Learning outcomes

After completing this module, you should know:

Understand the importance of early treatment of Kawasaki

disease

Know when to suspect a diagnosis of Kawasaki disease

Appreciate when and how to refer children with suspected

Kawasaki disease to hospital

Know the recommended hospital-based management of

Kawasaki disease

Appreciate the type of follow up arrangements that children

with Kawasaki disease may need.

How to complete this activity

Follow these steps to complete the activity and claim your credit:

1. Read the related article from The BMJ: Easily missed:

Kawasaki disease

2. Once you have read the article, click the 'Save and continue'
button on this page to go to the multiple choice questions.

Preguntas

A father brings his 4 year old son, James, to see you in

primary care. Five days ago James developed a sore throat.
At the time, his father measured his temperature using an ear
thermometer, and found that it was raised above 39C. He
thought this was most likely to be due to a viral sore throat,
and since then has been giving him regular doses of a
paracetamol suspension for children. However five days later
he is concerned that James still has a fever, which is why he
has brought him in to see you today.

On examination James is febrile, with a temperature of

39.5C. There is no evidence of tonsillitis, but his throat and
tongue look red, and his lips look a little sore and cracked.
You are unable to see any white spots inside his mouth. You
note that both of his eyes look red and bloodshot, but can
see no discharge to suggest conjunctivitis. He does not have
a rash. You can feel a single enlarged cervical lymph node on
the right, measuring around 2 cm in diameter.

What should be your provisional diagnosis?

1.

Viral sore throat

2.

Bacterial (streptococcal) sore throat

3.

Incomplete Kawasaki disease

4.
 Kawasaki disease

c : Incomplete Kawasaki disease

This child has had a fever of greater than 39C for five days, and
meets three of the additional diagnostic criteria for Kawasaki
disease, as he has bilateral conjunctival redness without
discharge, characteristic changes to the lips and oral cavity, and
cervical lymphadenopathy.

The diagnostic features of Kawasaki disease are [ 1 ] :

Fever (>39C) for at least four days and at least four of the
following features, after exclusion of other similar diseases.

Bilateral non-exudative bulbar conjunctival injection

Polymorphous exanthema

Changes in the extremities (acute - erythema of palms and

soles, oedema of hands and feet. Subacute - periungual
peeling of fingers and toes)

Changes to lips and oral cavity (red, fissured lips, strawberry

tongue, erythema of oropharyngeal mucosa, without exudates)

Cervical lymphadenopathy (1.5 cm, usually unilateral, rare

in infants)

Incomplete Kawasaki disease (fever but fewer than four diagnostic

criteria) is common (15-20% of all cases), so you should have a
high index of suspicion for any young child or infant with
prolonged fever and no clear diagnosis. Incomplete Kawasaki
disease is associated with an increased incidence of coronary
artery abnormalities, possibly caused by delayed
diagnosis. [ 1 ] [ 2 ]

The clinical presentation described above could also occur in a

bacterial or viral sore throat, but in this situation you should
assume that the child has incomplete Kawasaki disease until
proven otherwise. This would involve arranging for a prompt
(same day) hospital admission for further assessment.

Which of the following blood test abnormalities would you

most expect to see early on in the course of Kawasaki
disease?

1.

Raised urea and creatinine, and electrolyte abnormalities

2.

Clotting abnormalities

3.

Raised inflammatory markers, leucocytosis and

neutrophilia

4.

Thrombocytosis
c : Raised inflammatory markers, leucocytosis and
neutrophilia

There is no diagnostic test available for Kawasaki disease, but

certain blood test features may help with the diagnosis. In
practice, blood tests would usually be requested within a
secondary care setting, as GPs who suspect the diagnosis on
clinical grounds will usually need to refer patients promptly to
hospital for further assessment.

Leucocytosis and neutrophilia are usually found in patients with

Kawasaki disease. Inflammatory markers are typically raised and
mild abnormalities of liver function tests are common. White blood
cells are often present in the (sterile) urine or cerebrospinal fluid,
or both. A microbiologically confirmed infection is also present in a
third of patients with Kawasaki disease and should not preclude
the diagnosis. [ 3 ]

Thrombocytosis is common but occurs subacutely (week two to

three), so it is not helpful diagnostically.

Assuming that treatment is given promptly (within five to ten

days of the onset of symptoms), what is the risk that a child
with Kawasaki disease will develop coronary artery lesions?

1.

0%

2.

5%
3.

10%

4.

20%

b : 5%

It is important that Kawasaki disease is diagnosed early because

treatment with intravenous immunoglobulin within five to ten days
of fever onset reduces the incidence of coronary artery lesions
from 25% to approximately 5%. [ 1 ] [ 4 ] Delays in treatment might
lead to unnecessary morbidity and occasionally death. The
mortality rate is about 0.2% and is most commonly secondary to
thrombosis of giant aneurysms or later myocardial ischaemia and
infarction. [ 1 ]

Sophie is a 3 year old girl who was discharged from hospital

two days ago following treatment for Kawasaki disease. Her
parents come to see you in primary care. From the discharge
letter, you can see that she was treated with intravenous
immunoglobulin and aspirin. Sophies parents mention that
they were told at the hospital that some of Sophies routine
vaccinations may need to be delayed because of the type of
treatment she has received in hospital. There is nothing
documented on the discharge letter about this. You note that
Sophie is due receive her second measles mumps and
rubella (MMR) jab in two months time.
What should you advise?

1.

It is safe for Sophie to receive her MMR booster as usual in

two months time

2.

Sophies MMR booster will need to be delayed until five

months after her treatment

3.

Sophies MMR booster will need to be delayed until 11

months after her treatment

4.

The safest thing to do is to omit the MMR booster

altogether

c : Sophies MMR booster will need to be delayed until 11

months after her treatment

Live vaccines (such as measles, mumps, and rubella) should be

delayed for 11 months after treatment with intravenous
immunoglobulin. [ 1 ]
Sophie's parents are anxious that Sophie may be at risk of
developing Kawasaki disease again in future. Regarding the
risk of recurrence, which of the following should you advise
them?

C
Youranswer a

a.
ItisnotpossibletodevelopKawasakidiseaseasecondtime

b.
Itisrare(withalessthan1%risk)todevelopKawasakidiseaseasecondtime

c.
ItislikelythatshewillhaveanotherepisodeofKawasakidiseaseinfuture

d.
Sophieisnotatriskofdevelopingnewcardiacproblemsyearsinfuturerelatedtothisepisode
ofKawasakidisease

b : It is rare (with a less than 1% risk) to develop Kawasaki

disease a second time

Recurrence of Kawasaki disease is unlikely, and the risk of this is

less than 1%. [ 1 ]

Cardiac problems such as coronary artery abnormalities,

myocardial and valvular dysfunction and clinical important
pericardial effusions may be identified at presentation in patients
with severe Kawasaki disease, but usually develop in the
subacute phase (two to three weeks).

Echocardiography is performed at presentation, with follow-up

echocardiograms at two weeks - if there is concern - six weeks,
and often at six months. [ 5 ]
More patients affected by Kawasaki Disease in early childhood
are now entering adult life, and those with persistent or resolved
coronary aneurysms are at lifelong risk of acute coronary
syndromes (ACS). A patient safety alert emphasises the high risk
and atypical presentation of coronary artery aneurysms, coronary
thrombosis, and myocardial ischaemia or infarction in patients with
Kawasaki Disease. [ 6 ] It highlights the importance of seeking
specialist advice. Adults and children previously diagnosed with
coronary artery aneurysms following Kawasaki Disease should be
regularly reviewed by a specialist centre, and supplied with a
Person Specific Protocol (PSP) for use in emergencies. [ 6 ]

References
1. NewburgerJW,TakahashiM,GerberMA,GewitzMH,TaniLY,BurnsJC,etalDiagnosis,
treatment,andlongtermmanagementofKawasakidisease:astatementforhealthprofessionals
fromtheCommitteeonRheumaticFever,Endocarditis,andKawasakiDisease,Councilon
CardiovascularDiseaseintheYoung,AmericanHeartAssociation.Pediatrics2004:114:1708
33.

2. YeomJS,WooHO,ParkJS,ParkES,SeoJH,YounHS.Kawasakidiseaseininfants.KoreanJ
Pediatr2013;56:37782.

3. BenselerSM,McCrindleBW,SilvermanED,TyrrellPN,WongJ,YeungRS.Infectionsand
Kawasakidisease:implicationsforcoronaryarteryoutcome.Pediatrics2005;116:e7606.

4. TsudaE,HamaokaK,SuzukiH,SakazakiH,MurakamiY,NakagawaM,etal.Asurveyofthe
3decadeoutcomeforpatientswithgiantaneurysmscausedbyKawasakidisease.AmHeart
J2014;167:24958.

5. WoodLE,TullohRM.Kawasakidiseaseinchildren.Heart2009;95:78792.

6. NHS Improvement. Patient Safety Alert: Risk of death and

serious harm from failure to recognise acute coronary syndromes
in Kawasaki disease patients. 2016.

certificado
Video:https://www.youtube.com/watch?v=thdcueIequ0 http://www.kdfoundation.org/