PKD stands for Polycystic Kidney Disease. PKD is a genetic disorder. There will be structural abnormality in renal tubules resulting in growth of multiple cysts within the kidneys. The cysts are likely to be developed during childhood or adulthood. Some researches have also found the disease can also start developing during intrauterine life. Cysts are fluid filled tubules which can be as minute as microscopic or extensive in size. Since PKD is caused due to the abnormal gene which produces a protein which has a harmful effect on tubules of kidneys affecting its development.
Types of PKD:
PKD is broadly classified into 3 types:
Autosomal dominant PKD( ADPKD ): This type of PKD needs only one copy of an abnormal gene which is dominant, symptoms start after the age of 25-28 years but it can appear in childhood as well. 90% of the cases are ADPKD.
Autosomal recessive PKD( ARPKD ): This type of PKD is acquired by recessive inheritance. It starts during the fetal life and can present symptoms in the first few months of life, disease progresses rapidly and is very dangerous. It's a rare form of disorder which appears in among 1 in 20,000 people.
Acquired cystic kidney disease( ACKD ): This happens in cases where there is long term kidney damage, even those who are on dialysis tend to develop this. The main presenting symptom is blood in urine.
Genetic counseling is required in patients who tend to carry hereditary or genetic disorders to have an idea about the probability of passing on the gene to the coming generations.
Symptoms and Complications:
Signs and symptoms include abdominal pain, blood in urine, rise in blood pressure, pain while urinating and frequent urination. Some symptoms suffered during early stage, need to be controlled to delay the progression of the disease itself. Example:
Bladder infection, this needs to be treated using antibiotics to avoid further kidney damage. Controlling the blood pressure is necessary to prevent further damage of the kidneys and this can be done by maintaining a low fat and low sodium diet along with less intake of alcohol and refraining oneself from smoking. When the blood is noticed in urine plenty of fluid intake is recommended to avoid the formation of blood clots.
Complication in condition takes over when the patient of PKD starts developing other problems such as growth of cysts in the liver, development of aneurysm in the brain, chances of preeclampsia during pregnancy, heart problems, weakness or sac formation in colon and also there will be chances of developing malignancy.
External examination will show enlarged liver on palpation of abdomen, murmur in heart is heard, blood pressure is slightly elevated.
Further evaluation requires an ultrasound abdomen, CT scan, MRI to confirm the diagnosis.
Blood and urine tests include BUN, serum creatinine, urine culture etc.
Since it is one among the genetic disorders there is no FDA approved treatment for it. Certain dietary restrictions have proved helpful in controlling the progression of certain types of PKD. The nephrologist decides the course of treatment upon progression of disease. The treatment includes renal replacement therapy if the disease has reached its end stage like kidney failure, or chronic kidney disease. This includes dialysis in different frequencies and duration. Painkillers like acetaminophen are recommended for tackling pain caused due to Polycystic kidney disease. Kidney replacement is an option if a suitable donor kidney is available. Certain precautions need to be taken care of when ADPKD is being treated. The practitioner must rule out any kind of allergy from medicine or food before proceeding with the treatment. The infection within the cysts need to be controlled thus avoiding the spread to maximum extent. Use of bacteriostatic drugs and bacteriocidal drugs is recommended.
Being one among the common hereditary disorders it affects more than 550000 people across the United states alone. Nearly 9%-10% of people reach the end stage. Along with humans PKD is also known to affect animals as well.
1. What is the life expectancy of patients having PKD?
Most commonest form of PKD is the Autosomal Dominant Polycystic Kidney Disease in which the average life expectancy would be around 50-65 years depending upon the condition of the patient.
2. What are the foods to be avoided in PKD?
The foods like meat, poultry along with skin, fatty food which can trigger hypertension, eggs, milk and foods which cause obesity need to be avoided.