Polycystic kidney disease overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Serge Korjian, Yazan Daaboul
Overview
Autosomal dominant polycystic kidney disease (ADPKD), previously known as adult polycystic kidney disease, is a systemic disorder characterized primarily by multiple, bilateral renal cysts, cysts in other organs namely the liver and pancreas, and cardiovascular abnormalities including intracranial aneurysms and mitral valve prolapse.[1] ADPKD is one of the most common inherited disorders worldwide almost 15 times more common than cystic fibrosis in the general population.[2] It accounts for up to 3-5% of ESRD cases yearly.[3] Classically, ADPKD presents with hypertension and varying degrees of renal insufficiency. Around half of the patients diagnosed with ADPKD will reach end-stage renal disease (ESRD) by the age of 60 years.[1] Still, disease manifestation and severity is highly variable among patients even those within the same family.[2]
References
- ↑ 1.0 1.1 Harris PC, Torres VE. Polycystic Kidney Disease, Autosomal Dominant. 2002 Jan 10 [Updated 2011 Dec 8]. In: Pagon RA, Adam MP, Bird TD, et al., editors. GeneReviews™. Seattle (WA): University of Washington, Seattle; 1993-2013. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1246/
- ↑ 2.0 2.1 Gabow PA (1993). "Autosomal dominant polycystic kidney disease". N Engl J Med. 329 (5): 332–42. doi:10.1056/NEJM199307293290508. PMID 8321262.
- ↑ U.S. Renal Data System, 2013 Annual Data Report: Atlas of Chronic Kidney Disease and End-Stage Renal Disease in the United States, National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD, 2013.