Nephrotic Syndrome in Children
What is nephrotic syndrome?
A child with nephrotic syndrome may have the following characteristics that result from changes that occur to the small, functional structures in the kidneys:
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 abdomen (ascites)
High cholesterol levels in the blood
Decrease in frequency of urination
Weight gain from excess fluid
What are the different types of nephrotic syndrome?
The type of nephrotic syndrome that is most common in children is called idiopathic nephrotic syndrome. It accounts for about 90% of children with nephrotic syndrome. Idiopathic means that a disease occurs with no known cause. The most common type is called minimal-change nephrotic syndrome (MCNS). Idiopathic nephrotic syndrome is more common in boys than girls.
With MCNS, the child usually has relapses of the illness, 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 present in the first week of life called congenital nephrotic syndrome. Congenital nephrotic syndrome 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. The chance for carrier parents to have a child with congenital nephrotic syndrome is 1 in 4, or 25%, 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:
Fatigue and malaise
Weight gain and facial swelling
Abdominal swelling or pain
Fluid accumulation in the body spaces (edema)
Pale fingernail beds
Ears 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 doctor for a diagnosis.
How is nephrotic syndrome diagnosed?
In addition to a thorough physical exam and complete medical history, your child's doctor may advise the following tests:
Urine tests to check for protein
Blood tests to check the levels of cholesterol and a special blood protein called albumin
Renal ultrasound, a noninvasive test in which a transducer is passed over the kidney producing sound waves which bounce off of the kidney, transmitting a picture of the organ on a video screen. The test is used to determine the size and shape of the kidney and to detect a mass, kidney stone, cyst, or other obstruction or abnormalities.
Renal biopsy, a procedure where a small sample of tissue is taken from the kidney through a needle. The tissue is sent for special testing to determine the specific disease.
What is the treatment for nephrotic syndrome?
Your child’s health care provider will figure out the best treatment based on:
How old your child is
His or her overall health and medical history
How sick he or she is
How well your child can handle specific medications, procedures, or therapies
How long the condition is expected to last
Your opinion or preference
During the initial episode of nephrotic syndrome, your child may need to be hospitalized. He or she may need to be monitored if the swelling is severe or if he or she has blood pressure or breathing problems.
Medications may be needed to treat initial symptoms and during relapses. These medications may include:
Immunosuppressive drug therapy
Diuretics (to reduce the swelling)
Intravenous (IV) albumin. A blood protein that can be given to replace the low levels
A special diet that restricts salt and has other specifications
The medications used to treat nephrotic syndrome weaken the immune system so 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 occur throughout childhood. However, once a child reaches puberty, the disease usually stays in remission (complete or partial absence of symptoms). It is uncommon for symptoms to return during adulthood. However, it is possible.