title: Evaluation of Kawasaki disease risk-scoring systems for intravenous immunoglobulin resistance.
abstract: OBJECTIVE
  To assess the performance of 3 risk scores from Japan that were developed to predict, in children with Kawasaki disease, resistance to intravenous immunoglobulin (IVIG) treatment.
  
  
  METHODS
  We used data from a randomized trial of pulsed steroids for primary treatment of Kawasaki disease to assess operating characteristics of the 3 risk scores, and we examined whether steroid therapy lowers the risk of coronary artery abnormalities in patients prospectively classified as IVIG resistant.
  
  
  RESULTS
  For comparability with published cohorts, we analyzed the data of 99 patients who were not treated with steroids (16% IVIG-retreated) and identified male sex, lower albumin level, and higher aspartate aminotransferase level as independent risk factors for IVIG resistance. The Kobayashi score was similar in IVIG-resistant and -responsive patients, yielding a sensitivity of 33% and specificity of 87%. There was no interaction of high-risk versus low-risk status by treatment received (steroid versus placebo) with any of the 3 risk score algorithms.
  
  
  CONCLUSIONS
  Risk-scoring systems from Japan have good specificity but low sensitivity for predicting IVIG resistance in a North American cohort. Primary steroid therapy did not improve coronary outcomes in patients prospectively classified as being at high-risk for IVIG resistance.
authors.affiliation: New England Research Institutes, Watertown, MA, USA. lsleeper@neriscience.com
authors.forename: Lynn A
authors.initials: LA
authors.lastname: Sleeper
authors.forename: L Luann
authors.initials: LL
authors.lastname: Minich
authors.forename: Brian M
authors.initials: BM
authors.lastname: McCrindle
authors.forename: Jennifer S
authors.initials: JS
authors.lastname: Li
authors.forename: Wilbert
authors.initials: W
authors.lastname: Mason
authors.forename: Steven D
authors.initials: SD
authors.lastname: Colan
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Atz
authors.forename: Beth F
authors.initials: BF
authors.lastname: Printz
authors.forename: Annette
authors.initials: A
authors.lastname: Baker
authors.forename: Victoria L
authors.initials: VL
authors.lastname: Vetter
authors.forename: Jane W
authors.initials: JW
authors.lastname: Newburger
mesh_terms: D002675:Child, Preschool; D004311:Double-Blind Method; D004351:Drug Resistance; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007155:Immunologic Factors / Q000008:administration & dosage*; D007223:Infant; D007262:Infusions, Intravenous; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D011379:Prognosis; D012372:ROC Curve; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors