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Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
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Kawasaki Disease

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Overview of Kawasaki Disease

Overview of Kawasaki Disease

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  • 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
  • Stenotic lesions
  • Transcript

    • 1. Kawasaki Disease Androu Waheeb RCSI-Bahrain Medical Student Acknowledgement:Dr. Brian McCrindle at the Hospital for Sick Children, Toronto
    • 2. History Tomisaku Kawasaki, Red Cross Hospital, Tokyo, Japan (Jan 1961) Alternative Names  lymphnode syndrome  Mucocutaneous lymph node syndrome  Kawasaki syndrome Syndrome ?!
    • 3. Definition Auto-immune mediated vasculitis  Medium sized arteries  Favours coronary arteries
    • 4. 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
    • 5. 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
    • 6. Aetiology Unknown: infectious pattern Virus Bacterial(superantigenic toxin vs. conventional antigen) Genetics Auto-immune
    • 7. 6 cardinal symptoms/signs1. Fever • ≥ 5 days • ≥ 40oC • self-remitting • onset1. Non-purulent bilateral conjunctivitis • Onset after fever
    • 8. 6 cardinal symptoms/signs cont’d3. Oromucosal changes • Lips  Red  Cracked  Bleeding  Swollen • Strawberry tongue3. Erythematous rash • Truncal peripheral extremities
    • 9. 6 cardinal symptoms/signs cont’d5. Changes in extremities • Erythema of hands and feet  a/w pain  a/w brawny oedema dorsum of hand • Desquamation of fingers and toes
    • 10. 6 cardinal symptoms/signs cont’d6. Cervical lymphadenopathy • Least common • ≥ 1 lymph node, ≥ 1.5 cm in diameter • Unilateral • Anterior cervical triangle
    • 11. Diagnostics No specific diagnosis or test  Use clinical presentation  Not always simultaneous
    • 12. Diagnostic Criteria Japanese MOH  Complete KD Fever + 5/6 cardinal symptoms  Incomplete KD Fever + 4/6 cardinal symptoms + evidence of coronary artery abnormality  Echo: ≥ 3mm ≤ 5 yo; ≥ 4mm ≥ 5 yo  Internal diameter >1.5 x from adjacent segment More common in younger children
    • 13. Cardiac Findings Prominent in the acute phase leading cause of long-term morbidity and mortality. Involvement of 1. Pericardium 2. Myocardium 3. Endocardium 4. Valves 5. Coronary arteries
    • 14. Cardiac Findings cont’d Cardiac auscultation  hyperdynamic precordium  Tachycardia  a gallop rhythm  innocent flow murmur  Anemia  Fever  depressed myocardial contractility (myocarditis) significant MR  pansystolic regurgitation murmur low cardiac output syndrome or shock (poor myocardial function)
    • 15. Non-Cardiac Findings increased irritability vs. other febrile diseases Arthritis or arthralgia (first week)  multiplejoints  favours knees and ankles. Gastrointestinal complaints (33%)  Diarrhoea  Vomiting  abdominal pain, occur in approximately
    • 16. Non-Cardiac Findings cont’d Hepatic enlargement + jaundice Acute acalculous gallbladder distension  first two weeks  Cause hydrops  identified by abdominal ultrasound
    • 17. Non-Cardiac Findings cont’d siteof previous BCG erythema + induration  Common  Younger than 1 year perianal erythematous desquamation
    • 18. Characteristic Lab Findings acute stage (4-6w)  Leukocytosis  predominance of immature and mature granulocytes  Upregulated apoptosisDepleted peripheral lymphocytes.  Anemia  RBC indices,  particularly with a prolonged duration of active inflammation.  Elevated Acute Phase Reactants  ESR  CRP  Platelet count rapidly increases week 2-3, (can be > 1,000,000/mm3)  ANA and RF -ve
    • 19. Lab Findings cont’d serum transaminases moderately elevated Hypoalbuminemia  a/w with increased severity + duration Intermittent sterile pyuria  (Urinalysis not suprapubic)urethritis CSF pleocytosis  mononuclear cells predominance  normal glucose and protein Inflammatory synovial fluid Decreased plasma cholesterol, HDL, apolipoprotein A-I
    • 20. 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
    • 21. 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
    • 22. Treatment failure Retreatment IVIG 2g/kg Methylprednisolone IV pulse 30mg/kg 2-3 h od 1-3 d Infliximab (monoclonal anti-TNF-a antibody)
    • 23. Treatment Failure cont’d Ulinastatin Plasmapheresis Cytotoxic agents  Cyclophosphamide  Cyclosporin A MTX: dihydrofolate reductase inhibitor (anti-inflammatory)  10mg/BSA, once weekly  MOA unclear
    • 24. Management Serialstress test Follow up 4-6 w, 6m, 1 y  Echo 20% AneurysmstenosisMI  Percutaneous Transluminal Angioplasty  Rotational atherectomy  Stent  Bypass graft  transplant
    • 25. Complications Cardiac  Coronary artery dilations and aneurysms (5%)  Thrombosis  Myointimal proliferation  stenosis (20%)myocardial ischemia  1% giant aneurysms  Aspirin, clopidogrel, ticlopidine  LMW Heparin, Warfarin, anti platelet receptor monoclonal antibodies  Pericarditis + effusion  Myocarditis  Valvulitis (1%. Mitral Valve)
    • 26. 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
    • 27. Morbidity & Mortality Thrombosis principal cause MI highest rate 1 year after onset  LMCA  RCA + LAD Nocardiac sequelae within 1st mo  full recovery + asymptomatic
    • 28. 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
    • 29. 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.

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