Polysystic kidney disease is associated with all of the following except
Correct Answer: Renal cell carcinoma
Description: The incidence of RCC in patients with ADPKD is no higher than that in the general population ADPKD is the most common inheritable form of renal cystic disease. ADPKD most often becomes clinically apparent after age 30 years but may manifest at any age, including in utero. Ninety-nine percent of affected individuals have a mutation either of the PKD1 gene on chromosome 16 or, less often, of the PKD2 gene on chromosome 4 Cysts can be identified sonographically before age 20 years in almost all affected individuals. ADPKD is associated with a high incidence of liver cysts that increases with age. Signs or symptoms first occur between the ages of 30 and 50 years and include hematuria, flank pain, gastrointestinal symptoms, renal colic, and hypeension. Hepatic cysts occur in viually all patients with ADPKD by the age of 50 years and are rarely symptomatic. ICAs of the circle of Willis (berry aneurysm) occur in 10% to 30% of patients, and approximately 9% of these patients die because of subarachnoid hemorrhages. Tight BP control, the use of ACE inhibitors and/or ARB BP medication, and the possible use of tolvaptan are the most current treatment options. Pain (flank and/or abdominal) is the most common presenting symptom in adults. This results from a number of possible factors: mass effect (cysts impinging on abdominal wall or neighboring organs), bleeding into the cysts, urinary tract infection (including infected cysts), and nephrolithiasis. 20% to 30% of patients with ADPKD develop stones Hepatic cysts,are the most common extrarenal manifestation of ADPKD Other extra renal manifestations include : Intracranial (berry) aneurysms, colonic diveiculi; mitral valve prolapse; cysts of other organs (seminal vesicle, arachnoid membrane, pancreas) Campbell and Walsh Urology 11th edition , chapter 131 , pg no . 3017
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