title: Atypical hemolytic uremic syndrome due to factor H autoantibody.
abstract: Atypical hemolytic uremic syndrome (aHUS) is a disease caused by pathologies in the alternative complement system. The prevalence of aHUS is 10% of all aHUS cases. The subgroup of aHUS designated as DEAP (DEficiency of CFHR Proteins and CFH Autoantibody Positive)-HUS because of autoantibody to complement factor H (CFH) and CFH-related protein deficiency is seen very rarely, and the prevalence is 6% of all aHUS cases in the literature. We present here a female patient with DEAP-HUS. A 7.5-year-old girl with recurrent attacks of HUS had low C3 level. We initiated plasmapheresis treatment. After further analysis of the complement system, the result was compatible with DEAP-HUS, so we initiated immunosuppressive treatment. There were also family members with deficiency of CFHR-1 and CFHR-3, but they had no CFH autoantibody and no symptoms of HUS. In atypical cases of HUS, we should investigate complement status, especially for factor H autoantibody, for which treatment options differ from those of the other types of aHUS.
authors.affiliation: Department of Pediatrics, Akdeniz University Faculty of Medicine, Antalya, Turkey. arifeuslu2001@yahoo.com
authors.forename: Arife
authors.initials: A
authors.lastname: Uslu-Gökceoğlu
authors.forename: Cagla Serpil
authors.initials: CS
authors.lastname: Doğan
authors.forename: Elif
authors.initials: E
authors.lastname: Comak
authors.forename: Mustafa
authors.initials: M
authors.lastname: Koyun
authors.forename: Sema
authors.initials: S
authors.lastname: Akman
mesh_terms: D065766:Atypical Hemolytic Uremic Syndrome; D001323:Autoantibodies / Q000032:analysis; D001798:Blood Proteins / Q000172:deficiency; D002648:Child; D003180:Complement C3b Inactivator Proteins / Q000172:deficiency; D017242:Complement Factor H / Q000172:deficiency / Q000276:immunology*; D003404:Creatinine / Q000097:blood; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000188:drug therapy / Q000276:immunology*; D000081208:Hereditary Complement Deficiency Diseases; D006801:Humans; D007674:Kidney Diseases / Q000276:immunology*; D015432:Glomerulonephritis, Membranoproliferative
chemical_list: D001323:Autoantibodies; D001798:Blood Proteins; C517621:CFHR1 protein, human; C105970:CFHR3 protein, human; D003180:Complement C3b Inactivator Proteins; D017242:Complement Factor H; D003404:Creatinine