Polycystic liver disease 4 with or without kidney cysts
Polycystic liver disease 4 with or without kidney cysts
Definition
An autosomal dominant disease characterized by adult-onset of liver cysts arising from the bile duct epithelium, caused by heterozygous mutation in the LRP5 gene. Some patients may develop a few kidney cysts, but these are often incidental and do not result in renal failure.
Also known as polycystic liver disease 4 with or without kidney cysts — per MONDO