The symptoms can be classified into two categories, principal symptoms and other significant symptoms or findings: |
Principal symptoms |
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1 Fever that persists for 5 days or more (inclusive of cases in whom the fever has subsided before the fifth day in response to therapy); |
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2 Bilateral conjunctival congestion; |
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3 Changes of lips and oral cavity: reddening of the lips, strawberry tongue, and oral and pharyngeal mucosal diffuse injection; |
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4 Polymorphous exanthema; |
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5 Changes of peripheral extremities: (Initial stage) reddening of the palms and soles, indurative edema; (Convalescent stage) membranous fingertip desquamation; |
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6 Acute non-purulent cervical lymphadenopathy; |
At least 5 items of the 6 should be satisfied for diagnosis of KD. However, patients with 4 items of the principal symptoms can be diagnosed with KD when coronary aneurysm or dilatation is recognized by 2-D echocardiography or coronary angiography. |
Other significant symptoms or findings |
The following symptoms and findings should be considered in the clinical evaluation of suspected patients: |
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1 Cardiovascular: auscultation (heart murmur, gallop rhythm, distant heart sounds), electrocardiogram changes (prolonged PR/QT intervals, abnormal Q wave, low-voltage QRS complexes, ST-T changes, arrhythmias), chest X-ray findings (cardiomegaly), 2-D echo findings (pericardial effusion, coronary aneurysms), aneurysm of peripheral arteries other than coronary (eg, axillary), angina pectoris or myocardial infarction; |
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2 Gastrointestinal tract: diarrhea, vomiting, abdominal pain, hydrops of gall bladder, paralytic ileus, mild jaundice, slight increase of serum transaminase; |
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3 Blood: leukocytosis with shift to the left, thrombocytosis, increased erythrocyte sedimentation ratio, positive C-reactive peptide, hypoalbuminemia, increased α2-globulin, slight decrease in erythrocyte and hemoglobin levels; |
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4 Urine: proteinuria, increase of leukocytes in urine sediment; |
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5 Skin: redness and crust at the site of Bacille Calmette-Guèrin inoculation, small pustules, transverse furrows of the finger nails; |
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6 Respiratory: cough, rhinorrhea, abdominal shadow on chest X-ray; |
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7 Joint: pain, swelling; and |
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8 Neurological: cerebrospinal fluid pleocytosis, convulsion, unconsciousness, facial palsy, paralysis of the extremities. |
Remarks |
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1 For item 5 under principal symptoms, the convalescent stage is considered important. |
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2 Non-purulent cervical lymphadenopathy is less frequently encountered (approximately 65%) than other principal symptoms during the acute phase. |
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3 Male:Female ratio, 1.3-1.5 : 1; patients under 5 years of age, 80-85%; fatality rate, 0.1%. |
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4 Recurrence rate, 2-3%; proportion of siblings cases, 1-2%. |
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5 Approximately 10% of the total cases do not fulfil 5 of the 6 principal symptoms, in which other diseases can be excluded and KD is suspected. In some of these patients, coronary artery aneurysms (including so-called coronary artery ectasia) have been confirmed. |