Management of KAWASAKI disease.

Children suffering from the condition are usually cared for as inpatients on paediatric or paediatric-cardiology units and put on bed rest, due to the risk of myocardial events.

]The mainstays of management are the use of aspirin and intravenous immunoglobulin (IVIg) to reduce fever, and myocardial inflammation and to prevent or ameliorate cardiac sequelae (the main cause of morbidity and mortality associated with the condition).[/color]

Follow-up echocardiography is useful in determining whether or not there have been any coronary artery complications.

Percutaneous coronary intervention and coronary artery bypass grafting are usually avoided, particularly in the very young, as they are associated with relatively poor outcomes in the long term, in terms of maintenance of coronary artery perfusion; results in children aged >12 years are more encouraging.

Aspirin

The routine use of aspirin in the management of febrile children is not recommended due to the danger of Reye’s syndrome.

However, in Kawasaki disease, the antiplatelet and antipyretic effect of the drug provides the rationale for its use. The drug is used widely in a variety of high-dose and low-dose regimens, both in the acute and subsequent phases, and as long-term prophylaxis against coronary events in those who have coronary artery aneurysms.
There is a scant evidence base to support its use and little useful research to decide on the optimal regimen. The use of aspirin in Kawasaki disease does seem to be gaining favour.

Aspirin is firstly used in high dose for its anti-inflammatory properties and then in low dose for its anti-thrombotic effects.

One study has suggested that its use in the acute phase had no effect on preventing the failure of IVIg therapy, the formation of coronary artery aneurysms or in shortening the duration of fever.
A Cochrane review concludes that until good-quality randomised controlled trials are carried out, there is insufficient evidence to indicate whether children with Kawasaki disease should continue to receive aspirin as part of their treatment regimen.

IVIg

IVIg has been shown to reduce the incidence of coronary artery aneurysms from about 25% in the untreated to about 1-10% in treated patients in some series.
However, recent re-evaluations indicate that, although it remains an effective therapy, its potency may have been overstated, and that the timing of the infusion is critical in determining its efficacy.
There is no firm consensus on the optimal treatment regimen with some advocating high-dose therapy, and others suggesting that low-dose 1 g/kg regimens given as a single dose are just as effective. A recent randomised trial supports the view that a low-dose IVIg regimen has equal efficacy to a high-dose regimen in preventing coronary artery aneurysms.

Adjunctive therapy in refractory cases

The following agents have all been used in refractory cases:
Pentoxifylline.
Corticosteroids (avoided routinely as thought to increase complication rate).
Ulinastatin (human trypsin inhibitor derived from urine).
Abciximab (platelet glycoprotein IIb/IIIa receptor inhibitor).
Infliximab (monoclonal antibody acting as tumour necrosis factor-alpha antagonist).
Low-dose methotrexate (further trials called for).
On occasion, clopidogrel, dipyridamole and low-molecular weight heparins and/orwarfarin are used to treat children who develop large coronary artery aneurysms as prophylaxis against coronary events.

G Mohan.