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

被引:263
作者
Appel, GB
Cook, HT
Hageman, G
Jennette, JC
Kashgarian, M
Kirschfink, M
Lambris, JD
Lanning, L
Lutz, HU
Meri, S
Rose, NR
Salant, DJ
Sethi, S
Smith, RJH
Smoyer, W
Tully, HF
Tully, SP
Walker, P
Welsh, M
Würzner, R
Zipfel, PF
机构
[1] Columbia Univ, Dept Nephrol, New York, NY USA
[2] Univ London Imperial Coll Sci & Technol, Fac Med, Div Invest Sci, London, England
[3] Univ Iowa, Carver Coll Med, Dept Ophthalmol & Visual Sci, Iowa City, IA USA
[4] Univ N Carolina, Dept Pathol & Lab Med, Chapel Hill, NC USA
[5] Yale Univ, Sch Med, Dept Pathol, New Haven, CT 06510 USA
[6] Heidelberg Univ, Inst Immunol, D-6900 Heidelberg, Germany
[7] Univ Penn, Dept Pathol & Lab Med, Philadelphia, PA 19104 USA
[8] Kidneeds, Iowa City, IA USA
[9] Swiss Fed Inst Technol, Inst Biochem, Zurich, Switzerland
[10] Univ Helsinki, Dept Bacteriol & Immunol, Helsinki, Finland
[11] Univ Helsinki, Cent Hosp, Helsinki, Finland
[12] Johns Hopkins Sch Med, Ctr Autoimmune Dis Res, Baltimore, MD USA
[13] Boston Univ, Med Ctr, Dept Med, Boston, MA USA
[14] Univ Iowa, Carver Coll Med, Dept Pathol, Iowa City, IA USA
[15] Univ Iowa, Carver Coll Med, Dept Otolaryngol, Iowa City, IA USA
[16] Univ Michigan, Pediat Nephrol Div, Ann Arbor, MI USA
[17] Milagros Res Fund, New York, NY USA
[18] Nephropathol Associates, Little Rock, AR USA
[19] Univ Iowa, Carver Coll Med, Dept Internal Med, Iowa City, IA USA
[20] Innsbruck Med Univ, Dept Hyg Microbiol & Social Med, Innsbruck, Austria
[21] Hans Knoell Inst Nat Prod Res, Jena, Germany
来源
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY | 2005年 / 16卷 / 05期
关键词
D O I
10.1681/ASN.2005010078
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
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.
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收藏
页码:1392 / 1403
页数:12
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