This report describes general characteristics of the group of diseases classified as polycystic kidney disease (PKD). PKD is a genetically heterogeneous disorder with multiple causative genes. A list of subtypes, as defined by OMIM, can be found by following the link in the 'Related Diseases' section, below; links to reports for subtypes that have been investigated using fly models are included in the table.
[updated Jun. 2017 by FlyBase; FBrf0222196]
Polycystic kidney disease usually presents with progressive, bilateral, multiple cyst formation in the kidneys; liver cysts and intracranial aneurysm frequently develop. Acute and chronic pain, nephrolithiasis, and hypertension are common complications. The most serious renal complication is end-stage renal disease, which occurs in approximately 50% of patients by the age of 60 years. The typical age of onset is in middle life, but the range is from infancy to 80 years (summary by Wu and Somlo, 2000; pubmed:10655152). [from MIM:173900; 2017.06.22]
To date, 4 genes have been implicated in polycystic kidney disease. [from MIM:173900; 2017.06.22]