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Kawasaki Syndrome

History and Definition

Kawasaki syndrome (KS), also known as Kawasaki disease, is an acute febrile illness of unknown etiology that primarily affects children younger than 5 years of age. KS was first described in Japan by Tomisaku Kawasaki in 1967, and the first cases outside of Japan were reported in Hawaii in 1976.

KS is characterized by fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips, and throat. Serious complications of KS include coronary artery dilatations and aneurysms, and KS is a leading cause of acquired heart disease in the United States. The standard treatment with intravenous immunoglobulin and aspirin substantially decreases the development of these coronary artery abnormalities.

KS occurs worldwide, with the highest incidence in Japan, and it most often affects boys and younger children. KS may have a winter-spring seasonality, and community-wide outbreaks have been reported occasionally. In the continental United States, population-based and hospitalization studies have estimated an incidence of KS ranging from 9 to 19 per 100,000 children younger than 5 years of age. Approximately 4248 hospitalizations with KS, of which 3277 (77%) were for children under 5 years of age, were estimated among children younger than 18 years of age in the United States in the year 2000. In 2009, the estimated number of hospitalizations with KS was 5447 (standard error [SE]=297); 4040 (SE=227) for children <5 years of age.

CDC uses hospital discharge data, a passive KS surveillance system, and special studies to describe the incidence and epidemiology of KS in the United States. The KS surveillance system has been maintained by CDC since 1976 and is based on voluntary reporting of KS cases by health care providers and local and state health authorities. A standardized case report form is used to collect information on patients.

The KS case report form is available as a pdf document for health-care workers to submit a report. Health-care workers who wish to submit a report can do so by completing either the print form (i.e., print out and complete manually) or the fillable form (i.e., enter the data directly on the computer and then print out the completed form. Note: You may not be able to download and save the completed form if your computer’s software does not allow this feature.) The completed form should be sent to the mailing address provided on the form or sent by fax to (404) 639-3838.

Kawasaki Syndrome Case Definition

For epidemiologic surveillance, CDC defines a case of KS as illness in a patient with fever of 5 or more days duration (or fever until the date of administration of intravenous immunoglobulin if it is given before the fifth day of fever), and the presence of at least 4 of the following 5 clinical signs:

  • Rash
  • Cervical lymphadenopathy (at least 1.5 cm in diameter)
  • Bilateral conjuctival injection
  • Oral mucosal changes
  • Peripheral extremity changes.

Patients whose illness does not meet the above KS case definition but who have fever and coronary artery abnormalities are classified as having atypical or incomplete KS.

Articles

Callinan LS, Tabnak F, Holman RC, Maddox RA, Kim JJ, Schonberger LB, Vugia DJ, Belay ED. PDF Document Icon Kawasaki syndrome and factors associated with coronary artery abnormalities in California (PDF – 221 KB). Pediatr Infect Dis J. 2012 Sep;31(9):894-8.

Patel A, Holman RC, Callinan LS, Sreenivasan N, Schonberger LB, Fischer TK, Belay ED. PDF Document Icon Evaluation of clinical characteristics of Kawasaki syndrome and risk factors for coronary artery abnormalities among children in Denmark (PDF – 221 KB). Acta Paediatr. 2013 Apr;102(4):385-90. doi: 10.1111/apa.12142. Epub 2013 Jan 21.

Holman RC, Christensen KY, Belay ED, Steiner CA, Effler PV, Miyamura J, Forbes S, Schonberger LB, Melish M. Racial/Ethnic Differences in the Incidence of Kawasaki Syndrome among Children in Hawaii. Hawaii Medical Journal 2010;69:194-197.

Holman RC, Belay ED, Christensen KY, Folkema AM, Steiner CA, Schonberger LB. Hospitalizations for Kawasaki syndrome among children in the United States, 1997-2007. PIDJ 2010; 29: 483-488.

Belay ED, Maddox RA, Holman RC, Curns AT, Ballah K, Schonberger LB. PDF Document Icon Kawaskaki syndrome and risk factors for coronary artery abnormalities (PDF – 221 KB), United States, 1994-2003. Pediatr Infect Dis J 2006;25(3):245-249.

Holman RC, Curns AT, Belay ED, Steiner CA, Effler PV, Yorita KL, Miyamura J, Forbes S, Schonberger LB, Melish M. PDF Document Icon Kawaskaki syndrome in Hawaii (PDF – 163 KB). Pediatr Infect Dis J 2005;24(5):429-433.

Holman RC, Curns AT, Belay ED, Steiner CA, Schonberger LB. PDF Document Icon Kawasaki syndrome hospitalizations in the United States, 1997 and 2000 (PDF – 9 pages). Pediatrics 2003;112:495-501.

Gibbons RV, Parashar UD, Holman RC, Belay ED, Maddox RA, Powell KE, Schonberger LB. PDF Document Icon An evaluation of hospitalizations for Kawasaki syndrome in Georgia (PDF – 5 pages). Arch Pediatr Adolesc Med 2002;156:492-496.

Holman RC, Shahriari A, Effler PV, Belay ED, Schonberger LB. PDF Document Icon Kawasaki syndrome hospitalizations among children in Hawaii and Connecticut (PDF – 5 pages). Arch Pediatr Adolesc Med 2000;154:804-808.

Belay ED, Holman RC, Clarke MJ, DeStefano F, Shahriari A, Davis RL, Rhodes PH, Thompson RS, Black SB, Shinefield HR, Marcy SM, Ward JI, Mullooly JP, Chen RT, Schonberger LB. The incidence of Kawasaki syndrome in West Coast health maintenance organizations. Pediatr Infect Dis J 2000;19:828-832.

Rowley AH, Shulman ST. Kawasaki syndrome. Clin Microbiol Rev 1998;11:405-414.

Bradley DJ, Glode MP. Kawasaki disease: the mystery continues. West J Med 1998;168:23-29.

Bronstein DE, Dille AN, Austin JP, Williams CM, Palinkas LA, Burns JC. Relationship of climate, ethnicity and socioeconomic status to Kawasaki disease in San Diego County, 1994 through 1998. Pediatr Infect Dis J 2000;19:1087-1091.

Khan AS, Holman RC, Clarke MJ, Vernon LL, Gyurik TP, Schonberger LB. Kawasaki syndrome surveillance, United States, 1991-1993. In: Kato H, editor. Proceedings of the 5th International Kawasaki Disease Symposium; 1995 May 15-19; Fukuoka, Japan, Amsterdam. Elsevier,1995:80-84.

Melish ME, Hicks RM, Larson EJ. Mucocutaneous lymph node syndrome in the United States. Am J Dis Child 1976;130:599-607.

Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H. A new infantile acute febrile mucocutaneous lymph node syndrome prevailing in Japan. Pediatrics 1974;54:271-276.


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