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The long-term outcome of childhood nephrotic syndrome in Germany: a cross-sectional study

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Abstract

Background

Long-term outcomes of children with nephrotic syndrome have not been well described in the literature.

Methods

Cross-sectional study data analysis of n = 43 patients with steroid-sensitive (SSNS) and n = 7 patients with steroid-resistant (SRNS) nephrotic syndrome were retrospectively collected; patients were clinically examined at a follow-up visit (FUV), on average 30 years after onset, there was the longest follow-up period to date.

Results

The mean age at FUV was 33.6 years (14.4–50.8 years, n = 41). The mean age of patients with SSNS at onset was 4.7 years (median 3.8 years (1.2–14.5 years), the mean number of relapses was 5.8 (0 to 29 relapses). Seven patients (16.3%) had no relapses. Eleven patients were “frequent relapsers” (25.6%) and four patients still had relapses beyond the age of 18 years. Except of cataracts and arterial hypertension, there were no negative long-term outcomes and only one patient was using immunosuppressant therapy at FUV. 55% of patients suffered from allergies and 47.5% had hypercholesterolemia. Two patients suffered a heart attack in adulthood. A younger age at onset (< 4 years) was a risk factor for frequent relapses. An early relapse (within 6 months after onset) was a risk factor and a low birth weight was not a significant risk factor for a complicated NS course. The mean age of patients with SRNS at onset was 4.6 ± 4.4 years and 27.5 ± 9.9 years at FUV. Three patients received kidney transplantations.

Conclusions

The positive long-term prognosis of SSNS can reduce the concern of parents about the probability of the child developing a chronic renal disease during the clinical course after onset.

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Availability of data and material

The data sets used and/or analyzed during the current study are available from Dr. I. Franke/ Dr. R. Hagemann on reasonable request at Department of General Pediatrics, University Children’s Hospital Bonn, Germany, and will be shared with scientists/researcher upon request to Dr. I. Franke or Dr. R. Hagemann.

Abbreviations

AH:

Arterial hypertension

AP:

Alkaline phosphatase

BMI:

Body mass index

DPD:

Deoxypyridinoline

ESPED:

Erhebungseinheit für seltene pädiatrische Erkrankungen in Deutschland

FSGS:

Focal segmental glomerulosclerosis

FUV:

Follow-up visit

M:

Mean

MCNS:

Minimal change nephrotic syndrome

MG:

Membranous glomerulonephritis

NS:

Nephrotic syndrome

SSNS:

Steroid-sensitive nephrotic syndrome

SRNS:

Steroid-resistant nephrotic syndrome.

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Acknowledgements

***Ingo Franke †*** We thank our first author, colleague, and friend Ingo Franke for his excellent work in the field of Pediatric Nephrology in Germany. He taught us very enthusiastically, and he was a great pediatrician and researcher. Rest in Peace, dear Ingo! This publication should confirm your effort, your invested time, and energy. We all miss you!

Funding

This study was not funded by any private or institutional organizations/firms.

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Author notes

  1. Malik Aydin and Ingo Franke have contributed equally to this work and share the first authorship.

  2. Mark Born and Rebekka Hagemann have contributed equally to this work and share the senior authorship.

  3. Ingo Franke (R.I.P.) passed away during the submission process.

Authors and Affiliations

  1. Department of General Pediatrics, University Children’s Hospital Bonn, Bonn, Germany

    Malik Aydin, Ingo Franke, Lisa Kurylowicz, Rainer Ganschow, Michael Lentze & Rebekka Hagemann

  2. HELIOS University Medical Center Wuppertal, Children’s Hospital, Center for Clinical and Translational Research (CCTR), Center for Biomedical Education and Research (ZBAF), Witten/Herdecke University, Heusnerstr. 40, 42283, Wuppertal, Germany

    Malik Aydin

  3. Department of Radiology, Pediatric Radiology, University Hospital Bonn, Bonn, Germany

    Mark Born

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Contributions

IF and RH designed and performed the study. MA, MB, LK, RH, and IF performed the literature search, extracted data, and drafted the manuscript. ML and RG participated in manuscript writing and revision. All authors have read and approved the final manuscript.

Corresponding author

Correspondence to Malik Aydin.

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Conflict of interest

The authors have declared that they have no competing interests. This work has not been published before and it is not under consideration for publication anywhere else. Its publication has been approved by all co-authors.

Ethics approval and consent to participate

All retrospectively and prospectively analyzed data involving human participants were in accordance with the ethical standards and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Ethical approval was obtained by the Ethics Committee of the Faculty of Medicine, Rheinische Friedrich-Wilhelms-University, Bonn, Germany, and the study was assigned the human study registration number 081/05. The data extraction and analyses were performed pseudonymously; an additional participant informed consent was obtained from all individual participants included in the study. This article does not contain any studies with animals performed by any of the authors.

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Aydin, M., Franke, I., Kurylowicz, L. et al. The long-term outcome of childhood nephrotic syndrome in Germany: a cross-sectional study. Clin Exp Nephrol 23, 676–688 (2019). https://doi.org/10.1007/s10157-019-01696-8

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  • DOI: https://doi.org/10.1007/s10157-019-01696-8

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