• What is Nephrotic Syndrome?
  • Focal and Segmental
    Glomerulosclerosis (FSGS)
  • Minimal Change Disease (MCD)
  • Membranous Nephropathy (MN)
  • Glossary

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What is Nephrotic Syndrome?

Nephrotic Syndrome occurs when there is a malfunction in the kidney's filtering system (glomeruli) causing protein in the blood to leak into the urine (proteinuria). Nephrotic syndrome may be caused by many conditions, most commonly focal segmental glomerulosclerosis (FSGS), minimal change disease (MCD), and membranous nephropathy (MN).

The specific cause of nephrotic syndrome can only be determined with a kidney biopsy. Not all children require a kidney biopsy at initial diagnosis. In fact, many children are treated with steroids before a biopsy.

What are some of the symptoms of Nephrotic Syndrome?

Nephrotic syndrome causes excessive loss of protein in the urine which may lead to:

  • Swelling in parts of the body (edema) most visible in the head, hands, feet and abdomen
  • Low level of protein in the blood (hypoalbuminemia)
  • High blood cholesterol (hypercholesterolemia)
  • High blood pressure (hypertension)

What is Focal Segmental Glomerulosclerosis?

Focal Segmental Glomerulosclerosis (FSGS) is a disease that affects the kidney’s filtering system (glomeruli) causing scarring and loss of large amounts of protein in the urine. FSGS is a common cause of nephrotic syndrome.

 

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Who gets Focal Segmental Glomerulosclerosis?

Children and adults of all ages and races can get FSGS.


What causes FSGS?

There is no single cause of FSGS. When the cause is not known it is called idiopathic or primary FSGS. In some patients FSGS may be the result of a genetic or inherited disease, an infection such as parvovirus or HIV/AIDS, or other kidney diseases that lead to scarring.


What are some of the symptoms of FSGS?

FSGS causes excessive loss of protein in the urine which may lead to:

  • Swelling in parts of the body (edema) most visible in the head, hands, feet and abdomen
  • Low level of protein in the blood (hypoalbuminemia)
  • High blood cholesterol (hypercholestrolemia)
  • High blood pressure (hypertension)
  • Kidney failure

How is FSGS diagnosed?

With information obtained from blood tests, urine tests and a kidney biopsy, a physician can determine if a person has FSGS.


What is the treatment for FSGS?

Very few treatments are available for patients with FSGS. Most commonly, patients are treated with drugs called steroids and with blood pressure medicines. Prednisone and prednisolone are the most common steroid medicines used to treat FSGS. Some patients respond well to other medicines that suppress the immune system. To date, there is no treatment that is effective for every person with FSGS. Patients who do not respond to routine therapies are at risk for kidney failure.


Frequently Asked Questions
In some cases, FSGS does run in families, but most cases of FSGS are not inherited and cannot be passed down to children, and it is not contagious.

This varies from person to person. In general, there are several possible outcomes:

  • It may continue unchanged for many years, requiring only regular check-ups with blood tests.
  • Available therapies may control the disease.
  • It may go away on its own in rare cases.
  • The disease may worsen.
  • In some cases kidney failure develops and dialysis or transplantation is necessary.

What is Minimal Change Disease?

Minimal Change Disease is the most common cause of nephrotic syndrome in children. Minimal Change Disease is the diagnosis given when a patient has nephrotic syndrome and the kidney biopsy reveals little or no change to the structure of the kidney filters (glomeruli) or the surrounding kidney tissue. Tiny drops of a fatty substance called a lipid may be present, but no scarring has taken place within the kidney.

 

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Who gets Minimal Change Disease?

Children of all ages and even adults can get Minimal Change Disease, though it mostly affects young children under the age of 5. Boys are twice as likely to have it as girls.


What causes Minimal Change Disease?

The cause is not known but researchers are actively trying to learn more.


What are some of the symptoms of Minimal Change Disease?

The most common symptom is swelling around the eyes, face, abdomen and legs. A person with Minimal Change Disease may make less urine, gain weight and become swollen during active phases of the disease.


How is Minimal Change Disease diagnosed?

With information obtained from blood tests, urine tests and a kidney biopsy, a physician can determine if a person has Minimal Change Disease.


What is the treatment for Minimal Change Disease?

Usually the doctor will prescribe a drug called prednisone or prednisolone. This drug will help to stop the loss of protein in the urine and increase the amount of urine expelled from the body. Most patients will improve on this drug after several weeks of treatment.


Frequently Asked Questions
It is important to remember that most children and adults with this disease will not suffer long term kidney damage. If the disease does not recur for three years after the first spell, there is a good chance that it will not return. While some people will have only one attack of the disease, most children and adults will have at least two episodes. Even though there is no specific cure, most children will eventually "outgrow" Minimal Change Disease, with fewer and fewer episodes through the years until it no longer returns.
If prednisone does not work or if the side effects of the medications are too uncomfortable, the doctor may recommend another kind of medicine to lower the immune system function. Your doctor can discuss in detail the other treatment options. Diuretics may be prescribed to help the the body get rid of extra salt and water.

What is Membranous Nephropathy?

Membranous nephropathy (MN) is a kidney disease that is caused by an abnormal build up of immune proteins in the part of the kidney filter known as the glomerular “basement membrane.” This part of the glomerulus is the thin bit of tissue that helps separate the blood from the urine. In membranous nephropathy the basement membrane becomes thick and damaged, allowing large amounts of protein to leak out of the blood and into the urine.

 

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Who gets Membranous Nephropathy?

Anyone can have Membranous Nephropathy but it is more common in adults over the age of 40.


What causes Membranous Nephropathy?

The cause is not known but researchers are actively trying to learn more.


What are some of the symptoms of Membranous Nephropathy?

Symptoms occur gradually and can include swelling, weight gain, and high blood pressure.


How is Membranous Nephropathy diagnosed?

With information obtained from blood tests, urine tests and a kidney biopsy, a physician can determine if a patient has Membranous Nephropathy.


What is the treatment for Membranous Nephropathy?

Limiting salt in the diet and using medications to help the body get rid of extra salt and water is often helpful. Blood pressure medications such as ACE inhibitors are often used to try to decrease the loss of protein in the urine. Sometimes medicines are prescribed to decrease the chance of blood clots, which can be a problem in patients with nephrotic syndrome. For people with large amounts of protein in the urine, or who appear to be having long term damage to their kidneys, immunosuppression medications are frequently used. These include corticosteroids, cyclosporine, tacrolimus, and cyclophosphamide.


Frequently Asked Questions
Interestingly, MN sometimes goes away on its own. During the first 5 years after onset, this may happen in 1/3 of cases, even without any particular treatment. It is more likely if there is a mild or moderate amount of protein in the urine, and less likely if there is a large amount of protein in the urine. Another 1/3 of people seem to have a very stable course over many years, with no worsening and no improvement. And the remaining 1/3 have a slow decline in kidney function or worsening in the amount of protein in the urine, with up to 40% progressing to kidney failure after 15 years.

A

Abnormal: Differing from normal.

B

Biopsy (BY-op-see): A procedure in which a tiny piece of a body part, such as the kidney, is removed for examination under a microscope.

C

Creatinine (kree-AT-ih-nin): A waste product that is removed from blood by the kidneys. If kidney disease worsens, the level of creatinine in the blood increases.

Creatinine clearance: A test that measures how efficiently the kidneys remove creatinine from the blood. Low creatinine clearance indicates impaired kidney function.

G

Glomerulus (gloh-MEHR-yoo-lus): (Plural: glomeruli) A tiny set of looping blood vessels in the kidney where blood is filtered.

I

Immune (im-MYOON) system: The body’s system for protecting itself from viruses and bacteria or any “foreign” substances.

K

Kidney: Bean-shaped organ that filters wastes from the blood. The body has two kidneys located near the middle of the back. They create urine, which is delivered to the bladder through tubes called ureters.

Kidney Failure: Loss of kidney function.

N

Nephrologist (nef-RAHL-oh-jist): A doctor who treats patients with kidney problems or hypertension.

Nephron (NEF-rahn): A tiny part of the kidneys. Each kidney is made up of about 1 million nephrons, which are the working units of the kidneys, removing wastes and extra fluids from the blood.

Nephrotic (nef-RAH-tik) syndrome: A collection of symptoms that indicate kidney damage. Symptoms include high levels of protein in the urine, lack of protein in the blood, and high blood cholesterol.

P

Proteinuria (PRO-tee-NOOR-ee-uh): A condition in which the urine contains large amounts of protein, a sign that the kidneys are not functioning properly.

R

Renal (REE-nul): Of the kidneys. A renal disease is a disease of the kidneys. Renal failure means the kidneys have stopped working properly.

U

Urea (yoo-REE-uh): A waste product found in the blood. Urea is normally removed from the blood by the kidneys and then excreted in the urine. Urea accumulates in the body of people with kidney failure.

Uremia (yoo-REE-mee-uh): The illness associated with the buildup of urea in the blood because the kidneys are not working effectively. Symptoms include nausea, vomiting, loss of appetite, weakness, and mental confusion.

Urinalysis (yoor-in-AL-ih-sis): A test of a urine sample that can reveal many problems of the urinary system and other body systems. The sample may be observed for color, cloudiness, and concentration; signs of drug use; chemical composition, including sugar; the presence of protein, blood cells, or germs; or other signs of disease.