Nephrotic syndrome type 1

Overview

What is nephrotic syndrome type I?

Nephrotic syndrome type I is a rare inherited kidney disorder. It is also called congenital nephrotic syndrome of Finnish type because it has occurred most frequently in Finland. One of the main jobs of the kidneys is to filter excess water and waste products from the blood to be carried from the body in the urine. In nephrotic syndrome type I, the kidneys do not function properly; they allow protein to leak out. Usually, infants lose massive amounts of protein through the urine. The body responds to this abnormal loss of protein by retaining water. This leads to swelling because of fluid buildup in various parts of the body; this is called edema. The loss of protein can occur before birth and most infants are born prematurely. Infants also lose blood cells. They also lose antibodies, which are specialized proteins that help the body fight off infection. Because they lack the normal number of antibodies, infants and children are prone to developing repeated infections. They are also prone to developing blood clots in the body. The growth and development of infants and children is slowed and remains slowed despite treatment. They may need a feeding tube during infancy to ensure they get enough nutrients and calories. Nephrotic syndrome type I is a serious disorder and can be fatal in the first years of life. Kidney function usually continues to worsen until the kidneys fail completely. This is called end stage renal failure and will require a kidney transplant to fix. Even with treatment, most infants or children will require a kidney transplant.

References
  • Congenital nephrotic syndrome. Genetics and Home Reference website. Accessed June 22, 2017. https://ghr.nlm.nih.gov/condition/congenital-nephrotic-syndrome#
  • Niaudet P. Congenital and infantile nephrotic syndrome. UpToDate, Inc. website. Accessed May 24, 2017. http://www.uptodate.com/contents/congenital-and-infantile-nephrotic-syndrome
  • Hall G, Winn MP. Inherited Causes of Nephrotic Syndrome. In: Johnson RJ, Feehally J, Floeje J, eds. Comprehensive Clinical Nephrology. 5th ed. Philadelphia, PA: Elsevier Saunders;2015.
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Are there other names for nephrotic syndrome type I?

How common is nephrotic syndrome type I?

Are there other names for nephrotic syndrome type I?

There are several different types of nephrotic syndrome. Nephrotic syndrome type I has a few different names. The disorder has occurred most frequently in Finland and some names refer to the disorder as the Finnish type. Nephrotic syndrome type I is considered a congenital form; this means that symptoms develop by the first 3 months of an infant's life. Other names for nephrotic syndrome type I, include:

  • CNF
  • Congenital nephrotic syndrome, Finnish type
  • Congenital nephrotic syndrome of Finnish type
  • Congenital nephrotic syndrome type I
  • Finnish congenital nephrosis
  • Finnish-type congenital nephrosis
  • NPHS1
References
  • Hall G, Winn MP. Inherited Causes of Nephrotic Syndrome. In: Johnson RJ, Feehally J, Floeje J, eds. Comprehensive Clinical Nephrology. 5th ed. Philadelphia, PA: Elsevier Saunders;2015.
  • Niaudet P. Congenital and infantile nephrotic syndrome. UpToDate, Inc. website. Accessed May 24, 2017. http://www.uptodate.com/contents/congenital-and-infantile-nephrotic-syndrome
  • Holmberg C, Jalanko H. Congenital nephrotic syndrome and recurrence of proteinuria after renal transplantation. Pediatr Nephrol. 2014;29(12):2309-2317. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4212136/
  • Nephrotic Syndrome, Type I; NPHS1. The Online Mendelian Inheritance in Man (OMIM) website. Accessed June 22, 2017. http://www.omim.org/entry/256300
  • Niaudet P. Congenital nephrotic syndrome, Finnish type. OrphaNet website. Accessed June 20, 2017. http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=4048
How common is nephrotic syndrome type I?

Nephrotic syndrome type I is a rare disorder. Rare disorders often go unrecognized or misdiagnosed and how common they are in the world is often unclear or unknown. Nephrotic syndrome type I has occurred most often in Finland. Doctors estimate that there is about 1 new person diagnosed with the disorder for every 10,000 people in the population of Finland. Doctors don't know how many people have the disorder throughout the world, but it occurs much less often outside of Finland. The disorder also occurs with greater frequency among the Old Order Mennonites in Lancaster County, Pennsylvania. Nephrotic syndrome type I can affect people of any ethnic or racial background.

References
  • Niaudet P. Congenital and infantile nephrotic syndrome. UpToDate, Inc. website. Accessed May 24, 2017. http://www.uptodate.com/contents/congenital-and-infantile-nephrotic-syndrome
  • Nephrotic Syndrome, Type I; NPHS1. The Online Mendelian Inheritance in Man (OMIM) website. Accessed June 22, 2017. http://www.omim.org/entry/256300

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