Nephrotic Syndrome

Most people have two kidneys, one on either side of the body just beneath the ribcage. Healthy kidneys filter the blood and allow small particles of waste products and water to be excreted as urine. Kidneys also play important role in the control of blood pressure, maintenance of bone health and formation of red blood cells.

What is nephrotic syndrome?
Nephrotic syndrome occurs when the kidneys leak large amounts of protein (especially albumin) into the urine. It is these proteins which is mainly responsible for holding water in the blood vessels, and when they are lost in urine, their level decreases in the blood which causes the water to come out of the blood vessels and cause swelling (edema).

What causes Nephrotic Syndrome?
In most cases, the exact cause of nephrotic syndrome is not known. There is no relationship with diet or socioeconomic status of the family. It is non-infectious and does not transfer to other family members

What are the symptoms?
The most common symptom is swelling (edema). It first appears on the face, especially around the eyes which is most prominent in the morning when the child gets up and decreases by the evening.

Other symptoms include: Frothy urine, weakness and tiredness, passing less urine than usual, recurrent infections, diarrhoea.

What is the treatment?
Prednisolone (steroid) is the drug of choice when the child is first diagnosed. Most children respond to this drug with disappearance of the protein in the urine and loss of swelling within 1-2 weeks (we call this REMISSION). Other drugs like diuretics, ACE inhibitors, etc may be required for symptomatic treatment.

Those who do not respond to steroids are given second line drugs like Cyclophosphamide, MMF, Levamisole and other immunomodulators as decided by your doctor according to the individual patient profile.

Duration of treatment: First episode is treated for 3 months and subsequent episodes are treated as decided by your physician.

What are the side effects of the treatment?
Common Side Effects of Steroids are:

Decreased resistance to infections such as cough and cold
Increase in appetite
Flushed, swollen cheeks and stretch marks on the skin
Rise in blood pressure
Behavioral problems e.g temper tantrums, or mood changes.
However most of the side effects are reversible and wean off once the steroid is stopped.

Long term supervision and course of nephrotic syndrome
In most cases the child with nephrotic syndrome becomes completely well with prednisolone treatment, and there are very few who require second line treatment which includes immunomodulators and other drugs.

The child may remain well for several months or longer. During this period, the child should be regarded as being normal, and should not be made to feel different from other children.

In majority of cases, however, nephrotic syndrome recurs. The recurrence is indicated by appearance of swelling around the eyes, which, if untreated, gradually increases to involve the face, feet, legs and abdomen, and in such conditions, doctor should be consulted immediately. Relapses are common in early childhood but decrease with age.

More than 90% of children with nephrotic syndrome go into adulthood with their kidneys functioning normally.

What are parents expected to do at home?
Starting and stopping of medicines should be strictly done as advised by the doctor.
Daily urine protein monitoring by urine dipsticks till negative for three days then atleast thrice a week during remission.
Keep a diary of all urine results so that your doctor can review your child’s progress in clinic. The dose of Prednisolone and any other medications and other comments should also be recorded.
In remission phase immediately report to the doctor under following conditions:
Swelling around the eyes
Decreased urine output
Urine protein 1+ or more for persistent 2-3 days
Any sign of infection like cough, cold, coryza.

Dietary advice for your child:
The child should be given a diet with enough proteins. High protein food items include milk and milk products, dal, chana, soyabean, eggs, meat and fish.

If the child is edematous, the doctor might advise salt restriction. Once the swelling disappears, the child can have his usual diet. It must be understood that salt has no role in the causation of nephrotic syndrome. No benefit can be expected by prolonged restriction of salt unless advised by the doctor for some other reason (e.g., if the blood pressure is high).

When is kidney biopsy required?
Very few children require kidney biopsy. Certain conditions in which children do not respond to daily treatment or have persistent gross hematuria, persistent hypertension, low complement level (C3 / C4) require kidney biopsy.

What immunizations are necessary?
Children who are on steroid medications and other immunosuprressives can become very unwell if exposed to CHICKENPOX or MEASLES, therefore they should be immunized for the same and also receive pneumococcal vaccine.