In paediatric and adult populations, FIC-related variants may result in HCC and CCA, especially for ABCB11 (about 5–15% of children with PFIC2 develop HCC in their second- third year of life) [9, 70], ABCB4 [15] and TJP2 [9]. The gene discussed is ABCB4; the disease is cholangiocarcinoma.