Congenital nephrotic syndrome is a disorder that is passed down through families in which a baby develops protein in the urine and swelling of the body.
Nephrotic syndrome - congenital
Congenital nephrotic syndrome is an autosomal recessive genetic disorder. This means that each parent must pass on a copy of the defective gene in order for the child to have the disease.
Although congenital means present from birth, with congenital nephrotic syndrome, symptoms of the disease occur in the first 3 months of life.
Congenital nephrotic syndrome is a very rare form of nephrotic syndrome.
Nephrotic syndrome is a group of symptoms that include:
Children with this disorder have an abnormal form of a protein called nephrin. The kidney's filters (glomeruli) need this protein to function normally.
Symptoms of nephrotic syndrome include:
Early and aggressive treatment is needed to control this disorder.
Treatment may involve:
Fluids may be limited to help control swelling.
The provider may recommend removing the kidneys to stop protein loss. This may be followed by dialysis or a kidney transplant.
Kazumoto Iijima is in Kobe, Japan. Iijima is rated as an Elite expert by MediFind in the treatment of Congenital Nephrotic Syndrome. They are also highly rated in 99 other conditions, according to our data. Their top areas of expertise are Nephrotic Syndrome, Alport Syndrome, Congenital Nephrotic Syndrome, and Lowe Syndrome.
Kandai Nozu is in Kobe, Japan. Nozu is rated as an Elite expert by MediFind in the treatment of Congenital Nephrotic Syndrome. They are also highly rated in 62 other conditions, according to our data. Their top areas of expertise are Alport Syndrome, Nephrotic Syndrome, Congenital Nephrotic Syndrome, and Magnesium Deficiency.
Olivia Boyer is in Paris, France. Boyer is rated as an Elite expert by MediFind in the treatment of Congenital Nephrotic Syndrome. She is also highly rated in 27 other conditions, according to our data. Her top areas of expertise are Nephrotic Syndrome, Congenital Nephrotic Syndrome, Diffuse Mesangial Sclerosis, and Galloway-Mowat Syndrome.
The disorder often leads to infection, malnutrition, and kidney failure. It can lead to death by age 5, and many children die within the first year. Congenital nephrotic syndrome may be controlled in some cases with early and aggressive treatment, including an early kidney transplant.
Complications of this condition include:
Contact your provider if your child has symptoms of congenital nephrotic syndrome.
Published Date : July 27, 2021
Published By : Walead Latif, MD, Nephrologist and Clinical Associate Professor, Rutgers Medical School, Newark, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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Schlöndorff J, Pollak MR. Inherited disorders of the glomerulus. In: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. Brenner and Rector's The Kidney. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 43.
Vogt BA, Springel T. The kidney and urinary tract of the neonate. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine: Diseases of the Fetus and Infant. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 93.