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Kawasaki disease
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of Kawasaki disease are prepared by our editorial team based on guidelines from the Italian Society of Pediatrics (ISP 2021), the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019), and the American Heart Association (AHA 2017).
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2
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Screening and diagnosis
Diagnostic criteria: as per ISP 2021 guidelines, diagnose typical KD when fever lasting > 5 days is associated with ≥ 4 of the following clinical criteria:
bilateral non-exudative conjunctivitis
changes of lips and oral mucosa
changes of the extremities and perineal region
polymorphous exanthema
cervical lymphadenopathy).
E
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Classification and risk stratification
Diagnostic investigations
Electrocardiography: as per SHARE initiative 2019 guidelines, obtain ECG at baseline in all patients with suspected KD, as soon as the diagnosis is suspected.
B
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Echocardiography
Advanced imaging
Laboratory tests
Diagnostic procedures
Medical management
General principles: as per SHARE initiative 2019 guidelines, initiate treatment as soon as the diagnosis of complete or incomplete KD is made.
A
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IVIG
Aspirin
Corticosteroids
Monoclonal antibodies
Other immunosuppressive agents
Statins
Beta-blockers
Therapeutic procedures
Plasma exchange: as per SHARE initiative 2019 guidelines, do not perform plasma exchange in patients with KD, except on an individual basis after consultation with a specialist unit.
D
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Indications for revascularization
PCI
Surgical interventions
CABG: as per AHA 2017 guidelines, prefer CABG to PCI in patients with left main coronary artery disease, multi-vessel coronary artery disease with reduced LV function, multi-vessel coronary artery disease with lesions not amenable to PCI, and multi-vessel coronary artery disease in diabetic patients.
B
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Heart transplantation
Specific circumstances
Patient education
General counseling
As per AHA 2017 guidelines:
Consider providing general counseling regarding healthy lifestyle and activity promotion at every visit in all patients with or without coronary artery involvement.
C
Educate patients and families that having had KD is part of the patient's permanent medical history.
B
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Physical activity counseling
Pregnancy and contraception counseling
Preventative measures
Thromboprophylaxis: as per ISP 2021 guidelines, initiate dual anti-platelet prophylaxis with low-dose aspirin (at a single dose of 3-5 mg/kg/day) and clopidogrel (at a single dose of 0.2 mg/kg/day in pediatric patients aged < 24 months and up to 1 mg/kg/day in pediatric patients aged ≥ 24 months) in patients with medium-sized coronary artery aneurysms or with multiple and complex aneurysms.
E
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Immunizations
Follow-up and surveillance
Hospital discharge and follow-up: as per AHA 2017 guidelines, consider discharging patients without coronary artery involvement from cardiology care at 4-6 weeks after disease onset. Consider obtaining ongoing follow-up to 12 months.
C
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Clinical follow-up
Laboratory monitoring
ECG monitoring
Imaging follow-up
Surveillance angiography