Polycystic Kidney Diseases
The kidneys are two organs that filter waste and excess fluid in your blood which is then eliminated as urine. According to the Mayo Clinic, polycystic kidney disease occurs when clusters of noncancerous sacs of fluid develop in your kidneys. The sacs are called cysts and they may be very small or very large. The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) explains that cysts grow out of nephrons which are tiny filtering units inside your kidneys. Eventually the cysts separate from the nephrons and continue to enlarge. "The kidneys enlarge along with the cysts--which can number in the thousands--while roughly retaining their kidney shape," states the NIDDK.-
Significance
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Understanding the cause of polycystic kidney disease is important since the disease affects not only your kidneys but other vital organs. It can cause cysts to develop in your liver, pancreas in the membranes surrounding your brain and central nervous system and seminal vesicles, according to the Mayo Clinic. If you have polycystic kidney disease you are at risk for high blood pressure and kidney failure. Polycystic kidney disease is also associated with brain aneurysms and diverticula of the colon. Complications from the disease can be prevented by regular check ups.
Features
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Abnormal genes cause polycystic kidney disease, and the genetic defects mean the disease is inherited or passed down through families. The disease has two types, caused by different genetic flaws. It is unknown exactly what initiates the formation of cysts in the kidneys. In the early stages of polycystic kidney disease, the cysts may increase the kidneys' production of the hormone, erythropoietin, which stimulates production of red blood cells, resulting in too many red blood cells in your body.
Autosomal Dominant Polycystic Kidney Disease
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Autosomal dominant polycystic kidney disease (ADPKD), the first and most common type of polycystic kidney disease occurs in both children and adults, but more commonly in adults who often do not experience symptoms until they are in their 30s or 40s. According to the National Library of Medicine, as of 2009, ADPKD affects one in 1,000 Americans. "Autosomal dominant" means that if one parent has the disease, there is a 50 percent chance, the genetic defect will be passed on to their children, according to the NIDDK. "In some cases--perhaps 10 percent--autosomal dominant PKD occurs spontaneously in patients. In these cases, neither of the parents carries a copy of the disease gene," states the NIDDK.
Autosomal Recessive Polycystic Kidney Disease
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According to the National Library of Medicine (NLM), autosomal recessive form of polycystic kidney disease (ARPKD) appears in infancy or childhood. In this case, both parents have the defective genes that cause the disorder and each child has a 25 percent chance of developing polycystic kidney disease. ARPKD is less common than autosomal dominant polycystic kidney disease, occurring in one in 10,000 to 20,000 births in the United States, as of 2009. "This type tends to be very serious and progresses rapidly, resulting in end-stage kidney failure and generally causing death in infancy or childhood," states the NLM.
Symptoms and Diagnosis
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Symptoms of polycystic kidney disease include abdominal pain, blood in urine, excessive urination at night, pain in the back and on one or both sides, pain between the ribs and hips, drowsiness, headaches, high blood pressure, joint pain, nail abnormalities, unusual pain during menstruation. Your doctor will likely test for high blood pressure, do an abdominal ultrasound, computerized tomography (CT) scan or magnetic resonance imaging (MRI) scan to determine if your kidneys are enlarged and do a urinalysis to determine if blood or proteins are present that indicate polycystic kidney disease.
Treatment
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According to the Mayo Clinic, controlling high blood pressure is key in slowing the progression of polycystic kidney disease so it is important to be on a low-sodium and low-fat diet. In addition, your physician may prescribe high blood pressure medication which is in the category of angiotesin-converting enzyme inhibitors, such as benazepril or captopril, and a diurectic to eliminate excess fluid. If you develop a urinary tract infection as a result of the disease, it is crucial to treat it promptly with an antibiotic such as amoxicillin or ciprofloxacin. Depending on the severity of your disease you may have to have one or both of your kidneys removed. In end-stage kidney disease, kidney dialysis or a kidney transplant may become necessary.
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