**5. Clinical assessment**

The typical presentation is of an irritable child who has an unremitting high fever (often >39C) for 5 or more days, and some or all classical diagnostic of the clinical features (Table 1). The diagnosis can also be made by experienced clinicians on the fourth day of fever if classical diagnostic criteria are met.1 In most children the clinical features appear sequentially. Specific features for KD include perineal desquamation and erythema or crusting around the Bacille Calmette Guerin (BCG) inoculation site. Other extracardiac features such as respiratory or gastrointestinal symptoms occur frequently and make the diagnosis difficult.

The diagnosis requires the presence of fever for at least 5 days, and at least four of the following five criteria:


Table 1. The diagnostic criteria for Kawasaki disease (Newburger et al. 2004)

Incomplete KD should be considered in children who have an unexplained prolonged febrile illness and have not met diagnostic criteria (Newburger et al., 2004). This occurs more commonly in children less than 6 months or greater than 5 years of age, with the younger age group more likely to present with fewer clinical features and also a higher incidence of coronary artery abnormalities (Genizi et al., 2003).

Harnden, 2005). The epidemiology of Kawasaki disease, clustering of cases, community outbreaks and epidemics in the 1980s, support the hypothesis that an unknown infectious agent (or agents) triggers an abnormal inflammatory response in genetically susceptible individuals. Both conventional antigens and bacterial superantigens have been implicated as causative triggers in Kawasaki disease, however the triggering pathogen(s) remain

Initially, activated inflammatory cells, particularly monocytes, macrophages, T-cells, and subsequently platelets, adhere to the endothelial cells that line medium-size elastic arteries. Other mediators contribute to destruction of the extra-cellular matrix, leading to vessel dilatation, and subsequent smooth muscle proliferation in the media contributes to later pathology. There is destruction of the intimal layer of the affected artery with inflammatory infiltrate during the acute phase, and histologic findings of myocarditis and fibrosis are

The typical presentation is of an irritable child who has an unremitting high fever (often >39C) for 5 or more days, and some or all classical diagnostic of the clinical features (Table 1). The diagnosis can also be made by experienced clinicians on the fourth day of fever if classical diagnostic criteria are met.1 In most children the clinical features appear sequentially. Specific features for KD include perineal desquamation and erythema or crusting around the Bacille Calmette Guerin (BCG) inoculation site. Other extracardiac features such as respiratory or

The diagnosis requires the presence of fever for at least 5 days, and at least four of the



Incomplete KD should be considered in children who have an unexplained prolonged febrile illness and have not met diagnostic criteria (Newburger et al., 2004). This occurs more commonly in children less than 6 months or greater than 5 years of age, with the younger age group more likely to present with fewer clinical features and also a higher


Table 1. The diagnostic criteria for Kawasaki disease (Newburger et al. 2004)

incidence of coronary artery abnormalities (Genizi et al., 2003).

found in virtually all cases with Kawasaki disease (Yutani et al., 1981).

gastrointestinal symptoms occur frequently and make the diagnosis difficult.

unknown.

**4. Pathogenesis** 

**5. Clinical assessment** 

following five criteria:

illness onset)


or injected pharynx


Kawasaki disease shock syndrome has been recently described and is characterised by hypotension and haemodynamic instability, often requiring intensive care. (Dominguez et al., 2008; Yim et al., 2010) These patients may be at increased risk of delayed diagnosis and treatment, refractory disease and more severe coronary artery involvement (Kanegaye et al., 2009).
