Question

Congenital Nephrotic Syndrome - Know Signs Of It In Children!

Dr.Gaurav Sahai · Accepted Answer

As the name suggests, congenital nephrotic syndrome affects the kidneys of newborn babies. Though congenital means the disease must be present from birth, babies who develop nephrotic syndrome in the first three months are also said to suffer from this condition. This is a rare genetic disorder that is passed down from parents to their children. Boys are at a higher risk of suffering from this disorder than girls. The congenital nephrotic syndrome causes the kidneys to leak large amounts of protein into the baby s urine. This can lead to swelling of the body tissues and a weakened immune system.

Some of the common symptoms of this disease include

Low birth weight
Lack of appetite
Swelling of the body
Foamy or frothy urine
Lowered urine production
Cough
Pregnant women are usually screened for this condition before the baby is born. The doctor will be looking for a placenta that is larger than normal in ultrasound and high levels of alpha fetoprotein in amniotic fluid samples. If found; the doctor might ask for a genetic test to confirm the diagnosis.

Once the baby is born, he or she will show signs of fluid retention as the body begins to swell. Other signs your doctor will look out for are high blood pressure, signs of malnutrition and abnormal sounds from the heart and lungs. A urine analysis will also show high levels of protein in the urine through the overall protein levels in the body may be low. The doctor may also ask for a kidney biopsy in some cases to confirm the diagnosis.

Aggressive forms of treatment in the early stages of this disease can help control it. This may include antibiotics, blood pressure medication, ACE inhibitors and angiotensin receptor blockers to reduce protein leakage and diuretics to manage excess fluid build-up. Nonsteroidal anti-inflammatory drugs may also help reduce the amount of protein leaking into the urine. In addition, the doctor may advise you to limit fluid the baby s fluid intake to control the swelling. As a last resort, the kidneys may need to be removed to prevent protein loss. This will be followed by dialysis or a kidney transplant.

If left unchecked, this disorder can lead to a number of complications including acute kidney failure, blood clots, infections and diseases related to malnutrition. It can also lead to the death of a baby in the first year of catching the infection or before his or her fifth birthday.

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