Document Detail


Recognition of a Kawasaki disease shock syndrome.
MedLine Citation:
PMID:  19403470     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
John T Kanegaye; Matthew S Wilder; Delaram Molkara; Jeffrey R Frazer; Joan Pancheri; Adriana H Tremoulet; Virginia E Watson; Brookie M Best; Jane C Burns
Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural    
Journal Detail:
Title:  Pediatrics     Volume:  123     ISSN:  1098-4275     ISO Abbreviation:  Pediatrics     Publication Date:  2009 May 
Date Detail:
Created Date:  2009-04-30     Completed Date:  2009-05-26     Revised Date:  2010-09-24    
Medline Journal Info:
Nlm Unique ID:  0376422     Medline TA:  Pediatrics     Country:  United States    
Other Details:
Languages:  eng     Pagination:  e783-9     Citation Subset:  AIM; IM    
Affiliation:
Department of Pediatrics, School of Medicine, University of California, San Diego, La Jolla, California, USA. jkanegaye@rchsd.org
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MeSH Terms
Descriptor/Qualifier:
Child
Child, Preschool
Critical Care
Female
Humans
Hypotension / etiology*
Infant
Male
Mucocutaneous Lymph Node Syndrome / complications*,  physiopathology,  ultrasonography
Prospective Studies
Treatment Outcome
Grant Support
ID/Acronym/Agency:
K24 HL074864-04/HL/NHLBI NIH HHS; K24 HL074864-05/HL/NHLBI NIH HHS; K24-HL074864/HL/NHLBI NIH HHS; R01 HL069413-06/HL/NHLBI NIH HHS; R01 HL069413-07/HL/NHLBI NIH HHS; R01 HL69413/HL/NHLBI NIH HHS
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