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Long-term health-related quality of life outcomes of adults with pediatric onset of frequently relapsing or steroid-dependent nephrotic syndrome

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Abstract

Background

Long-term psychosocial outcomes and health-related quality of life (HRQOL) in adults with pediatric onset of frequently relapsing or steroid-dependent idiopathic nephrotic syndrome (FRNS or SDNS) remain to be determined.

Methods

In this prospective cohort study, 59 adults with pediatric onset of FRNS/SDNS and persistent active glomerular disease in adulthood completed the GEDEPAC-2 questionnaire exploring 11 well-being domains. Data were compared to the French general population (FGP) with standardized incidence ratio ([SIR]; adjusted for period, age, gender). Regression models were performed to identify predictive factors of psychosocial well-being.

Results

In 82% of cases, the questionnaire was completed while the participants (n = 59; 47 men; median age = 32 years; median number of relapses = 13) were in complete remission (under specific therapy in 76% of cases). Participants had higher educational degree than in the FGP (SIR = 6.3; p < 0.01) and more frequently a managerial occupation (SIR = 3.1; p < 0.01). Social integration was acceptable with regard to marital status and experience of sexual intercourse, but experiences of discrimination were far more frequent (SIR = 12.5; p < 0.01). The SF-12 mental component summary (MCS) score was altered (Z-score = − 0.6; p < 0.01) and mean multidimensional fatigue inventory (MFI-20) global fatigue score appeared high (12). Transfer from pediatric to adult healthcare was followed by a period of discontinued care for 33% of participants. Multivariate analysis revealed a close relationship between MFI-20, physical health, and MCS.

Conclusions

This study shows that pediatric onset FRNS and SDNS may have a long-term negative impact on mental HRQOL and highlights the impact of fatigue, which is often not adequately considered in routine care.

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Data availability

The datasets generated and/or analyzed during the current study are available from the corresponding author on reasonable request.

Code availability

Analyses were conducted using SAS®-v9.6 software (SAS Institute Inc., Cary, NC). Code generated during the current study is available from the corresponding author on reasonable request.

Abbreviations

CC:

Chronic condition

FGP:

French general population

FRNS:

Frequently relapsing nephrotic syndrome

HRQOL:

Health-related quality of life

INS:

Idiopathic nephrotic syndrome

MCS:

Mental component summary

NS:

Nephrotic syndrome

OR:

Odds ratio

PCS:

Physical component summary

SDNS:

Steroid-dependent nephrotic syndrome

SF-12:

Short Form questionnaire 12

SIR:

Standardized incidence ratio

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Acknowledgements

We thank the participants for taking the time to complete the questionnaire. We would also like to thank all the nephrologists involved in the medical care of the patients included in this study. The authors are grateful to Priscilla Boizeau, Sabrina Verchere and Adyla Yacoubi for data management assistance and The Fondation de France and The Association des Malades atteints de Syndrome Néphrotique (AMSN) for their funding.

Funding

This work was supported by a grant from Fondation de France and a grant from Association des Malades atteints de Syndrome Néphrotique (AMSN).

Author information

Author notes

  1. Vincent Audard and Hélène Mellerio have contributed equally to this work and are joint senior authors.

Authors and Affiliations

  1. Service de Néphrologie et Transplantation, Centre de Référence Maladie Rare “Syndrome Néphrotique Idiopathique”, Assistance Publique des Hôpitaux de Paris (AP-HP), Hôpitaux Universitaires Henri Mondor, Fédération Hospitalo-Universitaire “Innovative Therapy for Immune Disorders”, 51 Avenue du Marechal-de-Lattre-de-Tassigny, Créteil Cedex, 94010, Creteil, France

    Marie-Sophie Meuleman, Antoine Durrbach, Philippe Rémy, Dil Sahali & Vincent Audard

  2. Institut National de la Santé et de la Recherche Médicale (INSERM) U955, Institut Mondor de Recherche Biomédicale (IMRB), Université Paris Est Créteil, Equipe 21, Creteil, France

    Marie-Sophie Meuleman, Philippe Rémy, Dil Sahali & Vincent Audard

  3. Université de Paris, ECEVE UMR 1123, INSERM, 75010, Paris, France

    Sophie Guilmin-Crépon, Agnès Dumas, Aurélie Bourmaud, Corinne Alberti & Hélène Mellerio

  4. Unité d’Épidémiologie Clinique, CIC 1426, AP-HP.Nord, Hôpital Universitaire Robert Debré, Inserm, 75019, Paris, France

    Sophie Guilmin-Crépon, Fallou Leye, Aurélie Bourmaud & Corinne Alberti

  5. Service de Néphrologie, AP-HP, Hôpital Necker, Paris, France

    Aurélie Hummel

  6. Service de Néphrologie, AP-HP, Hôpital Bichat, Université de Paris, INSERM U1149, Paris, France

    Eric Daugas

  7. Service de Néphrologie Immunologie Clinique Transplantation, Centre de Recherche en Transplantation et Immunologie (CRTI), Centre Hospitalier Universitaire (CHU) de Nantes, Nantes, France

    Jacques Dantal

  8. Service de Néphrologie Transplantation, Dialyse et Aphérèses, CHU de Bordeaux, Bordeaux, France

    Claire Rigothier

  9. Service de Néphrologie, Hôpital Huriez, CHU de Lille, Lille, France

    François Provot

  10. Service de Néphrologie et Transplantation d’Organes, Hôpital de Rangueil et Centre de Référence Maladies Rénales Rares, CHU de Toulouse, Toulouse, France

    Dominique Chauveau

  11. APHM, INSERM, INRAe, C2VN, Centre de Néphrologie et Transplantation Rénale, Hôpital de la Conception, Aix Marseille University, Marseille, France

    Stéphane Burtey

  12. Service de Néphrologie et Transplantation Rénale, AP-HP, Hôpital Tenon, Paris, France

    Alexandre Hertig

  13. Service de Néphrologie et Dialyse, AP-HP, Hôpital Tenon, Paris, France

    Karine Dahan

  14. Service de Néphrologie Pédiatrique, AP-HP, Hôpital Robert Debré, Paris, France

    Claire Dossier

  15. Service de Néphrologie, AP-HP, Hôpital Européen Georges Pompidou, Université de Paris, Paris, France

    Alexandre Karras

  16. Service de Néphrologie, CHU de Rouen, Rouen, France

    Dominique Guerrot

  17. Service de Néphrologie, Hôpital Pasteur, CHU de Nice, Nice, France

    Vincent Esnault

  18. Service de Néphrologie, AP-HP, Hôpital Ambroise Paré, Boulogne-Billancourt, France

    Ziad A. Massy

  19. INSERM U1018 CESP, UVSQ, UPS Villejuif, Villejuif, France

    Ziad A. Massy

  20. Service de Néphrologie et Transplantation, AP-HP, Hôpital Pitié Salpêtrière, Paris, France

    Isabelle Tostivint

  21. Service de Néphrologie, Hôpital de Pontchaillou, CHU de Rennes, Rennes, France

    Marie-Pascale Morin

  22. Service de Néphrologie, Hémodialyse, Aphérèse et Transplantation Rénale, CHU de Grenoble Alpes, Université Grenoble-Alpes, Grenoble, France

    Philippe Zaoui

  23. Service de Néphrologie, Centre Hospitalier (CH) La Rochelle, La Rochelle, France

    Olivier Fritz

  24. Service de Néphrologie Dialyse et Transplantation Rénale, Hôpital Lapeyronie, CHU de Montpellier, Montpellier, France

    Moglie Le Quintrec

  25. Service de Néphrologie, CHU de Reims, Reims, France

    Alain Wynckel

  26. Service de Néphrologie Pédiatrique, Centre de Référence Maladie Rare “Syndrome Néphrotique Idiopathique”, Institut Imagine, AP-HP, Hôpital Necker-Enfants Malades, Université de Paris, Paris, France

    Olivia Boyer

  27. Service de médecine d’adolescent, Plateforme de Transition AD’venir, AP-HP, Hôpital Robert Debré, Paris, France

    Hélène Mellerio

  28. Groupe Français de Recherche en Médecine et Santé de l’Adolescent, Paris, France

    Hélène Mellerio

Authors
  1. Marie-Sophie Meuleman
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  2. Sophie Guilmin-Crépon
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  3. Aurélie Hummel
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Contributions

DS, CA, VA and HM designed the research study. M-SM, SG-C, AH, ED, JD, CR, FP, DC, SB, AH, KD, AD, CD, AK, DG, VE, PR, ZAM, IT, M-PM, PZ, OF, MLQ, AW, OB and DS collected the data provided clinical and biological information and critically reviewed the manuscript. M-SM, SG-C, AD, FL, VA and HM analyzed the data. M-SM, SG-C, FL, AB and HM performed statistical analysis. M-SM, AD, VA and HM wrote the manuscript. Each author contributed important intellectual content during manuscript drafting. All authors read and approved the final version of the manuscript.

Corresponding author

Correspondence to Marie-Sophie Meuleman.

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Conflict of interest

Vincent Audard has received consulting fees from Addmedica, unrelated to the present study. The remaining authors have no relevant financial interests to disclose.

Ethical approval

This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of “Assistance Publique - Hôpitaux de Paris” (No. 15.582).

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Written informed consent was obtained from all individual participants included in the study.

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There is no identifying information from participants in this article.

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Meuleman, MS., Guilmin-Crépon, S., Hummel, A. et al. Long-term health-related quality of life outcomes of adults with pediatric onset of frequently relapsing or steroid-dependent nephrotic syndrome. J Nephrol 35, 1123–1134 (2022). https://doi.org/10.1007/s40620-021-01111-0

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