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The incidence of the nephrotic syndrome in childhood in Germany

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Abstract

Background

The incidence of childhood nephrotic syndrome (NS) in Germany is not well known.

Methods

An ESPED-based nationwide collection of epidemiological data of children in 2005 and 2006.

Result

The mean age of NS at onset was 5.5 ± 3.7 years. The gender ratio of boys to girls was 1.8:1. The average length of stay was 15.5 ± 11.2 days, with younger children remaining significantly longer in hospital. Steroid-resistance was more common in children ≥8 years (p = 0.023). Focal-segmental glomerulosclerosis (FSGS) was more common in children >10 years (p = 0.029). The ratio of males to females with FSGS was 1:1.9, thus the FSGS risk for girls at onset was 3.3-times greater. Considering the available data, the incidence of NS in Germany is 1.2/100,000 in the population <18 years, of which 1.0/100,000 are steroid-sensitive.

Conclusion

Compared with international data, which primarily focused on regional and small populations, this is the largest study about the incidence of the childhood NS.

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Abbreviations

ALOS:

Average length of stay

APN:

Arbeitsgemeinschaft für Pädiatrische Nephrologie (Society of Paediatric nephrology)

ARF:

Acute renal failure

CNS:

Congenital nephrotic syndrome

DGKJ:

Deutsche Gesellschaft für Kinder- und Jugendmedizin

DMS:

Diffuse mesangial sclerosis

ESPED:

Erhebungseinheit für Seltene Pädiatrische Erkrankungen in Deutschland

FSGS:

Focal-segmental glomerulosclerosis

HIV:

Human imunnodeficiency virus

HSP:

Henoch-Schönlein purpura

LRTI:

Lower respiratory tract infection

MCNS:

Minimal-change-glomerulonephritis

MesPGN:

Mesangioproliferative glomerulonephritis

MPGN:

Membranoproliferative glomerulonephritis

NS:

Nephrotic syndrome

PNS:

Primary nephrotic syndrome

SNS:

Secondary nephrotic syndrome

SRNS:

Steroid-resistant nephrotic syndrome

SSNS:

Steroid-sensitive nephrotic syndrome

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Acknowledgements

The questionnaire was developed in consultation with the members of the Arbeitsgemeinschaft für Pädiatrische Nephrologie (APN) study committee and the survey was inaugurated as an APN-supported study. This study was funded by Bonner Elternverein für nierenkranke Kinder. We thank the Erhebungseinheit für Seltene Pädiatrische Erkrankungen in Deutschland (ESPED) for their exceptional cooperation and collaboration.

Author information

Author notes

  1. Ingo Franke and Malik Aydin contributed equally to this work.

Authors and Affiliations

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

    Ingo Franke, Malik Aydin, Corinna Elke Llamas Lopez, Lisa Kurylowicz, Rainer Ganschow & Michael Lentze

  2. Centre of Biomedical Education and Research, Helios University Children’s Hospital Wuppertal, University of Witten/Herdecke, Witten, Germany

    Malik Aydin

  3. Department of Radiology, Paediatric Radiology, University of Bonn, Bonn, Germany

    Mark Born

  4. Helios University Children’s Hospital Wuppertal, University of Witten/Herdecke, Heusnerstr. 40, 42283, Wuppertal, Germany

    Malik Aydin

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  1. Ingo Franke
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Corresponding author

Correspondence to Malik Aydin.

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Conflict of interest

The authors have declared that no conflicts of interest exist.

Human and animal rights

All procedures performed in studies involving human participants were in accordance with the ethical standards of the Faculty of Medicine, Rheinische Friedrich-Wilhelms-University, Bonn/Germany at which the studies were conducted (IRB Approval Number 081/05) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

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

Additional information

Corinna Elke Llamas Lopez: This present work has already been defended as a doctoral thesis.

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Franke, I., Aydin, M., Llamas Lopez, C.E. et al. The incidence of the nephrotic syndrome in childhood in Germany. Clin Exp Nephrol 22, 126–132 (2018). https://doi.org/10.1007/s10157-017-1433-6

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