kawasaki disease
Post on 03-Jun-2015
901 Views
Preview:
TRANSCRIPT
Prof DR Dr Arijanto Harsono SpAK
1967 Kawasaki Tomisaku reported 50 cases with febrile,
servical lymphadenopathy, conjunctival redness,
red tongue, cracked lips, erythema, and swollen hands and feet exfoliation followed
PENDAHULUAN
KAWASAKI DISEASE (KD)Previously called: MUCOCUTANEOUS LYMPH NODE SYNDROMEVasculitis DISEASES AFTER THE SECOND MOST HENOCH Schönlein Purpura
2Prof DR Dr Ariyanto Harsono SpA(K)
ETIOLOGY/PATHOGENESIS
Coronary vasculitis and medium blood vessels
Super-Antigen exposure HSP65
Activation of the immune system
Activation of endothelial cells and monocytes
Activation of T cells and B cells
Increased inflammatory cytokines
3Prof DR Dr Ariyanto Harsono SpA(K)
4Prof DR Dr Ariyanto Harsono SpA(K)
Prof DR Dr Ariyanto Harsono SpA(K)
ETIOLOGY/PATHOGENESIS...
6Prof DR Dr Ariyanto Harsono SpA(K)
ETIOLOGY/PATHOGENESIS...
7Prof DR Dr Ariyanto Harsono SpA(K)
Clinical Manifestations
8Prof DR Dr Ariyanto Harsono SpA(K)
Clinical Manifestations...
9Prof DR Dr Ariyanto Harsono SpA(K)
ClNICAL MANIFESTATION...
10Prof DR Dr Ariyanto Harsono SpA(K)
11Prof DR Dr Ariyanto Harsono SpA(K)
ClNICAL MANIFESTATION...
CLINICAL MANIFESTATION...
12Prof DR Dr Ariyanto Harsono SpA(K)
CLINICAL MANIFESTATIONS...
13Prof DR Dr Ariyanto Harsono SpA(K)
Prof DR Dr Ariyanto Harsono SpA(K)
14
CLINICAL MANIFESTATIONS...
Prof DR Dr Ariyanto Harsono SpA(K)
CLINICAL MANIFESTATIONS...
15Prof DR Dr Ariyanto Harsono SpA(K)
CLINICAL MANIFESTATIONS
16Prof DR Dr Ariyanto Harsono SpA(K)
CLINICAL MANIFESTATIONS……
CLINICAL MANIFESTATIONS...
17Prof DR Dr Ariyanto Harsono SpA(K)
18Prof DR Dr Ariyanto Harsono SpA(K)
CLINICAL MANIFESTATIONS……
CLINICAL MANIFESTATIONS …
19Prof DR Dr Ariyanto Harsono SpA(K)
20Prof DR Dr Ariyanto Harsono SpA(K)
CLINICAL MANIFESTATIONS……
21Prof DR Dr Ariyanto Harsono SpA(K)
CLINICAL MANIFESTATIONS……..
Prof DR Dr Ariyanto Harsono SpA(K)
22
CLINICAL MANIFESTATIONS……..
CLINICAL MANIFESTATIONS…….
23Prof DR Dr Ariyanto Harsono SpA(K)
CLINICAL MANIFESTATIONS…….
24Prof DR Dr Ariyanto Harsono SpA(K)
CLINICAL MANIFESTATIONS…….
MANIFESTASI KLINIS...
25Prof DR Dr Ariyanto Harsono SpA(K)
CLINICAL MANIFESTATIONS…….
Unusual manifestation
Prof DR Dr Ariyanto Harsono SpA(K) 26
Angus’ Rash
Unusual manifestation…..
Prof DR Dr Ariyanto Harsono SpA(K)
27Prof DR Dr Ariyanto Harsono SpA(K)
Unusual manifestation…..
Prof DR Dr Ariyanto Harsono SpA(K)
28
Beau’s line
Unusual manifestation…..
29Prof DR Dr Ariyanto Harsono SpA(K)
DIAGNOSISDiagnosis Criteria:
30
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
Polymorph EksantemCervical adenopathy
Typical KD: Fever 5 days with 4 or more of the following criteria
DIAGNOSISKriteria Diagnosis:
Typical KD: Demam 5 hari atau lebih disertai 4 dari kriteria dibawah ini
31
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
PolymorphEksantemCervical adenopathy
DIAGNOSISKriteria Diagnosis:
Typical KD: Demam 5 hari atau lebih disertai 4 dari kriteria dibawah ini
32
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
Polymorph ExanthemCervical adenopathy
DIAGNOSISKriteria Diagnosis:
Typical KD: Demam 5 hari atau lebih disertai 4 dari kriteria dibawah ini
33
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
Polymorph ExanthemCervical adenopathy
DIAGNOSISKriteria Diagnosis:
Typical KD: Demam 5 hari atau lebih disertai 4 dari kriteria dibawah ini
34
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
Polymorph ExanthemCervical adenopathy
DIAGNOSISKriteria Diagnosis:
Typical KD: Demam 5 hari atau lebih disertai 4 dari kriteria dibawah ini
35
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
Polymorph ExanthemCervical adenopathy
DIAGNOSISKriteria Diagnosis:
Typical KD: Demam 5 hari atau lebih disertai 4 dari kriteria dibawah ini
36
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
Polymorph ExanthemCervical adenopathy
DIAGNOSISKriteria Diagnosis:
Typical KD: Demam 5 hari atau lebih disertai 4 dari kriteria dibawah ini
37
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
Polymorph Eksanthem
Cervical adenopathy
DIAGNOSISKriteria Diagnosis:
Typical KD: Demam 5 hari atau lebih disertai 4 dari kriteria dibawah ini
38
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
Polymorph Eksanthem
Cervical adenopathy
DIAGNOSISKriteria Diagnosis:
39
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
Polymorph Eksanthem
Cervical adenopathy
DIAGNOSISDiagnosisCriteria:
40
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
Polymorph Eksanthem
Cervical adenopathy
DIAGNOSISDiagnosis Ceriteria:
41
Bilateral non-purulent conjunctivitis
Changes in lips and oral cavity
Peripheral limb abnormalities
Eksantem polymorphCervical adenopathy
Atypical KD:
Fever 5 days or more criteria +3 or lessFever 5 + days or more coronary abnormalities
42Prof DR Dr Ariyanto Harsono SpA(K)
• No history of sore throat or evidence of tonsillar exudate
• Streptococcal serology: negative.
.
• Scarlet fever is thought to be unlikely.
Prof DR Dr Ariyanto Harsono SpA(K)
43
Laboratry Examinations
Prof DR Dr Ariyanto Harsono SpA(K) 44
LED
CRP
Leukocyte
Neutrophil
Thrombocyte
CholesterolHDLTrigliserid
Plasma Cell IgAPerinuclear anti-neutrophilic cytoplasmic antibodies (P-ANCA): +
45Prof DR Dr Ariyanto Harsono SpA(K)
Pathology examination...
Intima and surrounding tissue necrosis. Areas of necrosisshowed fibrinoid change and basophilik. Inflammatory cell infiltrates-2 and the rest of the core seen in areas of necrosis.
Picture of early necrosis; smooth muscle showed cytoplasmic acidofiliaCore looks picnotic (chromatin condensation).Adventisia contained infiltrates of inflammatory cells.
46Prof DR Dr Ariyanto Harsono SpA(K)
Arteriole: fibrinoid necrosis (leukocytoclastic vasculitis): Note the pink staining material (fibrinoid necrosis) in multifocal areas of the
thickened wall of the venule. The material represents protein derived from the plasma that has deposited in the vessel wall owing to an increase in vessel
permeability from the inflammatory process.
• It is called fibrinoid because it looks like fibrin in a clot but it is really protein. Small vessel vasculitis is usually due to immune complex (IC) disease (type III hypersensitivity). ICs are deposited in the vessel wall and then activate the complement system. C5a, a chemotactic factor, attracts neutrophils (only a few are visible at around 7 o’clock).
Prof DR Dr Ariyanto Harsono SpA(K)
47
MANAGEMNT
Prof DR Dr Ariyanto Harsono SpA(K)
48
IVIG: Should be given within the
first 10 days after the diagnosis is established
Dosage: 2g/Kg single dose Children: 1g/kg BW should
be given "Single infusion" in 8-12 hours. If you already have heart problems given in divided doses 3-4 days
Prof DR Dr Ariyanto Harsono SpA(K)
49
Management…
Patients who are refractory to IVIG: Pulse methyl prednisolone 0.5-2 mg / kg bw
Aspirindose:50-80 mg / kg bw in the acute inflammatory phase3-5 mg / kg bw after fever resolved and platelets increased, maintained until cardiac abnormalities improved
Dipridamole: In patients who are intolerant to aspirinDose: 2-3 mg / kg bw
50Prof DR Dr Ariyanto Harsono
SpA(K)
Prof DR Dr Ariyanto Harsono SpA(K)
51
PROGNOSIS
Were largely complete recoveryLong-term observation of patients with aneurysms remaining 50% improved cardiac abnormalitiesMortality 1970: 1-2%; 1990: 0.4% due to the blockage of coronary / other cardiac abnormalities95% of deaths occurred after 6 months because of infection, the rest after 10 years
52Prof DR Dr Ariyanto Harsono SpA(K)
ProfilaksisDentists should remain alert
to features of the acute disease, and in patients with a history of Kawasaki disease, be aware of the possibility of recurrence and of heart valve defects requiring antibiotic prophylaxis prior to relevant dental treatment.Prof DR Dr Ariyanto Harsono SpA(K) 53
Prof Kawasaki Karlee
54)
Prof DR Dr Ariyanto Harsono SpA(K)
55
Prof Takashimura
56Prof DR Dr Ariyanto Harsono SpA(K)
top related