Kawasaki disease Flashcards Preview

Nelson - Rheumatic Diseases of Childhood > Kawasaki disease > Flashcards

Flashcards in Kawasaki disease Deck (40)
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1
Q

Kawasaki disease is aka

A

Mucocutaneous lymph node syndrome and infantile polyarteritis nodosa

2
Q

A vasculitis with a predilection for the coronary arteries

A

KD

3
Q

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

A

20-25

4
Q

KD: Predictors of poor outcome

A

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
Q

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

A

Medium

6
Q

3-phase process to the arteriopathy of KD

A

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
Q

Characteristic of KD fever

A

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

8
Q

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

A

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
Q

Most important manifestation of KD

A

Cardiac involvement

10
Q

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

A

In the 2nd to 3rd wk of illness

11
Q

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

A

> 8 mm internal diameter

12
Q

KD can be divided into 3 clinical phases

A

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

13
Q

KD: Acute febrile phase lasts

A

1-2 weeks

14
Q

KD: Phase associated with desquamation

A

Subacute

15
Q

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

A

Convalescent phase

16
Q

KD: Convalescent phase typically occurs about when

A

6-8 weeks after onset of illness

17
Q

KD: Phase with the highest risk of sudden death

A

Subacute

18
Q

KD: Phase associated with development of CAA

A

Subacute

19
Q

KD: Convalescent phase lasts until

A

Normal ESR

20
Q

KD: Leukocyte count

A

Elevated, predom neutrophils and immature forms

21
Q

KD: Anemia

A

Normo normo

22
Q

KD: Platelet count

A

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

23
Q

Diameter of small aneurysm

A

Less than 5mm internal diameter

24
Q

Diameter of medium aneurysm

A

5-8mm internal diameter

25
Q

z-score of a giant aneurysm

A

> /10

26
Q

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

A

Giant aneurysm

27
Q

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

A

2-3 wk of illness

28
Q

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

A

6-8 wk after onset of illness

29
Q

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

A

1 year later

30
Q

Atypical/incomplete KD is defined as

A

Persistent fever but fewer than 4 of the 5 principal characteristics

31
Q

KD: Age group with highest likelihood of CAA

A

Infants

32
Q

KD: Age group in which incomplete cases occur most frequently

A

Infants

33
Q

MC childhood infections that mimic KD

A

Adenovirus, measles, scarlet fever

34
Q

Treatment for KD acute stage

A

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
Q

Treatment for KD convalescent stage

A

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

36
Q

KD: LONG-TERM THERAPY FOR PATIENTS WITH CORONARY ABNORMALITIES

A

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

37
Q

KD Treatment: ACUTE CORONARY THROMBOSIS

A

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

38
Q

IVIG-resistant KD is defined as

A

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

39
Q

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

A

Reye syndrome

40
Q

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

A

11 mo