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Kawasaki Dis SemV

Jun 04, 2018

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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

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    EPIDEMIOLOGI

    * Asia khususnya Jepang dan Korea : 50-100/ tahun

    per 100,000 anak berusia 100 kasus, kebanyakan ras Cina

    * 80 % berusia 8 tahun

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    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)

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    BCG scar : redness and crust

    Cardiovascular findings during acute phase

    Tachycardia

    Cardiomegaly

    Pericardial effusion

    LV dysfunctionECG changes : PR int >, low QRS voltage

    ST depression/elevation

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    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 />

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    Convalescent phase

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

    and toenails

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    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 andcracking of lips

    4. Changes in the hands and feet : erythema andedema

    5. Polymorphous exanthem

    6. Unilateral cervical lymphadenopathy (1.5 cm)

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    Fever + more of remaining five criteria are present :

    KD is probable.

    Presence of coronary artery pathology may bediagnostic even when < 4 criteria are present

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    DIFFERENTIAL DIAGNOSIS

    Measles

    Stevens Johnson syndrome

    Staphylococcal scalded skin syndrome

    Drug reaction

    Scarlet feverHand Foot and Mouth Disease

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    Laboratory test not pathognomonicLeucocytosis 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

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    ECG

    Low voltage QRS

    ST elevation/depression

    QTc >

    Wide and deep Q wave : myocard infarct

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    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

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    Catheterization

    Selective

    Large or multiple aneurysm

    Sign of ischemia clinically or in ECG

    Suggest stenosis

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    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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