Published online April 27, 2009
PEDIATRICS Vol. 123 No. 5 May 2009, pp. e783-e789 (doi:10.1542/peds.2008-1871)

This Article Full Text Full Text (PDF) Submit a response View responses Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services E-mail this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My File Cabinet Download to citation manager Request Permissions Citing Articles Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Kanegaye, J. T. Articles by Burns, J. C. Search for Related Content PubMed PubMed Citation Articles by Kanegaye, J. T. Articles by Burns, J. C. Related Collections Infectious Disease & Immunity Social Bookmarking                         What's this?

ARTICLE

Recognition of a Kawasaki Disease Shock Syndrome

John T. Kanegaye, MD^a,b, Matthew S. Wilder, MD^a, Delaram Molkara, MD^a,c, Jeffrey R. Frazer, MD^a,c, Joan Pancheri, RN, BSN, CCRC^d, Adriana H. Tremoulet, MD^a,e, Virginia E. Watson, MD^a, Brookie M. Best, PharmD, MAS^a,f and Jane C. Burns, MD^a,g

^a Department of Pediatrics, School of Medicine
^f Department of Pharmacy, Skaggs School of Pharmacy and Pharmaceutical Sciences, University of California, San Diego, La Jolla, California; Divisions of
^b Emergency Medicine
^c Cardiology
^e Infectious Diseases
^g Allergy, Immunology, and Rheumatology
^d Center for Pediatric Clinical Research, Rady Children's Hospital San Diego, San Diego, California

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.

---

Key Words: Kawasaki disease (mucocutaneous lymph node syndrome) • shock • echocardiography • ventricular function

Abbreviations: KD—Kawasaki disease • IVIG—intravenous immunoglobulin • KDSS—Kawasaki disease shock syndrome

---

Accepted Jan 12, 2009.

CiteULike    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?

eLetters:

Read all eLetters

Shock syndrome, or macrophage activation syndrome, in children with Kawasaki disease

Randy Q Cron

Pediatrics Online, 13 May 2009 [Full text]