Membranoproliferative and C3-Mediated GN in Children

  • Christoph Licht
  • Magdalena Riedl
  • Matthew C. Pickering
  • Michael Braun
Reference work entry

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].

Keywords

Hepatitis Carbohydrate Angiotensin Cyclosporine Meningitis 

List of 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

Notes

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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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Christoph Licht
    • 1
    • 2
    • 3
  • Magdalena Riedl
    • 2
    • 4
  • Matthew C. Pickering
    • 5
  • Michael Braun
    • 6
  1. 1.Division of NephrologyThe Hospital for Sick Children, University of TorontoTorontoCanada
  2. 2.Research Institute, Cell Biology ProgramThe Hospital for Sick ChildrenTorontoCanada
  3. 3.Department of PaediatricsUniversity of TorontoTorontoCanada
  4. 4.Department of PaediatricsInnsbruck Medical UniversityInnsbruckAustria
  5. 5.Centre for Complement and Inflammation ResearchImperial CollegeLondonUK
  6. 6.Renal Section, Department of Pediatrics, Texas Children’s HospitalBalyor College of MedicineHoustonUSA

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