title: External validation of a risk score to predict intravenous immunoglobulin resistance in patients with kawasaki disease.
abstract: BACKGROUND
  we previously developed a new risk score to predict intravenous immunoglobulin (IVIG) resistance in Kawasaki disease. However, the IVIG dosage used in that study (1 g/kg/d for 2 consecutive days) differs from the single infusion of 2 g/kg recommended in the United States and elsewhere. Our aim was to assess the validity and applicability of our risk score in patients treated with a single infusion.
  
  
  METHODS
  we used a database of 1626 patients with Kawasaki disease given initial IVIG treatment at a dose of 1 g/kg/d for 2 consecutive days (n = 990; IVIG- 1 g/kg × 2) or 2 g/kg/d for 1 day (n = 636; IVIG- 2 g/kg × 1) across 17 hospitals in Japan. Patients received the total IVIG dose within 36 hours in IVIG- 1 g/kg × 2 and 24 hours in IVIG- 2 g/kg × 1. We stratified the patients according to a risk scoring system developed to predict IVIG unresponsiveness, based on scores of ≥ 5 points. We compared the accuracy of prediction between the 2 groups using receiver operating characteristic analysis.
  
  
  RESULTS
  baseline characteristics and clinical outcomes were similar between both groups. The areas under the receiver operating characteristic curve in IVIG- 2 g/kg × 1 were similar to those of IVIG- 1 g/kg × 2. Using a cut-off risk score of ≥ 5 points, we could identify IVIG resistance in terms of coronary artery abnormalities within 1 month and coronary artery abnormalities at 1 month with equivalent sensitivity and specificity in both groups.
  
  
  CONCLUSIONS
  our risk score can be used to predict IVIG unresponsiveness to a regimen based on a single infusion of 2 g/kg IVIG.
authors.affiliation: Department of Pediatrics, Gunma University Graduate School of Medicine, Gunma, Japan.
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Seki
authors.forename: Tohru
authors.initials: T
authors.lastname: Kobayashi
authors.forename: Tomio
authors.initials: T
authors.lastname: Kobayashi
authors.forename: Akihiro
authors.initials: A
authors.lastname: Morikawa
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Otani
authors.forename: Kazuo
authors.initials: K
authors.lastname: Takeuchi
authors.forename: Mamoru
authors.initials: M
authors.lastname: Ayusawa
authors.forename: Keiji
authors.initials: K
authors.lastname: Tsuchiya
authors.forename: Kenji
authors.initials: K
authors.lastname: Yasuda
authors.forename: Takahiro
authors.initials: T
authors.lastname: Suzuki
authors.forename: Shinya
authors.initials: S
authors.lastname: Shimoyama
authors.forename: Kentaro
authors.initials: K
authors.lastname: Ikeda
authors.forename: Yoichiro
authors.initials: Y
authors.lastname: Ishii
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Arakawa
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007223:Infant; D007262:Infusions, Intravenous; D007564:Japan; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000188:drug therapy*; D011379:Prognosis; D012372:ROC Curve; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous