Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A case of adolescent Kawasaki disease with Epstein-Barr virus-associated infectious mononucleosis complicated by splenic infarction

전염성 단핵구증과 비경색증이 동반된 청소년 난치성 가와사끼병 1예

  • Choi, Byeong Sam (Departments of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Kwon, Bo Sang (Departments of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Kim, Gi Beom (Departments of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Jeon, Yoon Kyung (Departments of Pathology, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Cheon, Jung-Eun (Departments of Radiology, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Bae, Eun Jung (Departments of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Noh, Chung Il (Departments of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Choi, Jung Yun (Departments of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Yun, Yong Soo (Departments of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine)
  • 최병삼 (서울대학교 의과대학 소아과학교실) ;
  • 권보상 (서울대학교 의과대학 소아과학교실) ;
  • 김기범 (서울대학교 의과대학 소아과학교실) ;
  • 전윤경 (서울대학교 의과대학 병리학교실) ;
  • 천정은 (서울대학교 의과대학 영상의학교실) ;
  • 배은정 (서울대학교 의과대학 소아과학교실) ;
  • 노정일 (서울대학교 의과대학 소아과학교실) ;
  • 최정연 (서울대학교 의과대학 소아과학교실) ;
  • 윤용수 (서울대학교 의과대학 소아과학교실)
  • Received : 2009.06.14
  • Accepted : 2009.08.14
  • Published : 2009.09.15
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Abstract

Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects children. There are few reports that describe the Epstein-Barr virus (EBV) as the possible infectious agent of KD. Here, we describe a case of KD in a 15-year-old boy complicated with giant coronary artery aneurysms, pericardial effusion, and splenic infarction. The clinical course of KD was refractory to intravenous gamma globulin and aspirin. Our patient also showed typical findings of concomitant EBV-associated infectious mononucleosis, such as hepatosplenomegaly and generalized lymphadenopathy, with EBV-positive atypical lymphoid hyperplasia. He improved dramatically after receiving intravenous methylprednisolone followed by oral prednisolone. Ultimately, the coronary artery aneurysms remained as the only sequelae. We report a rare case of adolescent KD with EBV-associated infectious mononucleosis and splenic infarction.

가와사끼병은 주로 소아기에 발생하는 급성 전신 혈관염으로, 원인은 아직 명확히 알려져 있지 않다. EBV는 전염성 단핵구증의 원인으로 잘 알려져 있으며, 또한 가와사키병의 원인으로도 보고된 바 있다. 가와사끼병으로 인한 합병증은 관상동맥류를 포함한 심혈관계 합병증이 주를 이루지만, 다양한 종류의 합병증들이 보고 되고 있다. 저자들은 EBV 감염과 연관된 전염성 단핵구증, 거대 관상동맥류, 심낭삼출, 비경색증이 동반된 난치성 가와사끼병 환자 1예를 경험하였기에 보고하는 바이다. 환자는 3번의 면역글로불린 치료에도 반응이 없었지만, pulse methylprednisolone 치료 후에 임상 증상은 급격히 호전되었고, 거대 관상동맥류만 지금까지 지속되고 있다.

Keywords

References

  1. Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics 2004;114:1708-33 https://doi.org/10.1542/peds.2004-2182
  2. Wallace CA, French JW, Kahn SJ, Sherry DD. Initial intravenous gammaglobulin treatment failure in Kawasaki disease. Pediatrics 2000;105:E78 https://doi.org/10.1542/peds.105.6.e78
  3. Park YW, Han JW, Park IS, Kim CH, Cha SH, Ma JS, et al. Kawasaki disease in Korea, 2003-2005. Pediatr Infect Dis J 2007;26:821-3 https://doi.org/10.1097/INF.0b013e318124aa1a
  4. Muta H, Ishii M, Sakaue T, Egami K, Furui J, Sugahara Y, et al. Older age 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
  5. Nakamura Y, Yashiro M, Uehara R, Watanabe M, Tajimi M, Oki I, et al. Use of laboratory data to identify risk factors of giant coronary aneurysms due to Kawasaki disease. Pediatr Int 2004;46:33-8 https://doi.org/10.1111/j.1442-200X.2004.01840.x
  6. Song D, Yeo Y, Ha K, Jang G, Lee J, Lee K, et al. Risk factors for Kawasaki disease-associated coronary abnormalities differ depending on age. Eur J Pediatr [Epub ahead of print] 2009 Jan 22. Available from: URL://http://www. springerlink.com/content/100415/
  7. Kim NY, Choi DY, Jung MJ, Jeon IS. A case of refractory Kawasaki disease complicated by peripheral ischemia. Pediatr Cardiol 2008;29:1110-4 https://doi.org/10.1007/s00246-008-9221-4
  8. Tabarki B, Mahdhaoui A, Selmi H, Yacoub M, Essoussi AS. Kawasaki disease with predominant central nervous system involvement. Pediatr Neurol 2001;25:239-41 https://doi.org/10.1016/S0887-8994(01)00290-9
  9. Beiler HA, Schmidt KG, von Herbay A, Loffler W, Daum R. Ischemic small bowel strictures in a case of incomplete Kawasaki disease. J Pediatr Surg 2001;36:648-50 https://doi.org/10.1053/jpsu.2001.22311
  10. Thabet F, Bellara I, Tabarki B, Kchaou H, Selmi H, Yacoub M, et al. Ischemic colitis and hemophagocytosis complicating Kawasaki disease. Arch Pediatr 2004;11:226-8 https://doi.org/10.1016/j.arcped.2003.12.017
  11. Huong DL, Gatfosse M, Papo T, Barjonet G, Lacombe P, Godeau P. Symptomatic splenic infarction complicating adult Kawasaki disease. Br J Rheumatol 1995;34:579-80 https://doi.org/10.1093/rheumatology/34.6.579
  12. Cho BK, Kwon SR, Yoon SJ, Chung MH, Lee SN, Lee SH. Adult-onset Kawsaki disease complicated by splenic infarction and coronary aneurysm. Korean J Infect Dis 2000;32: 388-92
  13. Uthman I, Khamashta M. The abdominal manifestations of the antiphospholipid syndrome. Rheumatology (Oxford) 2007;46: 1641-7 https://doi.org/10.1093/rheumatology/kem158
  14. van Hal S, Senanayake S, Hardiman R. Splenic infarction due to transient antiphospholipid antibodies induced by acute Epstein-Barr virus infection. J Clin Virol 2005;32:245-7 https://doi.org/10.1016/j.jcv.2004.07.013
  15. Gupta M, Johann-Liang R, Bussel JB, Gersony WM, Lehman TJ. Elevated IgA and IgM anticardiolipin antibodies in acute Kawasaki disease. Cardiology 2002;97:180-2 https://doi.org/10.1159/000063118
  16. Kikuta H, Nakanishi M, Ishikawa N, Konno M, Matsumoto S. Detection of Epstein-Barr virus sequences in patients with Kawasaki disease by means of the polymerase chain reaction. Intervirology 1992;33:1-5 https://doi.org/10.1159/000150224
  17. Kanegane H, Tsuji T, Seki H, Yachie A, Yokoi T, Miyawaki T, et al. Kawasaki disease with a concomitant primary Epstein-Barr virus infection. Acta Paediatr Jpn 1994;36:713-6 https://doi.org/10.1111/j.1442-200X.1994.tb03277.x
  18. Muso E, Fujiwara H, Yoshida H, Hosokawa R, Yashiro M, Hongo Y, et al. Epstein-Barr virus genome-positive tubulointerstitial nephritis associated with Kawasaki disease-like coronary aneurysms. Clin Nephrol 1993;40:7-15
  19. Pitetti RD, Laus S, Wadowsky RM. Clinical evaluation of a quantitative real time polymerase chain reaction assay for diagnosis of primary Epstein-Barr virus infection in children. Pediatr Infect Dis J 2003;22:736-9 https://doi.org/10.1097/01.inf.0000078157.90639.96
  20. Shulman ST. Is there a role for corticosteroids in Kawasaki disease? J Pediatr 2003;142:601-3 https://doi.org/10.1067/mpd.2003.258
  21. Dahlem PG, von Rosenstiel IA, Lam J, Kuijpers TW. Pulse methylprednisolone therapy for impending cardiac tamponade in immunoglobulin-resistant Kawasaki disease. Intensive Care Med 1999;25:1137-9 https://doi.org/10.1007/s001340051025