eprintid: 10040459 rev_number: 60 eprint_status: archive userid: 608 dir: disk0/10/04/04/59 datestamp: 2017-12-21 11:58:20 lastmod: 2021-10-09 22:37:53 status_changed: 2017-12-21 11:58:20 type: article metadata_visibility: show creators_name: Deschênes, G creators_name: Vivarelli, M creators_name: Peruzzi, L creators_name: Alpay, H creators_name: Alvaro Madrid, A creators_name: Andersen, R creators_name: Bald, M creators_name: Benetti, E creators_name: Berard, E creators_name: Bockenhauer, D creators_name: Boyer, O creators_name: Brackman, D creators_name: Dossier, C creators_name: Ekinci, Z creators_name: Emma, F creators_name: Enneman, B creators_name: Espinosa-Roman, L creators_name: Fila, M creators_name: Ghio, L creators_name: Groothoff, JW creators_name: Guigonis, V creators_name: Jankauskiene, A creators_name: Kagan, M creators_name: Kovacevic, M creators_name: Kemper, MJ creators_name: Levtchenko, E creators_name: Maringhini, S creators_name: Mir, S creators_name: Mitsioni, A creators_name: Mizerska-Wasiak, M creators_name: Wasiak, K creators_name: Moczulska, A creators_name: Montini, G creators_name: Murer, L creators_name: Nuutinen, M creators_name: Obukhova, V creators_name: Oh, J creators_name: Ozkaya, O creators_name: Papalia, T creators_name: Peco Antic, A creators_name: Pecoraro, C creators_name: Pena-Carrion, A creators_name: Petrossian, E creators_name: Pietrement, C creators_name: Prikhodina, L creators_name: Querfeld, U creators_name: Rittig, S creators_name: Saleem, MA creators_name: Saraga, M creators_name: Savenkova, N creators_name: Sever, L creators_name: Tullus, K creators_name: Ulinski, T creators_name: Vande Walle, J creators_name: Vara, J creators_name: Webb, N creators_name: Weber, LT creators_name: Zurowska, A title: Variability of diagnostic criteria and treatment of idiopathic nephrotic syndrome across European countries ispublished: pub divisions: UCL divisions: B02 divisions: C10 divisions: D17 divisions: G93 keywords: Proteinuria, Prednisone, Steroid dependency, Steroid resistance, Rituximab, Guidelines note: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. 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. date: 2017-03-16 date_type: published official_url: https://doi.org/10.1007/s00431-017-2891-2 oa_status: green full_text_type: other language: eng primo: open primo_central: open_green article_type_text: Journal Article verified: verified_manual elements_id: 1509032 doi: 10.1007/s00431-017-2891-2 lyricists_name: Bockenhauer, Detlef lyricists_id: DBOCK32 actors_name: Stacey, Thomas actors_id: TSSTA20 actors_role: owner full_text_status: public publication: European Journal of Pediatrics volume: 176 number: 5 pagerange: 647-654 issn: 1432-1076 citation: Deschênes, G; Vivarelli, M; Peruzzi, L; Alpay, H; Alvaro Madrid, A; Andersen, R; Bald, M; ... Zurowska, A; + view all <#> Deschênes, G; Vivarelli, M; Peruzzi, L; Alpay, H; Alvaro Madrid, A; Andersen, R; Bald, M; Benetti, E; Berard, E; Bockenhauer, D; Boyer, O; Brackman, D; Dossier, C; Ekinci, Z; Emma, F; Enneman, B; Espinosa-Roman, L; Fila, M; Ghio, L; Groothoff, JW; Guigonis, V; Jankauskiene, A; Kagan, M; Kovacevic, M; Kemper, MJ; Levtchenko, E; Maringhini, S; Mir, S; Mitsioni, A; Mizerska-Wasiak, M; Wasiak, K; Moczulska, A; Montini, G; Murer, L; Nuutinen, M; Obukhova, V; Oh, J; Ozkaya, O; Papalia, T; Peco Antic, A; Pecoraro, C; Pena-Carrion, A; Petrossian, E; Pietrement, C; Prikhodina, L; Querfeld, U; Rittig, S; Saleem, MA; Saraga, M; Savenkova, N; Sever, L; Tullus, K; Ulinski, T; Vande Walle, J; Vara, J; Webb, N; Weber, LT; Zurowska, A; - view fewer <#> (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 <https://doi.org/10.1007/s00431-017-2891-2>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10040459/1/Bockenhauer_bodytext-3%20final%20form.pdf document_url: https://discovery.ucl.ac.uk/id/eprint/10040459/8/Bockenhauer_Table%20I.pdf document_url: https://discovery.ucl.ac.uk/id/eprint/10040459/13/Bockenhauer_Table%20II.pdf document_url: https://discovery.ucl.ac.uk/id/eprint/10040459/21/Bockenhauer_Table%20IV.pdf