G284 Kawasaki disease bpsu survey in great britain and ireland

Abstract

Introduction Kawasaki disease (KD) is the commonest cause of acquired heart disease in the western world. We report here on the preliminary data for the first year of a 2-year national survey in Great Britain and Northern Ireland. Methods Using standard BPSU methodology, children with Complete KD (for the first year) presenting from January 1st to December 31st 2013 were studied with their treatments and complications. The steering committee reviewed cases that were unclear or incomplete. Results Excluding incomplete KD and incomplete data sets, there were 180 with complete KD (15/month). Peak presentation was in March, boy:girl ratio was 1.8 and 69% were 1 to 4 years old. The highest incidence was in children under 5 years especially in London and East Anglia (5.6 / 100,000) and lowest in the Midlands (2.8/100,000). Highest incidence was in Chinese (21.9/100,000), Black African and Caribbean (14.5/100,000) and Mixed ethnicity (15.3/100,000). 81% saw a GP at median 2 days after fever commencement and admission was median (range) of 2 (0–27) days later. Fever duration was 6 (1–28) days. 97% had rash, usually at the start of fever, which was on the trunk or widespread in 89% and was macular in 75%. Bilateral non-purulent conjunctivitis (91%) occurred at 4 (0–27) days and mucositis (97%) at 5 (0–27) days. Periphery involvement was up to 32 days in 156 (86%). Lymphadenopathy was least common (65%) often after 5 days. The BCG scar was inflamed in 11. CRP ranged from 1–501 mg/l, albumin from 17–45g/l, lowest platelet counts were below 150 × 10^9/l in 14. 31 (22%) had dilated coronary arteries, 9 had pericardial effusion and 12 had ECG abnormalities. Intravenous immunoglobulin (2g/kg) given within 7 days to 95%, but not to 9 complete cases (delayed diagnosis). Second dose was given to 13 children. High dose aspirin (30–50mg/kg) followed by low dose aspirin was given to 92%. 19 had sequelae, 15 with persistence of coronary artery dilation, with no deaths. Conclusions These preliminary results show a high incidence of coronary artery disease with the old guideline for KD and give new data on seasonality, ethnicity and correlation with clinical symptoms.