Focal Segmental Glomerulosclerosis (FSGS)

What are the signs and symptoms of FSGS?

Early stages of FSGS may not have obvious symptoms. Sometimes, the first signs and symptoms of FSGS may include swelling of the abdomen, face and legs, as well as frothy urine.

What tests are used to diagnose FSGS?

FSGS is a type of nephrotic syndrome, which is diagnosed by blood and urine testing as well as a physical examination. Laboratory testing may show extra protein in the urine, low protein levels in the blood, as well as elevated levels of fat in the blood. Lab testing may also show abnormal kidney function. Other conditions and forms of nephrotic syndrome may cause similar laboratory findings, so your child’s doctor needs do a kidney biopsy to make a specific diagnosis of FSGS. We confirm a diagnosis if the kidney biopsy shows the kind of scarring in the filtering units of the kidneys that is associated with FSGS.

In some cases, we can use genetic testing to identify specific changes in common genes coding for the protein building blocks in the kidney. These tests can sometimes help doctors make the diagnosis and guide treatment and prognosis.

What to expect from a kidney biopsy

Our hospital can perform a kidney biopsy as an outpatient procedure. When patients are scheduled for a kidney biopsy, we ask them to come with an empty stomach. We’ll place an IV in order to give medication during the procedure. Depending on the child, they will be sedated or asleep in order to eliminate stress and pain as much as possible.

For the procedure, we insert a needle through the back and onto the surface of the kidney. The doctor will be able to find the right position for the needle using real-time ultrasound. Using the needle, we’ll remove a few small pieces of kidney tissue for examination. This part of the biopsy takes about one hour. We’ll monitor the patient for several hours after the biopsy to ensure there are no complications, after which the child can go home.

Our team analyzes the tissue sample under a microscope and performs several tests, which takes a few days. Once the biopsy results are available a few days after the procedure, we will discuss them with the patient and family and develop a personalized treatment plan together.

Why choose us for FSGS testing?

Children’s Hospital Colorado offers a complete array of child-specific diagnostic and treatment options for FSGS. Kidney biopsies are performed by a team of experienced pediatric specialists, with sedation, anesthesia and pain control tailored to each child’s individual needs. For the final diagnosis, our expert team have vast experience performing kidney biopsies.

How is FSGS treated?

Treatment for FSGS will differ depending on what your child’s medical team determines to be causing the condition. If it is thought that FSGS is caused by an attack on the kidneys by the immune system, your child’s doctor will prescribe medications to temper the immune system such as steroids or immunosuppressant medication. Depending on the degree of severity, immunosuppressive therapy may or may not work to reverse excessive urinary protein losses or preserve kidney function.

If your child’s FSGS is caused by exposure to another disease or medication, your child’s doctor will work to treat the primary disease thought to be causing the FSGS, or they will remove the harmful medication. Kidney function may be affected by FSGS; in this case, your child may need treatment for health problems caused by decreased kidney function. These health problems can include high blood pressure, anemia, bone damage and high acid levels in the body. In severe cases where kidney function is about to be lost completely, dialysis and kidney transplantation may be needed.

FSGS caused by genetic abnormalities is often irreversible, and doctors can only offer supportive treatment to contain the amount of urinary protein loss and body swelling.

Why choose us for FSGS treatment?

The treatment of FSGS in children is highly specialized and often complex. Details depend on individual findings such as the severity of nephrotic syndrome, degree of kidney function impairment and other aspects. The worst-case scenario is destruction of the kidneys by FSGS, at which point dialysis or kidney transplantation would be required. The Kidney Center at Children’s Colorado has the only pediatric dialysis and kidney transplant programs in the region. From testing and diagnosis to kidney transplantation, all these factors help make us the optimal choice for comprehensive pediatric FSGS care.

Helpful resources

The National Kidney Foundation (NKF) acts as a lifeline for all people affected by kidney disease including FSGS. As pioneers of scientific research and innovation, NKF focuses on the whole patient through the lens of kidney health and works to enhance lives through action, education and accelerating change.