European Journal of Pediatrics

, Volume 176, Issue 5, pp 647–654 | Cite as

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

  • Georges DeschênesEmail author
  • Marina Vivarelli
  • Licia Peruzzi
  • On behalf of the ESPN Working Group on Idiopathic Nephrotic Syndrome
Original Article


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


Proteinuria Prednisone Steroid dependency Steroid resistance Rituximab Guidelines 



Arbeitgemeinshaft für Pediatrische Nephrologie


European Society of Pediatric Nephrology


German Society for Pediatric Nephrology


Kidney Disease Improving Global Outcomes


Idiopathic Nephrotic Syndrome


International Study of Kidney Disease in Childhood


Intravenous methylprednisolone




Société de Néphrologie Pédiatrique


Working Group


*Working Group on Idiopathic Nephrotic Syndrome—list of coauthors

Marmara University Medical Faculty, Istanbul, Turkey

Hospital Vall d’Hebron, Barcelona, Spain

Aarhus University Hospital, Aarhus, Denmark

Olgahospital, Stuttgart, Germany

Department of Woman’s and Child’s Health, AOP-University of Padova, Italy

Hôpitaux pédiatriques universitaires de Nice, Nice, France

Great Ormond Street Hospital, London, UK

Hôpital Necker, APHP, Université Paris-Descartes, Paris, France

Haukeland University Hospital, Bergen, Norway

APHP Robert-Debré, Paris France

Kocaeli Çocuk Nefroloji ve Romatoloji Bilim Dalı, Umuttepe, Türkiye

Bambino Gesù Children’s Hospital and Research Institute, Roma, Italy

KU Leuven and University Hospitals Leuven, Leuven, Belgium,

Paediatric Nephrology, Hospital Universitario La Paz, Madrid, Spain

Hôpital Arnaud de Villeneuve, Montpellier, France

U.O.C. Nefrologia e Dialisi Pediatrica - Fondazione IRCCS Cà Granda, Milan, Italy

Emma Children’s Hospital, Amsterdam, the Netherlands

Limoges University Hospital, Limoges, France

Pediatric Center, Vilnius University, Vilnius, Lithuania

Orenburg Regional Children’s Hospital, Orenburg, Russia

Institute for Child and Youth Health Care in Novi Sad, Serbia

University Medical Center Hamburg-Eppendorf, Hamburg, Germany

KU Leuven and University Hospitals Leuven, Leuven, Belgium

Ospedale dei Bambini “G. Di Cristina” A.R.N.A.S., Palermo, Italy

Ege University Faculty of Medicine, İzmir, Turkey

P. & A. Kyriakou Children’s Hospital, Athens, Greece

Medical University of Warsaw, Warsaw, Poland

Jagiellonian University Medical College, Cracow, Poland

University of Milan, Ospedale Maggiore Policlinico, Milan, Italy

Department of Woman’s and Child’s Health, AOP-University of Padova, Padua, Italy.

Department of Pediatrics, University of Oulu, Oulu, Finland

Research Institute of Pediatrics and Children Surgery, Moscow, Russia

University Medical Center Hamburg-Eppendorf, Hamburg, Germany

Fatih Sultan Mehmet Training and Research Hospital, Istanbul, Turkey

Azienda Ospedaliera, Cosenza, Italy

BEL MEDIC General Hospital, Belgrade, Serbia

Nephrology and Dialysis, Children Hospital Santobono-Pausilipon, Naples, Italy

Hospital Universitario La Paz, Madrid, Spain

Russian National Research Medical University, Moscow, Russia

Centre Hospitalier Universitaire de Reims, France

Pirogov Russian National Research Medical University, Moscow, Russia

Charité Universitätsmedizin, Berlin, Germany

Aarhus University Hospital, Aarhus, Denmark

School of Clinical Sciences, University of Bristol, UK

School of Medicine, University of Split, Split, Croatia

Saint-Petersburg State Pediatric Medical University, St Petersburg, Russia

İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey

Great Ormond Street Hospital, London, UK

Armand-Trousseau Hospital, Paris, France

Safepedrug, University Hospital Gent, Gent, Belgium

Martin Hospital Universitario 12 de Octubre, Madrid, Spain

University of Manchester, Manchester Academic Health Science Centre, Royal Manchester Children’s Hospital, Manchester, UK

University Hospital of Cologne, Cologne, Germany

Medical University Gdansk, Poland

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Authors’ contributions

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.

Compliance with ethical standards


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.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  1. 1.Pediatric Nephrology UnitUniversité Sorbonne Paris CitéParisFrance
  2. 2.Division of Nephrology and DialysisBambino Gesù Children’s Hospital and Research InstituteRomeItaly
  3. 3.Nephrology Dialysis Transplantation Unit, Città della Salute e della ScienzaRegina Margherita Children’s HospitalTurinItaly

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