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Clinical and Experimental Nephrology

, Volume 23, Issue 5, pp 676–688 | Cite as

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

  • Malik AydinEmail author
  • Ingo Franke
  • Lisa Kurylowicz
  • Rainer Ganschow
  • Michael Lentze
  • Mark Born
  • Rebekka Hagemann
Original Article
  • 214 Downloads

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.

Keywords

Childhood nephrotic syndrome Steroid-sensitive nephrotic syndrome Steroid-resistant nephrotic syndrome Outcome Complication 

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.

Notes

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!

Author 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.

Funding

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

Compliance with ethical standards

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.

Supplementary material

10157_2019_1696_MOESM1_ESM.docx (17 kb)
Supplementary material 1 (DOCX 16 KB)
10157_2019_1696_MOESM2_ESM.docx (20 kb)
Supplementary material 2 (DOCX 19 KB)
10157_2019_1696_MOESM3_ESM.jpg (40 kb)
Figure 3S Patients with first onset of nephrotic syndrome, n=59. (JPG 39 KB)

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Copyright information

© Japanese Society of Nephrology 2019

Authors and Affiliations

  • Malik Aydin
    • 1
    • 2
    Email author
  • Ingo Franke
    • 1
  • Lisa Kurylowicz
    • 1
  • Rainer Ganschow
    • 1
  • Michael Lentze
    • 1
  • Mark Born
    • 3
  • Rebekka Hagemann
    • 1
  1. 1.Department of General PediatricsUniversity Children’s Hospital BonnBonnGermany
  2. 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 UniversityWuppertalGermany
  3. 3.Department of Radiology, Pediatric RadiologyUniversity Hospital BonnBonnGermany

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