Nephrotic Syndrome in Children
What is nephrotic syndrome?
Nephrotic syndrome occurs when there is too much protein in the kidneys. This happens because small structures in the kidneys (glomeruli) stop working properly. A child with nephrotic syndrome may have the following symptoms:
Very high levels of protein in the urine
Low levels of protein in the blood due to its loss in the urine
Tissue swelling all over the body (edema), especially in the belly (ascites)
High cholesterol levels in the blood
Urinating less often
Weight gain from excess fluid
What are the different types of nephrotic syndrome?
About 90% of children with this disease have idiopathic nephrotic syndrome. Idiopathic means that the disease occurs with no known cause.
The most common type is called minimal change nephrotic syndrome (MCNS). With MCNS, the child usually has times when symptoms get worse (relapses). But the disease can usually be managed and the outlook is good. In rare cases, a child may develop kidney failure that requires dialysis.
There is a rare nephrotic syndrome that occurs in the first week of life called congenital nephrotic syndrome. This is inherited by an autosomal recessive gene. This means that males and females are equally affected, and the child inherited one copy of the gene from each parent, who are carriers. Carrier parents have a 25% chance of having a child with this syndrome with each pregnancy. The outcome for this type of nephrotic syndrome is very poor.
What are the symptoms of nephrotic syndrome?
The following are the most common symptoms of nephrotic syndrome:
Extreme tiredness (fatigue) and a general feeling of discomfort (malaise)
Weight gain and facial swelling
Belly swelling or pain
Fluid buildup in the body (edema)
Pale fingernail beds
Ear cartilage may feel less firm
Food intolerances or allergies
The symptoms of nephrotic syndrome may look like other conditions and medical problems. Always check with your child's healthcare provider for a diagnosis.
How is nephrotic syndrome diagnosed?
Your child’s healthcare provider will do a full physical exam and take your child’s medical history. The provider may order tests such as:
Urine tests. These are done to check for protein.
Blood tests. These check your levels of cholesterol, a special blood protein called albumin, and other blood chemistries.
Kidney ultrasound. For this noninvasive test, a probe (transducer) is passed over the kidney. This makes sound waves that bounce off of the kidney to create a picture of the organ on a video screen. The test is used to determine the size and shape of the kidney. It can also find a mass, kidney stone, cyst, or other blockage (obstruction) or abnormalities.
Kidney biopsy. A small tissue sample is taken from your child’s kidney. The sample is sent to a lab for special testing to figure out the specific disease.
What is the treatment for nephrotic syndrome?
Your child's healthcare provider will figure out the best treatment plan for your child based on:
Your child’s age, overall health, and medical history
How sick your child is
How well your child handles certain medicines, treatments, or therapies
If your child’s condition is expected to get worse
The opinion of the healthcare providers involved in your child's care
Your opinion and preference
During the first episode of nephrotic syndrome, your child may need to be hospitalized. Your child may need to be monitored if the swelling is severe or if he or she has blood pressure or breathing problems.
Medicines may be needed to treat initial symptoms and during relapses. These medicines may include:
Immunosuppressive drug therapy
Diuretics (to reduce the swelling)
IV (intravenous) albumin, blood protein that can be given to replace the low levels
A special diet that restricts salt and has other specifications
The medicines used to treat nephrotic syndrome weaken the immune system. Because of this, your child should not receive live vaccines. If your child has been exposed to chickenpox and has not already had the vaccine, the vaccine may be advised.
What is the long-term outlook for nephrotic syndrome?
Relapses do happen throughout childhood. But once a child reaches the teen years, the disease usually stays in remission. This means that symptoms decrease or go away entirely. In most cases, symptoms don’t return during adulthood.