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A patient's physical condition can be normal for many years after cysts start forming and physical checkups and blood tests may not show any additional signs of the autosomal dominant polycystic kidney disease (ADPKD). As the disease progresses, the kidneys gradually increase in size in addition to losing the ability to remove wastes and toxins from the blood, including creatinine. Since the buildup of toxins leads to uremia or "uremic poisoning" and precedes "end-stage renal disease" (ESRD), dialysis or renal transplantation is necessary to take over the functions of the kidneys. Renal failure usually develops between 40 and 60 years of age in ADPKD patients.

In general, the number and severity of symptoms and complications tend to increase with age. These include:

• Hypertension
• Pain
• Extrarenal cysts (cysts outside the kidney)
• Intracranial aneurysms
• Circulatory disturbances
• Diverticular disease

Hypertension

Hypertension (high blood pressure) is closely associated with ADPKD and develops in approximately 70% of adults and 10-15% of children before the onset of renal failure. It is often one of the earliest symptoms of PKD. A relationship appears to exist between the volume of the kidneys and the severity of hypertension.

It is thought that the rise in blood pressure may be secondary to renal damage caused by the cysts. Renal ischemia, the obstruction of the blood vessels due to compression by the cysts and resulting increased mass of the kidney, results in reduced blood flow and reduced delivery of oxygen to the kidney. This increases the release of renin and results in elevated blood pressure.

Hypertension ultimately contributes to elevating cardiovascular risk and to progression of renal failure.

Pain

Pain is one of the most common symptoms in polycystic kidney disease and is found in about half of PKD patients. Pain can be due to several causes, can be acute or chronic, and can range from mild to very severe. Imaging using computerized tomography (CT), magnetic resonance imaging (MRI), or ultrasound is most effective in pinpointing which cysts are largest or which may be the source of greatest pain.

The pain may be felt in the region of the kidneys (back, sides, and under the ribs) as well as in the abdomen due to the pressure resulting from the enlarged kidneys.

Acute Pain

Acute pain can be due many factors including:

• Infection - this may be due to a urinary tract infection or infection of the cysts.

• Hematuria - Gross hematuria (blood that is visible in the urine) occurs in approximately 50% of patients with PKD and is associated with rupture of renal cysts. This occurs when cysts rupture and hemorrhage into the urinary tract. It is associated with flank pain (pain between the upper abdomen and the back). Increased frequency of episodes of gross hematuria may indicate a worse prognosis. Episodes of hematuria usually last from 2-7 days and usually resolve with bedrest and hydration.

• Kidney Stones - Kidney stones (nephrolithiasis) occur in up to 30% of people with PKD and can cause considerable pain, usually in the flank. Impaired drainage of the kidney because of PKD may lead to crystal formation that may accumulate and develop into stones. Kidney stones are also associated with urinary tract infections and hematuria. Renal function in patients with polycystic kidney disease who develop kidney stones is usually worse than in PKD patients without stones. Some researchers correlate the development of kidney stones with individuals who exhibit large numbers of cysts and for whom cyst size is large.

Chronic Pain