Autosomal Recessive Polycystic Kidney Disease: Diagnosis, Prognosis, and Management

Autosomal recessive polycystic kidney disease (ARPKD) is the rare and usually early-onset form of polycystic kidney disease with a typical clinical presentation of enlarged cystic kidneys and liver involvement with congenital hepatic fibrosis or Caroli syndrome. ARPKD remains a clinical challenge in pediatrics, frequently requiring continuous and long-term multidisciplinary treatment. In this review, we aim to give an overview over clinical aspects of ARPKD and recent developments in our under-standing of disease progression, risk patterns, and treatment of ARPKD. (c) 2023 by the National Kidney Foundation, Inc. All rights reserved.