Characteristics of Fatal Atypical Kawasaki Disease – Less Contribution of Coronary Artery Aneurysm ?

  • Mamoru Ayusawa, Department of Pediatrics and Child Health, Nihon University School of Medicine, Japan
  • Takahiro Nakamura, Department of Pediatrics and Child Health, Nihon University School of Medicine, Japan
  • Junji Fukuhara, Department of Pediatrics and Child Health, Nihon University School of Medicine, Japan
  • Masaharu Matsumura, Japan
  • Osamu Abe, Department of Pediatrics and Child Health, Nihon University School of Medicine, Japan
  • Hiroshi Kanamaru, Department of Pediatrics and Child Health, Nihon University School of Medicine, Japan
  • Naokata Sumitomo, Department of Pediatrics and Child Health, Nihon University School of Medicine, Japan
  • Yoshikazu Nakamura, Department of Public Health, Jichi Medical College, Japan

    • Objectives: Atypical Kawasaki disease (AKD) occasionally present serious course and fatal outcome despite of 4 or less principle signs. To clarify characteristics of fatal cases with AKD, demographic data of them reported to Japan Nationwide Surveillance for Kawasaki Disease (JNSKD) were summarized. Methods: Ten fatal cases were reported as AKD (fAKD) including 3 females among 69,221 registrations in JNSKD during 1993-2002, while 34 fatal cases including 6 females were reported as ‘typical’ (fTKD). Age, duration until death, intravenous immunoglobulin (IVIG), coronary artery aneurysm (CAA) and reported clinical cause of death were compared between fAKD and fTKD. Results: Average age and duration until death in fAKD and fTKD were 13.4 vs. 16.3 months (NS) and 74.5 vs. 151 days (p<0.05), respectively. Initial IVIG day from onset and rate of IVIG treatment in fAKD and fTKD were 4.0 vs. 4.9 days (NS), and 4 (40%) vs. 2 (5.9%) cases (p<0.05), respectively. CAA was reported 3 cases (30%) in fAKD and 16 cases (47.1%) including 13cases of giant CAA. Reported causes of fAKD were 2 encephalopathy’s, 2 myocarditis', 1 aortic insufficiency, 1 heart failure, 1 multi-organ failure, 1 SIDS with moderate coronary aneurysm, and 1 drowning and 1 sudden death, without CAA. In contrast, 9 cases (26.4%) in fTKD were reported as death of acute myocardial infarction. Conclusion: Duration until death from onset of fAKD was shorter than that of fTKD. Indication of IVIG was insufficient. Although mild or moderate CAA complicate with AKD, its fatal contribution is considered to be negative.