Kawasaki Disease

There is no confirmatory test for Kawasaki disease (KD). North American clinical criteria require fever plus four of five principal clinical features; classic descriptions use at least 5 days of fever, but this duration should not force delay when the phenotype is sufficiently convincing. BMJ+1BMJManagement of Kawasaki disease | Archives of Disease in ChildhoodWolters KluwerIncomplete (atypical) Kawasaki disease

Principal manifestations include polymorphous rash, bilateral nonpurulent conjunctivitis, oral mucosal erythema or strawberry tongue, extremity erythema or edema, and unilateral cervical lymphadenopathy. BMJBMJKawasaki disease - Symptoms, diagnosis and treatment KD is an acute systemic vasculitis with particular clinical importance because coronary artery aneurysms are its major morbidity-producing complication. BMJBMJKawasaki disease - Symptoms, diagnosis and treatment

Laboratory findings support systemic inflammation but do not establish KD. Elevated C-reactive protein, erythrocyte sedimentation rate, or leukocytosis should heighten concern in a child with incomplete clinical features. Reported associated findings include early transaminase elevation, sterile pyuria, and thrombocytosis. BMJ+1BMJManagement of Kawasaki disease | Archives of Disease in ChildhoodWileyRevision of diagnostic guidelines for Kawasaki disease ...

Intravenous immune globulin (IVIG) and aspirin are established acute therapy. Randomized evidence indicates that IVIG plus aspirin reduces coronary artery abnormalities and systemic inflammation in acute KD. NEJMNEJMRandomized Trial of Pulsed Corticosteroid Therapy for ... A cited treatment report describes 2 g/kg IVIG as a single infusion as the standard initial regimen, but the supplied excerpts do not provide sufficient evidence to specify aspirin dose, duration, contraindications, or local protocol details. ScienceDirectScienceDirectEffective infliximab therapy for the early regression of ...

Failure to respond to initial IVIG identifies a group at increased risk for cardiac complications. ScienceDirectScienceDirectEffective infliximab therapy for the early regression of ... Second IVIG, infliximab, and corticosteroids have all been used for IVIG-resistant KD. ScienceDirectScienceDirectInfliximab versus second intravenous immunoglobulin for ... Selection should be directed with pediatric rheumatology and cardiology because the supplied sources do not establish a universal preferred rescue regimen.

All patients with KD should undergo echocardiography at diagnosis and at 6 to 8 weeks after illness onset; the cited review also recommends an intermediate echocardiogram at 10 to 14 days. BMJBMJManagement of Kawasaki disease | Archives of Disease in Childhood Early imaging can identify coronary vasculitis in incomplete presentations, but a normal early examination does not exclude KD. BMJBMJManagement of Kawasaki disease | Archives of Disease in Childhood

Coronary artery dimensions should be interpreted using body-size-adjusted Z scores. The supplied source categorizes dilation as Z score at least 2.0 to less than 2.5, small aneurysm as at least 2.5 to less than 5.0, medium aneurysm as at least 5 to less than 10 with absolute luminal dimension under 8 mm, and uses persistent Z scores below 2 as a no-involvement category. BMJBMJKawasaki disease - Symptoms, diagnosis and treatment

Young children may be at greatest risk of coronary involvement. The LancetThe LancetSubgroups of children with Kawasaki disease: a data ... Patients with aneurysms require continued cardiology-directed surveillance because coronary artery aneurysms account for substantial KD morbidity and mortality, and myocardial infarction risk is highest soon after disease onset. NEJM+1NEJMDiagnosing and Managing Kawasaki Disease | NEJM ClinicianBMJKawasaki disease - Symptoms, diagnosis and treatment

MIS-C is an important differential diagnosis in children with KD-like illness and has been incorporated into updated KD guidance. NEJMNEJMDiagnosing and Managing Kawasaki Disease | NEJM Clinician In a U.S. MIS-C cohort, 40% had KD-like features and 8% had coronary artery aneurysms defined by Z score of at least 2.5. NEJMNEJMMultisystem Inflammatory Syndrome in U.S. Children and ...

The distinction matters because treatment evidence and disease trajectory differ. In a comparative treatment study, initial treatment with IVIG alone in MIS-C was associated with delayed recovery and increased coronary aneurysm risk relative to treatment approaches that included glucocorticoids; this evidence applies to MIS-C rather than establishing treatment for classic KD. NEJMNEJMTreatment of Multisystem Inflammatory Syndrome in Children