Of these, 104 were diagnosed solely as PFIC3, one case was diagnosed as PFIC3 with cirrhosis and systemic amyloidosis, another as PFIC1 & PFIC3 with myotonic dystrophy, one as PFIC3 and multiple sclerosis, and one as PFIC3 and biliary atresia. This evidence concerns the gene ATP8B1 and primary systemic amyloidosis.