What is Congenital Kidney Disease?

Congenital kidney disease means being born with differences in the kidneys or urinary tract — such as a single kidney, reflux, or a blockage. These differences happen during pregnancy and can affect how well the kidneys work.

Doctors often group these conditions under the name CAKUT (Congenital Anomalies of the Kidney and Urinary Tract). While the term may be unfamiliar, it describes one of the leading causes of chronic kidney disease and kidney failure in children and young adults.

Common Conditions

Families usually know their child’s diagnosis by its specific name. These are some of the most common types of congenital kidney disease:

Hydronephrosis

Swelling in the kidney because urine cannot drain properly

Vesicoureteral reflux (VUR)

Urine flows backward from the bladder to the kidneys.

Dysplastic or hypoplastic kidneys

Kidneys that didn’t develop normally or are too small.

Single kidney (renal agenesis or kidney removal)

A child is born with one functioning kidney or has one removed early in life.

Multicystic dysplastic kidney

A kidney made up of fluid-filled sacs instead of working tissue.

Posterior urethral valves (PUV)

A blockage in boys that stops urine from flowing out normally.

Diagnosis

Kidney differences can be found:

Before birth (prenatal ultrasound at 16–20 weeks)
After birth (due to UTIs, abnormal bloodwork, or imaging)

Doctors may use:

Ultrasounds to check kidney size and shape
Urine tests for infection or protein
Blood tests for kidney function

Causes

Kidney differences can be found:

Genetic changes or family history
Maternal health during pregnancy (e.g. diabetes)
Certain medications during pregnancy

Parents did nothing wrong. Most cases occur simply due to how the baby developed.

How It Affects Children

Every child is different. Some live healthy lives with only monitoring. Others may:

Experience urinary tract infections (UTIs)
Develop high blood pressure
Slowly lose kidney function over time
In fact, congenital kidney disease is responsible for 40–50% of all kidney
failure in children. Early detection and monitoring make a big difference.

Adults Living With Congenital Kidney Disease

Although these conditions start before birth, many people are not diagnosed until adolescence or adulthood. Some only discover it after developing high blood pressure, abnormal labs, or through imaging for another reason. Signs it may still affect you as an adult:

Frequent UTIs in childhood
Only one kidney (or history of kidney removal)
High blood pressure at a young age
Elevated creatinine or low eGFR
Past diagnoses like reflux, PUV, or hydronephrosis

If this sounds familiar, talk to a nephrologist — especially if you have never had one follow your kidney health.

Treatment and Management

Congenital kidney differences cannot be reversed — a kidney that did not form typically cannot be remade. However, treatments and interventions can support and protect kidney function over time. Care focuses on monitoring, managing risks, and supporting overall health.

Mild cases → regular monitoring
Moderate cases → surgery for blockages or reflux, bladder training, catheterization, and growth support
Severe cases → frequent lab and blood pressure checks, and kidney replacement therapy such as dialysis or transplant, if needed

The challenge today is that there are limited therapies available to support kidneys and slow damage from getting worse. This is why KidneyFuture was created — to push for earlier action and better treatment options in the future.

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