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Médecine thérapeutique / Pédiatrie

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Steroid-resistant forms of idiopathic nephrotic syndrome Volume 18, issue 4, October-November-December 2015

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Authors
1 AP-HP
Hôpital universitaire Robert-Debré
Service de néphrologie pédiatrique
48, boulevard Sérurier
75935 Paris cedex 19
France
2 AP-HP
Hôpital universitaire Robert-Debré
Service d’anatomie et de cytologie pathologique
48, boulevard Sérurier
75935 Paris cedex 19
France
* Tirés à part

Steroid resistance manifests in hereditary diseases of the glomerular wall and steroid-resistant forms of idiopathic nephrotic syndrome which are of immune in origin. The assessment of a child with steroid-resistant nephrotic syndrome should include a genetic analysis. For patients with steroid-resistant idiopathic nephritic syndrome, focal segmental glomerulosclerosis is present in half the initial renal biopsies, but this has no formal prognostic value. The other half have minimal glomerular lesions. First-line treatment involves prolonged prednisone administration combined with a calcineurin antagonist, typically cyclosporine, and more recently tacrolimus. Response to treatment is the only prognostic factor. All patients with no response to first-line immunosuppressive therapy progress to end-stage renal disease (ESRD) within months. Patients with ESRD are treated with renal replacement therapy at least six months after the bilateral nephrectomy to prevent post-transplant complications associated with chronic nephrotic syndrome, particularly the risk of early renal graft thrombosis. The prognosis following renal transplantation is largely determined by the recurrence of nephrotic syndrome associated with the renal graft.