Background Kawasaki disease (KD) is the commonest acquired heart disease in the western world. The incidence is rising in all countries, doubling every 20 years. The last BPSU survey was 25 years ago and we wished to update the data and find if there was a link between phenotype, ethnicity, location, and outcome.
Methods The survey covered all children in the UK and Ireland who had a new diagnosis of KD between February 2013 and February 2015. Using BPSU ‘orange card’ methodology, patients were registered anonymously by their paediatrician who then completed a study questionnaire. Ethical and national information governance board approval was obtained avoiding need for patient consent.
Results 600 children with KD were notified, of whom 552 satisfied inclusion criteria, 322 boys and 215 girls (15 unknown), age range 2 months to 15.5 years. 389 had complete, 119 incomplete and 42 atypical disease. In those under 1 year of age, 48% had atypical disease (all with coronary artery changes by definition). 21% had incomplete disease. 355 (68%) were Caucasian, 47 (9%) were black African, 8% Indian Asian, 4% were Chinese/Japanese. One third of children were older than 5 years at presentation. Peak incidence in both years was February-March. 80% were seen by the GP at median (range) 2 (0–27) days from first symptom. Median time from onset to diagnosis was 7 (0–36) days and median time from admission to diagnosis was 1 (0–25) days. 24% of 523 had one or more dilated coronary arteries, the left in 90, the right in 64 and the circumflex in 17. 503/532 received immunoglobulin, 360/389 with complete KD, 42/42 with atypical and 101/119 with incomplete. 472/513 received high dose and 460/495 had low dose aspirin. There were 3 deaths and 49/549 children who survived had sequelae.
Conclusion The population-based data on this rare disease are presented for a 2 year period from the UK. This shows new information on ethnicity, phenotype, management and outcomes. There is a high rate of coronary artery disease suggesting an under-diagnosis or under-treatment of this important condition.
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.