Nephrotic Syndrome in Children

Key points about nephrotic syndrome:

• Nephrotic syndrome is a condition characterized by the loss of large amounts of a protein called albumin from the body in the urine. This indicates that one or both kidneys are damaged.
• The most common type is called ‘minimal change disease’.
• Your child may need to stay in the hospital during the first episode.
• Medications may be used to treat the initial symptoms and relapses.
• Salt and fluids are restricted in the diet.
• Rarely, kidney failure may develop in some cases and dialysis may be needed.

What is nephrotic syndrome?

• Nephrotic syndrome is a condition characterized by the loss of large amounts of a protein called albumin from the body in the urine. This indicates that one or both kidneys are damaged.
• The kidneys contain many tufts of capillaries called glomeruli. These structures, called glomeruli, are responsible for filtration. Nephrotic syndrome occurs when the glomeruli cannot work normally.
• Rarely, kidney failure may develop in some cases and dialysis may be needed.

What causes nephrotic syndrome in children?

In most patients the cause is unknown, that is, it is idiopathic.

Sometimes it can appear in the first week of a child's life. This is called congenital nephrotic syndrome, meaning nephrotic syndrome present from birth. It is caused by a gene inherited in an autosomal recessive pattern. This means that boys and girls are affected equally. It occurs in a child who receives one gene from a carrier mother and one from a carrier father. In other words, there is a chance that one in every 4 children of carrier parents will have the disease. The outcome in congenital nephrotic syndrome is generally not very good. It usually has a severe course.

What are the symptoms of nephrotic syndrome in children?

Symptoms may vary slightly from child to child:

• Extreme tiredness and weakness
• Excessive irritability
• Decreased appetite
• Weight gain and facial swelling
• Abdominal swelling or pain
• Foamy urine
• Fluid buildup in the body (edema)
• Fluid buildup in the abdomen (ascites)
• Pale nail beds
• Dull hair
• Food intolerances or allergies

Since these symptoms can also be caused by other diseases and conditions, you need to see your doctor for a definitive diagnosis.

How is nephrotic syndrome diagnosed in children?

After your doctor takes a detailed history and performs a physical examination, some tests may be requested:

• Urine test
• Blood test
• Kidney ultrasound
• Kidney biopsy

How is nephrotic syndrome treated in children?

Treatment depends on your child's symptoms, age, general health and the severity of the disease. In the initial stage of nephrotic syndrome, your child may need to stay in the hospital. Close monitoring of blood pressure, breathing problems and edema may be needed.

Medications may be used to treat the initial symptoms and relapses. These medications include:

• Corticosteroids
• Immunosuppressants
• Diuretics to reduce excess fluid buildup (ascites, edema, etc.)
• Intravenous albumin to raise low albumin levels in the blood
• A strict diet restricted in salt and fluids

The medications used to treat nephrotic syndrome can weaken the immune system. For this reason, your child should not be given live vaccines.

Talk to your doctor in detail about how the medications used in treatment work and their side effects.

Children with nephrotic syndrome may have trouble regulating their body's water balance. This can cause excessive fluid retention and, as a result, edema. For this reason, a diet restricted in salt and fluids should be followed. This makes it easier for the body to regulate water.

What are the possible complications of nephrotic syndrome?

Rarely, kidney failure may develop and dialysis may be needed.

How can I help my child with nephrotic syndrome?

You can help keep your child healthy in the following ways:

Keep your child away from people with respiratory illnesses such as colds and flu, because even a simple viral respiratory illness can cause a relapse, that is, a return of the disease.

• Measure the amount of protein in your child's urine every day. This way, you can catch a relapse early.
• Postpone your child's routine vaccinations until 3 months after steroid treatment and until your child is in remission. Talk to your pediatrician in detail about vaccinations and plan their timing.

Nephrotic syndrome may follow a good course, or it may worsen despite treatment. Patients go through periods of recovery and relapse. Family support is very important for a child's recovery. Usually, by the time children reach adolescence, the disease goes into remission. This means that the disease has become less active or has ended. In most patients, the disease does not return in adulthood.

When should I contact the hospital?

Be sure to contact your doctor if any of the following occur:

• Symptoms do not improve or get progressively worse
• New symptoms appear
• There is nausea, vomiting or abdominal pain
• There is unexpected weight gain
• There is edema in the legs, joints or around the eyes
• The urine becomes darker
• Urine output decreases or there is no urine at all

Be sure to call 112 if any of the following occur:

• Severe weakness, dizziness, headache, fainting, drowsiness or confusion
• Chest pain or shortness of breath