Definition: A genetic disorder with autosomal recessive inheritance, characterized by multiple CYSTS in both KIDNEYS and associated LIVER lesions. Serious manifestations are usually present at BIRTH with high PERINATAL MORTALITY.
Other names Polycystic Kidney Disease, Infantile, Type I; Kidney, Polycystic, Autosomal Recessive; Autosomal Recessive Polycystic Kidney Disease; Autosomal Recessive Polycystic Kidney; Polycystic Kidney and Hepatic Disease 1 (Autosomal; Polycystic Kidney and Hepatic Disease 1; Polycystic Kidney Disease, Infantile, Type 1; Polycystic Kidney Disease, Autosomal Recessive; ARPKD