-specific autoantibodies, thus complicating the differential diagnosis. The aim of our study was to identify the specific features for determination of antinuclear factor on the Hep-2 cell line and specific autoantibodies using a triple cell substrate in children with autoimmune hepatitis and WD. We have examined 62 pediatric patients with WD and 28 children with AIH. ANF was determined on the HEp-2 cell line (ANA-HEp-2, AESKUSLIDES®, Germany). Specific auto-antibodies were detected by means of a triple substrate obtained from rodents, which included samples of kidney, liver and stomach tissues (LKS Mouse, Germany) using indirect fluorescence reactions applying an automated analyzer HELIOS® (Germany). The analysis revealed ANF positivity in 15 out of 62 (24%) children with WD. Positive ANF results were obtained in 26 out of 28 children AIH (93%), being significantly more common in AIH compared with WD. The study of specific autoantibodies using a triple substrate revealed positive results in 34 children with WD (55%), versus 26 children with AIH (93%), Hence, the specific auto-antibodies on the triple substrate are more commonly found in children with AIH (p 0.001) than in children with WD. In children with suspected AIH, when a homogeneous type of luminescence is detected on the HEp-2 cell line, and with a combination of different luminescence types on a triple substrate, the presence of Wilson’s disease in patients cannot be excluded.