title: Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the Joint Committee of the Japanese Society of Nephrology and the Japan Pediatric Society.
abstract: Atypical hemolytic uremic syndrome (aHUS) is rare and comprises the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Recently, abnormalities in the mechanisms underlying complement regulation have been focused upon as causes of aHUS. The prognosis for patients who present with aHUS is very poor, with the first aHUS attack being associated with a mortality rate of ~25 %, and with ~50 % of cases resulting in end-stage renal disease requiring dialysis. If treatment is delayed, there is a high risk of this syndrome progressing to renal failure. Therefore, we have developed diagnostic criteria for aHUS to enable its early diagnosis and to facilitate the timely initiation of appropriate treatment. We hope these diagnostic criteria will be disseminated to as many clinicians as possible and that they will be used widely.
authors.affiliation: Department of Pediatrics, Shiga University of Medical Science, Otsu, Japan.
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Sawai
authors.forename: Masaomi
authors.initials: M
authors.lastname: Nangaku
authors.forename: Akira
authors.initials: A
authors.lastname: Ashida
authors.forename: Rika
authors.initials: R
authors.lastname: Fujimaru
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hataya
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Hidaka
authors.forename: Shinya
authors.initials: S
authors.lastname: Kaname
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Okada
authors.forename: Waichi
authors.initials: W
authors.lastname: Sato
authors.forename: Takashi
authors.initials: T
authors.lastname: Yasuda
authors.forename: Yoko
authors.initials: Y
authors.lastname: Yoshida
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fujimura
authors.forename: Motoshi
authors.initials: M
authors.lastname: Hattori
authors.forename: Shoji
authors.initials: S
authors.lastname: Kagami
mesh_terms: D065766:Atypical Hemolytic Uremic Syndrome / Q000097:blood / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology / Q000628:therapy; D015415:Biomarkers / Q000097:blood; D003167:Complement Activation; D003165:Complement System Proteins; D032921:Consensus; D042241:Early Diagnosis; D006801:Humans; D009398:Nephrology / Q000592:standards*; D010372:Pediatrics / Q000592:standards*; D010976:Platelet Count / Q000592:standards; D011237:Predictive Value of Tests; D011379:Prognosis; D012307:Risk Factors
chemical_list: D015415:Biomarkers; D003165:Complement System Proteins