Download - pediatrics.Kawasaki disease.(dr.khalid)
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Kawasaki Disease:
Dr.Khalid Hama salih, MDDr.Khalid Hama salih, MDPediatrics specialistPediatrics specialist
M.B.Ch. D. C.H B.F.I.B.M.S.pedM.B.Ch. D. C.H B.F.I.B.M.S.ped
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DrDr Tomisaku Kawasaki Tomisaku Kawasaki
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What is Kawasaki Disease?
Idiopathic multisystem disease characterized by vasculitis of small & medium blood vessels, including coronary arteries
A self-limited vasculitis of unknown etiology that predominantly affects children younger than 5 years. It is now the most common cause of acquired heart disease in children
*Burns, J. Adv. Pediatr. 48:157. 2001.*Burns, J. Adv. Pediatr. 48:157. 2001.
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Epidemiology• 80% of cases in children < 4 yrs• Males:females = 2:1• Positive family history in 1% but 13% risk of occurrence
in twins.
• in-hospital mortality ≈ 0.17%• Annual incidence of 4-15/100,000 children under 5 years
of age• Seasonal variation
– More cases in winter and spring but occurs throughout the year
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Kawasaki disease
Acute febril phase Subacute phase Convalescent phase
1- 2 wk 2 – 4 wk 6 – 8 wk 1- 2 wk
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Phases of Disease
• Acute (1-2 weeks from onset)– Febrile, irritable, toxic appearing– Oral changes, rash, edema/erythema of feet
• Subacute (2-8 weeks from onset)– Desquamation, may have persistent arthritis or
arthralgias– Gradual improvement even without treatment
• Convalescent (Months to years later)
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Kawasaki Disease: S&S• Respiratory
– Rhinorrhea, cough, pulmonary infiltrate
• GI– Diarrhea, vomiting, abdominal pain, hydrops of the
gallbladder, jaundice
• Neurologic– Irritability, aseptic meningitis, facial palsy, hearing loss
• Musculoskeletal– Myositis, arthralgia, arthritis
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Differential Diagnosis
• Infectious
– Measles & Group A beta-hemolytic strep can closely resemble KD
– Bacterial: severe staph infections w/toxin release
– Viral: adenovirus, enterovirus, EBV, roseola• Immunological/Allergic
– JRA (systemic onset)– Hypersensitivity reactions
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Kawasaki disease - AHA diagnostic criteriaFever of 5 days duration + four of five criteria
Oropharyngeal changes
(90%+ of cases)
1.
Changes in peripheralextremities
(90%+ of cases)
2.
Cervical lymphadenopathy
(~75% of cases)
5.
Polymorphous rash
(95%+ of cases)
4.
Bilateral non-purulent conjunctival injection
(90%+ of cases)
3.
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Atypical or Incomplete Kawasaki Disease
• Present with < 4 of 5 diagnostic criteria• Compatible laboratory findings• Still develop coronary artery aneurysms• No other explanation for the illness• More common in children < 1 year of age
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Trager, J. D. N Engl J Med 333(21): 1391. 1995.
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Han, R. CMAJ 162:807. 2000.
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Kawasaki Disease: Labs
• Early– Leukocytosis– Left shift– Mild anemia– Thrombocytopenia/
Thrombocytosis– Elevated ESR– Elevated CRP– Hypoalbuminemia– Elevated transaminases– Sterile pyuria
• Late– Thrombocytosis
– Elevated CRP
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Cardiovascular Manifestations of Acute Kawasaki Disease
• EKG changes– ArrhythmiasArrhythmias– Prolonged PR and/or QT intervalsProlonged PR and/or QT intervals– Low voltageLow voltage– ST-T–wave changes.ST-T–wave changes.
• CXR–cardiomegaly
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Coronary Arterial Changes
• Vary in severity from echogenicity due to thickening and edema or asymptomatic coronary artery ectasia to giant aneurysms
• May lead to myocardial infarction, sudden death, or ischemic heart disease
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Coronary Aneurysms
• • Patients most likely to develop aneurysms– Younger than 6 months, older than 8 years– Males– Fevers persist for greater than 14 days– Persistently elevated ESR– Thrombocytosis– Pts who manifest s/s of cardiac involvement
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Management Categories1.Pharmacologic therapy:
• IVIG: 2g/kg as one-time dose• Aspirin
– High dose (80-100 mg/kg/day) until afebrile x 48 hrs &/or decrease in acute phase reactants
– Decrease to low dose (3-5 mg/kg/day) for 6-8 weeks or until platelet levels normalize
2.Physical activity: decrease for 4-6wk
3.Follow-up and diagnostic testing
4.Invasive testing