Pediatric Nephrology

, Volume 20, Issue 9, pp 1340–1342

Congenital nephrotic syndrome responsive to angiotensin-converting enzyme inhibition

Brief Report

DOI: 10.1007/s00467-005-1918-5

Cite this article as:
Sreedharan, R. & Bockenhauer, D. Pediatr Nephrol (2005) 20: 1340. doi:10.1007/s00467-005-1918-5


Congenital nephrotic syndrome is a severe disorder caused by increased permeability of the glomerular capillary leading to massive proteinuria. Typically, this disorder presents in the first three months and is caused by inherited mutations in genes encoding structural proteins of the podocyte slit membrane and, as such, is usually irreversible. Medical management is often insufficient to stem the enormous losses of protein, and the patients require nephrectomies. Here, we present results for a patient with congenital nephrotic syndrome of unknown etiology which responded to treatment with an angiotensin-converting enzyme inhibitor alone. The patient’s proteinuria relapsed when the medication was stopped, but went into complete remission after restarting treatment. This remarkable response is discussed in the light of recent investigations into the effect of angiotensin II on podocyte integrity.


Congenital nephrotic syndrome ACE inhibitors Children Proteinuria Podocyte Slit diaphragm 

Copyright information

© IPNA 2005

Authors and Affiliations

  1. 1.Department of Pediatrics, Division of Nephrology, Yale University School of MedicineYale UniversityNew HavenUSA
  2. 2.Great Ormond Street HospitalLondonUK