9/27/22, 1:37 PM Childhood Nephrotic Syndrome

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Introduction

Minimal Change Disease

Other Conditions That Involve the Childhood Nephrotic

Syndrome

Hope Through Research

For More Information

Introduction Childhood nephrotic syndrome can occur at any age but is most

common between the ages of 1-1/2 and 8 years. It seems to affect

boys more often than girls.

A child with the nephrotic syndrome has these signs:

High levels of protein in the urine

Low levels of protein in the blood

Swelling resulting from buildup of salt and water.

The nephrotic syndrome is not itself a disease. But it can be the

first sign of a disease that damages the tiny blood-filtering units

(glomeruli) in the kidneys, where urine is made.

The kidneys are two bean-shaped organs found in the lower back.

They are about the size of a fist. They clean the blood by filtering

out excess water and salt and waste products from food. Healthy

kidneys keep protein in the blood, which helps the blood soak up

water from tissues. But kidneys with damaged filters may let

protein leak into the urine. As a result, not enough protein is left in

the blood to soak up the water. The water then moves from the

blood into body tissues and causes swelling. You may see swelling

around your child's eyes, belly, and legs. Your child may urinate

less often than usual and may gain weight from the excess water.

To diagnose childhood nephrotic syndrome, the doctor may ask for

a urine sample to check for protein. The doctor will dip a strip of

chemically treated paper into the urine sample. Too much protein

in the urine will make the

paper change color. Or

the doctor may ask for a

24-hour collection of

urine for a more precise

measurement of the

protein and other

substances in the urine.
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A strip of chemically treated paper will

change color when dipped in urine with too

much protein.

The doctor may take a

blood sample to see how

well the kidneys are

removing wastes. Healthy

kidneys remove

creatinine and urea

nitrogen from the blood.

If the blood contains high

levels of these waste

products, some kidney

damage may have already

occurred. But most

children with the

nephrotic syndrome do

not have permanent

kidney damage.

In some cases, the doctor may want to examine a small piece of

the child's kidney under a microscope to see if substances there are

causing the syndrome. The procedure of collecting a small tissue

sample from the kidney is called a biopsy, and it is usually

performed with a long needle passed through the skin. The child

will be awake during the procedure and receive calming drugs and

a local painkiller at the site of the needle entry. General anesthesia

is used in the very rare cases where open surgery is required. The

child will stay overnight in the hospital to rest and allow the health

care team to ensure that no problems occur.

Minimal

Change Disease

The most common form of the nephrotic syndrome in children is

called minimal change disease. Doctors do not know what causes

it. The condition is called minimal change disease because

children with this form of the nephrotic syndrome have normal or

nearly normal biopsies. If your child is diagnosed with minimal

change disease, the doctor will probably prescribe prednisone,

which belongs to a class of drugs called corticosteroids.

Prednisone stops the movement of protein from the blood into the

urine, but it does have side effects that the doctor will explain.

Following the doctor's directions exactly is essential to protect

your child's health. The doctor may also prescribe another type of

drug called a diuretic, which reduces the swelling by helping the

child urinate.

When protein is no longer present in the urine, the doctor will

begin to reduce the dosage of prednisone. This process takes

several weeks. Some children never get sick again, but most do

develop swelling and protein in the urine again, usually following

a viral illness. However, as long as the child continues to respond

to prednisone and the urine becomes protein free, he or she has an

excellent long-term outlook without kidney damage.

Children who relapse frequently, or who seem to be dependent on

prednisone or have side effects from it, may be given a second

type of drug called a cytotoxic agent. The agents most frequently
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used are cyclophosphamide, chlorambucil, and cyclosporine. After

reducing protein in the urine with prednisone, the doctor may

prescribe the cytotoxic agent for a while. Treatment with

cyclophosphamide and chlorambucil usually lasts for 8 to 12

weeks, while treatment with cyclosporine frequently takes longer.

The good news is that most children "outgrow" this disease by

their late teens with no permanent damage to their kidneys.

Other

Conditions That

Involve the

Childhood

Nephrotic

Syndrome

In about 20 percent of children with the nephrotic syndrome, the

kidney biopsy reveals scarring or deposits in the glomeruli. The

two most common diseases that damage these tiny filtering units

are focal segmental glomerulosclerosis (FSGS) and

membranoproliferative glomerulonephritis (MPGN). Very rarely, a

child may be born with a condition that causes the nephrotic

syndrome (congenital nephropathy).

Since prednisone is less effective in treating these diseases than it

is in treating minimal change disease, the doctor may use

additional therapies, including cytotoxic agents. Recent experience

with a class of drugs called ACE inhibitors (a type of blood

pressure drug) indicates that these drugs help prevent protein from

leaking into the urine and keep the kidneys from being damaged in

children with the nephrotic syndrome.

Hope Through

Research

The National Institute of Diabetes and Digestive and Kidney

Diseases (NIDDK) conducts and supports research to help many

kinds of people with kidney disease, including children. NIDDK's

Division of Kidney, Urologic, and Hematologic Diseases

maintains the Pediatric Nephrology Program, which supports

research into the causes, treatment, and prevention of kidney

diseases in children, including minimal change disease, congenital

nephrotic syndrome, primary glomerular disease, and postinfection

glomerulonephritis.

For More

Information

American Kidney Fund

6110 Executive Boulevard

Suite 1010

Rockville, MD 20852

(800) 638-8299 or (301) 881-3052

Email: helpline@akfinc.org

Internet: www.akfinc.org

National Kidney Foundation

30 East 33rd Street

New York, NY 10016

(800) 622-9010 or (212) 889-2210

Email: info@kidney.org

Internet: www.kidney.org
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National Kidney and Urologic Diseases

Information Clearinghouse

3 Information Way

Bethesda, MD 20892-3580

Email: National Kidney and Urologic Diseases

Information Clearinghouse

The National Kidney and Urologic Diseases Information

Clearinghouse (NKUDIC) is a service of the National Institute of

Diabetes and Digestive and Kidney Diseases (NIDDK). NIDDK is

part of the National Institutes of Health under the U.S. Department

of Health and Human Services. Established in 1987, the

clearinghouse provides information about diseases of the kidneys

and urologic system to people with kidney and urologic disorders

and to their families, health care professionals, and the public.

NKUDIC answers inquiries; develops, reviews, and distributes

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organizations and Government agencies to coordinate resources

about kidney and urologic diseases.

Publications produced by the clearinghouse are carefully reviewed

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NIH Publication No. 00-4695

May 2000

e-text posted: June 2000