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Polycystic kidney disease

Polycystic kidney disease in children 

About polycystic kidney disease 

Polycystic kidney disease (PKD) is a genetic disorder that causes fluid-filled cysts to grow in the kidneys. These cysts make the kidneys too big and eventually cause kidney failure. It is a serious condition that may require dialysis or a kidney transplant. There are two types of PKD: 

  • Autosomal dominant polycystic kidney disease (ADPKD) – A more common type of PKD that can be diagnosed anytime from in utero or adulthood 
  • Autosomal recessive polycystic kidney disease (ARPKD) – A very rare and serious genetic disorder that’s usually diagnosed in utero or shortly after birth 

At Children’s Hospital of Richmond at VCU, our nationally ranked nephrology team is known leaders in kidney care for kids – including diagnosing, treating and managing polycystic kidney disease. We work together with teams across our hospital, including urologists, dietitians, transplant teams and many others, to develop a plan that’s right for your child. 

Is polycystic kidney disease common in children? 

ARPKD is not common. It affects roughly 1 in 20,000 children. ADPKD is much more common. In fact, it’s the most common hereditary kidney disorder, which means it’s passed through family members. Researchers estimate that ADPKD affects 1 in every 400 to 1,000 people. 

Causes of polycystic kidney disease 

PKD is hereditary. Someone in the family must have the gene that causes PKD to pass it along to their children. 

  • ADPKD only requires one parent to have the PKD gene mutation to pass the condition to a child 
  • ARPKD requires that both parents have the PKD gene mutation and pass it to the child 

Signs of polycystic kidney disease 

ARPKD is often diagnosed in utero or shortly after birth. Your doctor may order additional testing if your baby presents any of these symptoms: 

  • Congenital hepatic fibrosis
  • Enlarged kidneys
  • Enlarged spleen and low blood counts
  • Excess urination
  • Growth failure
  • Hypertension (high blood pressure)
  • Low levels of amniotic fluid
  • Lung immaturity and breathing issues  

ADPKD can be diagnosed in utero, childhood and, sometimes, not until adulthood. Your child may show different symptoms based on the severity of disease and when it’s diagnosed. Symptoms of ADPKD include: 

  • Back or side pain
  • Blood in urine
  • Headaches
  • Hypertension (high blood pressure)
  • Kidney cysts
  • Kidney stones

Typically, at least one parent will know they have ADPKD. This can help diagnose the condition sooner and monitor it at an earlier stage.  

What can you do about polycystic kidney disease? 

PKD can affect many aspects of your child’s life. That’s why we work across specialties to connect your child, and entire family, to the care, support and services you need. Depending on the severity of your child’s condition and symptoms they experience, that may include: 

  • Breathing support and treatments
  • Medication and therapy to treat digestive tract problems, including diverticula
  • Medication and therapy to treat urinary tract infections or kidney stones
  • Medication to control high blood pressure
  • Monitoring heart valves and heart function
  • Monitoring the liver and pancreas for cysts
  • Nutritional support to encourage growth
  • Pain management  

Over time, kidney function may worsen and lead to kidney failure. Dialysis can help filter water and waste out of the blood until a kidney transplant is performed. Sometimes, PKD affects other organs. Children with severe complications may need a joint liver and kidney transplant

Learn more about CHoR’s polycystic kidney disease services and support