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INTRO

Pertama kali ditemukan oleh Tomisaku Kawasaki tahun 1967 di Jepang Insidensi tertinngi di Jepang Laki : perempuan 1.5 : 1 Komplikkasi: aneurisma arteri koronaria (20-40%) Etiologi: tak diketahui

EPIDEMIOLOGI * Asia khususnya Jepang dan Korea : 50-100/ tahun per 100,000 anak berusia <5 tahun * Indonesia > 100 kasus, kebanyakan ras Cina * 80 % berusia <4 tahun, * Sangat jarang berusia < 3 bulan atau > 8 tahun

CLINICAL MANIFESTATIONS
ACUTE PHASE (First 10 days) Fever (persistent), not responsive to antibiotic, may persist for 1-2/3-4 wks Conjunctivitis, bilateral, non exudative Changes in mouth and lips : strawberry tongue, red oral cavity, erythema and cracked lips Changes in the hands and feet :erythema and edema Polymorphous exanthem Cervical lymphadenopathy, unilateral (>1.5 cm)

Other associated findings Sterile pyuria (60 %) Liver dysfunction (40%) Arthritis of large joints (30%) Aseptic meningitis (25%) Abdominal pain with diarrhea Hydrops of gallbladder with jaundice CNS symptoms (irritable, lethargic, semicoma)

BCG scar : redness and crust Cardiovascular findings during acute phase Tachycardia Murmur / gallop Cardiomegaly Pericardial effusion LV dysfunction ECG changes : PR int >, low QRS voltage ST depression/elevation

Subacute phase (day 11-25) * Desquamation: tips of fingers and toes * Rash, fever, lymphadenopathy disappear Significant cardiovascular changes : coronary aneurysm,pericardial eff, myocard infarct * Thrombocytosis, peaking at 2 weeks />

Convalescent phase Lasts till ESR and platelet count return to normal. Deep transverse grooves (Beaus line) : finger nails and toenails

DIAGNOSTIC CRITERIA FOR KD 1. Remittent fever for 5 days/more 2. Bilateral conjunctival injection (no exudate) 3. Changes in the mouth and lips :strawberry tongue, diffuse reddening of oral cavity, erythema and cracking of lips 4. Changes in the hands and feet : erythema and edema 5. Polymorphous exanthem 6. Unilateral cervical lymphadenopathy (1.5 cm)

Fever + more of remaining five criteria are present : KD is probable. Presence of coronary artery pathology may be diagnostic even when < 4 criteria are present

DIFFERENTIAL DIAGNOSIS Measles Stevens Johnson syndrome Staphylococcal scalded skin syndrome Drug reaction Scarlet fever Hand Foot and Mouth Disease

Laboratory test not pathognomonic Leucocytosis with a shift to the left CRP, ESR, alpha1 antitrypsin : increase during acute phase * Thrombocytosis : subacute phase may > 1,000,000 * Pyuria (due to urethritis) Liver enzyme increase Elevated CPK : myocard infarction

ECG Low voltage QRS ST elevation/depression QTc > Wide and deep Q wave : myocard infarct

Echocardiography Most important Detect coronary artery aneurysm and cardiac dysfunction May reveal coronary artery changes, depressed LV function, regurgitation tricuspid, mitral, aortic and pericardial effusion N coronary size : baby 2 mm, toddler <3 mm adolescent 5 mm

Catheterization Selective Large or multiple aneurysm Sign of ischemia clinically or in ECG Suggest stenosis

Treatment Hospital admission with bed rest IVIG 2 g/kg BW in 10-12 hours Acetosal 80-100 mg/kgBW po 14 days or 2-3 days after fever subsides, -> 3-10 mg/kgBW once daily for 6-8 wks if echo N

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