Article
Glomerular sclerosis in kidneys with congenital nephrotic syndrome (NPHS1).
Kidney international - 1 Oct 2006
Kuusniemi A-M, Merenmies J, Lahdenkari A-T, Holmberg C, Salmela K, Karikoski R, Rapola J, Jalanko H
Abstract excerpt
Congenital nephrotic syndrome of the Finnish type (NPHS1) is a rare genetic disease caused by mutations in the NPHS1 gene encoding a major podocyte slit-diaphragm protein, nephrin. Patients with NPHS1 have severe nephrotic syndrome from birth and develop renal fibrosis in early childhood. In this work, we studied the development of glomerular sclerosis in kidneys removed from 4- to 44-month-old NPHS1 patients....
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