MANAGEMENT OF KAWASAKI-DISEASE IN THE BRITISH-ISLES

Kawasaki disease in the British Isles was surveyed by an active report ing scheme, based on all cases reported to the British Paediatric Surv eillance Unit that were diagnosed between 1 January and 31 December 19 90. The study was prompted by the need to investigate the high case fa tality rate of Kawasaki disease of 2% observed in 1988. One hundred an d sixty three patients were identified of whom six (3.7%) died. Forty five children (28%) suffered cardiac complications of which 39 (24%) w ere coronary artery abnormalities; five children were diagnosed at pos tmortem examination, and coronary artery abnormalities were detected b y echocardiography in 34. One hundred and forty nine children (93%) ha d echocardiography. High thrombocytosis, leucocytosis, duration of fev er, and younger age were associated with the presence of coronary arte ry abnormalities. Erythrocyte sedimentation rate, sex, and the number of diagnostic criteria were not. One hundred and thirty three children (87%) received aspirin. Ninety three children (61%) received intraven ous gammaglobulin (IVGG). Children were more likely to receive IVGG if they had thrombocytosis or typical Kawasaki disease. The incidence of coronary artery abnormalities was found to be similar in those treate d with IVGG (29%) and those untreated (20%), including those treated w ithin 10 days of onset. This may have reflected selection of the more serious cases to receive IVGG or that Kawasaki disease in the British Isles is a different illness to that experienced elsewhere. It may be, however, that IVGG is less effective in the treatment of British pati ents with Kawasaki disease than has been the experience in the United States and Japan. These observations emphasise the need for a therapeu tic trial of treatment modalities for Kawasaki disease in the UK and t he Republic of Ireland.