Idiopathic membranoproliferative glomerulonephritis in childhood

Membranoproliferative glomerulonephritis (MPGN), recognized since 1965, is now known to have three forms, designated types I, II, and III. The types are similar in the frequency of hypocomplementemia and clinical course but are dissimilar in glomerular ultrastructure, pathogenesis, mechanisms of com...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 1992, Vol.6 (1), p.96-103
Main Author: WEST, C. D
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child
Child, Preschool
Complement C3 - deficiency
Glomerulonephritis, Membranoproliferative - diagnosis
Glomerulonephritis, Membranoproliferative - drug therapy
Glomerulonephritis, Membranoproliferative - etiology
Humans
Infant
Medical sciences
Pharmacology. Drug treatments
Prednisone - therapeutic use
Urinary system
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Summary:Membranoproliferative glomerulonephritis (MPGN), recognized since 1965, is now known to have three forms, designated types I, II, and III. The types are similar in the frequency of hypocomplementemia and clinical course but are dissimilar in glomerular ultrastructure, pathogenesis, mechanisms of complement activation, predisposition to recur in the renal transplant, and, to some extent, in clinical presentation. Although glomerular proliferation is usually diffuse, it may be focal and segmental particularly in mild cases of MPGN I. Hypocomplementemia, present in about 80% of patients, is the result of hypercatabolism of C3 by three mechanisms as well as of diminished C3 synthesis. The hypocomplementemia is unrelated to clinical course or prognosis. Although MPGN I and III both have a high frequency of an extended haplotype on chromosome 6, which has known associations with autoimmune phenomena, and both have a high frequency of inherited complement defects, they are nevertheless dissimilar in glomerular ultrastructure, complement profile, and immunohistology in ways which suggest a wide difference in pathogenesis. Abnormalities in humoral immunity appear not to be involved in MPGN III. Treatment with anticoagulant, antiplatelet and cytotoxic drugs have, in controlled trials, been either ineffective or marginally effective. Long-term use of alternate-day prednisone in high dosage appears to be the most efficacious regimen in both controlled and uncontrolled studies.
ISSN:0931-041X
1432-198X
DOI:10.1007/BF00856851