Thrombotic microangiopathy mimicking membranoproliferative glomerulonephritis

被引：32

作者：

Brackman, Damien ^{[2
]}

Sartz, Lisa ^{[1
]}

Leh, Sabine ^{[3
]}

Kristoffersson, Ann-Charlotte ^{[1
]}

Bjerre, Anna ^{[4
]}

Tati, Ramesh ^{[1
]}

Fremeaux-Bacchi, Veronique ^{[5
]}

Karpman, Diana ^{[1
]}

机构：

[1] Lund Univ, Dept Pediat, Lund, Sweden

[2] Haukeland Hosp, Dept Pediat, N-5021 Bergen, Norway

[3] Haukeland Hosp, Dept Pathol, N-5021 Bergen, Norway

[4] Oslo Univ Hosp, Dept Pediat, Oslo, Norway

[5] Hop Europeen Georges Pompidou, AP HP, Dept Immunol, Paris, France

来源：

NEPHROLOGY DIALYSIS TRANSPLANTATION | 2011年 / 26卷 / 10期

基金：

瑞典研究理事会;

关键词：

complement; C3; haemolytic uraemic syndrome; membranoproliferative glomerulonephritis; eculizumab; HEMOLYTIC-UREMIC SYNDROME; FACTOR-H; MUTATIONS;

D O I：

10.1093/ndt/gfr422

中图分类号：

R3 [基础医学]; R4 [临床医学];

学科分类号：

1001 ; 1002 ; 100602 ;

摘要：

A 4-year-old boy presented with proteinuria and developed progressive renal failure over 6 years. In the patient's family, five individuals were affected with atypical haemolytic uraemic syndrome (aHUS) but not the patient. Renal biopsies (n = 3) showed glomerular basement membrane thickening with double contours, endothelial swelling and deposits of C3 and C1q. Electron microscopy revealed mesangial and subendothelial electron-dense deposits. Complement mutations in membrane cofactor protein (Y155D) and C3 (R713W and G1094R) were detected in all affected family members. The patient also had transient autoantibodies to factor H. The findings suggest that aHUS and glomerulopathy resembling membranoproliferative glomerulonephritis may have a common molecular background.

引用

页码：3399 / 3403

页数：5

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