Polycystic kidney disease (PKD) is passed down through families (inherited), usually as an autosomal dominant trait. If one parent carries the gene, the children have a 50% chance of developing the disorder.
Autosomal dominant PKD occurs in both children and adults, but it is much more common in adults. Symptoms often do not appear until middle age. It affects nearly 1 in 1,000 Americans. The actual number may be more, because some people do not have symptoms.
An autosomal recessive form of PKD also exists. It appears in infancy or childhood. This form is much less common than autosomal dominant PKS, but it tends to be very serious and gets worse quickly. It can cause serious lung and liver disease, end-stage kidney disease, and it usually causes death in infancy or childhood.
Persons with PKD have many clusters of cysts in the kidneys. What exactly triggers the cysts to form is unknown.
Porter CC, Avner Ed. Anatomic abnormalities associated with hematuria. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 515.
Torres VE, Grantham JJ. Cystic diseases of the kidney. In: Brenner BM, ed. Brenner and Rector's the Kidney. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 41.
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Herbert Y. Lin, MD, PhD, Nephrologist, Massachusetts General Hospital; Associate Professor of Medicine, Harvard Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.