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
N/A
Scopus Q Değeri
Cilt
37
Sayı
2