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The treatment of minimal change nephrotic syndrome: Lessons learned from multicentre co-operative studies

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Abstract

Minimal change nephrotic syndrome (MCNS) is the most frequent type of nephrotic syndrome in childhood. Treatment aims to induce remission, prevent relapses and avoid side-effects. MCNS is responsive to immunosuppressive therapy with glucocorticosteroids, alkylating drugs and cyclosporine A. From results of multicentre controlled studies it became evident that the initial treatment with prednisone should be intensive, i.e. for 12 weeks, in order to reduce the risk of subsequent relapses. Treatment of relapses should be standardized in order to categorise the patient's disease for further treatment. In case “frequent relapsers” develop signs of steroid-toxicity, they should be treated with alkylating drugs: frequent relapsers without steroid dependency with cyclophosphamide or chlorambucil for 8 weeks, steroid-dependent cases with cyclosphosphamide for 12 weeks. If frequent relapses re-occur after cytotoxic drug therapy, treatment with cyclosporine A should be considered. Starting dose is 100–150 mg/m2 per day and needs to be adjusted to the whole blood trough level. Treatment with cyclosporine A must be strictly controlled in order to avoid chronic nephrotoxicity. Cyclosporine A does not cure MCNS. It is only effective as long as it is administered; therefore long-lasting treatment is necessary in most cases.

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Abbreviations

APN:

Arbeitsgemeinschaft für Pädiatrische Nephrologie

CyA:

cyclosporin A

ISKDC:

International Study of Kidney Disease in Children

MCNS:

minimal change nephrotic syndrome

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Authors and Affiliations

  1. Department of Paediatric Nephrology and Metabolic Disorders, Children's Hospital, Medical School Hannover, Postfach 610180, W-3000, Hannover 61, Federal Republic of Germany

    J. Brodehl

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Presented in part as the Nülo Halman lecture in Helsinki, 15 November 1989

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Brodehl, J. The treatment of minimal change nephrotic syndrome: Lessons learned from multicentre co-operative studies. Eur J Pediatr 150, 380–387 (1991). https://doi.org/10.1007/BF02093714

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