title: Recognition of a Kawasaki disease shock syndrome.
abstract: OBJECTIVE
  We sought to define the characteristics that distinguish Kawasaki disease shock syndrome from hemodynamically normal Kawasaki disease.
  
  
  METHODS
  We collected data prospectively for all patients with Kawasaki disease who were treated at a single institution during a 4-year period. We defined Kawasaki disease shock syndrome on the basis of systolic hypotension for age, a sustained decrease in systolic blood pressure from baseline of > or =20%, or clinical signs of poor perfusion. We compared clinical and laboratory features, coronary artery measurements, and responses to therapy and analyzed indices of ventricular systolic and diastolic function during acute and convalescent Kawasaki disease.
  
  
  RESULTS
  Of 187 consecutive patients with Kawasaki disease, 13 (7%) met the definition for Kawasaki disease shock syndrome. All received fluid resuscitation, and 7 (54%) required vasoactive infusions. Compared with patients without shock, patients with Kawasaki disease shock syndrome were more often female and had larger proportions of bands, higher C-reactive protein concentrations, and lower hemoglobin concentrations and platelet counts. Evidence of consumptive coagulopathy was common in the Kawasaki disease shock syndrome group. Patients with Kawasaki disease shock syndrome more often had impaired left ventricular systolic function (ejection fraction of <54%: 4 of 13 patients [31%] vs 2 of 86 patients [4%]), mitral regurgitation (5 of 13 patients [39%] vs 2 of 83 patients [2%]), coronary artery abnormalities (8 of 13 patients [62%] vs 20 of 86 patients [23%]), and intravenous immunoglobulin resistance (6 of 13 patients [46%] vs 32 of 174 patients [18%]). Impairment of ventricular relaxation and compliance persisted among patients with Kawasaki disease shock syndrome after the resolution of other hemodynamic disturbances.
  
  
  CONCLUSIONS
  Kawasaki disease shock syndrome is associated with more-severe laboratory markers of inflammation and greater risk of coronary artery abnormalities, mitral regurgitation, and prolonged myocardial dysfunction. These patients may be resistant to immunoglobulin therapy and require additional antiinflammatory treatment.
authors.affiliation: Department of Pediatrics, School of Medicine, University of California, San Diego, La Jolla, California, USA. jkanegaye@rchsd.org
authors.forename: John T
authors.initials: JT
authors.lastname: Kanegaye
authors.forename: Matthew S
authors.initials: MS
authors.lastname: Wilder
authors.forename: Delaram
authors.initials: D
authors.lastname: Molkara
authors.forename: Jeffrey R
authors.initials: JR
authors.lastname: Frazer
authors.forename: Joan
authors.initials: J
authors.lastname: Pancheri
authors.forename: Adriana H
authors.initials: AH
authors.lastname: Tremoulet
authors.forename: Virginia E
authors.initials: VE
authors.lastname: Watson
authors.forename: Brookie M
authors.initials: BM
authors.lastname: Best
authors.forename: Jane C
authors.initials: JC
authors.lastname: Burns
mesh_terms: D002648:Child; D002675:Child, Preschool; D003422:Critical Care; D005260:Female; D006801:Humans; D007022:Hypotension / Q000209:etiology*; D007223:Infant; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D011446:Prospective Studies; D016896:Treatment Outcome; D014463:Ultrasonography