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

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

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Abbreviations

APN:

Arbeitgemeinshaft für Pediatrische Nephrologie

ESPN:

European Society of Pediatric Nephrology

GPN:

German Society for Pediatric Nephrology

KDIGO:

Kidney Disease Improving Global Outcomes

INS:

Idiopathic Nephrotic Syndrome

ISKDC:

International Study of Kidney Disease in Childhood

IVMP:

Intravenous methylprednisolone

MP:

Methylprednisolone

SNP:

Société de Néphrologie Pédiatrique

WG:

Working Group

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*Working Group on Idiopathic Nephrotic Syndrome—list of coauthors

Marmara University Medical Faculty, Istanbul, Turkey harika.alpay@gmail.com

Hospital Vall d’Hebron, Barcelona, Spain amadrid@vhebron.net

Aarhus University Hospital, Aarhus, Denmark rfa@clin.au.dk

Olgahospital, Stuttgart, Germany m.bald@klinikum-stuttgart.de

Department of Woman’s and Child’s Health, AOP-University of Padova, Italy elisabene@libero.it

Hôpitaux pédiatriques universitaires de Nice, Nice, France berard.e@chu-nice.fr

Great Ormond Street Hospital, London, UK d.bockenhauer@ucl.ac.uk

Hôpital Necker, APHP, Université Paris-Descartes, Paris, France olivia.boyer@aphp.fr

Haukeland University Hospital, Bergen, Norway damien.marie.michel.brackman@helse-bergen.no

APHP Robert-Debré, Paris France claire.dossier@aphp.fr

Kocaeli Çocuk Nefroloji ve Romatoloji Bilim Dalı, Umuttepe, Türkiye zekinci@outlook.com

Bambino Gesù Children’s Hospital and Research Institute, Roma, Italy francesco.emma@opbg.net

KU Leuven and University Hospitals Leuven, Leuven, Belgium, benedicte.eneman@uzleuven.be

Paediatric Nephrology, Hospital Universitario La Paz, Madrid, Spain lespinosar@salud.madrid.org

Hôpital Arnaud de Villeneuve, Montpellier, France m-fila@chu-montpellier.fr

U.O.C. Nefrologia e Dialisi Pediatrica - Fondazione IRCCS Cà Granda, Milan, Italy luciana.ghio@policlinico.mi.it

Emma Children’s Hospital, Amsterdam, the Netherlands j.w.groothoff@amc.uva.nl

Limoges University Hospital, Limoges, France v.guigonis@gmail.com

Pediatric Center, Vilnius University, Vilnius, Lithuania augustina.jankauskiene@mf.vu.lt

Orenburg Regional Children’s Hospital, Orenburg, Russia mkaganorenburg@yahoo.com

Institute for Child and Youth Health Care in Novi Sad, Serbia dr.maja.kovacevic@gmail.com

University Medical Center Hamburg-Eppendorf, Hamburg, Germany kemper@uke.de

KU Leuven and University Hospitals Leuven, Leuven, Belgium elena.levtchenko@uzleuven.be

Ospedale dei Bambini “G. Di Cristina” A.R.N.A.S., Palermo, Italy s.maringhini@ospedalecivicopa.org

Ege University Faculty of Medicine, İzmir, Turkey sevgi.mir@ege.edu.tr

P. & A. Kyriakou Children’s Hospital, Athens, Greece amitsioni@icloud.com

Medical University of Warsaw, Warsaw, Poland wasiaczki@wp.pl

Jagiellonian University Medical College, Cracow, Poland amoczulska@cm-uj.krakow.pl

University of Milan, Ospedale Maggiore Policlinico, Milan, Italy giovanni.montini@unimi.it

Department of Woman’s and Child’s Health, AOP-University of Padova, Padua, Italy. luisa.murer@sanita.padova.it

Department of Pediatrics, University of Oulu, Oulu, Finland mnuutine@cc.oulu.fi

Research Institute of Pediatrics and Children Surgery, Moscow, Russia obuhova.v@mail.ru

University Medical Center Hamburg-Eppendorf, Hamburg, Germany j.oh@uke.de

Fatih Sultan Mehmet Training and Research Hospital, Istanbul, Turkey ozanozkaya@yahoo.com

Azienda Ospedaliera, Cosenza, Italy teresapapalia@alice.it

BEL MEDIC General Hospital, Belgrade, Serbia amira@udk.bg.ac.rs

Nephrology and Dialysis, Children Hospital Santobono-Pausilipon, Naples, Italy pecoraro@unina.it

Hospital Universitario La Paz, Madrid, Spain antonia.pena@salud.madrid.org

Russian National Research Medical University, Moscow, Russia ed3565@ya.ru

Centre Hospitalier Universitaire de Reims, France cpietrement@chu-reims.fr

Pirogov Russian National Research Medical University, Moscow, Russia lprikhodina@yahoo.com

Charité Universitätsmedizin, Berlin, Germany uwe.querfeld@charite.de

Aarhus University Hospital, Aarhus, Denmark rittig@clin.au.dk

School of Clinical Sciences, University of Bristol, UK m.saleem@bristol.ac.uk

School of Medicine, University of Split, Split, Croatia msaraga@kbsplit.hr

Saint-Petersburg State Pediatric Medical University, St Petersburg, Russia savenkova.n.spb@mail.ru

İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey severlale@hotmail.com

Great Ormond Street Hospital, London, UK Kjell.Tullus@gosh.nhs.uk

Armand-Trousseau Hospital, Paris, France tim.ulinski@aphp.fr

Safepedrug, University Hospital Gent, Gent, Belgium Johan.VandeWalle@uzgent.be

Martin Hospital Universitario 12 de Octubre, Madrid, Spain julia.vara@salud.madrid.org

University of Manchester, Manchester Academic Health Science Centre, Royal Manchester Children’s Hospital, Manchester, UK Nicholas.Webb@cmft.nhs.uk

University Hospital of Cologne, Cologne, Germany lutz.weber@uk-koeln.de

Medical University Gdansk, Poland azur@gumed.edu.pl

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Author information

Georges Deschênes and Licia Peruzzi conceived the questionnaires and wrote the paper.

Marina Vivarelli wrote the paper with LP and GD.

Coauthors of the ESPN Working Group on Idiopathic Nephrotic Syndrome answered the surveys.

Among coauthors, Olivia Boyer, Detlef Bockenhauer, Etienne Berard, Lutz Weber, and Lale Sever kindly reviewed the paper.

Correspondence to Georges Deschênes.

Ethics declarations

Funding

This study has been supported by a grant of 1000€ from the European Society of Pediatric Nephrology

Conflicts of interest

The authors declare that they have no conflict of interest and no financial relationship with the ESPN.

The coauthors in the list declare no conflict of interest and no financial relationship with the ESPN.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

It is clear that this paper obviously reports the routine clinical experience of medical teams on the treatment of human patients affected of a human disease, but this article does not contain any experimental studies with human participants or animals performed by any of the authors. It is also clear that several thousand human patients are the core of this clinical experience, but this paper does not report any specific information contained in patient files. Consequently, informed consent has not been obtained from individual participants.

Additional information

Revisions received: 13 February 2017; 24 February 2017

Communicated by Mario Bianchetti

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Deschênes, G., Vivarelli, M., Peruzzi, L. et al. Variability of diagnostic criteria and treatment of idiopathic nephrotic syndrome across European countries. Eur J Pediatr 176, 647–654 (2017). https://doi.org/10.1007/s00431-017-2891-2

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Keywords

  • Proteinuria
  • Prednisone
  • Steroid dependency
  • Steroid resistance
  • Rituximab
  • Guidelines