Congenital Nephrotic Syndrome: Case Report and Review of Literature

Küçük Resim Yok

Tarih

2012

Dergi Başlığı

Dergi ISSN

Cilt Başlığı

Yayıncı

Cukurova Univ, Fac Medicine

Erişim Hakkı

info:eu-repo/semantics/closedAccess

Özet

Congenital nephrotic syndrome (CNS) is a rare kidney disease which reveals itself after birth and characterized with severe proteinuria, hypoalbuminemia and edema. It is a genetic disorder that occurs with deterioration of glomerular filtration barrier especially as a result of mutation that develops in genes called nephrin and podocin. CNS could be as a result of perinatal infection as well as a part of a genetic syndrome. Immune suppressive treatment is ineffective in genetically originated CNS, however renal transplantation yields curative treatment. In many cases to prevent from life threatening edema, daily albumin infusion is needed. Additionally, high caloric diet, thyroxin and mineral supply are applied. Also prophylaxis of thrombolytic complications and opportunistic infection that could develop due to immune deficiency is needed. In this report we discussed a case with the review of literature by reporting a newborn which had CNS diagnosis as a result of persistent hypoalbuminemia and proteinuria without edema.

Açıklama

Anahtar Kelimeler

Congenital Nephrotic Syndrome, Hypoalbuminemia, Proteinuria, Neonatal

Kaynak

Cukurova Medical Journal

WoS Q Değeri

Scopus Q Değeri

Cilt

37

Sayı

2

Künye