How to Treat Autosomal Recessive Polycystic Kidney Disease?

  • February 12, 2024
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How to Treat Autosomal Recessive Polycystic Kidney Disease?

What Is Autosomal Recessive Polycystic Kidney Disease (ARPKD) ?

Autosomal recessive polycystic kidney disease (ARPKD) is a rare problem that happens in about 1 in every 20,000 children. It starts when fluid-filled cysts form in the kidneys, making them big and not work well, even before birth. Doctors can find this problem early because it shows up in babies. Sometimes, babies with ARPKD can have trouble breathing and may not survive the first week after birth. But if they make it past that, they usually grow up into adults. However, they might need medical help all their life. ARPKD also affects the liver because of changes in a gene called PKHD1, which is needed for making a special protein for the kidneys and liver. This means people with ARPKD need to see doctors regularly to manage their health and stay as healthy as possible.

Why Treat ARPKD?

Treating ARPKD is crucial to prevent or manage complications associated with the condition. Without treatment, ARPKD can progress to kidney failure, which may require dialysis or kidney transplantation. Additionally, individuals with ARPKD are at risk of developing high blood pressure, urinary tract infections, and liver complications such as portal hypertension and liver fibrosis. Early detection and intervention can help improve outcomes and quality of life for individuals living with ARPKD.

How to Treat ARPKD?

  1. Management of Symptoms: Treatment for ARPKD focuses on managing symptoms and complications associated with the condition. This may include medications to control blood pressure, antibiotics to treat infections, and dietary modifications to support kidney function.
  2. Monitoring: Regular monitoring of kidney and liver function is essential for individuals with ARPKD. This may involve blood tests, urine tests, imaging studies (such as ultrasound or MRI), and kidney biopsies to assess the progression of the disease and detect any complications early.
  3. Supportive Care: Individuals with ARPKD may require supportive care to manage symptoms and improve quality of life. This may include pain management, nutritional support, and access to specialized healthcare providers, such as nephrologists, hepatologists, and genetic counselors.
  4. Dialysis: In cases where ARPKD progresses to kidney failure, dialysis may be necessary to remove waste products and excess fluid from the blood. Dialysis can help improve symptoms and prolong life while awaiting a kidney transplant.
  5. Kidney Transplantation: Kidney transplantation is the most effective treatment for ARPKD-related kidney failure. During a kidney transplant, a healthy kidney from a donor is surgically implanted into the recipient's body to replace the damaged kidneys. A successful kidney transplant can restore kidney function and improve quality of life for individuals with ARPKD.