What is Nephrotic Syndrome?

• A kidney condition with excessive loss of protein in the urine leading to low protein levels in the blood.
• Low protein levels in the blood will lead to swelling (oedema) around the eyes, abdomen and lower legs.
• It is not very common – it occurs in about 2-15 per 100,000 children each year.

What causes it?

• In children most of the time the cause is unknown but it is most likely due to an immune disorder.
• Rarely, the disease may be inherited ie genetic basis for nephrotic syndrome.
• It is not caused by specific infection,or the wrong food.
• In adults and older children it may be secondary to other conditions like diabetes, or Systemic Lupus Erythematosus.

Investigations at presentation

• Urine analysis.
• Quantification of the amount of protein lost in the urine per day either with 24 hour urine collection done commonly in older children and adults or a spot urine sample for young children who are not toilet trained. Blood test for the level of protein (albumin), kidney function test, and serum cholesterol. Serum cholesterol is high in children with nephrotic syndrome.
• Other tests will depend on your child’s associated features and will be ordered by your paediatrician.

Medication and Monitoring

• The specific medicine for nephrotic syndrome in childhood is a type of steroid known as prednisolone.
• This must be prescribed by a paediatrician. The dose is calculated based on the child’s body weight and height.
• The side effects of prednisolone are:
  • Increased appetite
  • Moon face and fat around the trunk of the body
  • increased activity
  • abdominal pain
• Less common side effects particularly if prednisolone is given at high doses over prolonged period of time include:
  • cataracts
  • stretch marks
  • muscle weakness
  • acne
  • short stature
  • increased blood pressure
• The majority of children with nephrotic syndrome will respond to the prednisolone. However, nephrotic syndrome in children is very prone to recurrence – otherwise known as relapse. The first sign of response to steroid treatment is the disappearance of protein in the urine. The first sign of relapse is the re-appearance of protein in the urine. Hence to monitor the response to treatment as well as relapse, the patient or carer will be taught how to check for protein in the urine at home every day using a dipstick.

• If the dipstick is negative for 3 days consecutively this means the child is responding to the steroid and is in remission. This usually occurs after about 7-10 days of steroid treatment and the oedema will disappear.
• If the proteinuria is there for 3 consecutive days the oedema will recur too and this is called relapse.
• Whenever the child has relapse; it is necessary to bring him or her back to the doctor who will prescribe another course of prednisolone.
• The doctor may also prescribe an antibiotic – penicillin whenever the child has a relapse to prevent a bacterial infection of the abdomen, which children with nephrotic syndrome are prone to have.

Other advice

• A normal protein, adequate calories and no added salt diet is recommended.
• Your child can go to school or nursery as usual. However while on steroid, if your child comes into contact with other children with measles or chicken pox, you should inform your doctor immediately.
• You should also consult the doctor before your child gets any immunization because certain vaccines should be avoided while your child is on steroid treatment. Pneumococcal vaccine currently not prescribed in our routine Ministry of Health Immunisation Schedule is also recommended for children with nephrotic syndrome.
• Continue home urine albumin monitoring by doing once daily dipstick testing of the first morning urine sample. Consult the doctor if the dipstick is >2+ for 3 consecutive days.
• It is also advisable to consult the doctor whenever the child has oedema regardless of the urine dipstick result.

Prognosis:

• Prognosis is good for children who respond to steroid treatment (steroid sensitive). Steroid sensitive neprhotic syndrome does not lead to kidney failure. The disease will eventually disappear in late childhood in majority.
• For those children who do not respond to 4 weeks of high dose steroid treatment, they are called steroid resistant. These children will need to undergo a kidney biopsy to evaluate further the type of renal disease and involvement. They should be referred to the nephrologist as they tend to progress to renal failure with time.