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Renal manifestations of autosomal dominant polycystic kidney disease

Authors
Arlene B Chapman, MD
Frederic F Rahbari-Oskoui, MD, MSCR
William M Bennett, MD
Section Editor
Ronald D Perrone, MD
Deputy Editor
Alice M Sheridan, MD

INTRODUCTION

Autosomal dominant polycystic kidney disease (ADPKD) often leads to progressive renal failure due in part to continued enlargement of the cysts. Kidney size typically increases to more than five times normal in the years prior to the loss of kidney function, and measured total kidney volume is the strongest predictor for the development of renal insufficiency [1]. Other renal manifestations that can occur include hypertension, urinary tract infection, concentrating defect, hematuria, nephrolithiasis, and acute or chronic flank and abdominal pain; protein excretion is generally not a prominent feature [2,3]. All complications relate directly to the extent of renal cyst involvement, which can be assessed by total kidney volume measurements. (See "Course and treatment of autosomal dominant polycystic kidney disease" and "Hypertension in autosomal dominant polycystic kidney disease" and "Urinary tract infection in autosomal dominant polycystic kidney disease".)

HEMATURIA

Hematuria, which is often grossly visible, occurs at some time in the course in approximately 35 to 50 percent of patients with ADPKD, usually occurs prior to loss of kidney function, and may be the presenting symptom of the disease [2,4]. A precipitating event, such as a urinary tract infection or strenuous activity, can often be identified, and recurrent episodes are not uncommon [4]. Gross hematuria is associated with more rapid progression of kidney disease in ADPKD [4].

Rupture of a cyst into the collecting system is thought to be responsible for the development of hematuria. Although hemorrhage into a cyst is also common, the typical presentation is pain, rather than hematuria, since many cysts do not communicate with the collecting system [2]. The hematuria due to cyst rupture generally resolves within two to seven days with conservative therapy. Conservative therapy consists of bedrest, hydration, and analgesics that exclude nonsteroidals. In some cases, antihypertensive medications may be held [4]. Occasionally, bleeding can persist for several weeks. With unusual and severe bleeding, percutaneous arterial embolization or even nephrectomy may become necessary [5].

Gross hematuria is more likely among individuals with larger kidneys (particularly when >15 cm in length), hypertension, and higher plasma creatinine concentrations [4,6]. Frequent episodes of gross hematuria before 30 years of age may be associated with worse renal outcomes; this may possibly reflect accelerated cyst expansion [7].

Nephrolithiasis is another cause of hematuria in ADPKD patients. The hematuria associated with nephrolithiasis is usually microscopic. The hematuria should resolve with passage or removal of the stone. Prolonged or recurrent hematuria, particularly in a man over 50 years of age, raises the possibility of an underlying renal cell carcinoma (RCC) [2] or of an unrelated problem, such as bladder cancer, prostate cancer, or immunoglobulin A (IgA) nephropathy [8]. (See 'Renal cancer' below.)

         

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Literature review current through: Nov 2016. | This topic last updated: Wed Oct 05 00:00:00 GMT+00:00 2016.
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