Polycystic Kidney Disease (PKD)
Polycystic Kidney Disease (PKD)
Polycystic kidney disease (PKD) is an inherited disorder in which clusters of cysts develop primarily within your kidneys. Cysts are noncancerous round sacs containing water-like fluid. The cysts vary in size and, as they accumulate more fluid, they can grow very large.
Although kidneys usually are the most severely affected organs, polycystic kidney disease can cause cysts to develop in your liver and elsewhere in your body. The disease causes a variety of serious complications.
Types of Polycystic kidney disease (PKD)
There are two types of polycystic kidney diseases.That includes
1. Autosomal dominant Polycystic Kidney disease (ADPKD)
2. Autosomal Recessive Polycystic Kidney Disease (ARPKD)
Rare and usually fatal before 1 year of life.
Autosomal dominant polycystic kidney disease (ADPKD). Signs and symptoms of ADPKD often develop between the ages of 30 and 40. In the past, this type was called adult polycystic kidney disease, but children can develop the disorder.
Only one parent needs to have the disease in order for it to pass along to the children. If one parent has ADPKD, each child has a 50 percent chance of getting the disease. This form accounts for about 90 percent of cases of polycystic kidney disease.
Autosomal recessive polycystic kidney disease (ARPKD). This type is far less common than is ADPKD. The signs and symptoms often appear shortly after birth. Sometimes, symptoms don’t appear until later in childhood or during adolescence.
Both parents must have abnormal genes to pass on this form of the disease. If both parents carry a gene for this disorder, each child has a 25 percent chance of getting the disease.
Clinical features of ADPKD
Pain in the renal angles, haematuria, headache
Gastronitestinal complaints
Nocturia, frequently, polyuria
Slowly developing hypertension
Both kidneys palpated and surface nodular
Uremia in late stages
Other systemic involvement in ADPKD
- Genetourinary
Hypernephroma, renal calculi, ovarian cyst
B. Gastro intestinal
Hepatic cysts, pancreatic cysts, diverticuli
C. C.V.S
Hypertension, aortic and mitral valve abnormalities, thoracic aortic aneurysm
D. Central Nervous System
Berry aneursms (Subarachnois haemorrahage)
E. Haematopoietic
Erythrocytosis
Diagnosis of Polycystic Kidney Disease (PKD)
Clinical features and family history of polycystic disease.
Renal ultra sonogrphy : Demonstrates bilateral renal cysts; follow up renal ltrasound in 2-3 years may show increasing renal cystic involvement.
Total abdominal ultrasonography : may be done to demonstrate hepatic, pancreatic, ovarian cysts.
CT Scan of abdomen
Treatment for PKD
Haematuria : Bed rest, analgesics, hydration
Bladder and renal parenchymal infection,hypertension, subarachnois haemorrahage
Avoiding renal trauma.
Complications of Polycystic Kidney Disease
A common complication of polycystic kidney disease is high blood pressure. Kidney failure is another common problem for people with polycystic kidney disease.
Polycystic kidney disease varies greatly in its severity, and some complications are preventable. Lifestyle changes and medical treatments may help reduce damage to your kidneys from complications, such as high blood pressure.