22 - 142 - KAWASAKI DISEASE Flashcards
This nail finding is seen during the 3rd to 6th week after illness
A. Onychomadesis
B. Terry nails
C. Splinter hemorrhage
D. Beau’s lines
D
Diagnostic criteria for classic kawaski disease includes the following except
A. Periungual desquamation
B. High spiking and intermittent fever >/= 5 days
C. Cervical lymphadenopathy >/= 1.5 cm
D. Bilateral exudative conjuctival injection
D
Least commonly identified clnical feature of KD
A. Conjuctival injection
B. Strawberry tongue
C. Cervical lymphadenopathy
D. Desquamation of the entire hand and foot
C
T/F: KD is more common in males
TRUE
T/F: KD has a higher attake rate in Japanese children who adopt a western diet and lifestyle
TRUE
T/F: Bullae, vesicles and ulcerations are seen in KD
FALSE
Peak time to detect coronary artery dilatation in KD
A. first week of fever
B. 6-8 weeks after fever onset
C. 2-3 weeks after fever onset
D. 4-6 weeks after fever onset
C
most common cause of acquired heart disease in children in developed nations
Kawasaki disease
KD affects all blood vessels in the body, but primarily damages what size of BV?
medium-sized muscular arteries such as the coronary arteries
In kawasaki disease, treatement with IVIG and aspirin should be initiated within how many days of fever?
First 10 days of fever
reduces the prevalence of coronary artery abnormalities from 25% in those treated with aspirin alone, to 5% in those who receive IVIG with aspirin.
Long-term complications of kawasaki
thrombosis and stenosis of the major coronary arteries with myocardial ischemia.
80% of KD patients occur in what age?
6 months to 5 years
Peak age of illness in KD
9 to 11 months
3 forms of kawasaki
- Morbilliform
- Targetoid
- Scarlatiniform
What primary lesions are not observed in kawasaki
Bullae, vesicles, ulcers
groin erythema and desquamation are commonly observed are frequently observed in what phase of kawasaki
Acute febrile phase
Classic periungual desquamation of the fingers and toes does not begin until how many weeks after fever begins
second to third week after fever begins, and can progress to involve the entire hand and foot
transverse lines across the fingernails (Beau lines) is apparent after how many weeks of illness
third to sixth week after illness
Fever characteristics in kawasaki
fever in KD is daily, high spiking, intermittent, and lasts for 1 to 2 weeks
3 stages of kawasaki
- Acute febrile
- Subacute
- begins when fever resolves and continues until all clinical features have normalized - Convalescent
- follows the subacute phase and continues until the erythrocyte sedimentation rate [ESR] normalizes, usually at 6 to 8 weeks after the onset of fever
Conjunctival injection in KD
Bilateral, nonexudative
There may be limbal sparing (Fig. 142-5). Photophobia is a common accompanying feature.
Oral findings in kawasaki
red, swollen, dry, cracked lips that may bleed (Fig. 142-6), a “strawberry” tongue, and erythema of the mouth and throat
Oral ulcers are not a feature of KD.
Associated Clinical Features of Kawasaki Disease
Three linked pathologic processes characteristic of KD vasculopathy
- neutrophilic necrotizing arteritis, which occurs in the first 2 weeks after fever onset;
- subacute/chronic vasculitis, which begins in the first 2 weeks but can persist for months to years and is comprised of lymphocytes (predominately CD8 T lymphocytes25 ), plasma cells (particularly immunoglobulin [Ig] A plasma cells26,27 ), eosinophils, and macrophages; and
- luminal myofibroblastic proliferation, which is closely associated with subacute/chronic vasculitis and can result in progressive arterial stenosis
Echocardiography should be performed in all children with suspected KD, and should be performed when?
- At diagnosis
- at 2 to 3 weeks after fever onset,
- at 6 to 8 weeks after fever onset
peak time to detect coronary artery dilation
2 to 3 weeks after onset of fever, during the subacute phase of illness
Electrocardiogram in the acute febrile phase of illness most often shows
prolonged PR interval and/or nonspecific ST- and T-wave changes
Diagnostic Criteria for Classic Kawasaki Disease
refers to children with prolonged fever and fewer than 4 of the other features of illness who have a laboratory profile compatible with KD
Incomplete (or atypical) KD
Criteria for Diagnosis of Incomplete Kawasaki Disease
Treatment of Acute Kawasaki Disease
When is aspirin discontinued in kawasaki
Aspirin is discontinued if echocardiograms at 2 to 3 weeks and 6 to 8 weeks are normal and when acute-phase reactants have normalized.
Clinical Course and prognosis of KD
Approximately 85% of KD children treated with IVIG and aspirin within the first 10 days of illness respond with rapid resolution of fever and other clinical signs. The vast majority of KD patients who are promptly diagnosed and treated do well, without developing cardiac complications. However, approximately 15% of KD children treated within the first 10 days of illness continue to have fever following a single infusion of IVIG with aspirin and require additional therapy; these patients have a higher risk of developing coronary artery abnormalities.
Antiinflamatory dose of aspirin
80 to 100 mg/kg/day (high dose)
Antithrombotic dose of aspirin
3 to 5 mg/kg/day
n patients who do not respond to initial therapy and are already in a high-risk category because of the presence of coronary artery dilation, what management can we do
second dose of IVIG (2 mg/kg) given with prednisolone in a tapering regimen over 2 to 3 weeks should be considered. Other options for IVIG nonresponders include highdose intravenous methylprednisolone once daily for 3 days or infliximab
what cardiac sequelae can happen in approximately 25% of untreated children
coronary artery abnormalities, including dilation and aneurysms that can lead to myocardial infarction and sudden death
intravenous immunoglobulin (IVIG) with aspirin, when given in the first 10 days of fever, reduces the prevalence of coronary artery abnormalities from 25% in untreated patients to how many percent?
5%
phase that begins when fever resolves and continues until all clinical features have normalized
subacute phase
phase that follows the subacute phase and continues until the erythrocyte sedimentation rate [ESR] normalizes, usually at 6 to 8 weeks after the onset of fever)
convalescent phase
Thrombocytosis, with platelet counts sometimes exceeding 1,000,000/mm3, is characteristic of what phase?
subacute phase
sometimes used to follow clinical response in patients refractory to IVIG therapy.
CRP
Once IVIG is given, the ESR cannot be used to follow clinical response, because IVIG itself transiently increases the ESR.
best single test to differentiate measles rash from kawasaki
measles IgM antibody is virtually always positive by the appearance of the measles rash
