22 - 142 - KAWASAKI DISEASE

Question 1

This nail finding is seen during the 3rd to 6th week after illness

A. Onychomadesis

B. Terry nails

C. Splinter hemorrhage

D. Beau’s lines

Answer 1

D

Question 2

Diagnostic criteria for classic kawaski disease includes the following except

A. Periungual desquamation

B. High spiking and intermittent fever >/= 5 days

C. Cervical lymphadenopathy >/= 1.5 cm

D. Bilateral exudative conjuctival injection

Answer 2

D

Question 3

Least commonly identified clnical feature of KD

A. Conjuctival injection

B. Strawberry tongue

C. Cervical lymphadenopathy

D. Desquamation of the entire hand and foot

Answer 3

C

Question 4

T/F: KD is more common in males

Answer 4

TRUE

Question 5

T/F: KD has a higher attake rate in Japanese children who adopt a western diet and lifestyle

Answer 5

TRUE

Question 6

T/F: Bullae, vesicles and ulcerations are seen in KD

Answer 6

FALSE

Question 7

Peak time to detect coronary artery dilatation in KD

A. first week of fever

B. 6-8 weeks after fever onset

C. 2-3 weeks after fever onset

D. 4-6 weeks after fever onset

Answer 7

C

Question 8

most common cause of acquired heart disease in children in developed nations

Answer 8

Kawasaki disease

Question 9

KD affects all blood vessels in the body, but primarily damages what size of BV?

Answer 9

medium-sized muscular arteries such as the coronary arteries

Question 10

In kawasaki disease, treatement with IVIG and aspirin should be initiated within how many days of fever?

Answer 10

First 10 days of fever

reduces the prevalence of coronary artery abnormalities from 25% in those treated with aspirin alone, to 5% in those who receive IVIG with aspirin.

Question 11

Long-term complications of kawasaki

Answer 11

thrombosis and stenosis of the major coronary arteries with myocardial ischemia.

Question 12

80% of KD patients occur in what age?

Answer 12

6 months to 5 years

Question 13

Peak age of illness in KD

Answer 13

9 to 11 months

Question 14

3 forms of kawasaki

Answer 14

1. Morbilliform
2. Targetoid
3. Scarlatiniform

Question 15

What primary lesions are not observed in kawasaki

Answer 15

Bullae, vesicles, ulcers

Question 16

groin erythema and desquamation are commonly observed are frequently observed in what phase of kawasaki

Answer 16

Acute febrile phase

Question 17

Classic periungual desquamation of the fingers and toes does not begin until how many weeks after fever begins

Answer 17

second to third week after fever begins, and can progress to involve the entire hand and foot

Question 18

transverse lines across the fingernails (Beau lines) is apparent after how many weeks of illness

Answer 18

third to sixth week after illness

Question 19

Fever characteristics in kawasaki

Answer 19

fever in KD is daily, high spiking, intermittent, and lasts for 1 to 2 weeks

Question 20

3 stages of kawasaki

Answer 20

1. Acute febrile
2. Subacute
   - begins when fever resolves and continues until all clinical features have normalized
3. Convalescent
   - follows the subacute phase and continues until the erythrocyte sedimentation rate [ESR] normalizes, usually at 6 to 8 weeks after the onset of fever

Question 21

Conjunctival injection in KD

Answer 21

Bilateral, nonexudative

There may be limbal sparing (Fig. 142-5). Photophobia is a common accompanying feature.

Question 22

Oral findings in kawasaki

Answer 22

red, swollen, dry, cracked lips that may bleed (Fig. 142-6), a “strawberry” tongue, and erythema of the mouth and throat

Oral ulcers are not a feature of KD.

Question 23

Associated Clinical Features of Kawasaki Disease

Answer 23

Question 24

Three linked pathologic processes characteristic of KD vasculopathy

Answer 24

1. neutrophilic necrotizing arteritis, which occurs in the first 2 weeks after fever onset;
2. subacute/chronic vasculitis, which begins in the first 2 weeks but can persist for months to years and is comprised of lymphocytes (predominately CD8 T lymphocytes25 ), plasma cells (particularly immunoglobulin [Ig] A plasma cells26,27 ), eosinophils, and macrophages; and
3. luminal myofibroblastic proliferation, which is closely associated with subacute/chronic vasculitis and can result in progressive arterial stenosis

Question 25

Echocardiography should be performed in all children with suspected KD, and should be performed when?

Answer 25

1. At diagnosis
2. at 2 to 3 weeks after fever onset,
3. at 6 to 8 weeks after fever onset

Question 26

peak time to detect coronary artery dilation

Answer 26

2 to 3 weeks after onset of fever, during the subacute phase of illness

Question 27

Electrocardiogram in the acute febrile phase of illness most often shows

Answer 27

prolonged PR interval and/or nonspecific ST- and T-wave changes

Question 28

Diagnostic Criteria for Classic Kawasaki Disease

Answer 28

Question 29

refers to children with prolonged fever and fewer than 4 of the other features of illness who have a laboratory profile compatible with KD

Answer 29

Incomplete (or atypical) KD

Question 30

Criteria for Diagnosis of Incomplete Kawasaki Disease

Answer 30

Question 31

Question 32

Treatment of Acute Kawasaki Disease

Answer 32

Question 33

When is aspirin discontinued in kawasaki

Answer 33

Aspirin is discontinued if echocardiograms at 2 to 3 weeks and 6 to 8 weeks are normal and when acute-phase reactants have normalized.

Question 34

Clinical Course and prognosis of KD

Answer 34

Approximately 85% of KD children treated with IVIG and aspirin within the first 10 days of illness respond with rapid resolution of fever and other clinical signs. The vast majority of KD patients who are promptly diagnosed and treated do well, without developing cardiac complications. However, approximately 15% of KD children treated within the first 10 days of illness continue to have fever following a single infusion of IVIG with aspirin and require additional therapy; these patients have a higher risk of developing coronary artery abnormalities.

Question 35

Antiinflamatory dose of aspirin

Answer 35

80 to 100 mg/kg/day (high dose)

Question 36

Antithrombotic dose of aspirin

Answer 36

3 to 5 mg/kg/day

Question 37

n patients who do not respond to initial therapy and are already in a high-risk category because of the presence of coronary artery dilation, what management can we do

Answer 37

second dose of IVIG (2 mg/kg) given with prednisolone in a tapering regimen over 2 to 3 weeks should be considered. Other options for IVIG nonresponders include highdose intravenous methylprednisolone once daily for 3 days or infliximab