Kawasaki disease

Question 1

Kawasaki disease is aka

Answer 1

Mucocutaneous lymph node syndrome and infantile polyarteritis nodosa

Question 2

A vasculitis with a predilection for the coronary arteries

Answer 2

KD

Question 3

Approximately ___ % of untreated children with KD develop coronary artery abnormalities

Answer 3

20-25

Question 4

KD: Predictors of poor outcome

Answer 4

1) Young age 2) Male gender 3) Persistent fever 4) Poor response to IVIG 5) Thrombocytopenia 6) Transaminitis 7) Hyponatremia 8) Hypoalbuminemia 9) Elevated N-terminal-probrain natriuretic protein 10) Elevated C-reactive protein levels

Question 5

KD is a vasculitis that predominantly affects the ___-size arteries

Answer 5

Medium

Question 6

3-phase process to the arteriopathy of KD

Answer 6

1) Neutrophilic necrotizing arteritis occurring in the 1st 2 wk of illness that begins in the endothelium and moves through the coronary wall 2) Subacute/chronic vasculitis driven by lymphocytes, plasma cells, and eosinophils, which may last weeks to years and results in fusiform aneurysms

Question 7

Characteristic of KD fever

Answer 7

1) High (≥38.3°C [101°F]) 2) Unremitting 3) Unresponsive to antibiotics

Question 8

In addition to fever, the 5 principal clinical criteria of KD are

Answer 8

At least 4 days and at least 4 of the ff: 1) Bilateral nonexudative conjunctival injection with limbal sparing 2) Erythema of the oral and pharyngeal mucosa with strawberry tongue and red, cracked lips 3) Edema and erythema of the hands and feet 4) Rash of various forms (maculopapular, erythema multiforme, or scarlatiniform); and 5) Nonsuppurative cervical lymphadenopathy, usually unilateral, with node size >1.5 cm

Question 9

Most important manifestation of KD

Answer 9

Cardiac involvement

Question 10

KD: CAA develop in up to 25% of untreated patients when?

Answer 10

In the 2nd to 3rd wk of illness

Question 11

Giant coronary artery aneurysms pose the greatest risk for rupture, thrombosis or stenosis, and myocardial infarction and is defined as

Answer 11

> 8 mm internal diameter

Question 12

KD can be divided into 3 clinical phases

Answer 12

1) Acute febrile phase 2) Subacute phase 3) Convalescent ohase

Question 13

KD: Acute febrile phase lasts

Answer 13

1-2 weeks

Question 14

KD: Phase associated with desquamation

Answer 14

Subacute

Question 15

KD: Phase associated with disappearance of all clinical signs of illness

Answer 15

Convalescent phase

Question 16

KD: Convalescent phase typically occurs about when

Answer 16

6-8 weeks after onset of illness

Question 17

KD: Phase with the highest risk of sudden death

Answer 17

Subacute

Question 18

KD: Phase associated with development of CAA

Answer 18

Subacute

Question 19

KD: Convalescent phase lasts until

Answer 19

Normal ESR

Question 20

KD: Leukocyte count

Answer 20

Elevated, predom neutrophils and immature forms

Question 21

KD: Anemia

Answer 21

Normo normo

Question 22

KD: Platelet count

Answer 22

1st week Normal; 2nd-3rd week Rapidly increases sometimes exceeding 1,000,000

Question 23

Diameter of small aneurysm

Answer 23

Less than 5mm internal diameter

Question 24

Diameter of medium aneurysm

Answer 24

5-8mm internal diameter

Question 25

z-score of a giant aneurysm

Answer 25

>/10

Question 26

Threshold at which anticoagulation should be initiated in an aneurysm as in KD

Answer 26

Giant aneurysm

Question 27

KD: Echocardiography should be performed at diagnosis and again after ___

Answer 27

2-3 wk of illness

Question 28

KD: If the results are normal at 2-3 wk of illness, a repeat study should be performed when

Answer 28

6-8 wk after onset of illness

Question 29

KD: In patients without coronary abnormalities at any time during the illness, performance of echocardiography and a lipid profile is recommended when

Answer 29

1 year later

Question 30

Atypical/incomplete KD is defined as

Answer 30

Persistent fever but fewer than 4 of the 5 principal characteristics

Question 31

KD: Age group with highest likelihood of CAA

Answer 31

Infants

Question 32

KD: Age group in which incomplete cases occur most frequently

Answer 32

Infants

Question 33

MC childhood infections that mimic KD

Answer 33

Adenovirus, measles, scarlet fever

Question 34

Treatment for KD acute stage

Answer 34

2 g/kg of IVIG and high- dose aspirin (80-100 mg/kg/day divided q6h) within 10 days of disease onset and ideally as soon as possible after diagnosis

Question 35

Treatment for KD convalescent stage

Answer 35

Aspirin 3-5 mg/kg (antithrombotic dose) once daily orally until 6-8 wk after illness onset if normal coronary findings throughout course

Question 36

KD: LONG-TERM THERAPY FOR PATIENTS WITH CORONARY ABNORMALITIES

Answer 36

Aspirin 3-5 mg/kg once daily orally; Clopidogrel 1 mg/kg/day (maximum: 75 mg/day)

Question 37

KD Treatment: ACUTE CORONARY THROMBOSIS

Answer 37

Prompt fibrinolytic therapy with tissue plasminogen activator or other thrombolytic agent under supervision of a pediatric cardiologist

Question 38

IVIG-resistant KD is defined as

Answer 38

Persistent or recrudescent fever 36 hr after completion of the initial IVIG infusion

Question 39

KD: Patients undergoing long-term aspirin therapy should receive annual influenza vaccination to reduce the risk of ___

Answer 39

Reye syndrome

Question 40

As IVIG may interfere with the immune response to live virus vaccines as a result of specific antiviral antibody, the measles-mumps-rubella and varicella vaccinations should generally be deferred until how long after IVIG administration

Answer 40

11 mo