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Polycystic Kidney Disease

What is Polycystic Kidney Disease?

Polycystic Kidney Disease (PKD) is a disorder in which numerous groups of cysts develop within the inner outline of the kidneys. Cysts are noncancerous lesions which are rounded and fluid-filled.

Polycystic Kidney Disease may occur in different bodily organs aside from the kidneys, although kidneys are the most commonly associated with severe affectation of the disease. PKD could also lead to the development of cysts to the liver and in other organs of the body.

Common complications of polycystic kidney disease include hypertension, although kidney failure could also arise from people with polycystic kidney disease.

Polycystic kidney disease could vary widely in its severity, and some of the known complications could be prevented through proper treatment and referral. Lifestyle modifications and medical interventions could help reduce kidney tissue damage from complications such as hypertension.

At present, polycystic kidney disease is considered one of the most frequently inherited disorders; it affects approximately 600,000 Americans and a total of 12,000,000 of the world’s population. Most clients fall from the autosomal dominant type of the disease. It is the fourth leading cause of renal failure and 10% of all end-stage renal disease (ESRD), the age range is usually clients 40 to 60 years old. It equally affects any gender and race.

Symptoms of Polycystic Kidney Disease

Symptoms of polycystic kidney disease may include:

  • Back pain or flank pain
  • Blood in the urine (hematuria)
  • Frequent urination (polyuria)
  • Headache
  • Hypertension
  • Increased abdominal size
  • Kidney failure
  • Kidney stones
  • Urinary tract or kidney infection

It is possible for people to have polycystic kidney disease for a long period of time without manifesting any signs and symptoms which make them remain unknowledgeable of their current condition.

If any of the given signs and symptoms begin to manifest, it is recommended that one have a visit to his/her doctor in order for them to be appropriately diagnosed. It is recommended to discuss the screening process to the physician, especially if the client is a first-degree relative to another client diagnosed with polycystic kidney disease.

What Causes Polycystic Kidney Disease?

Cysts are benign (noncancerous) lesions; they are sac-like and contain water-like fluid. They vary in size and they can grow extremely large as they begin to accumulate more fluid. A normal kidney weighs one-third pound or less (approximately three-quarters of a kilogram), while a kidney affected with a number of cysts could weigh as much as 30 pounds.

Abnormal genes cause polycystic kidney disease, and the disease being genetic, means it runs hereditarily through the family tree. Genetic mutation could also be a cause of polycystic kidney disease, but this are just in rare cases. There are two types of polycystic kidney disease, each caused by different genetic abnormalities:

  • Autosomal dominant polycystic kidney disease (ADPKD). Signs and symptoms of ADPKD often develop to clients between ages 30 and 40. This was recently called adult polycystic kidney disease, but was changed as children could also develop the disorder. Only one parent needs to have the disease in order for it to be passed along the next generation. If one parent has ADPKD, each client of the next generation has a 50 percent risk of obtaining the disease. 90 percent of cases of polycystic kidney disease fall to this criterion.
  • Autosomal recessive polycystic kidney disease (ARPKD). This a less common type of polycystic kidney disease. The signs and symptoms often occur to neonates (newborns) or infants. In some cases, symptoms don’t appear later in childhood or during the client’s adolescence. Both parents must have the polycystic kidney disease causing gene to pass this form of the disease. If both parents carry a gene for this criterion, each client of the next generation has a 25 percent chance of obtaining the same type of the disease.

Research and studies have identified two genes associated with ADPKD and one associated with ARPKD.

There are cases where a client with ADPKD has no family history of the said disease. It is also possible, however, that an affected member of the family had the disease but did not manifest any signs or symptoms before dying of complications or other causes. In a rare percentage of cases where no familial risk is present, ADKD results from a spontaneous gene mutation.

Diagnosis of Polycystic Kidney Disease

Diagnostic techniques are available to identify the size and number of kidney cysts as well as to assess the amount of normal kidney tissue.

  • Ultrasound examination. In this diagnostic test, a wand-like instrument called a transducer is placed on the area to be visualized. It produces inaudible sound waves that are then reflected back to the transducer. A device then would translate the data collected into images of the kidneys.
  • Computerized Tomography (CT) scan. The client would be asked to lie on a moveable table, which the client would then be guided inside a dome-like device that project various cross-sectional x-ray beams through the client. It would then be analyzed by health-care professionals through cross-sectional images of the kidneys.
  • Magnetic Resonance Imaging (MRI) scan. As you lie inside a dome-like device, magnetic fields and radio-waves create cross-sectional views of the kidneys.

Treatment of Polycystic Kidney Disease

The treatment of polycystic kidney disease is symptomatic, and is also driven to prevent or treat its complications.

  • Hypertension is controlled by giving the client a low-sodium and low fat diet with moderate protein and calorie intake. Lifestyle changes like smoking cessation, increase exercise and stress reduction may help control blood pressure. Medications given are Angiotensin-converting Enzymes (ACE) inhibitors.
  • Pain is managed by non-pharmaceutical pain management and by taking acetaminophen containing drugs. Surgery is also recommended if the cysts grew large enough to cause pressure to the organ causing pain. They are also surgically removed when they begin to obstruct blood flow to the organ.
  • Bladder or kidney infections are treated using appropriate antibiotics.
  • Blood in the urine or hematuria is initially managed with increased fluid intake to dilute the urine. In this way, obstructive clots would be prevented from forming in the urinary tract. Bed rest may also reduce the occurrence of bleeding.
  • Dialysis or kidney transplant is usually done for clients that progressed to kidney failure.
  • In cases of liver cysts, hormone replacement therapy is discouraged. Drainage of the cysts is performed when they are of small numbers. Partial removal of the liver or even liver transplantation could also be considered.
  • Regular screening for intracranial aneurysms might also be recommended if the client with PKD has a history of intracranial aneurysms. If an aneurysm is found, surgical clipping may be an option to reduce bleeding.

How to Prevent Polycystic Kidney Disease

In clients with polycystic kidney disease who are considering having offspring, they could ask the help of genetic counselors to help in assessing their risk of passing the disease to their next generation.

Keeping the kidneys physiologically normal as possible may help in preventing complications of the disease. One intervention is blood pressure management.

  • Proper adherence to the medication regimen provided by the physician.
  • A low-salt and low-fat diet with high-fiber content.
  • Maintaining a normal body weight.
  • Smoking cessation
  • Regular exercise

 

Reference:

http://www.healthcommunities.com/polycystic-kidney-disease/overview-of-pkd.shtml

http://www.mayoclinic.com/health/polycystic-kidney-disease/DS00245