Polycystic Kidney Disease (PKD)

Home » Polycystic Kidney Disease (PKD) » Signs and Symptoms, Diagnosis, Treatment, Prognosis

Signs and Symptoms

Symptoms of PKD include those that affect the kidney as well as some that affect other organs, including the brain and liver. Though little is known about kidney-related cyst formation in other organs, it is probably related to abnormalities in the glomerular membrane.

Autosomal Dominant PKD

• Abdominal, back, or side pain caused by:
  • Enlarged kidney
  • Hemorrhage into cyst
  • Infected cyst
  • Kidney stone
  • Rupture of cyst
• Aneurysm (weakened blood vessel in the brain)
• Chronic renal failure and end-stage renal disease (ESDR)
• Hematuria
• Hypertension
• Pancreatic and hepatic (in the liver) cysts

Autosomal Recessive PKD (infantile PKD)

• Enlarged cystic kidney at birth
• Hepatic fibrosis (abnormally small liver with scar tissue)
• Hypertension (high blood pressure)

Diagnosis

A nephrologist uses CT scan, x ray, and ultrasound to view kidney cysts. Autosomal dominant PKD is diagnosed when cysts are found in other organs as well as the kidney.

Scarring (sclerosis) of the liver distinguishes PKD recessive type and can be seen in a developing fetus.

Treatment

Treating PKD involves the following:

• Controlling pain, headaches
• Curing urinary tract infection (UTI)
• Controlling hypertension

Surgical reduction (removal) of cysts reduces pain in some people, especially if the cysts are large, but it does not alter the course of PKD and cannot stop cysts from developing. Surgery is performed with a laparacsope through a small incision in the abdomen. Headaches may be caused by vasculitis (i.e., swollen blood vessels in the head). Antibiotics are used to cure UTI before it spreads to the cysts and ACE inhibitors are used to lower blood pressure.

Renal replacement therapy, which involves kidney dialysis or transplantation, may be required in ESDR to restore kidney function. If the prospective kidney comes from someone in the family, genetic counseling and assessment is necessary. Cysts do not develop in healthy transplanted kidneys.

Prognosis

Many infants and children with recessive PKD die from hapatic fibrosis, which obstructs blood flow and causes bile buildup in the liver. Its symptoms are enlargement of the liver and the spread of a fibrous connective tissue over the liver. Those who survive into their 20s may develop splenic, pancreatic, and vascular problems. Children with recessive PKD often have smaller than average stature.

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