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Variability of diagnostic criteria and treatment of idiopathic nephrotic syndrome across European countries

Deschênes, G; Vivarelli, M; Peruzzi, L; Alpay, H; Alvaro Madrid, A; Andersen, R; Bald, M; ... Zurowska, A; + view all (2017) Variability of diagnostic criteria and treatment of idiopathic nephrotic syndrome across European countries. European Journal of Pediatrics , 176 (5) pp. 647-654. 10.1007/s00431-017-2891-2. Green open access

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Abstract

The aim of the surveys conducted by the Idiopathic Nephrotic Syndrome Working Group of the ESPN was to study the possible variability of treatment in Europe at different stages of the disease by means of questionnaires sent to members of the Working Group. Four surveys have been completed: treatment of the first flare, treatment of the first relapse and the issue of steroid dependency, use of rituximab, and the management of steroid-resistant patients. A uniform treatment of the first flare was applied in only three countries, and ten additional centers have adopted one of the three main protocols. Reported treatment of the first relapse was relatively uniform, whereas the use of additional immunosuppressants in steroid dependency was widely variable. Rituximab had already been used in hundreds of patients, although the formal evidence of efficiency in steroid dependency was relatively recent at the time of the survey. The definition of steroid resistance was variable in the European centers, but strikingly, the first-line treatment was uniform throughout the centers and included the combination of prednisone plus calcineurin antagonists. Conclusion: The variability in the approach of idiopathic nephrotic syndrome is unexpectedly large and affects treatment of the first flare, strategies in the case of steroid dependency, as well as the definitions of steroid resistance.What is Known:• Steroids and immunosuppressants are the universal treatment of idiopathic nephrotic syndrome.What is New:• The variability of treatments and strategy of treatment in European centers of pediatric nephrology.

Type: Article
Title: Variability of diagnostic criteria and treatment of idiopathic nephrotic syndrome across European countries
Open access status: An open access version is available from UCL Discovery
DOI: 10.1007/s00431-017-2891-2
Publisher version: https://doi.org/10.1007/s00431-017-2891-2
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Proteinuria, Prednisone, Steroid dependency, Steroid resistance, Rituximab, Guidelines
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Renal Medicine
URI: https://discovery.ucl.ac.uk/id/eprint/10040459
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