Clinical fetures of kawasaki disease in school-aged children

학동기 아동에서의 가와사끼병의 임상 특징

  • Park, Eun Young (Department of Pediatrics, School of Medicine, Ewha Womans University) ;
  • Kim, Ji Hye (Department of Pediatrics, School of Medicine, Ewha Womans University) ;
  • Kim, Hae Soon (Department of Pediatrics, School of Medicine, Ewha Womans University) ;
  • Shon, Sejung (Department of Pediatrics, School of Medicine, Ewha Womans University)
  • 박은영 (이화여자대학교 의과대학 소아과학교실) ;
  • 김지혜 (이화여자대학교 의과대학 소아과학교실) ;
  • 김혜순 (이화여자대학교 의과대학 소아과학교실) ;
  • 손세정 (이화여자대학교 의과대학 소아과학교실)
  • Received : 2006.09.07
  • Accepted : 2006.09.27
  • Published : 2007.03.15

Abstract

Purpose : Kawasaki disease (KD) rarely occurs in school-aged children. We clarified the characteristics of KD in this age group to provide tips for a high index of suspicion. Methods : Features of 38 patients with KD who were 7 years of age or older were retrospectively reviewed. Results : The incidence of the KD patients ${\geq}7years$ was 4.9 percent. The ratio of male to female was 2.5:1. Of the 38 patients, nine patients (24.0 percent) were diagnosed with typical KD and 29 patients (76.0 percent) with incomplete KD. In incomplete KD patients, cervical lymphadenopathy (69.0 percent) occurred most frequently, followed by conjunctival injection (62.0 percent) and polymorphous rash (45.0 percent). These patients occasionally presented with other additional symptoms including abdominal pain, headache, vomiting and arthralgia. Incomplete KD was initially diagnosed as cervical lymphadenitis (34.0 percent), viral infection (14.0 percent), scarlet fever (7.0 percent), meningitis (7.0 percent), and Kikuchi disease (7.0 percent). Coronary complications were noted in 15 patients (39.0 percent). Of the 37 patients treated with intravenous immunoglobulin, five (14.0 percent) were resistant to the therapy and all had coronary abnormalities. Conclusion : Most patients with KD ${\geq}7years$ of age have incomplete presentations. They tend to have a higher incidence of initial presentations of unilateral neck mass and coronary artery involvement. In school-aged children, fever and cervical lymphadenitis or suspected neck infection unresponsive to intravenous antibiotics should signal the possibility of KD. A high index of suspicion and prompt treatment is essential in this age group of patients.

목 적 : 학동기 아동에서의 가와사끼병은 드물게 나타나며 대부분 불완전한 양상을 보이므로 진단이 늦어지고 심혈관계 합병증의 발생이 증가한다. 본 연구에서는 이들의 임상 특징을 조사하여 이 연령군에서 가와사끼병의 조기 진단에 도움이 되고자 하였다. 방 법 : 1995년 6월부터 2006년 5월까지 가와사끼병으로 입원하여 치료받은 7세 이상의 소아 38명을 대상으로 임상 특징을 후향적으로 조사하였다. 결 과 : 연구 기간 중 7세 이상의 환아는 4.9%이었고, 남녀 비는 2.5:1이었고, 연령 분포는 7-12세였다. 76%가 불완전형 가와사끼병으로 이 때에는 발열을 제외하고 경부 림프절 비대가 가장 흔히 나타났고(69%), 다음으로 양측성 결막 충혈(62%), 부정형 발진(45%)의 순이었다. 또한 복통, 두통, 구토 및 관절통 등 다른 증상을 동반하는 경우가 있었다. 불완전형 가와사끼병의 초기 진단명은 경부 림프절염이 10명(34%)으로 가장 많았고, 그 외에 바이러스 감염 4명(14%), 성홍열 2명(7%), 뇌수막염 2명(7 %), Kikuchi 병(Kikuchi disease) 2명(7%)의 순이었다. 관상동맥 합병증은 15명(39%)에서 나타났고, 첫번째 IVIG 치료에 반응하지 않은 경우는 5명(14%)이었으며 이들에서 모두 관상동맥 병변이 발생하였다. 결 론 : 학동기 아동의 가와사끼병은 대부분 불완전형으로, 경부 림프절 비대가 흔하게 나타나며 심혈관계 합병증의 발생률이 높고 비특이적인 증상들을 동반하는 경우가 많아 다른 감염성 질환으로 오진되기 쉽다. 따라서, 항생제 치료에 반응하지 않고 발열이 지속될 경우 가와사끼병의 가능성을 항상 염두에 두어야 하며 조기 진단 및 치료를 함으로써 심혈관계 합병증의 위험성을 감소시켜야 한다.

Keywords

References

  1. Kato H, Sugimura T, Akagi T, Sato N, Hashino K, Maeno Y, et al. Long-term consequences of Kawasaki disease. A 10- to 21-year follow-up study of 594 patients. Circulation 1996;94:1379-85 https://doi.org/10.1161/01.CIR.94.6.1379
  2. Dajani AS, Taubert KA, Gerber MA, Shulman ST, Ferrieri P, Freed M, et al. Diagnosis and therapy of Kawasaki disease in children. Circulation 1993;87:1776-80 https://doi.org/10.1161/01.CIR.87.5.1776
  3. Yanagawa H, Yashiro M, Nakamura Y, Kawasaki T, Kato H. Epidemiologic pictures of Kawasaki disease in Japan: from the nationwide incidence survey in 1991 and 1992. Pediatrics 1995;95:475-9
  4. Stockheim JA, Innocentini N, Shulman ST. Kawasaki disease in older children and adolescents. J Pediatr 2000;137:250-2 https://doi.org/10.1067/mpd.2000.105150
  5. Muta H, Ishii M, Sakaue T, Egami K, Furui J, Sugahara Y, et al. Older ages is a risk factor for the development of cardiovascular sequelae in Kawasaki disease. Pediatrics 2004;114:751-4 https://doi.org/10.1542/peds.2003-0118-F
  6. Ayusawa M, Sonobe T, Uemura S, Ogawa S, Nakamura Y, Kiyosawa N, et al. Revision of diagnostic guidelines for Kawasaki disease(the 5th revised edition). Pediatr Int 2005; 47:232-4 https://doi.org/10.1111/j.1442-200x.2005.02033.x
  7. Dajani AS, Taubert KA, Takahashi M, Bierman FZ, Freed MD, Ferrieri P, et al. Guidelines for long-term management of patients with Kawasaki disease. Circulation 1994;89:916-22 https://doi.org/10.1161/01.CIR.89.2.916
  8. Lee H, Kim H, Kim HS, Sohn S. NT-proBNP: a new diagnostic screening tool for Kawasaki disease. Korean J Pediatr 2006;49:539-44 https://doi.org/10.3345/kjp.2006.49.5.539
  9. Heo MY, Choi SJ, Kim HS, Sohn S. Clinical features of atypical Kawasaki disease. J Korean Pediatr Soc 2002;45: 376-82
  10. Fukushige J, Ueda Y. Incidence and clinical features of incomplete Kawasaki disease. Proceedings of the Third International Kawasaski Disease Symposium; 1988; Tokyo, Japan: Kawasaki Foundation 1988:287-9
  11. Boven K, De Graeff-Meeder ER, Spliet W, Kuis W. Atypical Kawasaki disease: an often missed diagnosis. Eur J Pediatr 1992;151:577-80 https://doi.org/10.1007/BF01957725
  12. Fan PC, Chiu CH, Yen MH, Huang YC, Li CC, Lin TY. School-aged children with Kawasaki disease: high incidence of cervical lymphadenopathy and coronary artery involvement. J Paediatr Child Health 2003;39:55-7 https://doi.org/10.1046/j.1440-1754.2003.00085.x
  13. Pannaraj PS, Turner CL, Bastian JF, Burns JC. Failure to diagnose Kawasaki disease at the extremes of the pediatric age range. Pediatr Infect Dis J 2004;23:789-91 https://doi.org/10.1097/01.inf.0000134312.39744.a4
  14. Anderson MS, Todd JK, Glod MP. Delayed diagnosis of Kawasaki syndrome: an analysis of the problem. Pediatrics 2005;115;428-33 https://doi.org/10.1542/peds.2004-1824
  15. Shim SY, Heo MY, Kim HS, Sohn S. High-dose intravenous immunoglobulin retreatment in Kawasaki disease. J Korean Pediatr Soc 2002;45:1273-7
  16. Burns JC, Capparelli EV, Brown JA, Newburger JA, Glode MP. Intravenous gamma-globulin treatment and retreatment in Kawasaki disease. US/Canadian Kawasaki Syndrome Study Group. Pediatr Infect Dis J 1998;17:1144-8 https://doi.org/10.1097/00006454-199812000-00009
  17. Hashino K, Ishii M, Iemura M, Akagi T, Kato H. Re-treatment for immune globulin-resistant Kawasaki disease: a comparative study of additional immune globulin and steroid pulse therapy. Pediatr Int 2001;43:211-7 https://doi.org/10.1046/j.1442-200x.2001.01373.x
  18. Ahn SY, Kim DS. Treatment of intravenous immumoglobulin-resistant Kawasaki disease with methotrexate. Scand J Rheumatol 2005;34:136-9
  19. Burns JC, Mason WH, Hauger SB, Janai H, Bastian JF, Wohrley JD, et al. Infliximab treatment for refractory Kawasaki disease. J Pediatr 2005;146:662-7 https://doi.org/10.1016/j.jpeds.2004.12.022