Autosomal recessive polycystic kidney disease (ARPKD, previously called infantile polycystic kidney disease), is an inherited disorder characterized by kidney cysts, kidney failure, and high blood pressure. In contrast to autosomal dominant polycystic kidney disease, which tends to affect kidney function in older people (adults aged 30 or over), autosomal recessive polycystic kidney disease is a more common type of polycystic kidney disease in infants and children. The cysts impair normal function in the kidneys, including clearing waste of the body and maintaining normal blood pressure. In some children, liver function might be damaged as well. Because of impaired kidney functions, the lungs are often unable to develop completely because of an absence of fluid surrounding the baby in the womb before birth, which leads to decreased lung function after birth. In the most severe cases, death occurs before birth. Some infants will develop kidney disease in the first few years of life that will eventually lead to kidney failure. This requires dialysis or kidney transplantation to survive. In general, intelligence is usually not affected by ARPKD. There is no cure for ARPKD, although aggressive treatment and improved transplantation methods have significantly increased survival beyond infancy into adolescence. ARPKD is caused by pathogenic variants in PKHD1 gene.