kawasaki disease Flashcards
what is kawasaki
self limiting condition
non infectious medium vessel vasculitits with a predilectuon for coronary vessels
fever and manifestations of acute inflammation lasting 12 days without therapy
early treatment required to prevent complications
complications of kawasaki
coronary artery synndromes - infants under 12 months are at increased risk of coronary artery aneurysm
depressed myocardial contractility and heart failure
myocardial infarction
arrythmias
peripheral arterial occlusion
diagnosis
Fever and 4 of 5 of the following clinical features – CRASH & BURN
C: Bilateral non-purulent conjunctivitis
R: generalised variable rash
A: Cervical lymphadenopathy – unilateral > bilateral
S: strawberry tongue, Oral mucosa changes – erythema, pharyngitis, dry cracked lips
H: hand and feet swelling, erythema of palms/soles, desquamation
associated non-specific symptoms of kawasaki disease
diarrhoea, vomiting, abdo pain
irritability
cough or rhinorrhea
joint pain
weakness
inflammation and crusting of BCG site
sterile pyuria
aseptic meningitis
hydrops of gall bladder
incomplete (atypical) kawasaki disease
diagnostic crteria not fulfilled but otherwise similar clinical picture to that of classic kawasaki disease
still at risk of cardiovascular complications
laboritory findings
elevated acute phase reactants (CRP, ESR)
elevated WCC with predominant neutrophilia
elevated platelets
normocytic, normochromic anaemia
sterile pyuria
invesstigations
there are no diagnostic tests for kawasaki disease but can be supportive orr used to exclude other caauses of fever
CPR, ESR, FBC, ALT, Albumin
AASOT/antiDNAase B
urinalysis
blood culture
echocardiography is required - complications inlcude coronary artery aneurysms
differential diagnosis
adenovirus
EBV
scarlett fever
toxic shock syndrome
steven - johnson syndrome
measles
initial management
IV immunoglobulin IVIG
low gose aspirin
further management
second dose IVIG may be given is incomplete treatment response, particulalry in high risk age group and atypical kawasaki disease
refer to cardiology for echocardiogram and reppeat echo at 4-8 weeks post treatment
kawasaki disease can be diagnosed with less that 4 additional features if
coronary artery abnormalities are also present
features of conjunctival injection
bilateral, non-exudative, painless
often with limbic sparing (zone around the iris is clear)
rash
reyes syndrome
aspirin is usually contraindicated in children under 16 due to the risk of reye’s syndrome, a rare acute encephalopathy associated with liver fialure
however, inn kawasaki’s disease, the benefits are thought to outweigh the risk
how gets kawasakis
most common ages 1-4, more common in children of asain descent
how common are coronary artery aneurysms as a conseuence of kawasaki’s
aneurysms develop in 20% if not treated
kawasaki rash
erythematous polymorphous rash occuring in the first few days
involving trunk and extremities
variable presentations
bullous, vasicular or petechial rashes are not typical in KD
oral changes in kawasakis
strewberry tongue
erythemea, dryness, cracking and bleeding of the lips
diffuse oropharyngeal erythema
exudates ate not typical of KD
extremity changes in KD
hyperaemia and painful oedema of the hands and feet that progresses to desquamation from the second week of illness
lymphadenopathy
cervical, most commonly unilateral, tender
at least one node >1.5cm
less common feature and seen on older children
giving IVIg
should be given within the first 10 days of illness or after if there iis ongoing fever/inflammation
a second dose should be given if the child doesn’t respond to the first dose
IVIg must be ordered through national blood authority
haemolytic anaemia is an uncommon advserse effect of IVIg
post IVIg: defer live vaccines
