KAWASAKI-DISEASE IN INFANTS LESS-THAN ONE-YEAR OF AGE

Objective: To identify risk factors for severe sequelae, analyze disea se characteristics, and assess efficacy of intravenously administered immune globulin (IVGG) therapy in infants less than 12 months of age w ith Kawasaki disease. Design: Retrospective chart review of children l ess than 12 months of age with Kawasaki disease between 1980 and 1993. Results: Of 443 patients with Kawasaki disease, 57 (13%) were less th an 1 year of age, including 14 (3%) less than 6 months. Age at onset w as a predictor of the development of coronary artery aneurysms (CAA) a nd of giant (>8 mm) aneurysms: 11 (79%) of 14 children <6 months and 1 7 (44%) of 39 children 6 to 12 months of age acquired CAA (p = 0.06), and 5 (37%) of 14 children <6 months and 2 (5%) of 39 children 6 to 12 months of age acquired giant CAA (p <0.01). No specific clinical or l aboratory features predicted the development of CAA, which was found i n 7 (29%) of 24 patients treated with IVGG by illness day 10 and in 21 (73%) of 29 patients treated after day 10 or never treated with IVGG (p <0.01). Only 1 (4%) of 24 patients treated by day 10 but 6 (21%) of 29 children treated after day 10 or never treated with IVGG acquired giant CAA (p = 0.01). Persistent (>1 year) CAA were present in 4 (17%) of 24 IVGG-treated children by day 10 and in 14 (48%) of 29 children not treated by day 10 or never treated with IVGG (p <0.025). There was no difference in outcome if IVGG was given by illness day 7 or on ill ness days 8 to 10. Conclusions: Patients with Kawasaki disease less th an 6 months of age are at particularly increased risk of having CAA an d giant CAA. Therapy with IVGG, given by illness day 10, is associated with substantial reduction in the frequency of CAA and giant CAA in t his high-risk population.