Polycystic Kidney Disease
Polycystic Kidney Disease is a topic covered in the Washington Manual of Medical Therapeutics.
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General Principles
- Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disorder resulting in cystic enlargement of the kidney and occurs in approximately 1 in 1000 persons. Approximately 20% of patients with ADPKD do not have a positive family history.
- There are two well-described mutations in the polycystin genes, PKD1 and PKD2. PKD1 is the most common and present in approximately 85% of ADPKD. PKD2 is associated with a later onset of disease.
- The mechanism by which cysts form is unclear. The polycystin gene products primarily localize to the cilia of the tubular apical membrane. Disordered regulation of cell division and planar cell polarity may lead to overgrowth of the tubular segment, eventually pinching off from the rest of the collecting system. In abnormal cells, cyclic AMP imparts a proliferative phenotype as well as inducing chloride extrusion into the cyst lumen. Cyst formation affects only a relatively small percentage of tubules, suggesting a “two-hit” hypothesis where a sporadic mutation of the wild-type allele results in local cystogenesis.
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Citation
Williams, Dominique, et al., editors. "Polycystic Kidney Disease." Washington Manual of Medical Therapeutics, 35th ed., Wolters Kluwer Health, 2016. Washington Manual, www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602278/all/Polycystic_Kidney_Disease.
Polycystic Kidney Disease. In: Williams D, Ramgopal R, Gdowski M, et al, eds. Washington Manual of Medical Therapeutics. 35th ed. Wolters Kluwer Health; 2016. https://www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602278/all/Polycystic_Kidney_Disease. Accessed December 7, 2019.
Polycystic Kidney Disease. (2016). In Williams, D., Ramgopal, R., Gdowski, M., Dretler, A., & Bhat, P. (Eds.), Washington Manual of Medical Therapeutics. Available from https://www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602278/all/Polycystic_Kidney_Disease
Polycystic Kidney Disease [Internet]. In: Williams D, Ramgopal R, Gdowski M, Dretler A, Bhat P, editors. Washington Manual of Medical Therapeutics. Wolters Kluwer Health; 2016. [cited 2019 December 07]. Available from: https://www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602278/all/Polycystic_Kidney_Disease.
* Article titles in AMA citation format should be in sentence-case
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