Membranoproliferative glomerulonephritis type III

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Classification according to ICD-10
N00.5 Diffuse Mesangiocapillary Glomerulonephritis - Membranoproliferative Glomerulonephritis, Types I and III, or onA
ICD-10 online (WHO version 2019)

The Membranoproliferative glomerulonephritis type III is a very rare form of membranoproliferative glomerulonephritis with immune complex deposits subepithelial and subendothelial.

As in type I, the deposits lie between the endothelium and the basement membrane and additionally between the basement membrane and podocytes, as in membranous glomerulonephritis .

Synonyms are: Ig-mediated MPGN; Immunoglobulin-mediated membranoproliferative glomerulonephritis; English Nephrotic Syndrome, Type 7; NPHS7; Nephrotic Syndrome, Type 7 With Membranoproliverative Glomerulonephritis

root cause

Of the disease are, in some cases mutations in DGKE - gene on 17 chromosome locus based q22.

Furthermore, a genetic defect was found on chromosome 1 in a form that occurs in families .

pathology

It is assumed that changes in the capillary wall lead to complement activation (C3 component) as activation of the alternative path .

Prospect of healing

There are only a few studies with contradicting results on the prognosis .

literature

  • N. Nishizaki, S. Fujinaga, D. Hirano, H. Murakami, K. Kamei, Y. Ohtomo, T. Shimizu, K. Kaneko: Membranoproliferative glomerulonephritis Type 3 associated with Kabuki syndrome. In: Clinical nephrology. Vol. 81, No. 5, May 2014, pp. 369-373, doi: 10.5414 / CN107464 , PMID 23149249 .

Individual evidence

  1. ↑ Glomerulonephritis, membranoproliferative, immunoglobulin-mediated. In: Orphanet (Rare Disease Database).
  2. ^ Nephrotic syndrome, type 7.  In: Online Mendelian Inheritance in Man . (English)
  3. ^ A b J. Neary, A. Dorman, E. Campbell, M. Keogan, P. Conlon: Familial membranoproliferative glomerulonephritis type III. In: American Journal of Kidney Diseases . Vol. 40, No. 1, July 2002, p. E1, doi: 10.1053 / ajkd.2002.33932 , PMID 12087587 .
  4. ↑ a b Emedicine Medscape