title: Autoimmune-type atypical hemolytic uremic syndrome treated with eculizumab as first-line therapy.
abstract: We report a case of atypical hemolytic uremic syndrome (aHUS) in a 4-year-old boy. Although the patient had the typical triad of aHUS (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury), urgent dialysis was not indicated because he had neither oliguria nor severe electrolyte abnormality. He was given eculizumab as first-line therapy, which led to significant clinical improvement, thus avoiding any risk of complications associated with plasma exchange and central venous catheterization. Retrograde functional analysis of the patient's plasma using sheep erythrocytes indicated an increase in hemolysis, suggesting impairment of host cell protection by complement factor H. The use of eculizumab as first-line therapy in place of plasma exchange might be reasonable for pediatric patients with aHUS.
authors: Department of Nephrology, Chiba Children's Hospital, Chiba, Japan.
authors: Masataka
authors: M
authors: Hisano
authors: Akira
authors: A
authors: Ashida
authors: Eiji
authors: E
authors: Nakano
authors: Mamiko
authors: M
authors: Suehiro
authors: Yoko
authors: Y
authors: Yoshida
authors: Masanori
authors: M
authors: Matsumoto
authors: Toshiyuki
authors: T
authors: Miyata
authors: Yoshihiro
authors: Y
authors: Fujimura
authors: Motoshi
authors: M
authors: Hattori
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome / Q000188:drug therapy*; D002675:Child, Preschool; D017242:Complement Factor H / Q000276:immunology; D051056:Complement Inactivating Agents / Q000627:therapeutic use*; D006801:Humans; D008297:Male
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C493495:CFH protein, human; D051056:Complement Inactivating Agents; D017242:Complement Factor H; C481642:eculizumab
keywords: atypical hemolytic uremic syndrome; eculizumab; plasma exchange