Background Kawasaki disease (KD) is an acute, self-limited vasculitis commonly affecting children <5 years. Over the last years several cases of KD associated with hemodynamic instability or Kawasaki disease shock syndrome (KDSS) have been described
Methods Retrospective descriptive study of patients diagnosed with KDSS in our hospital (January 2009 - December 2014). KDSS was defined as those patients with KD who showed clinical signs of hypoperfusion (cold extremities, oliguria, reduced pulse amplitude, prolonged capillary refill time >2 second, tachycardia and/or hypotension for age (<2 SD of the mean) and need for ICU admission.
Results During a six-year period a total of 5 patients met the criteria for KDSS. 4/5 of the children presented at an atypical age (<6 months or >5 years). 3/5 of the children presented with incomplete KD. 3/5 did not respond to the first dose of IVIG and 4/5 received high dose steroids. All children achieved full remission of symptoms. Only one patient has cardiac sequelae
Conclusions KDSS is an unusual but severe form of KD presentation. In children with suspected toxic shock, negative cultures and/or poor response to antibiotics, KDSS should be considered as possible differential diagnosis, even when the classical criteria of KD are not met. Early diagnosis is essential in order to initiate appropriate and aggressive therapy thereby reducing the risk of coronary arteries aneurysm, more frequently occurring in children with KDSS.
Dominguez et al. Pediatrics 2008; 122; e786e790.
Kanegaye et al. Pediatrics 2009; 123;e783-e789.
Disclosure of Interest None declared