Kawasaki disease Flashcards
Kawasaki disease is aka
Mucocutaneous lymph node syndrome and infantile polyarteritis nodosa
A vasculitis with a predilection for the coronary arteries
KD
Approximately ___ % of untreated children with KD develop coronary artery abnormalities
20-25
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
KD is a vasculitis that predominantly affects the ___-size arteries
Medium
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
Characteristic of KD fever
1) High (≥38.3°C [101°F]) 2) Unremitting 3) Unresponsive to antibiotics
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
Most important manifestation of KD
Cardiac involvement
KD: CAA develop in up to 25% of untreated patients when?
In the 2nd to 3rd wk of illness
Giant coronary artery aneurysms pose the greatest risk for rupture, thrombosis or stenosis, and myocardial infarction and is defined as
> 8 mm internal diameter
KD can be divided into 3 clinical phases
1) Acute febrile phase 2) Subacute phase 3) Convalescent ohase
KD: Acute febrile phase lasts
1-2 weeks
KD: Phase associated with desquamation
Subacute
KD: Phase associated with disappearance of all clinical signs of illness
Convalescent phase
KD: Convalescent phase typically occurs about when
6-8 weeks after onset of illness
KD: Phase with the highest risk of sudden death
Subacute
KD: Phase associated with development of CAA
Subacute
KD: Convalescent phase lasts until
Normal ESR
KD: Leukocyte count
Elevated, predom neutrophils and immature forms
KD: Anemia
Normo normo
KD: Platelet count
1st week Normal; 2nd-3rd week Rapidly increases sometimes exceeding 1,000,000
Diameter of small aneurysm
Less than 5mm internal diameter
Diameter of medium aneurysm
5-8mm internal diameter
z-score of a giant aneurysm
> /10
Threshold at which anticoagulation should be initiated in an aneurysm as in KD
Giant aneurysm
KD: Echocardiography should be performed at diagnosis and again after ___
2-3 wk of illness
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
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
Atypical/incomplete KD is defined as
Persistent fever but fewer than 4 of the 5 principal characteristics
KD: Age group with highest likelihood of CAA
Infants
KD: Age group in which incomplete cases occur most frequently
Infants
MC childhood infections that mimic KD
Adenovirus, measles, scarlet fever
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
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
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)
KD Treatment: ACUTE CORONARY THROMBOSIS
Prompt fibrinolytic therapy with tissue plasminogen activator or other thrombolytic agent under supervision of a pediatric cardiologist
IVIG-resistant KD is defined as
Persistent or recrudescent fever 36 hr after completion of the initial IVIG infusion
KD: Patients undergoing long-term aspirin therapy should receive annual influenza vaccination to reduce the risk of ___
Reye syndrome
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
