Child health- Kawasaki disease

Question 1

define kawasaki disease

Answer 1

acute, self limiting systemic vasculitis, affecting children <5years old, particularly of East Asain origin.

more common in boys than girls

Question 2

incidence of kawasaki disease

Answer 2

8 per 100,000 children under 5 in the UK
>200 per 100,000 in Japan

Question 3

which vessels are affected by KD?

Answer 3

medium sized arteries, especially coronary.
risk of CAA and sudden cardiac death

Question 4

what are the clinical deatures of kawasaki disease?

Answer 4

fever >5 days plus 4/5 of
muscositis, conjuctivitis, rash, peripheral changes, cervical lymphadenopathy

TEMPERS

Question 5

what are the phases of kawasaki disease?

Answer 5

ACUTE
SUBACUTE
CONVALESCENT

Question 6

describe the acute phase of KD

Answer 6

1-2 weeks: high fever, irritability, rash, mucositis, peripheral erythema/oedema

Question 7

describe the subacute phase of KD

Answer 7

2-4 weeks: afebrile, desquamation, highest risk for cardiac complications

Question 8

convalascent phase

Answer 8

4-8 weeks: asymptomatic, resolution of clinical features, possible coronary artery aneurysm improvement

Question 9

differential diagnoses for kawasaki disease

Answer 9

scarlet fever. toxic shock syndrome, Paediatric Inflammatory Multisystem Syndrome (PIMS-TS), viral exanthems, measles, systemic-onset juvenile idiopathic arthritis, Stevens-Johnson syndrome

Question 10

investigations for kawasaki disease

Answer 10

bedside: ECG (arrythmias, ST-T changes), urinalysis (sterile pyuria)
Laboratory: FBC (anaemia, raised WBC, platelets), U&Es, LFTs, ESR/CRP, ASOT.
Imaging: echocardiogram (coronary artery aneurysms, valvular disease, thrombosis, myocarditis, pericardial effusion).

Question 11

management for kawasaki disease

Answer 11

hospital admission, oral aspirin (high dose to resolve fever, then continue on low dose)
intravenous immunoglobulin within 10 days, possibly corticosteroids or infliximab for refractory cases

Question 12

complications of kawasaki disease

Answer 12

coronary artery aneurysms, myocarditis, pericarditis, arrhythmias, valvular disease, coronary artery thrombosis, myocardial infarction, sudden cardiac death.

Question 13

what is the main theory about origin of kawasaki disease

Answer 13

infectious trigger causes an immune mediated response in a genetically susceptible child.
no genetic variants or infectious agents have been identified

Question 14

what are the risk factors for kawasaki disease

Answer 14

asain ethnicity, especially japanese
aged <5 years (80% of cases)
male sex (male to female ratio 1.5:1)

Question 15

what are the investigations for kawasaki disease

Answer 15

no diagnostic test
clinical diagnosis but all patients need an echocardiogram.

Question 16

what is a complication/risk of using aspirin in children under 16?

Answer 16

reyes syndrome
a rare acute encephalopathy
associated with liver failure
benefits outweigh the risk

Question 17

what is the biggest risk with kawasaki disease

Answer 17

developing a CAA
15-25% untreated