kawasaki disease

Question 1

what is kawasaki

Answer 1

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

Question 2

complications of kawasaki

Answer 2

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

Question 3

diagnosis

Answer 3

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

Question 4

associated non-specific symptoms of kawasaki disease

Answer 4

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

Question 5

incomplete (atypical) kawasaki disease

Answer 5

diagnostic crteria not fulfilled but otherwise similar clinical picture to that of classic kawasaki disease
still at risk of cardiovascular complications

Question 6

laboritory findings

Answer 6

elevated acute phase reactants (CRP, ESR)
elevated WCC with predominant neutrophilia
elevated platelets
normocytic, normochromic anaemia
sterile pyuria

Question 7

invesstigations

Answer 7

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

Question 8

differential diagnosis

Answer 8

adenovirus
EBV
scarlett fever
toxic shock syndrome
steven - johnson syndrome
measles

Question 9

initial management

Answer 9

IV immunoglobulin IVIG
low gose aspirin

Question 10

further management

Answer 10

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

Question 11

kawasaki disease can be diagnosed with less that 4 additional features if

Answer 11

coronary artery abnormalities are also present

Question 12

features of conjunctival injection

Answer 12

bilateral, non-exudative, painless
often with limbic sparing (zone around the iris is clear)

Question 13

rash

Question 14

reyes syndrome

Answer 14

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

Question 15

how gets kawasakis

Answer 15

most common ages 1-4, more common in children of asain descent

Question 16

how common are coronary artery aneurysms as a conseuence of kawasaki’s

Answer 16

aneurysms develop in 20% if not treated

Question 17

kawasaki rash

Answer 17

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

Question 18

oral changes in kawasakis

Answer 18

strewberry tongue
erythemea, dryness, cracking and bleeding of the lips
diffuse oropharyngeal erythema
exudates ate not typical of KD

Question 19

extremity changes in KD

Answer 19

hyperaemia and painful oedema of the hands and feet that progresses to desquamation from the second week of illness

Question 20

lymphadenopathy

Answer 20

cervical, most commonly unilateral, tender
at least one node >1.5cm
less common feature and seen on older children

Question 21

giving IVIg

Answer 21

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