Membranoproliferative glomerulonephritis

Pediatr Nephrol. 2010 Aug;25(8):1409-18. doi: 10.1007/s00467-009-1322-7. Epub 2009 Nov 12.

Abstract

Membranoproliferative glomerulonephritis is an uncommon kidney disorder characterized by mesangial cell proliferation and structural changes in glomerular capillary walls. It can be subdivided into idiopathic and secondary forms, which are differentially diagnosed by a review of clinical features, laboratory data, and renal histopathology. Three types-I, II, and III-have been defined by pathologic features. All three types are associated with hypocomplementemia, but they manifest somewhat different mechanisms of complement activation. Type II, also known as "dense deposit disease", is associated with the presence of C3-nephritic factor. Membranoproliferative glomerulonephritis primarily affects children and young adults, with patients presenting with nephrotic or nephritic syndrome or with asymptomatic renal disease. This type of glomerulonephritis often progresses slowly to end-stage renal disease, and it tends to recur after renal transplantation, especially type II. The efficacy of various forms of treatment remains controversial; however, long-term steroid treatment seems to be effective in children with nephrotic-range proteinuria. Improvement in renal outcomes largely relies on the evaluation of more selective agents in carefully controlled studies.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adult
  • Child
  • Complement Activation
  • Complement C3 Nephritic Factor
  • Glomerulonephritis / pathology
  • Glomerulonephritis, Membranoproliferative / diagnosis
  • Glomerulonephritis, Membranoproliferative / drug therapy*
  • Glomerulonephritis, Membranoproliferative / pathology*
  • Humans
  • Kidney / pathology
  • Kidney Diseases / pathology
  • Kidney Failure, Chronic / pathology
  • Kidney Glomerulus / pathology
  • Kidney Transplantation

Substances

  • Complement C3 Nephritic Factor