simple presentation

1. .
BY
DR/ MOHAMEDABDEL RAZEK
SHAQRA GENERAL HOSPITAL
PEDIATRIC EGYPTIAN Fellowship

2. 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 in the United States and Japan.”
Mucocutaneous lymph node syndrome:
• Involves the skin, mouth, and lymph nodes

3. Epidemiology
 Median age of affected children = 2.3 years
 80% of cases in children < 4 yrs. 5% of cases in children > 10 yrs.
 Males: females = 1.5-1.7:1
 Recurs in 3%
 Positive family history in 1% but 13% risk of occurrence in twins.
 Annual incidence of 4-15/100,000 children under 5 years of age
 More in Asian-Americans, African Americans next most prevalent
 Seasonal variation
– More cases in winter and spring but occurs throughout the year

4. What is the Etiologyof Kawasaki Disease?
 Infectious agent most likely (why):
• Age-restricted susceptible population
• Seasonal variation
• Well-defined epidemics
• Acute self-limited illness similar to known infections
 ( BUT) No causative agent identified
Bacterial, retroviral, super antigenic bacterial toxin
Immunologic response triggered by one of several microbial agents( MAY BE)
 New Haven Coronavirus
• Identified a novel human coronavirus in respiratory secretions from a 6-month-old with typical
Kawasaki Disease
• Suggests association between viral infection & Kawasaki disease

5. Diagnostic Criteria
 Fever for at least 5 days
 At least 4 of the following 5 features:
1. Changes in the extremities Edema, erythema,
desquamation
2. Polymorphous exanthema, usually truncal
3. Conjunctival injection
4. Erythema&/or fissuring of lips and oral cavity
5. Cervical lymphadenopathy
Illness not explained by other known
disease process

6. “Strawberry” tongue
White coating
Red bumps - swollen
Red lips
Swollen
Chapped

7. SKIN RASH
.

8. Swelling of the palms of the hands and
soles of the feet
.

9. Skin peels from hands and feet
.

10. Bilateral non-purulent conjunctival
injection
.

11. Unilateral large cervical lymph
node
.

13. Atypical or Incomplete Kawasaki Disease
1.Present with < 4 of 5 diagnostic criteria
2.Compatible laboratory findings
3.Still develop coronary artery aneurysms
4.No other explanation for the illness
5.More common in children < 1 year of age

14. Kawasaki Disease: S&S
Respiratory
• Rhinorrhea, cough, pulmonary infiltrate
GIT
• Diarrhea, vomiting, abdominal pain, hydrops of the
gallbladder, jaundice
 Neurologic
• Irritability, aseptic meningitis, facial palsy, hearing loss
Musculoskeletal
• Myositis, arthralgia, arthritis

15. Differential Diagnosis
 Infection:
 Measles & Group A beta-hemolytic strep can closely resemble KD
 Bacterial: severe staph infections w/toxin release
 Viral: adenovirus, enterovirus, EBV, roseola
 Spirochetal: Lyme disease, Leptospirosis
 Parasitic: Toxoplasmosis
 Rickettsial : Rocky Mountain spotted fever, Typhus
 Immunological/Allergic
 JRA (systemic onset)
 Atypical ARF
 Hypersensitivity reactions
 Stevens-Johnson syndrome
 Toxins
 Mercury

16. 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 arthralgia.
 Gradual improvement even without treatment.
Convalescent (Months to years later)
 DUE TO CORONARY ARTERY ANEURYSM

17. Kawasaki Disease: Lab findings
Early
 Leukocytosis
 Left shift
 Mild anemia
 Thrombocytopenia
 Elevated ESR
 Elevated CRP
 Hypoalbuminemia
 Elevated transaminases
 Sterile pyuria
Late
 Thrombocytosis
 Elevated CRP
 Thrombocytosis

18. CardiovascularManifestationsof AcuteKawasaki Disease
ECG changes
 Arrhythmias
 Abnormal Q waves
 Prolonged PR and/or QT intervals
 Low voltage
 ST-T–wave changes.
CXR–cardiomegaly

19. CardiovascularManifestationsof AcuteKawasaki Disease
None
Suggestive of myocarditis (50%)
• Tachycardia, murmur, gallop rhythms
• Disproportionate to degree of fever & anemia
Suggestive of pericarditis
• Present in 25% although symptoms are rare
• Distant heart tones, pericardial friction rub, tamponade

20. Role of Cardiology in the AcuteSetting
• Usually just to document baseline
coronary artery status (not an
emergency)
• If myocarditis suspected (an
emergency)
• Can help diagnose “atypical” disease

21. EchocardiographicFindings
• Myocarditis with dysfunction
• Pericarditis with an effusion
• Valvar insufficiency
• Coronary arterial changes

22. CoronaryArterial Changes
• 15% to 25 % of untreated patients
develop coronary artery changes
• 3-7% if treated in first 10 days of
fever with IVIG
• Most commonly proximal, can be
distal

23. CoronaryArterial 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
 Size
 Small = <5 mm diameter
 Medium = 5-8 mm
 Giant = ≥ 8 mm
 Shape :SaccularOR Fusiform
 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
 Cardiac involvement with manifestation

24. Coronary Aneurysm
Approximately 50% of aneurysms resolve
 Smaller size
 Fusiform morphology
 Female gender
 Age less than 1 year
Giant aneurysms (>8mm) worst prognosis

25. CoronaryAneurysm

26. Cardiovascular Sequelae
 0.3-2% mortality rate due to cardiac disease
10% from early myocarditis
 Aneurysms may thrombose, cause MI/death
 MI is principal cause of death in KD
• 32% mortality
• Most often in the first year
• Majority while at rest/sleeping
• About 1/3 asymptomatic

27. AcuteKawasaki Disease: Treatment
 IVIG: 2g/kg as one-time dose
• Mechanism of action is unclear
• Significant reduction in CAA in pts treated with IVIG plus
aspirin vs aspirin alone (15-25% VS 3-5%)
• Efficacy unclear after day 10 of illness
• 70-90% defervesce & show symptom resolution within 2-3
days of treatment
• Retreat those with failure of response to 1st dose or recurrent
symptoms
• Up to 2/3 respond to a second course

28. Treatment
Aspirin
• High dose (80-100 mg/kg/day) until afebrile FOR 48 hrs &/or
decrease in acute phase reactants
• Need high doses in acute phase due to malabsorption of ASA
• Decrease to low dose (3-5 mg/kg/day) for 6-8 weeks or until
platelet levels normalize
• No evidence of effectiveness on CAA when used alone
Aggressive support with diuretics & inotropes
for some patients with myocarditis
Antibiotics while excluding bacterial infection

29. Treatment
Conflicting data about steroids
• Reports of higher incidence of aneurysms &
more ischemic heart disease in pts with
aneurysms
• Case report of KD refractory to IVIG but
responsive to high-dose steroids &
cyclosporine
Physical activity: decrease for 4-6wk
Follow-up

30. Dr TomisakuKawasaki
.