All of the following are true about adult polycystic kidney disease, EXCEPT:
Correct Answer: Hypeension is rare
Description: Autosomal dominant polycystic kidney disease (ADPKD) is an autosomal dominantly transmitted disease. The severity of structural abnormality of ADPKD generally correlates with the renal manifestations including pain, hematuria, hypeension, and renal dysfunction. Hypeension (HTN) occurs before the onset of renal failure in more than 80% of ADPKD patients. Gross hematuria may be the initial presenting symptom. In addition to its renal manifestations, ADPKD is a multisystemic disorder with prominent extrarenal cystic and noncystic manifestations including: Polycystic liver disease Cysts in diverse organ systems (pancreas, spleen, arachnoid membrane, pineal gland, and seminal vesicles) Intracranial saccular aneurysms Thoracic aoic aneurysms and dissections Coronary aery aneurysms Mitral and/or tricuspid valve prolapse Aoic valve insufficiency Aoic root dilation Colonic diveicula Ref: Qian Q., Torres V.E. (2009). Chapter 46. Cystic Diseases of the Kidney. In E.V. Lerma, J.S. Berns, A.R. Nissenson (Eds), CURRENT Diagnosis & Treatment: Nephrology & Hypeension.
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