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Five-year outcome of children with idiopathic nephrotic syndrome: the NEPHROVIR population-based cohort study

  • Claire DossierEmail author
  • Jean-Daniel Delbet
  • Olivia Boyer
  • Patrick Daoud
  • Bettina Mesples
  • Beatrice Pellegrino
  • Helène See
  • Gregoire Benoist
  • Anne Chace
  • Anis Larakeb
  • Julien Hogan
  • Georges Deschênes
Original Article

Abstract

Background

The optimal therapeutic regimen for children at onset of idiopathic nephrotic syndrome (INS) is still under debate. A better knowledge of the disease’s course is necessary to design more appropriate and/or personalized treatment protocols.

Methods

We report the 5-year outcome of patients included from December 2007 to May 2010 in the prospective multicentric and multiethnic population-based NEPHROVIR study. Patients were treated at onset according to the French steroid protocol (3990 mg/m2, 18 weeks). Data were collected at 5 years or last follow-up.

Results

Out of the 188 children with nephrotic syndrome (121 boys, 67 girls; median age 4.1 years), 174 (93%) were steroid-sensitive. Six percent of steroid-sensitive patients required intravenous steroid pulses to get into remission. Relapse-free rate for steroid-sensitive patients was 21% (36/174) at last follow-up (median 72 months). A first relapse occurred in138 steroid sensitive patients (79%) with a median time of 8.3 months (IQ 3.4–11.3). Out of the 138 relapsers, 43 were frequent relapsers. Age at onset below 4 years was the only predictive factor of relapse, while gender, ethnicity, and delay to first remission were not. At 96 months of follow-up, 83% of frequent relapsers were still under steroids and/or immunosuppressive drugs.

Conclusions

The treatment of the first flare deserves major improvements in order to reduce the prevalence of relapsers and the subsequent long-lasting exposure to steroids and immunosuppression.

Keywords

Nephrotic syndrome Children Steroid-sensitive Frequent relapser Immunosuppressive drug Methylprednisolone pulse Nephrovir 

Notes

Acknowledgments

We acknowledge all pediatricians from participating centers for their help and contribution to this study: Dr. Azib (Department of Pediatrics, Centre Hospitalier René-Dubos, Pontoise), Dr. Mahdi (Department of Pediatrics, Centre Hospitalier Intercommunal, Créteil), Dr. Khelfaoui (Department of Pediatrics, Centre Hospitalier Sainte Camille, Bry sur Marne), Dr. Nathanson (Department of Pediatrics, Centre Hospitalier de Versailles), Dr. May (Department of Pediatrics, Centre Hospitalier d’Evry), Dr. Breant (Department of Pediatrics, Centre Hospitalier de Melun), Dr. Ait Boudaoud (Department of Pediatrics, Centre Hospitalier de Villeneuve Saint Georges), Dr. Elias (Department of Pediatrics, Centre Hospitalier de Gonnesse), Dr. Laoucine (Department of Pediatrics, Centre Hospitalier d’Aulnay), Dr. Gouraud (Department of Pediatrics, Centre Hospitalier de Meaux), Dr. Furioli (Department of Pediatrics, Centre Hospitalier de Mantes la Jolie), Dr. Mahfoufi (Department of Pediatrics, Centre Hospitalier deMontfermeil), Dr. Seaume (Department of Pediatrics, Centre Hospitalier de Longjumeau), Dr. Blanc (Department of Pediatrics, Centre Hospitalier de Poissy-Saint Germain), Dr. Colin-Gorski, Dr. Bensaid (Department of Pediatrics, Centre Hospitalier d’Argenteuil), Dr. de Pontual, Dr. Galerne (Department of Pediatrics, Jean Verdier Hospital), Dr. Saunier (Department of Pediatrics, Centre Hospitalier de Fontainebleau), Dr. Zenkhri (Department of Pediatrics, Hopital Bicetre), Dr. de Gennes, Dr. Cheminee (Department of Pediatrics, Centre Hospitalier d’Orsay), Dr. Muller (Department of Pediatrics, Centre Hospitalier de Rambouillet), Dr. Amira, Dr. Escoda (Department of Pediatrics, Centre Hospitalier de Saint Denis), Dr. Gajdos (Department of Pediatrics, Antoine-Beclère Hospital, APHP), Dr. Akil (Department of Pediatrics, Centre Hospitalier de Neuilly), Dr. Monier (Department of Pediatrics, Centre Hospitalier de Montmorency), Dr. MBuila (Department of Pediatrics, Centre Hospitalier des Portes de l’Oise), Dr. Abdelhadi (Department of Pediatrics, Centre Hospitalier de Provins), Dr. Branca (Department of Pediatrics, Centre Hospitalier de Montereau), Dr. Tahiri (Department of Pediatrics, Centre Hospitalier de Dourdan), Dr. Chalvon, Dr. Sfez (Department of Pediatrics, Centre Hospitalier de Lagny), Dr. Nacer (Department of Pediatrics, Centre Hospitalier de Coulommiers), Dr. Saf (Department of Pediatrics, Centre Hospitalier d’Arpajon), and Dr. Gaagliardone (Department of Pediatrics, Hopital Franco-Britannique).

Compliance with ethical standards

The study was reviewed and accepted by local Ethics Committee. Informed consent was obtained from all participants included in the study.

Conflict of interest

The authors declare that they have no competing interests.

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Copyright information

© IPNA 2018

Authors and Affiliations

  • Claire Dossier
    • 1
    Email author return OK on get
  • Jean-Daniel Delbet
    • 1
  • Olivia Boyer
    • 2
  • Patrick Daoud
    • 3
  • Bettina Mesples
    • 4
  • Beatrice Pellegrino
    • 5
  • Helène See
    • 6
  • Gregoire Benoist
    • 7
  • Anne Chace
    • 8
  • Anis Larakeb
    • 9
  • Julien Hogan
    • 10
  • Georges Deschênes
    • 10
  1. 1.Department of Pediatric NephrologyHôpital Armand-Trousseau, APHPParisFrance
  2. 2.Department of Pediatric NephrologyHôpital Necker-Enfants-Malades, APHPParisFrance
  3. 3.Department of Pediatrics and NeonatologyCentre Hospitalier Intercommunal André-GrégoireMontreuilFrance
  4. 4.Department of PediatricsHôpital Louis-Mourier, APHPColombesFrance
  5. 5.Department of PediatricsCH Francois-QuesnayMantes-La-JolieFrance
  6. 6.Department of PediatricsCentre Hospitalier Intercommunal Robert-BallangerAulnay Sous BoisFrance
  7. 7.Department of PediatricsHôpital Ambroise-Paré, APHPBoulonge-BillancourtFrance
  8. 8.Department of PediatricsCentre Hospitalier Intercommunal de Villeneuve Saint-GeorgesVilleneuve Saint-GeorgesFrance
  9. 9.Department of PediatricsCentre Hospitalier de MeauxMeauxFrance
  10. 10.Department of Pediatric NephrologyHôpital Robert-Debré, APHPParisFrance

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