Table 1.

Comparative epidemiological and genetic mutation characteristics of autosomal dominant polycystic kidney disease associated polycystic liver disease and isolated polycystic liver disease

Characteristics	ADPKD associated PLD	Isolated PLD
Prevalence	0.20%	< 0.01%
Type of inheritance	AD	AD
Gene mutated	PKD1; PKD2	PRKCSH; SEC63
Encoded product	Polycystin-1; Polycystin-2	Hepatocystin; Sec63 protein
Chromosome locus	216p13.3; 4q21	19p13.2; 6q21

AD: Autosomal dominant; ADPKD: Autosomal dominant polycystic kidney disease; PLD: Polycystic liver disease.