Renal Transplantation Under Prophylactic Eculizumab in Atypical Hemolytic Uremic Syndrome With CFH/CFHR1 Hybrid Protein

被引：57

作者：

Krid, S. ^{[1
]}

Roumenina, L. T. ^{[2
,3
]}

Beury, D. ^{[4
]}

Charbit, M. ^{[1
]}

Boyer, O. ^{[1
,3
]}

Fremeaux-Bacchi, V. ^{[4
]}

Niaudet, P. ^{[1
,3
]}

机构：

[1] Hop Necker Enfants Malad, Paris, France

[2] Cordeliers Res Ctr, INSERM, UMRS 872, Paris, France

[3] Univ Paris 05, Paris, France

[4] Hop Europeen Georges Pompidou, AP HP, Lab Immunol, Paris, France

来源：

AMERICAN JOURNAL OF TRANSPLANTATION | 2012年 / 12卷 / 07期

关键词：

Atypical HUS; Eculizumab; recurrence; renal transplantation; ANTIBODY-MEDIATED REJECTION; FACTOR-H; FUNCTIONAL-CHARACTERIZATION; COMPLEMENT; MUTATIONS; DELETION;

D O I：

10.1111/j.1600-6143.2012.04051.x

中图分类号：

R61 [外科手术学];

学科分类号：

摘要：

We report the first observation of successful kidney transplantation under pre-emptive eculizumab treatment in a 7-year-old boy with atypical hemolytic uremic syndrome (aHUS) and a known hybrid CFH/CFHR1 gene, who was dependent on plasma therapy during the 3-year dialysis period. The hybrid CFH/CFHR1 protein has an altered C3b/C3d binding, is incapable to protect cells from complement attack and is directly implicated in aHUS pathogenesis. There was no evidence of recurrence during the first 16-month follow-up period. We conclude that eculizumab alone, without plasma therapy (plasma infusion and/or plasma exchange), is sufficient to prevent recurrence of aHUS and to maintain long-term graft function.

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收藏

页码：1938 / 1944

页数：7

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