Autosomal-dominant polycystic kidney disease occurs throughout the world.
The prognosis in adults is extremely variable. Progression may be slow, even after symptoms of renal insufficiency appear. However, after uremic symptoms develop, polycystic kidney disease is usually fatal within 4 years unless the patient receives treatment with dialysis, a kidney transplant, or both.
The prevalence in reported series varies between 1 in 400 and 1 in 1,000 in people of European descent. The age and adjusted yearly incidence rate in the United States is 1.38 in 100,000. This figure almost doubles if those people in whom the diagnosis was made at autopsy are included.
Overall, approximately 600,000 Americans have the disease, making Autosomal-dominant polycystic kidney disease one of the most common hereditary disorders in the United States.
Although both types of polycystic kidney disease are genetically transmitted, the incidence in two distinct age-groups and different inheritance patterns suggest two unrelated disorders. The infantile type appears to be inherited as an autosomal recessive trait; the adult type, as an autosomal dominant trait. Both types affect males and females equally.
The frequency of the disease in people of non-European ethnic background is not known; it may be less frequent in blacks.
Autosomal-dominant polycystic kidney disease is an important cause of ESRD; it accounts for approximately 4% of ESRD in the United States and 8% to 10% in Europe and Australia .
Polycystic kidney disease can't be cured. The primary goal of treatment is preserving renal parenchyma and preventing infectious complications. Management of secondary hypertension will also help prevent rapid deterioration in function. Progressive renal failure requires treatment similar to that for other types of renal disease, including dialysis or, rarely, a kidney transplant.
In Japan, approximately 5% of patients with ESRD have Autosomal-dominant polycystic kidney disease.
Polycystic kidney disease is an inherited disease that affects the kidneys. It may occur as a non-inherited, acquired form as a result of long-term kidney problems, dialysis, and old age. The disease can cause cysts to develop in your liver, pancreas, membranes that surround your brain and central nervous system, and seminal vesicles. These cysts can grow so large and so numerous that they crowd out the normal kidney tissue, rendering the kidneys unable to function normally. It is one of the most common genetic diseases in humans. PKD is the most common inherited disease in the United States. It is a genetic disorder characterized by the growth of numerous cysts in the kidneys. If too many cysts grow or if they get too big, the kidneys become damaged. The cysts may also cause pain or may get infected. Three factors determine cyst classification: its cause (acquired, inherited), its features (complicated, simple, multiple, single), and its location (outer [cortical] or inner [medullary] kidney tissue). The disease can also damage the liver , pancreas and rarely the heart and brain . The two major forms of polycystic kidney disease are distinguished by their patterns of inheritance.
PKD can also cause cysts in the liver and problems in other organs , such as the heart and blood vessels in the brain. The disease varies greatly in its severity, and some complications are preventable. Defective polycystins appear to contribute to cyst formation by affecting epithelial cell maturation, resulting in the development of cysts of varying sizes in the cortex and medulla. The two major forms of polycystic kidney disease are distinguished by the usual age of onset and their pattern of inheritance. About one-half of people with the major type of PKD progress to kidney failure, also called end-stage renal disease (ESRD). Persons with autosomal dominant PKD often exhibit no symptoms and the disease can not be diagnosed by routine blood work in its early stages. The autosomal recessive form of polycystic kidney disease (sometimes called ARPKD) is much rarer and is often lethal early in life. Children of parents with PKD have a 50% chance of getting the disease. Regular checkups can lead to treatments to reduce damage to your kidneys from complications, such as high blood pressure.
Causes of Polycystic Kidney Disease
The comman causes of Polycystic Kidney Disease include the following:
- PKD is caused by a genetic defect.
- The disease can cause cysts to develop in your liver, pancreas, membranes that surround your brain and central nervous system, and seminal vesicles.
- High blood sugar ( diabetes ).
- Long-term use of medicines that can damage the kidneys.
- The cysts may cause the kidneys to increase production of erythropoietin resulting in too many red blood cells, rather than the expected anemia of chronic kidney disease.
- When the kidneys stop working, doctors use a treatment called dialysis to remove waste products and extra water from patients with chronic kidney failure.
Symptoms of Polycystic Kidney Disease
Some sign and symptoms related to Polycystic Kidney Disease are as follows:
- Attacks of sudden and severe pain in the abdomen and/or lower back.
- Urinary tract infections.
- Flank pain on one or both sides.
- Blood in the urine.
- Kidney failure.
- High blood pressure.
- Aneurysms in the brain and diverticulosis.
- Abdominal pain or tenderness.
- Vague discomfort or aching in the abdomen and/or lower back.
- Increase in the size of your abdomen.
- Painful menstruation.
Treatment of Polycystic Kidney Disease
Here is list of the methods for treating Polycystic Kidney Disease:
- In rare cases, surgery is used to stop bleeding of the cysts and to remove tumors or suspected tumors.
- High blood pressure is generally treated with medications and a low salt diet. Patients with anemia are given iron supplements, and infections are treated with antibiotics.
- You'll need to drink lots of fluids as soon as blood in the urine is noted, in order to dilute the urine. Dilution may help prevent obstructive clots from forming in your urinary tract.
- In order to optimize ventilation, fluid overload can be managed with diuretics, continuous renal replacement therapy, and nephrectomy.
- Antibiotics are used to treat urinary tract infections.
- Surgical removal of one or both kidneys may be required. Treatment of end-stage kidney disease may include kidney dialysis or kidney transplantation.