Objective: as Kawasaki Disease (KD) has gained the worldwide interest due the new clinic form and differences between several countries we performed to review of epidemiological, clinics, outcome´s features of firsttime and recurrence´s KD of 1 patient (pt) despite the rarely as this case.
Case reports: Brazil boy, 10 years that since 3y had severe typical clinic form´s KD, Harada´s score:7 and cardiac´s exams not altered. A currently the standard therapy for the acute stage (2g/kg) gama-globulin (gIg) and after 30mg/kg/dose/3x´s methylprednisolone were used due to non responsive, though controversy about steroid´s efficacy. In 2º month (mo) all exams were normal, but the Echo showed giant aneurysm in the all trunk of left coronary artery (23x19mm). In 3ºmo he had aphasia due to left and right lose hearing by neurossensorial deficiency and remained using antiplaquetary aspirin´s doses since then. The pt stayed well until to 8y when developed the KD typical recurrence and newly underwent to conventional therapy. In followings founded thrombosis asymmetric left and right (bigger) axillary artery aneurysm with significant distal pulse reduction. Anticoagulation therapy and 30 mg/kg/day aspirin were maintained. In last 2y the pt has been well, memmbers preserved distal blood flow without hearing.
Conclusions: except the echo, the usual exams were poor in to shown complications and we specula an abnormal immunologic response and genetically susceptibility individuals in the regard. To discovery of the KD exact cause better therapy and prevention might appears for these severe unusual form of disease.