Kawasaki disease Flashcards Preview

Nelson - Rheumatic Diseases of Childhood > Kawasaki disease > Flashcards

Flashcards in Kawasaki disease Deck (40):
1

Kawasaki disease is aka

Mucocutaneous lymph node syndrome and infantile polyarteritis nodosa

2

A vasculitis with a predilection for the coronary arteries

KD

3

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

20-25

4

KD: Predictors of poor outcome

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

5

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

Medium

6

3-phase process to the arteriopathy of KD

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

7

Characteristic of KD fever

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

8

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

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

9

Most important manifestation of KD

Cardiac involvement

10

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

In the 2nd to 3rd wk of illness

11

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

>8 mm internal diameter

12

KD can be divided into 3 clinical phases

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

13

KD: Acute febrile phase lasts

1-2 weeks

14

KD: Phase associated with desquamation

Subacute

15

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

Convalescent phase

16

KD: Convalescent phase typically occurs about when

6-8 weeks after onset of illness

17

KD: Phase with the highest risk of sudden death

Subacute

18

KD: Phase associated with development of CAA

Subacute

19

KD: Convalescent phase lasts until

Normal ESR

20

KD: Leukocyte count

Elevated, predom neutrophils and immature forms

21

KD: Anemia

Normo normo

22

KD: Platelet count

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

23

Diameter of small aneurysm

Less than 5mm internal diameter

24

Diameter of medium aneurysm

5-8mm internal diameter

25

z-score of a giant aneurysm

>/10

26

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

Giant aneurysm

27

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

2-3 wk of illness

28

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

6-8 wk after onset of illness

29

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

1 year later

30

Atypical/incomplete KD is defined as

Persistent fever but fewer than 4 of the 5 principal characteristics

31

KD: Age group with highest likelihood of CAA

Infants

32

KD: Age group in which incomplete cases occur most frequently

Infants

33

MC childhood infections that mimic KD

Adenovirus, measles, scarlet fever

34

Treatment for KD acute stage

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

35

Treatment for KD convalescent stage

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

36

KD: LONG-TERM THERAPY FOR PATIENTS WITH CORONARY ABNORMALITIES

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

37

KD Treatment: ACUTE CORONARY THROMBOSIS

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

38

IVIG-resistant KD is defined as

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

39

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

Reye syndrome

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

11 mo