Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Polycystic Kidney Disease

Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_4667-2

Synonyms

Definition

Polycystic kidney disease is a common genetic disease characterized by accumulation of multiple fluid-filled cysts in each kidney and other organs. The renal cysts originate from the renal tubular epithelial cells lined by a single layer of cells that have higher rates of cellular proliferation and apoptosis and are less differentiated than the normal tubular cells. Progression of cysts in the kidneys could ultimately cause end-stage renal disease.

Characteristics

PKD (Polycystic Kidney Disease) consists of two forms: ADPKD (Autosomal Dominant Polycystic Kidney Disease) and ARPKD (Autosomal Recessive Polycystic Kidney Disease). ADPKD is the most common genetic disease affecting 1 in 500 to 1 in 1,000 in adults of all ethnic groups worldwide. ARPKD is far less common, affecting 1 in 6,000 to 1 in 40,000 at a far younger age, including newborns, infants, and children. While kidney cysts arise from all the segments of the...

Keywords

Autosomal Dominant Polycystic Kidney Disease Polycystic Kidney Disease Primary Cilium Kidney Volume Autosomal Recessive Polycystic Kidney Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Section of NephrologyYale University School of MedicineNew HavenUSA