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Kawasaki Disease

Kawasaki Disease . Morning Report 7/13/09. Kawasaki Disease. Acute febrile vasculitic syndrome of early childhood Affecting all blood vessels in the body but mostly medium and small vessels with a preferential involvement of the coronary arteries.

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Kawasaki Disease

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  1. Kawasaki Disease Morning Report 7/13/09

  2. Kawasaki Disease • Acute febrile vasculitic syndrome of early childhood • Affecting all blood vessels in the body but mostly medium and small vessels with a preferential involvement of the coronary arteries. • Exact etiology unknown but thought to be infectious in nature • Immune response thought to be oligoclonal or antigen driven

  3. Epidemiology • Race: Japanese > Blacks, Polynesians, Filipinos > Whites • Gender: Male:Female~ 3:2 • Age: • 90-95% <10years old • Peak incident 18-24months

  4. Diagnosis • Presence of 5 or more days of fever + 4 or more of the 5 principle clinical features

  5. Peeling, Erythema, Edema of Hands and Feet

  6. Changes in Lips and Oral Cavity (Erythema, Stawberry Tongue)

  7. Polymorphous Exanthema

  8. Bilateral Bulbar Conjunctival Injection without Exudate

  9. Cervical Lymphadenopathy

  10. Other Clinical Findings • Arthritis/arthralgia • Irritability • Diarrhea, Vomiting, Abdominal Pain • Hepatomegally, Jaundice • Pleural Effusions, infiltrates • Stiff Neck secondary to aseptic meningitis

  11. Incomplete or Atypical Kawasaki Disease • Children with unexplained fever for more than 5 days associated with 2-3 of the principle clinical features • More common in young infants • May be supported by laboratory evidence of systemic inflammation

  12. Differential Diagnosis • Viral Infections (Measles, adenovirus, enterovirus, EBV) • Scarlet Fever • Staphylococcal scalded skin syndrome • Bacterial cervical lymphadenitis • Rocky Mountain Spotted Fever • Leptospirosis

  13. Lab Studies • Moderate to high WBC count with left shift • Anemia • Elevated ESR, CRP • Thrombocytosis • Mild-Moderate elevation in transaminases • Sterile Pyuria

  14. Differential Diagnosis cont… • Toxic Shock Syndrome • Drug Hypersensitivity • Steven-Johnson syndrome • Juvenile idiopathic arthritis • Juvenile Polyarteritis Nodosa • Mercury hypersensitivity reaction

  15. Cardiac Imaging • Echocardiogram is critical for the evaluation of all patients suspected of having KD. • Baseline echo during acute stage to r/o coronary artery aneurysms and evidence of myocarditis, valvulitis, or pericardial effusion • Echo should be repeated in 2nd-3rd week of illness and again 1 month after (or once all lab values normalize) • Prior to treatment 20-25% of patients had Cardiac involvement with mortality rate 0.1-2% • With IVIG risk reduced to 5%

  16. Treatment • Standard therapy is IVIG with Asprin • During the acute phase of illness • IVIG (2gm/kg) and • Asprin 80-100mg/kg /day • Continue high dose asprin until day 14 of illness if still afebrile • Continue asprin 3-5mg/kg/day until no evidence of coronary changes by 6-8 weeks

  17. ~10% fail to respond to initial IVIG therapy (persistence of fever after 36hrs) • Retreatment with IVIG at same dose recommended

  18. Other Options • 3rd dose IVIG • Pulse Steroids (Methylprednisolone mg/kg for 2-3 hours qday x3days) • Infliximab (monoclonal ab against tumor necrosis factor) • Cyclophosphamide • Methotrexate

  19. Long Term Follow Up • MI caused by thrombotis occlusion of abnormal coronary artery Is principle cause of death • Usually occurs within first year • Children at high risk need frequent ECHO evaluations • Small solitary aneurysms-long term asprin therapy • Giant aneurysms or multiple complex aneurysms-long term antiplatelet therapy and anticoagulation • Primary surgical management is coronary artery bypass graft

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