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Paediatrics: Kawasaki disease

Kawasaki disease is the commonest cause of acquired heart disease in children in the UK.

Kawasaki disease


Kawasaki disease is the commonest cause of acquired heart disease in children in the UK. It affects 3.4/100,000 children under 5yrs (boys > girls) and in the UK mortality is 3.7%. It is a systemic vasculitic disease with coro-nary arteritis leading to coronary artery aneurysms as the most important complication (20–30%). Other complications include coronary thrombo-sis, myocardial infarction, and dysrrhythmias.


Diagnostic criteria


The diagnosis can be made in children with fever (>38.5*C) present for at least 5 days, without other explanation, in the presence of 4 of the 5 following criteria. Criteria may not be present at the same time (history is important) and misery is a very common feature.


   Bilateral congestion of the ocular conjunctivae (94%): non-purulent.

   Changes of the lips and oral cavity with at least one of the following: dryness, erythema, fissuring of lips (70%); strawberry tongue (71%); diffuse erythema of oral and pharyngeal mucosa without discrete lesions (70%).

   Changes of the extremities with at least one of the following: erythema of palms and soles (80%); indurative oedema (67%); periungual desquamation of fingers and toes (29%).

   Polymorphous exanthem (92%).

   Non-suppurative cervical lymphadenopathy >1.5cm (42%).


Note: The percentage values indicate the proportion of patients manifest-ing this clinical sign within the first 10 days after onset of fever.



Differential diagnosis


The differential diagnosis for Kawasaki disease includes the following:


   Streptococcal and staphylococcal toxin-mediated diseases


   Adenovirus and other viral infections (enterovirus, measles)


   Drug reactions or Stevens–Johnson syndrome




   Yersinia pseudotuberculosis infection


   Rickettsial infection


   Reiter’s syndrome


·  IBD


   Post-infectious immune complex disease (e.g. post-meningococcaemia)



Associated features


In addition to the diagnostic criteria of Kawasaki disease, the other fea-tures of the condition include the following.

   Renal: urethritis with sterile pyuria.


Musculoskeletal: arthralgia and arthritis (35% of patients).

CNS: aseptic meningitis with mild CSF pleocytosis and normal CSF glucose and protein; sensorineural hearing loss (transient high frequency loss or permanent loss).


GI: diarrhoea and vomiting; hydrops of the gall bladder with or without obstructive jaundice.


Cardiac: congestive heart failure, myocarditis, pericardial effusion, arrhythmias, mitral insufficiency, acute myocardial infarction (up to 73%) within 1yr of disease.


·Coronary aneurysms: incidence of coronary artery aneurysms varies (15–25% in untreated patients) and resolution varies with age at onset and size and shape of aneurysm.




Haematology: leucocytosis with left shift common in acute phase; thrombocytosis peaks in the 3rd to 4th week; normocytic, normochromic anaemia present early and persists until inflammatory process begins to subside; reticulocyte count low.

Coagulation: increased coagulability, platelet turnover, and depleted fibrinolysis.

Urine: mononuclear cells with cytoplasmic inclusions are abundant in the urine early in the disease. These cells are not detected by dipstick methods for ‘WBC’, which only detect polymorphs.

Acute phase reactants: elevated ESR persists beyond the acute febrile period and gradually returns to normal over 1–2mths. CRP may also be elevated.

Biochemistry: elevated liver transaminases; hypoalbuminaemia.


Immunology: marked activation of circulating monocyte/macrophages; B cell activation elevated immunoglobulin production; T cell lymphopenia.


Cardiology: ECG usually normal, but strain, ischaemia, and/or infarct can be present.


Echocardiography: aneurysms may first be seen from 7–21 days post-onset of fever.




High dose IV immunoglobulin is the treatment of choice. 2g/kg over 12hr as a single infusion. Consider repeat dose after 48hr if no deferevescence.


Aspirin: 30—50mg/kg/day (divided qds) reducing to 3–5mg/kg as fever resolves.


The role of steroids and novel biological therapies is not clear.


Follow-up is very important for cardiac review.


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