title: The clinical and laboratory features of Chinese Han anti-factor H autoantibody-associated hemolytic uremic syndrome.
abstract: Anti-complement factor H (CFH) autoantibody-associated hemolytic uremic syndrome (HUS) is a severe sub-type of HUS.
  
  
  
  We assessed the clinical and renal pathological features, circulating complement levels, and genetic background of Chinese pediatric patients with this sub-type of HUS. Thirty-three consecutive patients with acute kidney injury who tested positive for serum anti-CFH autoantibodies were enrolled in this study.
  
  
  
  All 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 (SC5b-9) levels. The CFH-anti-CFH immunoglobin G (IgG) circulating immunocomplex (CFH-CIC) titers were more closely correlated with CFH plasma levels than anti-CFH 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 follow-up, nine patients reached the combined end-points, including two with end-stage renal disease and seven with relapses.
  
  
  
  Plasma C3a, C5a, and SC5b-9 levels predicted disease activity in anti-CFH autoantibody-associated HUS patients enrolled in this study. These patients responded well to plasma therapy combined with immunosuppression.
authors.affiliation: Renal Division, Department of Medicine, Peking University Institute of Nephrology, Peking University First Hospital, Beijing, People's Republic of China.|Key laboratory of Renal Disease, Ministry of Health of China, Beijing, People's Republic of China.|Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Ministry of Education of China, Beijing, 100034, People's Republic of China.
authors.forename: Di
authors.initials: D
authors.lastname: Song
authors.affiliation: Department of Nephrology, Beijing Children's Hospital affiliated to Capital Medical University, West District Nan Li Shi Lu 56th, Beijing, 100045, People's Republic of China. desin2000@sina.com.
authors.forename: Xiao-Rong
authors.initials: XR
authors.lastname: Liu
authors.affiliation: Department of Nephrology, Beijing Children's Hospital affiliated to Capital Medical University, West District Nan Li Shi Lu 56th, Beijing, 100045, People's Republic of China.
authors.forename: Zhi
authors.initials: Z
authors.lastname: Chen
authors.affiliation: Department of Pediatrics, Peking University First Hospital, Beijing, People's Republic of China.
authors.forename: Hui-Jie
authors.initials: HJ
authors.lastname: Xiao
authors.affiliation: Department of Pediatrics, Peking University First Hospital, Beijing, People's Republic of China.
authors.forename: Jie
authors.initials: J
authors.lastname: Ding
authors.affiliation: Department of Pediatrics, Shandong Provincial Hospital affiliated with Shandong University, Jinan, People's Republic of China.
authors.forename: Shu-Zhen
authors.initials: SZ
authors.lastname: Sun
authors.affiliation: Department of Nephrology, Renmin Hospital of Wuhan University, Wuhan, People's Republic of China.
authors.forename: Hong-Yan
authors.initials: HY
authors.lastname: Liu
authors.affiliation: Renal Division, Department of Medicine, Peking University Institute of Nephrology, Peking University First Hospital, Beijing, People's Republic of China.|Key laboratory of Renal Disease, Ministry of Health of China, Beijing, People's Republic of China.|Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Ministry of Education of China, Beijing, 100034, People's Republic of China.
authors.forename: Wei-Yi
authors.initials: WY
authors.lastname: Guo
authors.affiliation: Renal Division, Department of Medicine, Peking University Institute of Nephrology, Peking University First Hospital, Beijing, People's Republic of China.|Key laboratory of Renal Disease, Ministry of Health of China, Beijing, People's Republic of China.|Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Ministry of Education of China, Beijing, 100034, People's Republic of China.
authors.forename: Su-Xia
authors.initials: SX
authors.lastname: Wang
authors.affiliation: Renal Division, Department of Medicine, Peking University Institute of Nephrology, Peking University First Hospital, Beijing, People's Republic of China. yufengevert1@sina.com.|Key laboratory of Renal Disease, Ministry of Health of China, Beijing, People's Republic of China. yufengevert1@sina.com.|Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Ministry of Education of China, Beijing, 100034, People's Republic of China. yufengevert1@sina.com.|Department of Nephrology, Peking University International Hospital, Beijing, 102206, People's Republic of China. yufengevert1@sina.com.
authors.forename: Feng
authors.initials: F
authors.lastname: Yu
authors.affiliation: Renal Division, Department of Medicine, Peking University Institute of Nephrology, Peking University First Hospital, Beijing, People's Republic of China.|Key laboratory of Renal Disease, Ministry of Health of China, Beijing, People's Republic of China.|Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Ministry of Education of China, Beijing, 100034, People's Republic of China.|Peking-Tsinghua Center for Life Sciences, Beijing, People's Republic of China.
authors.forename: Ming-Hui
authors.initials: MH
authors.lastname: Zhao
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology / Q000276:immunology; D000293:Adolescent; D044466:Asian People / Q000235:genetics; D001323:Autoantibodies / Q000032:analysis*; D001483:Base Sequence; D002648:Child; D002675:Child, Preschool; D017242:Complement Factor H / Q000276:immunology*; D003165:Complement System Proteins / Q000032:analysis / Q000276:immunology; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics / Q000276:immunology* / Q000503:physiopathology*; D006801:Humans; D007166:Immunosuppressive Agents; D007223:Infant; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D057049:Thrombotic Microangiopathies / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D017242:Complement Factor H; D003165:Complement System Proteins
keywords: Autoantibody; Complement factor H; Hemolytic uremic syndrome; Immunosuppression; Plasma therapy; Thrombotic microangiopathies