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Membranoproliferative and C3-Mediated GN in Children

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Abstract

Membranoproliferative glomerulonephritis (MPGN) describes a histopathological pattern characterized by mesangial and endocapillary proliferation and capillary wall remodeling with formation of double contours. This injury pattern is a result of deposition of immunoglobulins (Ig)/immune complexes (IC) and/or complement proteins in the mesangium and/or along the capillary wall of the glomerulus [1, 2].

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Abbreviations

aHUS:

Atypical hemolytic uremic syndrome

AMD:

Age-related macular degeneration

AP:

Alternative pathway (of complement)

aPL:

Acquired partial lipodystrophy

C:

Complement

C3G:

C3 glomerulopathy

C3GN:

C3 glomerulonephritis

C3NeF:

C3 nephritic factor

CFB:

Complement factor B

CFH:

Complement factor H

CFHR1-5:

Complement factor H-related protein 1–5

CFI:

Complement factor I

CFP:

Properdin

CP:

Classical pathway (of complement)

DDD:

Dense deposit disease

DEAP-HUS:

Deficiency of CFHR plasma proteins and CFH autoantibody positive HUS

ESRD:

End-stage renal disease

FFP:

Fresh frozen plasma

GN:

Glomerulonephritis

IC:

Immune complexes

Ig:

Immunoglobulins

IVCP:

Intravenous cyclophosphamide

LP:

Lectin pathway (of complement)

MCP:

Membrane cofactor protein

MPGN:

Membranoproliferative glomerulonephritis

NeF:

Nephritic factor

PE:

Plasma exchange

PI:

Plasma infusion

PIGN:

Postinfectious glomerulonephritis

SCR:

Short consensus repeat

TMA:

Thrombotic microangiopathy

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Acknowledgments

The authors wish to thank Dr. Paul Thorner, The Hospital for Sick Children and Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada for preparing Fig. 1 and discussing pathohistological aspects of C3G.

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Authors and Affiliations

  1. Division of Nephrology, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

    Christoph Licht

  2. Research Institute, Cell Biology Program, The Hospital for Sick Children, Toronto, ON, Canada

    Christoph Licht & Magdalena Riedl

  3. Department of Paediatrics, University of Toronto, Toronto, ON, Canada

    Christoph Licht

  4. Department of Paediatrics, Innsbruck Medical University, Innsbruck, Tyrol, Austria

    Magdalena Riedl

  5. Centre for Complement and Inflammation Research, Imperial College, London, UK

    Matthew C. Pickering

  6. Renal Section, Department of Pediatrics, Texas Children’s Hospital, Balyor College of Medicine, Houston, TX, USA

    Michael Braun

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  1. Christoph Licht
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  2. Magdalena Riedl
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  3. Matthew C. Pickering
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  4. Michael Braun
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Correspondence to Christoph Licht .

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Editors and Affiliations

  1. Department of Pediatrics, Medical College of Wisconsin, Children’s Research Institute, Children’s Hospital, Health System of Wisconsin, Milwaukee, Wisconsin, USA

    Ellis D. Avner

  2. Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts, USA

    William E. Harmon

  3. Service de Néphrologie Pédiatrique, Hôpital Necker-Enfants Malades, Université Paris-Descartes, Paris, France

    Patrick Niaudet

  4. Department of Pediatrics, Wakayama Medical University, Wakayama City, Japan

    Norishige Yoshikawa

  5. Division of Nephrology, Bambino Gesù Children’s Hospital – IRCCS, Rome, Italy

    Francesco Emma

  6. Division of Nephrology and Hypertension, The Heart Institute, Cincinnati Children’s Hospital Medical Center, College of Medicine, Cincinnati, Ohio, USA

    Stuart L. Goldstein

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Licht, C., Riedl, M., Pickering, M.C., Braun, M. (2016). Membranoproliferative and C3-Mediated GN in Children. In: Avner, E., Harmon, W., Niaudet, P., Yoshikawa, N., Emma, F., Goldstein, S. (eds) Pediatric Nephrology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43596-0_29

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  • DOI: https://doi.org/10.1007/978-3-662-43596-0_29

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