Infectious pattern: peak at 1 y and v. little before and after 5; seasonal variation;
E: swelling of the dorsa of the feet + erythema of soles H: perianal desquamation
Help identify KD
Antinuclear antibody, infections, cancer, lung disease, gastrointestinal diseases, hormonal diseases, blood diseases, skin disease, elderly people, family history of rheumatic disease. 5% occurrence naturally Rheumatoid Factor for RA Pleocytosis: more than regular number of cells in CSF
Kawasaki Disease Androu Waheeb RCSI-Bahrain Medical Student Acknowledgement:Dr. Brian McCrindle at the Hospital for Sick Children, Toronto
History Tomisaku Kawasaki, Red Cross Hospital, Tokyo, Japan (Jan 1961) Alternative Names lymphnode syndrome Mucocutaneous lymph node syndrome Kawasaki syndrome Syndrome ?!
Epidemiology 85% younger than 5 years Uncommon in 1 year < age < 5 years Male:female ratio 1.6:1 2-5 Increased prevalence in Asian decent (10x) Japan highest (2006: 188.1/100k < 4 yo) 6 Korea 2nd (2005: 104.6/100k < 5 yo)7 Vs USA (2000:17.1/100k < 5 yo) 8
Epidemiology cont’d Average recurrence 3% Average sibling occurrence 1% Average mortality < 1% Racial Demographic Asian and Pacific Island descent > non-Hispanic African-American > Hispanics > Caucasian
Treatment Conventional=IVIG + Aspirin IVIG Reduces the prevalence of coronary artery abnormalities MOA unknown (neutralization of antigen or immune inhibition?) 2g/kg infusion 10-12 h Higher dose Less infusions Best within 7 days (earlier better) Live vaccines 11 mo reduced immunogenicity
Treatment cont’d Aspirin Anti-platelet and anti-inflammatory; no reduction of CAA incidence 80-100 mg/kg/d/4 doses 3-5 mg/kg/d afebrile 72 hours + ≥ day 14 of illness. D/C no CAA 6 week follow-up Reye syndrome a/w varicella and influenza vaccine Steroids Controversial Combined (30mg/kg steroids + conventional) No significant
Treatment failure Retreatment IVIG 2g/kg Methylprednisolone IV pulse 30mg/kg 2-3 h od 1-3 d Infliximab (monoclonal anti-TNF-a antibody)
Coronary Aneurysms Smaller with time Risk factors Male 8 year < Age < 1 year Fever non-responsive to IVIG Decreased Hb Serum[albumin] Increased WBC Band CRP
Morbidity & Mortality Thrombosis principal cause MI highest rate 1 year after onset LMCA RCA + LAD Nocardiac sequelae within 1st mo full recovery + asymptomatic
Take Home Message Auto-immune mediated vasculitis (mucuopurulent lymphadenopathy) 6 Cardinal Symptoms, don’t have to occur in totality (Japanese Diagnostic Criteria) Etiology unknown: behaves as infectious and genetic Devastating cardiac complications MI, anurysms, vasculitis Treatment: IVIG + Aspirin + Complications
References1. Kim, D. S. (2006). "Kawasaki Disease." Yonsei Medical Journal 47(6): 759-772.2. Li, X.-h., X.-j. Li, et al. (2008). "Epidemiological Survey of Kawasaki Disease in Sichuan Province of China." Journal of tropical pediatrics 54(2): 133-136.3. Huang, W.-C., L.-M. Huang, et al. (2009). "Epidemiologic Features of Kawasaki Disease in Taiwan, 2003-2006." Pediatrics 123(3): e401-405.4. Chang, R.-K. R. (2002). "Epidemiologic characteristics of children hospitalized for Kawasaki disease in California." Pediatric Infectious Disease Journal 21(12): 1150-1155.5. Morens, D. M., L. J. Anderson, et al. (1980). "National Surveillance of Kawasaki Disease." Pediatrics 65(1): 21-25.6. Yosikazu Nakamura, M. Y., Ritei Uehara, Izumi Oki, Makoto Watanabe, and Hiroshi Yanagawa (2008). "Epidemiologic Features of Kawasaki Disease in Japan: Results from the Nationwide Survey in 2005-2006." J Epidemiol 18(4): 167-172.7. Park, Y., J. Han, et al. (2007). "Kawasaki Disease in Korea, 2003-2005." Pediatric Infectious Disease Journal 26(9): 821-823.8. Robert C. Holman, A. T. C., Ermias D. Belay, Claudia A. Steiner and Lawrence B. (2003). "Kawasaki Syndrome Hospitalizations in the United States, 1997 and 2000." Pediatrics 112(3): 495-501.