title: Risk factors and derived formosa score for intravenous immunoglobulin unresponsiveness in Taiwanese children with Kawasaki disease.
abstract: OBJECTIVE
  Kawasaki disease (KD) is the most common pediatric vasculitis. The study aimed to identify the risk factors of intravenous immunoglobulin (IVIG) unresponsiveness from the initial clinical parameters of the Taiwanese KD patients.
  
  
  METHODS
  We enrolled 248 KD (development dataset: 181, validation: 67) patients who received IVIG within 10 days after fever onset. IVIG unresponsiveness was defined by persistent fever beyond 24 hours after IVIG or recrudescent fever with KD symptoms.
  
  
  RESULTS
  From the development dataset (181 patients), IVIG unresponsiveness was noted in 22 patients (12.1%). The preIVIG levels of albumin, percentage of neutrophils, and positive lymphadenopathy were identified with highest risk for IVIG unresponsiveness. These three variables were used to construct a three-variable logistic regression model, which yielded an area under the receiver-operating-characteristics curve of 0.87. These three variables were further used to generate a composite scoring model (Formosa score) which yielded a sensitivity of 90.9% and specificity of 81.3% for a cut-off point of three or more. Validation in an independent cohort (67 KD patients) yielded sensitivity and specificity of 71.4% and 81.0%, respectively.
  
  
  CONCLUSIONS
  We have established a simple three-variable Formosa score for KD patients to identify early those at risk of IVIG unresponsiveness for timely aggressive immunomodulation initially.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan.
authors.forename: Ming-Tai
authors.initials: MT
authors.lastname: Lin
authors.affiliation: Department of Medical Research, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: Chin-Hao
authors.initials: CH
authors.lastname: Chang
authors.affiliation: Department of Pediatrics, Cardinal Tien Hospital, Taipei, Taiwan.
authors.forename: Li-Chuan
authors.initials: LC
authors.lastname: Sun
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan.
authors.forename: Hsin-Min
authors.initials: HM
authors.lastname: Liu
authors.affiliation: National Translational Medicine and Clinical Trial Resource Center, Taipei, Taiwan.
authors.forename: Huei-Wen
authors.initials: HW
authors.lastname: Chang
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan.
authors.forename: Chun-An
authors.initials: CA
authors.lastname: Chen
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan.
authors.forename: Shuenn-Nan
authors.initials: SN
authors.lastname: Chiu
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan.
authors.forename: Chun-Wei
authors.initials: CW
authors.lastname: Lu
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan.
authors.forename: Luan-Yin
authors.initials: LY
authors.lastname: Chang
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan.
authors.forename: Jou-Kou
authors.initials: JK
authors.lastname: Wang
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan. Electronic address: wumh@ntu.edu.tw.
authors.forename: Mei-Hwan
authors.initials: MH
authors.lastname: Wu
mesh_terms: D000418:Albumins / Q000032:analysis; D002097:C-Reactive Protein / Q000032:analysis; D002675:Child, Preschool; D004351:Drug Resistance; D005260:Female; D005334:Fever / Q000628:therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007223:Infant; D016015:Logistic Models; D000072281:Lymphadenopathy / Q000175:diagnosis; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000628:therapy*; D015999:Multivariate Analysis; D012372:ROC Curve; D012189:Retrospective Studies; D012307:Risk Factors; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D013624:Taiwan; D017211:Treatment Failure
chemical_list: D000418:Albumins; D016756:Immunoglobulins, Intravenous; D002097:C-Reactive Protein
keywords: Kawasaki disease; albumin; immunoglobulin; lymphadenopathy; score