Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease.
Luisa Berenise Gamez-GonzalezIsabel Moribe-QuinteroMartin Cisneros-CastoloJavier Varela-OrtizMireya Muñoz-RamírezMartin Garrido-GarcíaMarco Antonio Yamazaki-NakashimadaPublished in: Pediatrics international : official journal of the Japan Pediatric Society (2018)
The presence of coronary aneurysms was significantly and positively correlated with male gender, IVIG resistance, inotrope treatment, cardiac failure, abdominal pain and neurological symptoms. IVIG-resistant patients had higher neutrophil : lymphocyte ratio. Abdominal symptoms, hypoalbuminemia and elevated C-reactive protein were present in almost all of the patients. Multisystem involvement with atypical presentation in KDSS is frequent. An important differential diagnosis is TSS. Mechanical ventilation, gastrointestinal and neurological symptoms were associated with IVIG resistance and the presence of coronary aneurysms. The first line of treatment includes IVIG and pulse corticosteroids; in severe cases, infliximab, anakinra, cyclosporine or plasmapheresis are alternative treatment options.
Keyphrases
- end stage renal disease
- mechanical ventilation
- ejection fraction
- newly diagnosed
- chronic kidney disease
- coronary artery disease
- coronary artery
- abdominal pain
- prognostic factors
- intensive care unit
- blood pressure
- acute respiratory distress syndrome
- mental health
- left ventricular
- patient reported outcomes
- aortic stenosis
- physical activity
- atrial fibrillation
- transcatheter aortic valve replacement
- cerebral ischemia