Inherited kidney diseases — also known as hereditary kidney diseases — are genetic conditions that affect kidney function and are often passed from biological parents to their children. There are more than 300 genetic kidney disorders, many of which are very rare. Some affect only the kidneys, while others affect other organs as well.
The experts at UPMC specialize in treating all types of inherited kidney disease. Our team will develop a treatment plan to manage your symptoms and reduce your risk of complications.
Inherited kidney diseases — also known as hereditary kidney diseases — are genetic conditions that affect kidney function and are often passed from biological parents to their children. There are more than 300 genetic kidney disorders, many of which are very rare. Some affect only the kidneys, while others affect other organs as well.
The most common inherited kidney disease is autosomal dominant polycystic kidney disease (ADPKD). People with ADPKD form cysts on their kidneys, which fill with fluid and damage the kidneys over time.
Doctors can usually diagnose ADPKD before age 40 when kidney function is still normal. But it's crucial not to wait until kidney function begins to decline to start treatment for ADPKD.
Early treatment can delay the progression of the disease by many years. People with ADPKD can also develop kidney infections, kidney stones, and blood in their urine for other reasons that may require additional treatment.
Other common inherited kidney diseases include:
Congenital abnormalities of the kidney and ureteral tract (CAKUT) are a group of disorders that cause you to have either:
Doctors most often diagnose CAKUT during childhood, but the disease doesn't show up in some infants. When CAKUT affects adults, they either have a single kidney or chronic kidney disease of unknown cause.
Each person has around 25,000 genes in every cell, including two copies of each gene — one copy from each parent.
Changes in your genes or the chromosomes containing those genes can lead to inherited kidney disease. While parents may pass these mutations to their children, inherited kidney disease can also occur randomly when genes mutate, even without a family history of kidney disease.
Recessive hereditary kidney diseases need a mutation on both copies of a gene to cause disease, with one mutation coming from each parent.
The parents might not have had the disease but are “carriers" of it. A person is a carrier if one copy of their gene has a mutation and one copy does not.
Most people with recessive hereditary kidney diseases have parents who do not have the disease but may have another family member who has it.
Dominant hereditary kidney diseases only need a mutation on one copy of a gene to cause disease.
Adults with these mutations also have a normal copy of the gene. The chance that each child will inherit the copy with a mutation is 50%.
Family history is the biggest risk factor for hereditary kidney disease. However, other health problems may impact the severity of your symptoms and whether you’ll develop complications such as kidney failure.
Conditions that increase risk include:
Left untreated, inherited kidney conditions can damage your kidneys over time and lead to end-stage kidney disease. End-stage means your kidney function can no longer support your body's needs, and you need dialysis or a kidney transplant.
Living a healthy lifestyle and controlling conditions like high blood pressure and diabetes can help manage inherited kidney disease symptoms.
Most inherited kidney diseases are very rare.
Inherited kidney disease symptoms can vary from mild to severe. Some people don't have any symptoms at first. Over time, they may start or get worse.
Check with your doctor if you have any of these symptoms:
Fluid and waste buildup in your body can cause these symptoms. They may be a sign that your kidneys aren't working as they should.
If you have symptoms of kidney disease, you should schedule an appointment with your doctor right away.
Some types of inherited kidney disease are diagnosed at birth or cause symptoms right away. However, other types do not cause noticeable symptoms and are diagnosed later in life during routine physical exams or testing for other conditions.
If you have symptoms of kidney disease, your doctor will perform a physical exam, talk to you about your health history, and order tests to diagnose your condition.
During your visit, your doctor will:
Your doctor may order blood, urine, or other tests to check for signs of inherited kidney disease.
Blood tests include:
Urine tests include:
Genetic tests include:
Other tests include:
Your prognosis will depend on the type of inherited kidney disease you have and the severity of your symptoms. Your doctor will discuss your prognosis with you.
Although there is no cure for inherited kidney disease, the goal of treatment is to keep your condition from getting worse and prevent permanent kidney damage. Your treatment will depend on your type of inherited kidney disease and the severity of your symptoms.
Treatment may include:
Lifestyle changes may help slow the progression of inherited kidney disease. Your kidney care team will provide detailed guidance on how to change your diet and lifestyle.
Your doctor may recommend:
Your doctor may refer you to other medical specialists, such as a registered dietitian or a cardiologist, to help you manage your condition.
You might need medicine if you have high blood pressure, high blood sugar, or albumin in your urine.
Your doctor may prescribe medications such as:
Our experts in supportive care can also prescribe medicine to ease other painful symptoms.
End-stage kidney disease — also called kidney failure — can cause toxic waste to build up in your body. If your inherited kidney disease leads to kidney failure, you may need dialysis, also called renal replacement therapy, to filter and clean your blood.
Dialysis cleans waste from your blood and helps control your blood pressure. There are two common types of dialysis:
If you have end-stage kidney disease, dialysis is a lifesaving treatment option. Without it, toxins and fluid would build up in your body and cause death within days or weeks.
However, for most people, dialysis is only a short-term treatment. In time, you may need a kidney transplant.
Although dialysis treatments are lifesaving for many people, they can be hard and sometimes painful for others.
For people with advanced kidney disease who want to avoid dialysis treatment, renal supportive care can offer hope and comfort.
Supportive care aims to:
If you have kidney failure caused by inherited kidney disease, a kidney transplant may be the key to getting you back to your healthy life. We partner with UPMC kidney transplant experts to provide care before, during, and after transplant.
When you choose UPMC for inherited kidney disease care, you will receive:
By UPMC Editorial Staff. Last reviewed on 2025-04-24 by Faizan Syed, MD.
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