Polymorphism of transmembrane region of MICA gene and Kawasaki disease

Kawasaki disease is a febrile disease of children complicated with vasculit is of the coronary arteries and potential aneurysm formation. It has been r ecognized worldwide and appears to be increasing in frequency. Studies have found that Kawasaki disease is associated with major histocompatibility co mplex (MHC) class I B antigens. The MHC-class-I-chain-related gene A (MICA) is located near HLA-B. It has a triplet repeat microsatellite polymorphism in the transmembrane region. We investigated the microsatellite polymorphi sm in children with Kawasaki disease and controls. Seventy children (46 boy s), age at diagnosis 1.68 +/- 1.69 years, with Kawasaki who were treated wi th aspirin as well as intravenous gamma-globulin were enrolled. Control sub jects consisted of 154 children (87 boys), age 2.81 +/- 2.12 years. Phenoty pe frequency of allele A4 in patients with aneurysm formation was significa ntly lower than in patients without aneurysms [relative risk (RR) = 0.06, 9 5% confidence interval (CI) = 0.01-0.48, p = 0.00469, pc = 0.0232] and show ed a similar tendency when compared with controls. Gene frequency of allele A4 was also significantly lower in patients who developed aneurysms than i n patients who did not (RR = 0.07, 95% CI = 0.01-0.57, p = 0.0057, pc = 0.0 282). Gene frequency of allele A5 showed a tendency to be higher in patient s who developed aneurysms than in controls (RR = 2.35, 95% CI = 0.98-5.63, p = 0.0486, pc = 0.220). Allele A5.1 tended to be negatively associated wit h Kawasaki disease (RR = 0.57, 95% CI = 0.35-0.93, p = 0.022, pc = 0.105). Our study showed that allele A4 was negatively associated with coronary ane urysm formation in Kawasaki disease. This suggests that allele A4 protects the children with Kawasaki disease from developing coronary aneurysms after aspirin and gamma globulin therapy. Copyright (C) 2000 S. Karger AG,Basel.