Pediatric Case of the Week 13: Let the Bad Times Roll... WEEKEND UPDATE

Kawasaki's disease is never as easy as it was initially presented (pictures of all the physical exam findings and references to a motorcycle company bearing the same name.) We, as emergency department providers, are at a significant disadvantage when trying to make this diagnosis. Frankly, KW is difficult to diagnose.

For starters, KW is relatively rare, therefore not often on the forefront of our differential diagnosis in patients who present with conjunctivitis and fever. In fact, you can imagine these patients being triaged as "pink eye" or a "viral syndrome."  Also, Kawasaki's can present with some variation (in other words) symptoms may evolve at different times rather than all at once. Unfortunately, we get ONE SHOT to make the right call. Missing this diagnosis can lead to tragic outcomes: multiple coronary artery aneurysms resulting in significant morbidity and mortality.  So please, consider KD in any child with prolonged fever (technically 5 days) and any 4 of the following:

1. Conjunctival injection, usually bilateral and nonpurulent
2. Cervical lymphadenopathy >1.5cm
3. Oral mucosal changes (cracking or bleeding lips) or strawberry tongue
4. Polymorphous rash and /or swelling of the hands and feet with late desquamation
5. No other diagnosis to explain these symptoms


The differential includes staphylococcal and streptococcal toxic shock syndromes, streptococcal scarlet fever, staphylococcal scalded skin syndrome, measles, febrile viral exanthems, adenovirus infection, hypersensitivity reactions (including Stevens-Johnson syndrome, erythema multiforme minor and serum sickness), and systemic onset juvenile rheumatoid arthritis.

BUT THE POINT OF THIS EXERCISE IS TO GET YOU TO THINK KAWASAKI FIRST, SECOND, AND THIRD in kids with combinations of these symptoms.  As a paranoid ED provider, I'd rather you consider this disease and talk yourself out of it. This is truly an incident in which it is beneficial to think WORST FIRST.


KD presents mostly in kids age 1-2 (rarely before 3 months or after age 8). KD is a systemic vasculitis with preference for the coronary arteries.  Laboratory studies should be directed at evaluating this process: ESR, CRP, Platelets, WBC, AST, ALT; All can be elevated but none are pathognomonic.  Additionally, 2 urine proteins: meperin A and filamin C may become usesful as biomarkers of KW.  (As of yet they are not validated in any diagnostic algorithm.) 

1. Henderson D. Kawasaki Disease Diagnosed by Urine Proteins? Medscape Medical News. December 26, 2012. Available at http://www.medscape.com/viewarticle/776728. Accessed January 8, 2013.
2. Kentsis A, Shulman A, Ahmed S, Brennan E, Monuteaux MC et al. Urine proteomics for discovery of improved diagnostic markers of Kawasaki disease. EMBO Mol Med. Dec 20 2012;[Medline].

If you are uncertain, I would get an ECHO in the ED (or transfer the patient to someplace with this capability.)  Worth noting however, if it is early in the disease process, there may not be echocardiographic evidence of aneurysm.

So, either way, admit patients who you suspect may have Kawasaki's or "incomplete Kawasaki's" and start IVIG ASAP  2g/kg over 8-12 hours.  Also, high dose aspirin is recommended 80-100 mg/kg/day divided into 4 doses.

If you are going to be seeing kids, you need to know about Kawasaki's disease.  You need to think about Kawasaki's disease.   You need to make this diagnosis or get this patient to someone who can.

Hopefully with the right training, thoughtfulness, foresight, and a little bit of luck, you can make the diagnosis and treatment of Kawasaki's Disease look EASY.

BUT PLEASE...WEAR A HELMET!!!