Definition

What is Childhood nephrotic syndrome?

Nephrotic syndrome is a condition that causes the kidneys to leak large amounts of protein into the urine. This can lead to a range of problems, including swelling of body tissues and a greater chance of catching infections.

Other characteristics of this disease include:

Nephrotic syndrome isn’t itself a disease. Diseases that damage blood vessels in your kidneys cause this condition.

How common is Childhood nephrotic syndrome?

Around 1 in every 50,000 children are diagnosed with the condition each year. Although nephrotic syndrome can affect people of any age, it’s usually first diagnosed in children aged between 2 and 5 years old. It affects more boys than girls. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Childhood nephrotic syndrome?

The common symptoms of Childhood nephrotic syndrome are:

  • Swelling (edema) in your ankles and feet, and around your eyes
  • Foamy urine
  • Weight gain from fluid buildup in your body
  • Fatigue
  • Appetite loss
  • High cholesterol and triglyceride levels

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you or your loved one has any signs or symptoms listed above or you have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Childhood nephrotic syndrome?

Your kidneys are filled with tiny blood vessels, called glomeruli. As your blood moves through these vessels, extra water and waste products are filtered into the urine. Protein and other substances that your body needs stay in your bloodstream.

Nephrotic syndrome happens when the glomeruli are damaged and can’t properly filter your blood. Damage to these blood vessels allows protein to leak into your urine.

Albumin is one of the proteins lost in the urine. Albumin helps pull extra fluid from your body into your kidneys. This fluid is then removed in the urine. Without albumin, your body holds onto the extra fluid. This causes swelling (edema) in your legs, feet, ankles, and face.

Some conditions that cause nephrotic syndrome only affect the kidneys. These are called primary causes of nephrotic syndrome. These conditions include:

Focal segmental glomerulosclerosis. A condition in which the glomeruli become scarred from disease, a genetic defect, or an unknown cause.

Membranous nephropathy. In this disease, the membranes in the glomeruli thicken. The cause of the thickening isn’t known, but it may occur along with lupus, hepatitis B, malaria, or cancer.

Minimal change disease. In this condition, the kidney tissue looks normal under a microscope, but for some unknown reason, it doesn’t filter properly.

Renal vein thrombosis. In this disorder, a blood clot blocks a vein that drains blood out of the kidney.

Other diseases that cause nephrotic syndrome affect the whole body. These are called secondary causes of nephrotic syndrome. These conditions include:

Diabetes. In this disease, uncontrolled blood sugar can damage blood vessels all over your body, including in your kidneys.

Lupus. Lupus is an autoimmune disease that causes inflammation in the joints, kidneys, and other organs.

Amyloidosis. This rare disease is caused by a buildup of the protein amyloid in your organs. Amyloid can build up in, and damage your kidneys.

Some medications, including antibiotics and nonsteroidal anti-inflammatory drugs (NSAIDs), have also been linked to nephrotic syndrome.

Risk factors

What increases my risk for Childhood nephrotic syndrome?

The condition tends to be more common in families with a history of allergies or those of an Asian background, although it’s unclear why.

Please consult with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Childhood nephrotic syndrome diagnosed?

Nephrotic syndrome can usually be diagnosed after dipping a dipstick into a urine sample. If there are large amounts of protein in a person’s urine, there will be a colour change on the stick.

A blood test showing a low level of a protein called albumin will confirm the diagnosis.

In some cases, when the initial treatment doesn’t work, your child may need a kidney biopsy. This is when a very small sample of kidney tissue is removed using a needle so it can be studied under a microscope.

How is Childhood nephrotic syndrome treated?

The main treatment for nephrotic syndrome is steroids, but additional treatments may also be used if a child develops significant side effects.

Most children have relapses until their late teens and need to take steroids when these occur.

Your child may be referred to a childhood kidney specialist (paediatric nephrologist) for tests and specialist treatment.

Steroids

Children diagnosed with nephrotic syndrome for the first time are normally prescribed at least a 4-week course of the steroid medicine prednisolone, followed by a smaller dose every other day for 4 more weeks.

This stops protein leaking from your child’s kidneys into their urine.

When prednisolone is prescribed for short periods, there’s usually no serious or long-lasting side effects, although some children may experience:

  • Increased appetite
  • Weight gain
  • Red cheeks
  • Mood changes

Most children respond well to treatment with prednisolone, with the protein often disappearing from their urine and the swelling going down within a few weeks. This period is known as remission.

Diuretics

Diuretics, or “water tablets”, may also be given to help reduce the fluid build-up. They work by increasing the amount of urine produced.

Penicillin

Penicillin is an antibiotic, and may be prescribed during relapses to reduce the chances of an infection.

Dietary changes

You may be advised to reduce the amount of salt in your child’s diet to prevent further water retention and oedema. This means avoiding processed foods and not adding salt to what you eat.

Get tips on how to cut down on salt.

Vaccinations

Children with nephrotic syndrome are advised to have the pneumococcal vaccine. Some children may also be recommended the varicella (chickenpox) vaccination between relapses.

Live vaccines, such as MMR, chickenpox and BCG, shouldn’t be given while your child is taking medication to control their symptoms.

Additional medication

Other medications may be used alongside or in place of steroids if your child’s remission can’t be maintained with steroids or they experience significant side effects.

Additional medications that may be used include:

  • Levamisole
  • Cyclophosphamide
  • Ciclosporin
  • Tacrolimus
  • Mycophenolate
  • Rituximab

Albumin infusions

Most of the protein lost in nephrotic syndrome is a type called albumin. If your child’s symptoms are severe, they may be admitted to hospital to receive albumin infusions.

Albumin is slowly added to the blood over a few hours through a thin plastic tube called a cannula, which is inserted into one of the veins in their arm.

Children who are born with nephrotic syndrome (congenital nephrotic syndrome), may eventually need a kidney transplant.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Childhood nephrotic syndrome?

If your child has been diagnosed with nephrotic syndrome, you’ll need to monitor their condition on a daily basis to check for signs of relapses.

You’ll need to use a dipstick to test your child’s urine for protein the first time they urinate each day.

The results of a dipstick test are recorded as either:

  • Negative – 0mg of proteinuria per decilitre of urine (mg/dl)
  • Trace – 15-30mg/dl
  • 1+ – 30-100mg/dl
  • 2+ – 100-300mg/dl
  • 3+ – 300/1,000mg/dl
  • 4+ – over 1,000mg/dl

The result for each day needs to be written down in a diary for your doctor or specialist nurse to review during your outpatient appointments.

You should also note down the dose of any medication they’re taking and any other comments, such as whether your child is feeling unwell.

If the dipstick shows 3+ or more of protein in the urine for 3 days in a row, this means your child is having a relapse.

If this happens, you either need to follow the advice given about starting steroids or contact your doctor.

You should seek immediate medical advice if:

  • Your child has come into contact with someone who has chickenpox or measles and your doctor has told you that your child is not immune to these illnesses
  • Your child is unwell or has a fever
  • Your child has diarrhea and is vomiting if you have any questions, please consult with your doctor to better understand the best solution for you

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: April 11, 2018 | Last Modified: April 11, 2018

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