Treatment of Nephrotic Syndrome">Congenital Nephrotic Syndrome
Unfortunately, there is no specific treatment. In cases associated with infections, treatment of the infection is enough to also make the nephrotic syndrome disappear. The cases without clear cause sometimes just get better with time. However, in inherited cases, no specific treatment exists to repair the glomeruli. Some medications can help reduce the protein losses, but not enough to prevent the symptoms. Depending on the severity of the disease and the decisions you make with the doctors, there are two main treatment possibilities:

1. Intensive treatment
This involves a long-term admission to hospital. The aim is to allow the child to grow and develop and this is done initially by protein supplementation. The main protein lost in the urine is called albumin and this can be given as an infusion.

Typically, this treatment involves a small operation to place a central venous catheter (thin plastic tube) into one of the large veins in the arm or neck. This will make giving the daily infusion of albumin much easier and more comfortable for your child.

Additionally, medications can be given to increase the excretion of salt and water (diuretics). Obviously, because of the leak in the kidney, a substantial amount of the infused albumin is also lost in the urine, but nevertheless these infusions can stabilise the albumin levels in the blood, minimise the swelling (oedema) and provide building blocks for the child’s growth and development.

Unfortunately, this treatment can have complications. Having a plastic catheter enter the body gives potential for infections, and the loss of antibodies in the urine makes the child even more vulnerable to infection. As the bacteria can directly enter the bloodstream, these infections can be life threatening, but can usually be treated with antibiotics. Often removal of the catheter is needed to get rid of the infection totally.

Another complication is the potential for a blood clot to develop at the site of the infected catheter that blocks the vein. With recurrent infections it can become impossible to find open veins in which to place a new catheter.
To prevent this happening, your doctors may recommend removing the child’s kidney(s) as the only way to stop the protein losses.

Since congenital nephrotic syndrome typically leads to kidney failure eventually, this radical approach merely pre-empts the inevitable. However, it makes the child dependent on dialysis at an age when this is much more complicated than in older children. In milder cases, it may be enough to remove only one kidney. While protein losses will continue in the remaining kidney, the total loss is reduced and this may allow for adequate growth and discharge home. Yet in most cases, both kidneys are removed, typically when the baby has reached a size where dialysis is feasible, usually around 5kg.

Dialysis is then carried out until the child receives a kidney transplant, which is usually possible when he or she reaches 10kg in weight.

2. Conservative treatment
The intensive treatment described above is very demanding on everybody involved. It typically involves the child staying in hospital for the first six to 12 months of life and many further hospital admissions after that. This puts an enormous strain on the family, especially if one parent stays with the child in hospital and the other with the other children at home.

Some parents feel that such long-term hospitalisation with multiple interventions, operations and potential complications is not the right treatment for their child and would rather take their child home. This obviously increases the risk that the child may not grow and develop normally and, in fact, may die.

The child can still be checked in clinic regularly and receive medications to minimise the consequences of the congenital nephrotic syndrome but there is a very real risk of irreversible loss of growth and development and even death. However, the child would be in the natural environment of family and home. Yet, even if the child survives the initial time, kidney failure would typically occur after a few years as part of the natural course of congenital nephrotic syndrome. However, if active treatment such as transplantation were decided at that point, this would occur at an age where it has fewer complications compared to in infancy.