Download Kawasaki Disease

PAEDIATRIC POLICY
CARDIAC: 2.5
July 2000
KAWASAKI DISEASE
Originally written:
Revised Edition:
Review Date:
Protocol Discussed with:
Louise Kyne February 1997
Colin Dunkley July 2000
July 2002
Professor N Rutter
Epidemiology
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1.5 per 100,000 children (UK)
80% < 4 years of age
Peak incidence: End of 1st year of life
Increase in oriental races, increase in siblings < 2 years old
Male - Female ratio 1.5 : 1
Sudden death in up to 2% of affected children
Diagnostic Criteria
(a)
Fever of 5 or more days duration
(b)
Presence of 4 of the following 5 features:
i)
Bilateral conjunctival injection
ii)
Change(s)* in the mucus membranes of the upper respiratory tract, such as
injected pharynx, dry cracked lips or strawberry tongue
iii)
Change(s)* in the peripheral extremities, such as eczema, erythema or desquamation
iv)
Polymorphous rash
v)
Cervical lymphadenopathy
c)
Illness not explained by other known disease process
Note:
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* one of these is sufficient
these features do not necessarily all appear at once, they can emerge consecutively
conjunctival injection - mainly bulbar and not purulent
cervical lymphadenopathy - seen 50-75% of patients. One node > 1.5 cms is necessary.
Usually tender, non-fluctuant
20% of children with echo proven coronary artery disease did not fulfil the classic diagnostic
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criteria – consider immunoglobulin therapy in this group.
Additional Features
1)
Extreme misery is characteristic
2)
Desquamation affecting genital area highly suggestive but late feature
3)
Raised platelet count is common, but usually after day 10.
4)
Others -
5)
: heart murmur, pericardial effusion, myocardial infarction
: joint pain and swelling
: abdominal pain, hydrops of gall bladder, proteinuria, diarrhoea
: CNS involvement
: meatal inflammation in boys
: reddening of BCG scars
ECG changes : ST segment flattening and depression
T wave inversion
Conductive disturbances eg heart block
Investigations
Note:
FBC
Blood culture
U&E
Throat swab
ESR
Urinalysis
LFT
ASOT
Viral Titre - inc. varicella titre
ECG
ECHO
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EC
HO
- At
first
sus
picion – many demonstrate pericardial effusion or myocarditis
- 6 weeks later – may demonstrate coronary artery aneurysms
- Further Echo's only if earlier Echo's abnormal
Predictive factors for coronary artery involvement:
 age < 1 year
 fever > 16 days
 recurrent fever after afebrile period of 48 hours
 arrhythmia
 cardiomegaly
 caucasian
 anaemia
 hypoalbuminaemia
 thrombocytosis marked
 leukocytosis
marked
Management
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1.
IMMUNOGLOBULIN
: 2 g / kg iv given over 12 hours. NB See product information or
preparations may vary. Infusion rates may be altered according to
patient response.
: Most beneficial when given in 1st 10 days of illness.
: Administer cautiously in view of such large volume of fluids,
particularly if evidence of myocardial dysfunction.
: Consider second dose immunoglobulin if remain pyrexial > 48hrs
following first dose.
2.
ASPIRIN
: 100 mgs / kg / day in 4 divided doses.
: Use until 14th day of illness or when temp settled.
Then : Use aspirin 5 mgs / kg / day in a single dose.
: Discontinue after 6 - 8 weeks after verifying absence of coronary
aneurysms.
: If aneurysms detected, use aspirin long term and involve cardiologist
3.
Long term management including selective coronary arteriography should be
individualised to the coronary artery status.
4.
Recurrences have been reported to occur in 0.8% of children and may be associated with an
increased risk of coronary artery disease.
5.
Delay live vaccines at least 6 months following immunoglobulin as less effective.
6.
Parent support group. Fact sheet.
c/o Mrs Sue Davidson
KD Support Group
13 Norwood Grove
Potters Green
Coventry CV2 2FR
Tel: 01203 612178
Fax: 024 7661 2178
E-mail: [email protected]
References:
- Dajani AS et al. Guidelines for long-term management of patients with Kawasaki disease. Report from the Committee on
Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart
Association. Circulation 89(2): 916-22, 1994 Feb
- Rowley AH. Shulman ST. Kawasaki syndrome. Pediatric Clinics of North Amercia. 46(2): 313-29, 1999 Apr.
- AHA – Diagnostic Guidelines for Kawasaki Disease. American Journal of Diseases in Children vol 144, Nov 1990
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