Recognition of a Kawasaki Disease Shock Syndrome

Abstract

Abstractobjective-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%] 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. KeywordsKawasaki disease (mucocutaneous lymph node syndrome); shock; echocardiography; ventricular function What's Known on This SubjectAlthough the cardiac complications of KD are well known, hemodynamic instability is unusual in the acute phase of illness, except as a complication of intravenous IVIG administration. What This Study AddsWe have observed shock and hypotension with increasing frequency in newly diagnosed KD. Compared with hemodynamically normal KD, KDSS is associated with increased inflammation, platelet consumption, IVIG resistance, coronary artery abnormalities, mitral regurgitation, and myocardial dysfunction.KAWASAKI disease (KD) is the most common cause of acquired heart disease in the pediatric age group and results in permanent damage to the coronary arteries in up to 25% of untreated children. Although no patients with KD presented with shock in the prospective database before 2003, we postulated that earlier cases might have been misdiagnosed and therefore never included in our database. To identify patients with KD who were treated for shock but were misclassified with other diagnoses, we conducted a computerized search, on the basis of International Classification of Diseases, Ninth Revision codes, for patients discharged from the critical care unit between January 1, 2001, and Augus...

Immunoglobulin Resistance in Kawasaki Disease

“…20,21 Several factors have been reported to be associated with refractory cases, including younger age, 22 male gender, 23 earlier presentation, 3 initial treatment before day 4 of fever, lower platelets, 3,22 lower hemoglobin, 3,24 higher white blood cell 25 and bands counts, 3 elevated C-reactive protein 3,19,22,24,26,27 and erythrocyte sedimentation rate, 18 elevated lactate dehydrogenase, 19,24 abnormal liver function, 3,19,22,23,26,27 lower serum albumin 23,28,29 and certain biomarkers, 30-33 among others. 3 Thus, failure to reduce inflammation and fever after immunoglobulin therapy are markers for immunoglobulin resistance in Kawasaki disease.…”

Kawasaki disease shock syndrome: three cases

“…Kawasaki disease (KD) is a disease of unknown aetiology affecting medium sized blood vessels 1 . It is the commonest type of acquired heart disease in children and 15-25% of patients have coronary artery involvement 2 .…”

“…It is the commonest type of acquired heart disease in children and 15-25% of patients have coronary artery involvement 2 . Kawasaki disease shock syndrome (KDSS) is a rare haemodynamically unstable phenomenon in the acute stages of KD which was first described by Kenagaye et al in 2009 1 . Possible causes for the haemodynamic instability are vasculitis with capillary leak, myocardial dysfunction and cytokine dysregulation 1 .…”

“…Kawasaki disease shock syndrome (KDSS) is a rare haemodynamically unstable phenomenon in the acute stages of KD which was first described by Kenagaye et al in 2009 1 . Possible causes for the haemodynamic instability are vasculitis with capillary leak, myocardial dysfunction and cytokine dysregulation 1 . KDSS may be misdiagnosed as it mimics other causes of shock 3 .…”

Kawasaki disease shock syndrome: a rare and severe complication of Kawasaki disease

“…The cause of KDSS is unknown as KD, but capillary leakage due to vasculitis, myocardial dysfunction, and cytokine dysregulation are thought to be responsible. The patients are hypotensive and show signs and symptoms of poor perfusion 3 . This report describes two children with KDSS, the first one had the diagnosis of KD but the second one initially had the diagnosis of toxic shock syndrome.…”