RESUMO
The association of atypical hemolytic uremic syndrome [HUS] with idiopathic nephrotic syndrome has been exceptionally described and its physiopathology is not yet understood
Observation: We report the observation of a four year and two month old boy descended of consanguineous parents which have since the age of three years a steroid-resistant idiopathic nephrotic syndrome with minimal glomerular lesions treated favorably by cyclosporine A during 12 months but whose ulterior evolution was marked by relapse of the disease and after a few months by the installation of an atypical hemolytic uremic syndrome and afterwards a terminal renal insufficiency
Conclusion: Idiopathic nephrotic syndrome may constitute the terrain of an atypical HUS. Its physiopathology, complex and not yet clarified, does probably incriminate an infectious factor and/or a genetic predisposition that remains to confirm