Taken from Reference Medscape 2011: Autosomal Polycystic Kidney Disease

http://emedicine.medscape.com/article/244907-medication

Medical Care

Ensure that a patient with ADPKD who is nonhypertensive and has normal renal function undergoes blood testing and ultrasonographic scanning once a year.

Schedule more frequent follow-up studies for patients with high blood pressure. Hypertension is common, occurring in as many as 50-70% of patients before the onset of renal failure.

Patients with renal failure require more frequent monitoring, based on the severity of their condition.

A study by Torres et al identified serum high-density lipoprotein (HDL) cholesterol, urine sodium excretion (UNa V), and 24-hour osmolality as having a likely effect on ADPKD progression.[11] Whether modification of these components influences the clinical course of ADPKD remains unclear.

Medical therapy is necessary to accomplish the following:

• Control blood pressure. In patients with renal disease, the goal is a blood pressure of less than 130/88 mm Hg. If more than 1 g/d of urinary protein is present, the target blood pressure is less than 125/75 mm Hg. Achieving good blood pressure control helps slow the progression of renal disease. A study by Patch et al showed that, when intensity and coverage of antihypertensive therapy was increased, mortality decreased for patients with ADPKD.[12]
• The best drugs for this condition are ACE inhibitors (ie, captopril, enalapril, lisinopril) or angiotensin II receptor antagonist blockers (ie, telmisartan, losartan, irbesartan, candesartan). Calcium channel blockers are not recommended.
• Control abnormalities related to renal failure (ie, hyperkalemia,hyperphosphatemia, hypocalcemia, hyperparathyroidism, acidosis).
• Treat urinary tract infections, which occur in 30-50% of patients and most frequently in women. Gram-negative bacteria are the most common pathogens.
• Reduce abdominal pain produced by enlarged kidneys.
• Avoid nonsteroidal anti-inflammatory drugs (NSAIDs).
• With heart murmurs, institute routine American Heart Association antibiotic prophylaxis.
• Treatment involves surgical cyst decompression, which is effective for pain relief in 60-80% of patients. See Surgical Care.
• Distinguishing between infections of the bladder, renal parenchyma, and cysts is important because the treatment for each condition is different. Treating infected cysts requires antibiotics that penetrate into the cyst. Useful agents are ciprofloxacin, trimethoprim-sulfamethoxazole, clindamycin, and chloramphenicol.
• Patients with ADPKD and ESRD may undergo hemodialysis, peritoneal dialysis, or renal transplantation.

Surgical Care

Infected renal or hepatic cysts

• If infected cysts do not respond to conventional antibiotic therapy, surgical drainage may be necessary.
• This procedure is usually performed with ultrasonographically guided puncture.

Large cysts causing abdominal pain

• Cysts may become large enough to cause abdominal discomfort or pain. Typically, acute pain is from cyst hemorrhage or an obstruction by a clot, stone, or infection.[13]
• When one or more cysts can be identified as causing the pain, the symptoms can often be abated by open- or fiber optic–guided surgery to excise the outer walls and to drain them.
• In approximately one half of patients, however, candidate cysts cannot be identified as directly causing the pain. In these cases, indiscriminate excision of dozens of cyst walls that abut the capsule have produced complete symptomatic relief for months or years. Volumetric reduction of these kidneys usually exceeds 50% but still leaves kidneys larger than normal size. Not every cyst can be removed, and, with time, the residual cysts enlarge and symptoms may reappear.
• Approximately one quarter of patients with the most severe pain do not gain relief from surgery or pharmacologic therapy with narcotics. These individuals usually have inaccessible cysts in the medullary portions of the kidneys. Nephrectomy is used as a last resort to control the pain in these patients.

Massive polycystic liver diseases (See the image below.)

• When the liver becomes so large that it prevents the patient from obtaining normal nutrition or causes severe abdominal discomfort, a surgical procedure is necessary.
• Surgical intervention may range from unroofing several cysts to a partial hepatectomy.
• Partial hepatectomy is difficult because of the characteristics of the polycystic liver. Only expert surgeons should proceed with this surgical procedure.
• When the polycystic liver causes portal hypertension or is very large with nonresectable areas, liver transplantation may be necessary.
• Special attention should be paid when bilateral nephrectomy has to be carried out in patients with severe liver involvement. Several cases of refractory ascites after bilateral nephrectomy have been reported in these patients.

Consultations

• Nephrologist upon evidence of renal insufficiency, hypertension, microalbuminuria, or concentrating defect
• Invasive radiologist for cyst sclerosis or drainage
• General surgeon for nephrectomy, cyst decompression, unroofing, or surgical hepatic procedures
• Neurosurgeon for ICAs
• Cardiologist for valvular abnormalities

Diet

Although a low-salt diet is recommended when hypertension or renal failure is present, no other special diet reportedly is of benefit.

Activity

Patients should avoid contact sports in which direct trauma to the back or abdomen is likely. This is especially important with larger, palpable kidneys in order to minimize the risk of rupture