Kawasaki Disease

Question 1

What is Kawasaki disease (KD)?

Answer 1

It is defined as an acute systemic, medium-sized vessel vasculitis, which tends to affect the coronary arteries

Question 2

What four risk factors are associated with Kawasaki disease?

Answer 2

Children < 5 Years Old

Male Gender

Asian Ethnicity

Family History

Question 3

What are the seven clinical features of Kawasaki disease?

Answer 3

High Fever > 39C For > 5 Days

Erythematous Maculopapular Rash

Palm & Sole Desquamation

Strawberry Tongue

Bright Red Cracked Lips

Bilateral Conjunctivitis

Cervical Lymphadenopathy

Question 4

What is palm & sole desquamation?

Answer 4

It is defined as peeling of the skin

Question 5

What is a strawberry tongue?

Answer 5

It is defined as a red tongue, with large papillae

Question 6

What are the three phases of Kawasaki disease?

Answer 6

Acute Phase

Subacute Phase

Convalescent Phase

Question 7

When does the acute phase of Kawasaki disease?

Answer 7

It occurs within 1 -2 weeks of fever onset

Question 8

What is the acute phase of Kawasaki disease?

Answer 8

It results in the presentation of high fever, irritability, rash, mucositis, peripheral erythema and oedema

Question 9

When does the subacute phase of Kawasaki disease occur?

Answer 9

It occurs within 2 - 4 weeks of fever onset

Question 10

What is the subacute phase of Kawasaki disease?

Answer 10

In this phase, individuals are afebrile and most clinical features begin to resolve

However, there is development of hand/feet desquamation and polyarthralgia

Question 11

When does the convalescent phase of Kawasaki disease occur?

Answer 11

It occurs within 4 - 8 weeks of fever onset

Question 12

What is the convalescent phase of Kawasaki disease?

Answer 12

This is an asymptomatic period, in which most clinical features have resolved

Question 13

In which phase of Kawasaki disease, is the risk of developing cardiac complications highest?

Answer 13

Subacute Phase

Question 14

What three investigations are used to diagnose Kawasaki disease?

Answer 14

Blood Tests

Urinalysis

ECHO Scans

Question 15

What nine bood test results indicate Kawasaki disease?

Answer 15

Decreased Haemoglobin Levels

Increased Platelet Levels

Increased White Cell Levels

Increased ALP Levels

Increased ALT Levels

Increased AST Levels

Decreased Albumin Levels

Increased CRP Levels

Increased ESR Levels

Question 16

What is the feature of Kawasaki disease on urinalysis?

Answer 16

There is increased white blood cells without infection

Question 17

What is the gold standard investigation used to diagnose Kawasaki disease?

Answer 17

ECHO Scans

Question 18

When should ECHO scans be used to diagnose Kawasaki disease?

Answer 18

It should be conducted in all suspected patients without delay

Following diagnosis, it is used to monitor disease progression and complication development

Question 19

What are the five features of Kawasaki disease on ECHO scans?

Answer 19

Coronary Artery Aneurysms

Coronary Artery Thrombosis

Valvular Disease

Pericardial Effusion

Decreased Ventricular Function

Question 20

What is important to note about the use of ECHO scans when diagnosing Kawasaki disease?

Answer 20

In early disease, there may be normal ECHO results

Question 21

What does the ‘American Heart Association’ criteria state for a diagnosis of Kawasaki disease to be obtained?

Answer 21

A diagnosis can be obtained in cases where individuals have a high fever > 39C for at least five days, plus at least four out of five key clinical features…

• Mucositis: Erythema & Cracking of Lips, Strawberry Tongue, Oral Erythema
• Conjunctivitis: Bilateral Conjunctivitis Without Exudate
• Rash: Maculopapular, Erythroderma, Erythema Multiforme
• Peripheral Changes: Erythema, Oedema, Desquamation
• Cervical Lymphadenopathy

Question 22

What is the most appropriate management step in suspected Kawasaki disease?

Answer 22

There should be urgent secondary care admission

Question 23

What are the two pharmacological management options of Kawasaki disease?

Answer 23

Oral High Dose Aspirin

IV Immunoglobulins

Question 24

What is the gold standard management option of Kawasaki disease?

Answer 24

Oral high dose aspirin + single dose of IV immunoglobulin

Question 25

How is aspirin used to manage Kawasaki disease?

Answer 25

It is used to reduce the risk of coronary artery aneurysms/thrombosis

Question 26

What aspirin regime should be administered to manage Kawasaki disease?

Answer 26

A high dose aspirin is administered until the fever has resolved for a period of 48 hours

Following this, low dose aspirin should be administered for approximately 6 weeks

Question 27

Why is aspirin usually contraindicated in children < 16 years old?

Answer 27

Reye’s Syndrome

Question 28

What is Reye’s syndrome?

Answer 28

It is a rare acute encephalopathy associated with liver failure

Question 29

How is IV immunoglobulin used to manage Kawasaki disease?

Answer 29

It is used to reduce the risk of coronary artery aneurysms

Question 30

What IV immunoglobulin regime should be administered to manage Kawasaki disease?

Answer 30

A single dose is administered within the first 10 days of illness

However, if there is no clinical feature improvement within 36 hours, a second dose can be administered

Question 31

What are the seven complications are associated with Kawasaki disease?

Answer 31

Coronary Artery Aneurysms

Coronary Artery Thrombosis

Myocardial Infarction

Valvular Heart Disease

Myocarditis

Pericarditis

Arrhythmias