Membranoproliferative glomerulonephritis type II (dense deposit disease): an update

Gerald B Appel; H Terence Cook; Gregory Hageman; J Charles Jennette; Michael Kashgarian; Michael Kirschfink; John D Lambris; Lynne Lanning; Hans U Lutz; Seppo Meri; Noel R Rose; David J Salant; Sanjeev Sethi; Richard J H Smith; William Smoyer; Hope F Tully; Sean P Tully; Patrick Walker; Michael Welsh; Reinhard Würzner; Peter F Zipfel

doi:10.1681/ASN.2005010078

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Membranoproliferative glomerulonephritis type II (dense deposit disease): an update

Journal article

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Membranoproliferative glomerulonephritis type II (dense deposit disease): an update

Gerald B Appel, H Terence Cook, Gregory Hageman, J Charles Jennette, Michael Kashgarian, Michael Kirschfink, John D Lambris, Lynne Lanning, Hans U Lutz, Seppo Meri, …

Journal of the American Society of Nephrology, Vol.16(5), pp.1392-1403

05/2005

DOI: 10.1681/ASN.2005010078

PMID: 15800116

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Abstract

Membranoproliferative glomerulonephritis type II (MPGN II) is a rare disease characterized by the deposition of abnormal electron-dense material within the glomerular basement membrane of the kidney and often within Bruch's membrane in the eye. The diagnosis is made in most patients between the ages of 5 and 15 yr, and within 10 yr, approximately half progress to end-stage renal disease, occasionally with the late comorbidity of visual impairment. The pathophysiologic basis of MPGN II is associated with the uncontrolled systemic activation of the alternative pathway (AP) of the complement cascade. In most patients, loss of complement regulation is caused by C3 nephritic factor, an autoantibody directed against the C3 convertase of the AP, but in some patients, mutations in the factor H gene have been identified. For the latter patients, plasma replacement therapy prevents renal failure, but for the majority of patients, there is no proven effective treatment. The disease recurs in virtually all renal allografts, and a high percentage of these ultimately fail. The development of molecular diagnostic tools and new therapies directed at controlling the AP of the complement cascade either locally in the kidney or at the systemic level may lead to effective treatments for MPGN II.

Glomerulonephritis, Membranoproliferative - pathology

Glomerulonephritis, Membranoproliferative - physiopathology

Humans

Retinal Diseases - pathology

Kidney Glomerulus - pathology

Retinal Diseases - physiopathology

Child

Glomerulonephritis, Membranoproliferative - therapy

Retina - pathology

Details

Title: Subtitle: Membranoproliferative glomerulonephritis type II (dense deposit disease): an update
Creators: Gerald B Appel - Columbia University, Department of Nephrology, New York, New York, USA
H Terence Cook
Gregory Hageman
J Charles Jennette
Michael Kashgarian
Michael Kirschfink
John D Lambris
Lynne Lanning
Hans U Lutz
Seppo Meri
Noel R Rose
David J Salant
Sanjeev Sethi
Richard J H Smith
William Smoyer
Hope F Tully
Sean P Tully
Patrick Walker
Michael Welsh
Reinhard Würzner
Peter F Zipfel
Resource Type: Journal article
Publication Details: Journal of the American Society of Nephrology, Vol.16(5), pp.1392-1403
DOI: 10.1681/ASN.2005010078
PMID: 15800116
NLM abbreviation: J Am Soc Nephrol
ISSN: 1046-6673
eISSN: 1533-3450
Publisher: United States
Grant note: R13 DK071183 / NIDDK NIH HHS
Language: English
Date published: 05/2005
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Neurology; Molecular Physiology and Biophysics; Pulmonary, Critical Care, and Occupational Medicine; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Fraternal Order of Eagles Diabetes Research Center; Neurosurgery; Otolaryngology; Internal Medicine
Record Identifier: 9984006413802771

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