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What is ARPKD?

What is autosomal recessive polycystic kidney disease (ARPKD)?

ARPKD is an extremely rare type of polycystic kidney disease (PKD) passed from parent to child by recessive inheritance. Primarily affecting infants and children, ARPKD is characterized by the development of renal cysts within collecting ducts and congenital hepatic fibrosis.1-3

ARPKD can result in infant mortality, due in part to large polycystic kidneys that can interfere with childbirth.4,5

  • Children who survive the first 12 months of life have as much as a 78% chance of living past the age of 15 years6
    • They may suffer from complications related to renal tubular dysfunction and hypertension3
  1. The National Kidney Foundation. Polycystic kidney disease. https://www.kidney.org/atoz/content/polycystic. Accessed October 3, 2018.

  2. Patel V, Chowdhury R, Igarashi P. Advances in the pathogenesis and treatment of polycystic kidney disease. Curr Opin Nephrol Hypertens. 2009;18(2):99-106. doi:10.1097/MNH.0b013e3283262ab0

  3. Harris PC, Torres VE. Polycystic kidney disease. Annu Rev Med. 2009;60:321-337.

  4. Li X, ed. Polycystic Kidney Disease. Brisbane, Australia. Codon Publications; 2015.

  5. Torres VE, Grantham JJ. Cystic diseases of the kidney. In: Taal MW, Chertow GM, Mardsen PA, Skorecki K, Yu ASL, Brenner BM, eds. Brenner & Rector’s The Kidney. Philadelphia, PA: Elsevier Saunders; 2012:1626-1667.

  6. Kaplan BS, Kaplan P, Rosenberg HK, Lamothe E, Rosenblatt DS. Polycystic kidney diseases in childhood. J Pediatr. 1989;115(6):867-880.