Nephrotic Syndrome


Nephrotic syndrome is a type of kidney disorder wherein, the body passes out the excess amount of proteins through urine, owing to the damage caused to the tiny clusters of blood vessels in the kidneys.

The symptoms include putting on excessive weight as a result of fluid retention, foamy urine and severe swelling around the region of the feet, ankles and eyes.

The main feature of nephrotic syndrome is that the kidneys leak a lot of protein. Normally, urine contains virtually no protein.

In nephrotic syndrome the urine contains large amounts of protein. What happens is that filters in the kidneys (the glomeruli) become 'leaky' and protein, instead of remaining in the blood, leaks out into the urine.

Protein in the urine is called proteinuria.

The other key features of nephrotic syndrome are:
A low level of protein in the blood as a result of protein loss in the urine. Although there is a drop in many of the proteins normally found in the bloodstream, the main protein that leaks from the blood into the urine is called albumin. A low blood level of albumin is main feature of nephrotic syndrome.
Fluid retention (oedema). This is a consequence of the low level of albumin in the bloodstream, and other complex factors not fully understood.

A high blood level of cholesterol and other fats (lipids).

This is due to the change in the balance of various protein levels in the blood due to the protein leakage.

Normal kidney function, at least initially. This means that the 'waste clearing' function of the kidneys is not affected - at least not at first. However, some of the conditions that cause nephrotic syndrome can progress to cause chronic kidney disease.

Other typical symptoms and signs of nephrotic syndrome are discussed later.

Common causes behind it
Minimal change disease (also known as nil disease) leads to the abnormal functioning of the kidneys.

Most common cause  almost 90% of children have this entity.
No body knows why the Minimal change disease nephrotic syndrome occurs.

Other reasons for nephrotic syndrome are infections , autoimmunity mediated and few medications

Nephrotic syndrome (NS) is the name given to a collection of kidney-related findings in your child’s body. These include:
proteinuria—high levels of protein in the urine (based on your child’s age and size) hypoalbuminemia—low levels of protein in your child’s blood, since it’s being passed out of his body in his urine edema—swelling.

This happens because the proteins in your child’s blood act as a sponge to keep fluid in the blood. With fewer proteins to do this, the fluid may leak out of the blood into your child’s tissues, causing them to swell, especially around the stomach area.

high cholesterol (blood fat) levels—low levels of protein in the blood stimulate the body to overproduce certain kinds of blood fats
While nephrotic syndrome is not a disease, it can be the first sign of disease that can damage the kidney's tiny blood-filtering unit (glomeruli) where urine is made.
Here’s what you need to know about nephrotic syndrome:
In the vast majority of children, NS is idiopathic, meaning that doctors don’t yet know what causes it.
Nephrotic syndrome always affects both kidneys.

It usually appears between the toddler and elementary school years, although it may appear later.
There are thought to be two forms of nephrotic syndrome, minimal change disease (MCD) and focal sclerosis (FSGS).

MCD is much more common in children, and likely to respond to therapy.

FSGS is a more aggressive disease, and may lead to kidney damage.
Most children with NS outgrow it by young adulthood.

Treatment Available
Pediatric Nephrotic syndrome is mainly treated by high dose steroids. So we classify Nephrotic syndrome as Steroid sensitive Nephrotic syndrome and Steroid Resistant Nephrotic syndrome. As long as the Nephrotic syndrome is steroid sensitive, kidneys usually do not fail and eventually the child recovers.

Other aspect of management of Nephrotic syndrome in kids is, that is a relapsing and remitting disease in more than 2/3 of patients, so parents must be making a Nephrotic diary as suggested by the doctor so that the relapse of disease is picked before the swelling appears.

Target of Nephrotic syndrome treatment is to make the child grow normally and using steroid sparing agents in kids with frequent relapses and Steroid dependence else they can develop high blood pressure, short stature , cataract, glaucoma and diabetes which all are irreversible.

Never start or stop the steroids on your own considering the side effect profile.

All vaccines including oral polio drops are not to be given in children receiving daily dose steroids.

Article Compiled By :
Dr. Amit Agarwal
Pediatric Nephorologist in Noida