Renal Dysplasia and Cystic Diseases & Can They Be Cured?

How are these different types of renal conditions and diseases treated?

Renal agenesis: There is no specific treatment for renal agenesis. Dietary precautions are likely to have a limited influence on the remaining single kidney and are not practical to be maintained long term. Restriction in contact sports is recommended, along with avoiding activity that places the individual at substantial risk due to injury (e.g., motorcycle riding, all-terrain vehicles and trampolines). Your doctor should discuss with you appropriate restrictions and limitations.

Renal hypoplasia: There is no treatment for renal hypoplasia. Any associated ureteral problems should be treated to reduce the risk of infection and rapid decrease in kidney function. Unfortunately, this will not reverse the process of hypoplasia. Therapy consists of maintaining fluid and electrolyte balance and correcting abnormal acidity levels in the blood.

Autosomal recessive polycystic kidney disease (ARPK): There is no cure for ARPK. Treatment for this childhood illness focuses on supportive measures – such as dialysis – for kidney failure. If the organs are enlarged, some children benefit from removing them, especially if the size is causing difficulty with breathing, feeding or peritoneal dialysis.

Autosomal dominant polycystic kidney disease (ADPK): Since there is also no cure for ADPK, treatment centers on controlling symptoms. Approximately 60 percent of patients with this disease have high blood pressure that, if left untreated, can lead to heart disease and brain hemorrhages or stroke. More than half of these patients will have pain that can be very intense, occur suddenly and persist for a long time. Pain can be due to the presence of stones, blood clots or infection. Also, individuals (particularly women) with ADPK are at high risk for urinary tract infections. But treating these infections can be difficult, given the limited concentration of antibiotics within the cyst fluid.

When such an infection does not respond to medication, your doctor may recommend draining the cyst by needle aspiration, laparoscopy or an open surgical procedure. While draining the cysts connected to this disease can be beneficial, the fluid is likely to accumulate again. In any case, you will be followed closely over time since the disease is progressive and renal failure can occur, which will require supportive measures.

Juvenile nephronophthisis and medullary cystic disease complex: Because patients with either juvenile nephronophthisis or medullary cystic disease complex experience excessive thirst and water loss, they will need to replace salt in their diets. Since medical researchers have not found a cause for either of these conditions, the likely outcome is kidney failure, which will require supportive measures.

Tuberous sclerosis (TS): Unfortunately there is no cure for TS but there is treatment for a number of its symptoms and patients should be monitored regularly so they can be treated appropriately. Anti-epileptic drugs may be used to control seizures, and medications may be prescribed for behavior problems.

Von Hippel-Lindau disease: There is no cure for this disease and the treatment is symptomatic support. If a urologist suspects a cyst is actually a tumor, they will remove the growth and a portion of the kidney. When lesions are larger than four centimeters, a more aggressive approach – such as a removal of the organ (nephrectomy) – might be required.

Multicystic dysplastic kidney (MCDK): The treatment for a multicystic kidney varies. Some pediatric urologists favor early renal tumor removal that can often be done as an outpatient procedure. Others favor observation since long-term problems associated with unilateral MCDK are rare and the kidneys will often become smaller.

Renal cyst: Observation is the treatment for a simple renal cyst. Only when symptoms develop, do urologists drain or remove it.

Medullary sponge kidney: There is no cure for medullary sponge kidney. Instead, treatment centers on management of stone formation and urinary infections. Water pills may be effective in decreasing the calcium output and limiting stone formation. Patients who have recurrent urinary infections may benefit from long-term, low-dose antibiotics.

Acquired renal cystic disease: Individuals who exhibit symptoms may benefit from removal of the kidney, particularly if they are already undergoing dialysis.

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