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Kawasaki Disease: An Update of diagnosis and treatment

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Kawasaki Disease: An Update of diagnosis and treatment This algorithm is a guide to evaluation of patients with suspected incomplete Kawasaki disease (KD). – PowerPoint PPT presentation

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Title: Kawasaki Disease: An Update of diagnosis and treatment


1
Kawasaki DiseaseAn Update of diagnosis and
treatment
2
What is Kawasaki Disease?
  • Idiopathic multisystem disease characterized by
    vasculitis of small medium blood vessels,
    including coronary arteries

3
Diagnostic Criteria
  • Fever for at least 5 days
  • At least 4 of the following 5 features
  • Changes in the extremities
  • Edema, erythema, desquamation
  • 2. Polymorphous exanthem, 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

Modified from Centers for Disease Control.
Kawasaki Disease. MMWR 2961-63, 1980
4
Atypical or Incomplete Kawasaki Disease
  • Present with lt 4 of 5 diagnostic criteria
  • Compatible laboratory findings
  • Still develop coronary artery aneurysms
  • No other explanation for the illness
  • More common in children lt 1 year of age
  • 2004 AHA guidelines offer new evaluation and
    treatment algorithm

5
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6
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)

7
  • AHA classify coronary arteries aneurysms
  • Small (5 mm internal diameter),
  • medium (5 to 8 mm internal
  • diameter),
  • or giant (8 mm internal diameter).
  • The Japanese Ministry of Health Classify coronary
    arteries asabnormal
  • the internal lumen diameter is 3 mm in children 5
    years old or 4 mm in children 5 years old
  • the internal diameter of a segment measures 1.5
    times that of an adjacent segment

8
Abnormal coronary artery
Diameter of CA /BSA
9
Coronary Artery Involvement in Children With
Kawasaki Disease Risk Factors
10
Harada et al risk score
(1) white blood cell count 12 000/mm3 (2) platelet count 350 000/mm3 (3) CRP 3 (4) hematocrit 35 (5) albumin 3.5 g/dL (6) age 12 months (7) male sex.
? 4/7 high risk
11
ASAI
Symtomps 0 di?m 1 di?m 2 di?m
Sex Age Days of fever Recurrent fever Recurrent rash Recurrent bong da Anemie (Hb lt 10g/dL) WBC(X 103/ mm3) VS(mm) VS and PLT high for a long time(months ) Enlarge CI Abnormal rymth Ischemic myocady pericarditis N? ? 1 lt 14 - - - - lt 26 lt 60 lt 1 - - - - Nam gt 1 14 -15 26 30 60 100 ? 16 gt 30 gt 100 gt1
? 9/23 di?m high risk
12
ÐI?U TR? ASPIRIN
  • AHA-2004 80-100 mg/kg.
  • Pediatrics-1995 meta-analysis.

Control Ratio Dilated CA Ratio Dilated CA
after 30 days (n2547) After 60 days (n4151)
ASA 22.8 ( 95 CI 20.6-25) 17.1(95 CI 13.6-20.7)
ASAIVIG 1g/kg 17.3(95 CI 14.3-20.2) 11.1(95 CI 8.7-13.6)
ASAIVIG gt1g/kg 10.3( 95 CI 8.3-12.3) 4.4 (95 CI 2.8-6)
ASA IVIG gt1g/kg lieàu duy nhaát 2.3(95 CI 0.5-4.2) 2.4(95 CI 0.5-4.2)
IVIG gt1g/kg ASA lt80 mg/kg 13(95 CI 9-17) 4.8(95 CI 2.3-7.4)
IVIG gt1g/kg ASA gt80mg/kg 9.1 (95 CI 6.9-11.4) 4(95 CI 2.-6.1)
13
Dilated CA in 30 days Dilated CA in 60 days
IVIG (2G/KG/D) lt IVIG 1G/KG lt ASA IVIG HIGH DOSE ASA HIGH DOSE IVIG HIGH DOSE ASA LOW DOSE IVIG (2G/KG/D) lt IVIG 1G/KG lt ASA IVIG HIGH DOSE ASA HIGH DOSE IVIG HIGH DOSE ASA LOW DOSE
14
ASPIRIN vs IVIG
T? L? T?N THUONG M?CH VÀNH
15
CORTICOID
  1. Initial CORTICOID vs ASPIRIN.
  2. Initial CORTICOID ASPIRIN IVIG vs
    ASPIRINIVIG.
  3. Resistance IVIG.

16
IVIGASPIRIN vs IVIGASPIRIN METHYPREDNISOLON
Randomized Trial of Pulsed Corticosteroid Therapy
for Primary Treatment of Kawasaki Disease. N Engl
J Med 2007356663-75.
- 30 mg/kg over 2 to 3 hours - IVIG 2g/kg. -
Aspirin 80-100mg/kg.
17
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18
Effect and result
  • Response with IVIG 90
  • No response with IVIG 10

19
Prediction of Intravenous Immunoglobulin
Unresponsiveness in Patients With Kawasaki
disease. Circulation 20061132606-2612
published online May 30, 2006
http//circ.ahajournals.org/cgi/content/full/113/2
2/2606.
Kobayashi-2006
20
Prediction of Intravenous Immunoglobulin
Unresponsiveness in Patients With Kawasaki
disease. Circulation 20061132606-2612
published online May 30, 2006
http//circ.ahajournals.org/cgi/content/full/113/2
2/2606.
TIÊN ÐÓAN T?N THUONG M?CH VÀNH
21
ANTI IVIG
  • IVIG ONLY 2 g/kg (evidence level C).
  • STEROID ONLY.
  • PULSE STEROID IVIG Hashino et al RCT.
  • 17 patients who did not respond to an initial
    infusion of 2 g/kg IVIG plus aspirin followed by
    an additional IVIG infusion of 1 g/kg.
  • Randomized to receive either a single additional
    dose of IVIG (1 g/kg) or pulse steroid therapy.
  • RESULT
  • Patients in the steroidgroup had a shorter
    duration of fever and lower medical costs.
  • No significant difference in the incidence of
    coronary arteryaneurysms was noted between the 2
    groups, but power to detect a difference was
    limited.

22
KHÁNG IVIG
  • AHA-2004 recommends
  • Steroid treatment berestricted to children in
    whom 2 infusions of IVIG have been ineffective in
    alleviating fever and acute inflammation
    (evidence level C).
  • The most commonly used steroid regimen is
    intravenous pulse methylprednisolone, 30 mg/kg
    for 2 to 3 hours, administered once daily for 1
    to 3 days.

23
Acute Kawasaki Disease Conclusion for Treatment
( AHA 2004)
  • IVIG 2g/kg as one-time dose
  • Beneficial effect 1st reported by Japanese
  • Mechanism of action is unclear
  • Significant reduction in CAA in pts treated with
    IVIG plus aspirin vs. aspirin alone (15-25?3-5)

24
Acute Kawasaki Disease Treatment
  • IVIG
  • 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

25
Acute Kawasaki Disease Treatment
  • Aspirin
  • High dose (80-100 mg/kg/day) until afebrile x 48
    hrs /or decrease in acute phase reactants
  • Need high doses in acute phase due to
    malabsorption of ASA
  • Dosage of ASA in acute phase does not seem to
    affect subsequent incidence of CAA

26
Acute Kawasaki Disease Treatment
  • Aspirin
  • Decrease to low dose (3-5 mg/kg/day) for 6-8
    weeks or until platelet levels normalize (
    evidence level C).
  • No evidence /effect on CAA when used alone
  • Due to potential risk of Reye syndrome instruct
    parents about symptoms of influenza or varicella

27
  • In case of persistent or recrudescent
    fever   Repeat dose of IVIG 2 g/kg as single
    infusion consider IV methylprednisolone 30 mg/kg
    once a day may be repeated as necessary up to a
    total of three doses
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