Prognosis

Prognosis, especially for those who survive the newborn period is far less bleak than once thought. Infants with ARPKD who survive mechanical ventilation have a good chance of survival. The 5-year survival rate is 80 to 95% for those who survive the neonatal period (pertaining to the first 4 weeks after birth). Because of improved ESRD (End Stage Renal Disease) treatment, and more effective control of both systemic and portal hypertension, survival into adulthood is common.

Correlation of disease symptoms with age at time of diagnosis has been suggested. It was thought that ARPKD individuals who were diagnosed early in life exhibited more kidney manifestations, with little, or sometimes no apparent liver involvement, while those diagnosed later in life, up to adolescence, (seldom into adulthood), manifested more clinical liver complications with little or no kidney manifestations. This description does not however take into account the prominent liver manifestations that occur in children with early kidney transplants who initially exhibited no liver problems.

Because of continued improvements in mechanical ventilation, neonatal support, control of hypertension, management of ESRD and transplantation, the ARPKD population is now living well into adulthood.