Autosomal dominant polycystic kidney disease. Definition

Medical Definition: autosomal dominant polycystic kidney disease

It is responsible for 10% of patients on dialysis and the third leading cause of ESRD. Incidence: 1/400-1.000 births. Autosomal dominant, bilateral in 96% of cases, associated with cysts in other organs (liver in 50%, pancreas in 10%, circles of Willis in 10-40%). In 90% of cases there is a genetic defect located on the short arm of chromosome 16. In the remaining 10% is due to a genetic defect is not located, but situated on the short arm of chromosome 16. Progresses inexorably to chronic renal failure to the 40-60 years (45% are on dialysis at 60 years). From a clinical perspective, 30% of children and 60% of adults who have not reached are hypertensive renal failure. When suffering from terminal renal insufficiency, 80% were hypertensive. The radiological diagnosis by ultrasonography or CT urography. Are objectified two kidneys, significantly enlarged, and filled with a countless number of cysts.

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