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Membranoproliferative glomerulonephritis

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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.

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Acknowledgments

This work was supported by the NIHR Cambridge Biomedical Research Centre.

Author information

Authors and Affiliations

  1. Renal Unit, Addenbrooke’s Hospital, Box 118, Hills road, Cambridge, CB2 0QQ, UK

    Bassam Alchi & David Jayne

Authors
  1. Bassam Alchi
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  2. David Jayne
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Corresponding author

Correspondence to David Jayne.

Additional information

Answers:

1) C

2) B

3) A

4) D

5) C

Questions:

Questions:

(Answers appear following the question list)

  1. 1.

    Which of the following statements regarding the prevalence of MPGN is true?

    1. a)

      MPGN is more common in Europe and North America

    2. b)

      MPGN usually affects children and young adults with a male to female ratio of 3:1

    3. c)

      MPGN accounts for approximately 5% of the primary renal causes of nephrotic syndrome in children and adults

    4. d)

      MPGN is a common cause of ESRD in children

  2. 2.

    Which of the following statements regarding C3NeF is correct?

    1. a)

      C3NeF is an IgM autoantibody

    2. b)

      C3NeF can be found in healthy subjects

    3. c)

      C3NeF prevents the inactivation of C3 convertase (C3bBb) by factor I

    4. d)

      C3NeF is a surrogate marker of disease progression

  3. 3.

    Which of the following histological features is the most characteristic of MPGN?

    1. a)

      Duplication of the glomerular basement membrane

    2. b)

      Crescent formation

    3. c)

      Effacement of the podocyte foot process

    4. d)

      Positive staining for C4 along the glomerular capillaries

  4. 4.

    Which of the following statements regarding the recurrence of MPGN in kidney transplantation is true?

    1. a)

      More common in type I MPGN

    2. b)

      Is usually benign

    3. c)

      Nephrotic-range proteinuria is a risk factor for recurrence

    4. d)

      Can morphologically resemble transplant glomerulopathy

  5. 5.

    Which of the following regarding the treatment of idiopathic MPGN is true?

    1. a)

      The rarity of MPGN makes the randomized controlled study design easy to implement

    2. b)

      There is a good evidence that the combination of aspirin and dipyridamole slows the progression of the disease

    3. c)

      Steroid therapy has been proven effective for children but not for adults

    4. d)

      Cyclosporine is generally recommended in patients with rapidly progressive renal failure

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Alchi, B., Jayne, D. Membranoproliferative glomerulonephritis. Pediatr Nephrol 25, 1409–1418 (2010). https://doi.org/10.1007/s00467-009-1322-7

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