Recognition of a Kawasaki Disease Shock Syndrome

American Academy of Pediatrics (AAP) - Tập 123 Số 5 - Trang e783-e789 - 2009
John T. Kanegaye1,2, Matthew S. Wilder1, Delaram Molkara3,1, Jeffrey R. Frazer3,1, Joan Pancheri4, Adriana H. Tremoulet1,5, Virginia E. Watson1, Brookie M. Best1,6, Jane C. Burns7,1
1Department of Pediatrics, School of Medicine
2Emergency Medicine
3Cardiology
4Center for Pediatric Clinical Research, Rady Children's Hospital San Diego, San Diego, California
5Infectious Diseases
6Department of Pharmacy, Skaggs School of Pharmacy and Pharmaceutical Sciences, University of California, San Diego, La Jolla, California; Divisions of
7Allergy, Immunology, and Rheumatology

Tóm tắt

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 ≥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.

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American Academy of Pediatrics (AAP)

Tập 123 Số 5

e783-e789

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