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Title of Journal: Pediatr Nephrol

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Abbravation: Pediatric Nephrology

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Springer Berlin Heidelberg

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DOI

10.1007/bf00774493

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1432-198X

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The clinical and laboratory features of Chinese Ha

Authors: Di Song Xiaorong Liu Zhi Chen Huijie Xiao Jie Ding Shuzhen Sun Hongyan Liu Weiyi Guo Suxia Wang Feng Yu Minghui Zhao On Behalf of the Chinese Renal–TMA Network Institutes
Publish Date: 2016/12/29
Volume: 32, Issue: 5, Pages: 811-822
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Abstract

We assessed the clinical and renal pathological features circulating complement levels and genetic background of Chinese pediatric patients with this subtype of HUS Thirtythree consecutive patients with acute kidney injury who tested positive for serum antiCFH autoantibodies were enrolled in this studyAll of the eight patients who underwent renal biopsies presented with changes typical of thrombotic microangiopathy especially changes in chronic characteristics Compared to patients in remission and normal control subjects patients with acute disease had significantly lower plasma CFH levels and significantly higher plasma complement 3a C3a C5a and terminal complement complex SC5b9 levels The CFH–antiCFH immunoglobin G IgG circulating immunocomplex CFHCIC titers were more closely correlated with CFH plasma levels than antiCFH IgG levels Of the 22 patients four 18 were homozygous for CFHR3–1Δ and ten were heterozygous for CFHR1 or CFHR3 deletions Most patients responded well to a combination of plasma and immunosuppressive therapies with a remission rate of 87 At the end of the followup nine patients reached the combined endpoints including two with endstage renal disease and seven with relapsesWe gratefully acknowledge the contributions of Jie AO Xin ZHENG and Lei QU of the Peking University First Hospital in our histopathological studies This work was supported by grants from the National Natural Science Foundation of China to Innovation Research Group No 81321064 National Natural Science Foundation of China No 81470932 No81500526 No 81670639 and No 81670640 The Capital Health Research and Development of Special Grant No 201622094 and the Research on the Application of Capital Clinical Characteristics Program of Beijing Municipal Science and Technology Commission No Z161100000516106

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