142 - Kawasaki Disease

Question 1

Leading cause of acquired heart disease in children in developed nations

Answer 1

Kawasaki disease

Question 2

Classic KD is diagnosed in a patient with prolonger fever and _____ of _____ clinical features

Answer 2

4

5

Question 3

Other terms for KD

Answer 3

Mucocutaneous lymph node syndrome

Infantile periarteritis nodosa

Question 4

Attack rate of KD is highest in _____ children

Answer 4

Asian, particularly Japanese, Korean, and Chinese

Question 5

KD is predominantly an illness of young children, with 80% of cases occurring in children ages

Answer 5

6 months to 5 years

Question 6

Y/N: Girls are more commonly affected by KD than girls at a ratio of 3:2

Answer 6

No - Boys are more commonly affected than girls

Question 7

Peak age of KD

Answer 7

9 months to 11 months

Question 8

Forms of generalized exanthem in KD

Answer 8

Morbilliform
Targetoid
Scarlatiniform (diffuse erythema)

Question 9

In the acute febrile phase of KD, _____ erythema and desquamation are commonly observed

Answer 9

Groin

Question 10

Classic periungual desquamation of the fingers and toes does not begin until

Answer 10

Second to third week after fever begins

Question 11

In the third to sixth week after illness in KD, transverse lines across the fingernails (_____) are often apparent

Answer 11

Beau lines

Question 12

A common finding in children with KD is erythema and swelling at the site of ______ vaccine administration

Answer 12

Bacille Calmette-Guerin

Question 13

Stages of KD

Answer 13

Acute febrile phase
Subacute phase
Convalescent phase

Question 14

Begins when fever resolves and continues until all clinical features have normalized

Answer 14

Subacute phase

Question 15

Follows the subacute phase and continues until the ESR normalizes

Answer 15

Convalescent phase

Question 16

Least commonly observed clinical feature, occurring in approximately 75% of children with classic KD

Answer 16

Cervical lymphadenopathy

Question 17

More than 50% of KD patients have myocarditis during the acute febrile phase, manifested clinically as

Answer 17

Tachycardia disproportionate to fever

Question 18

A complete blood count reveals either a _____ white blood cell count with a _____ predominance

Answer 18

Normal or elevated

Neutrophil

Question 19

Thromobocyto(-sis/-penia) has been reported to be associated with a more severe outcome

Answer 19

-penia

Question 20

Thrombocytosis, with platelet counts sometimes exceeding 1,000,000/mm3 is characteristic of the _____ phase of KD

Answer 20

Subacute

Question 21

Once IVIG is given, the _____ cannot be used to follow clinical response, because IVIG itself transiently increases the _____

Answer 21

ESR

Question 22

A CBC and CRP or ESR should be performed at _____, and the CRP repeated at ______

Answer 22

Baseline

2 to 3 weeks and 6 to 8 weeks after onset

Question 23

Imaging that should be performed in all children with suspected KD

Answer 23

Echocardiography

Question 24

Echocardiography should be performed at

Answer 24

Diagnosis, at 2 to 3 weeks after fever onset, and at 6 to 8 weeks after fever onset

Question 25

Electrocardiogram in the acute febrile phase of illness most often shows

Answer 25

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

Question 26

Diagnostic criteria for Kawasaki disease:

Fever lasting 5 or more days, high spiking and intermittent, with at least 4 of the 5 clinical features:

Answer 26

1. Bilateral, nonexudative conjunctival injection
2. Oral mucosal changes, including red, dry, cracked lips, pharyngeal erythema, and/or strawberry tongue
3. Changes of the hands and feet: erythema of palms and soles and/or swelling of the hands and feet during the acute phase, and/or periungual desquamation of the fingers and toes during the subacute phase
4. Rash: erythematous morbilliform, scarlatiniform, or targetoid
5. Cervical lymphadenopathy at least 1.5 cm in diameter

Question 27

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 27

Incomplete (or atypical) KD

Question 28

Y/N: Infants 6 months of age or older can have mild or subtle clinical findings with KD, but have a high risk of developing coronary artery abnormalities

Answer 28

No - younger

Question 29

Approximately 85% of KD children treated with IVIG and aspirin within the first _____ days of illness respond with rapid resolution of fever and other clinical signs

Answer 29

10

Question 30

Treatment of acute Kawasaki disease

Answer 30

2g/kg of IVIG infused over 10-12 hours

Aspirin 80-100 mg/kg/day every 6 hours orally

Question 31

Regimen of IVIG and aspirin when administered to children with KD within the first 10 days of fever, was shown to reduce the prevalence of coronary artery abnormalities from _____% in untreated patients to _____% in those who receive therapy

Answer 31

25

5

Question 32

Aspirin is given in high doses during acute KD for _____ effect

Answer 32

Antiinflammatory

Question 33

Aspirin is generally continued at 80 to 100 mg/kd/day until

Answer 33

14th illness day or

Until the patient has beed afebrile for at least 2 days

Question 34

Aspirin is then reduced to 3 to 5 mg/kg/day given in a single daily dose, for its _____ effect

Answer 34

Antithrombotic

Question 35

Aspirin is discontinued at

Answer 35

6 to 8 weeks after onset if all echocardiograms have been normal and acute-phase reactants have normalized

Question 36

Approximately _____% of acute KD patients do not respond to initial therapy

Answer 36

15

Question 37

Most patients with “refractory” KD will respond to

Answer 37

Second 2 g/kg IVIG infusion

Question 38

In patients who do not respond to initial therapy and are already in a high-risk category because of the presence of coronary artery dilation, _____ should be considered

Answer 38

Second dose of IVIG given with prednisolone in a tapering regimen over 2 to 3 weeks