常染色体显性遗传性多囊肝病分子学研究进展

常染色体显性遗传性多囊肝病（autosomal dominant polycystic liver disease,ADPLD,MIM174050）,简称多囊肝（polycystic liver disease,PCLD or PLD）,过去曾称为成人多囊肝病（adult polycystic liver disease,APLD）,可作为常染色体显性遗传性多囊肾病（autosomal dominant polycystic kidney disease,ADPKD,MIM173900,173910）的肾外表现而存在,亦可单独发病,前者称为多囊肾相关型多囊肝病（polycystic liver disease associated with ADPKD）后者称为独立型多囊肝病（isolated polycystic liver disease）.