title: Using the Electronic Medical Record to Correlate Kawasaki Disease Phenotypes With Clinical Outcomes.
abstract: BACKGROUND
  We sought to systematically standardize the documentation of clinical and laboratory features in Kawasaki disease (KD) on the day of initial treatment and correlate the presentation with clinical outcomes.
  
  
  METHODS
  Kawasaki disease features and classification were documented by the attending physician using a standardized documentation tool on the day of treatment for KD, including confidence in the KD diagnosis on a 4-point scale. Incomplete KD was further classified using American Heart Association (AHA) criteria (sufficient or insufficient) and baseline echocardiogram data. We prospectively recorded intravenous immunoglobulin (IVIG) resistance, coronary artery abnormalities (CAAs), periungual peeling, and retrospectively identified subsequent diagnoses of autoimmune/inflammatory disease.
  
  
  RESULTS
  From November 2012 to October, 2015, 162 patients were treated for KD: 105 with complete KD (Group 1), 7 with incomplete KD based on CAAs on day of KD diagnosis (Group 2), 23 with incomplete KD meeting AHA criteria (Group 3), and 27 with incomplete KD and insufficient AHA criteria (Group 4). Group 4 patients had lower baseline median C-reactive protein levels (Group 4 median 4.65 mg/dL [interquartile range {IQR}, 2.3-13.6] vs Group 1 median 8.0 mg/dL [IQR, 4.5-17], Group 2 median 13.9 mg/dL [IQR, 1.4-18.2], Group 3 median 13.3 mg/dL [IQR, 4.9-20.2]), and no coronary abnormalities developed, although 11% had IVIG resistance. Group 4 had higher rates of subsequent autoimmune/inflammatory conditions diagnosed (11.1% in Group 4 vs <5% for all others, P = .02).
  
  
  CONCLUSIONS
  Standardized documentation and classification of KD features may be useful to correlate with clinical outcomes, including subsequent diagnosis of autoimmune/autoinflammatory disease. Among patients with incomplete KD who did not meet AHA criteria and had a normal baseline echocardiogram, the IVIG resistance rate may have been related to a lower likelihood of an accurate diagnosis of KD.
authors.affiliation: Division of 1Infectious Diseases, Nationwide Children's Hospital, The Ohio State University, Columbus.
authors.forename: Preeti
authors.initials: P
authors.lastname: Jaggi
authors.affiliation: Division of Cardiology, Nationwide Children's Hospital, The Ohio State University, Columbus.
authors.forename: Michelle
authors.initials: M
authors.lastname: Grcic
authors.affiliation: Division of Cardiology, Nationwide Children's Hospital, The Ohio State University, Columbus.
authors.forename: John
authors.initials: J
authors.lastname: Kovalchin
authors.affiliation: Division of Cardiology, Nationwide Children's Hospital, The Ohio State University, Columbus.
authors.forename: Carolyn M
authors.initials: CM
authors.lastname: Wilhelm
authors.affiliation: Division of Rheumatology, Nationwide Children's Hospital, The Ohio State University, Columbus.
authors.forename: Cagri
authors.initials: C
authors.lastname: Yildirim-Toruner
authors.affiliation: Division of Cardiology, Nationwide Children's Hospital, The Ohio State University, Columbus.
authors.forename: Karen
authors.initials: K
authors.lastname: Texter
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D003330:Coronary Vessel Anomalies / Q000150:complications; D003937:Diagnosis, Differential; D004282:Documentation / Q000379:methods*; D004452:Echocardiography; D057286:Electronic Health Records; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007223:Infant; D009080:Mucocutaneous Lymph Node Syndrome / Q000145:classification / Q000150:complications / Q000000981:diagnostic imaging* / Q000188:drug therapy*; D010641:Phenotype; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous