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What Is Congenital Nephrotic Syndrome?

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  • Written By: Meshell Powell
  • Edited By: Melissa Wiley
  • Last Modified Date: 30 November 2016
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Congenital nephrotic syndrome is a rare inherited disorder in which a baby develops protein in the urine and swelling of the body shortly after birth. Some additional symptoms of this disorder may include low birth weight, poor appetite, or low urine output. Treatment for congenital nephrotic syndrome varies according to specific symptoms and may include the use of medications, fluid restriction, or kidney transplant. Any questions or concerns about congenital nephrotic syndrome or the most appropriate treatment methods for an individual situation should be discussed with a doctor or other medical professional.

This kidney disorder is most commonly found among family members with a Finnish ancestry, although this is not always the case. Parents who have a family history of congenital nephrotic syndrome have an increased risk of having a baby who is born with this condition. Prenatal testing can sometimes reveal the presence of this disorder, although it is most frequently diagnosed after the birth of the child.

During a routine ultrasound, the placenta may be larger than normal if the baby has congenital nephrotic syndrome. Screening tests or genetic tests may be performed if this condition is suspected. In many cases, the baby is not officially diagnosed until additional observation and testing is performed after birth.

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A baby who is born with congenital nephrotic syndrome will often develop high blood pressure and generalized swelling soon after birth. A stethoscope will usually reveal abnormal heart and lung sounds. The infant may exhibit signs of malnutrition and have problems feeding or gaining weight. Protein and fat may be present in the urine with lower than normal levels of protein in the blood.

Treatment for congenital nephrotic syndrome depends on the severity of the symptoms and the overall health of the patient. If infection is present, antibiotics are given, usually through an IV. Medications known as non-steroidal inflammatory drugs, or NSAIDS, may be used in an effort to slow the buildup of protein in the kidneys. Blood pressure medications or diuretics may be prescribed as well.

If there is significant damage to the kidneys due to congenital nephrotic syndrome, dialysis or transplant may become necessary. Dialysis involves connecting the patient to a machine that filters waste materials from the blood before returning the blood to the body. Kidney transplantation is a surgical procedure in which one or both diseased kidneys are replaced with functional donor kidneys. Dialysis is often used until the child is old enough and healthy enough to undergo transplantation surgery.

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