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Kawasaki Disease
Kawasaki Disease
Kawasaki Disease
Conventional investigations
should be considered together
Full blood count and film
Erythrocyte sedimentation rate
C reactive protein
Blood cultures
ASOT and anti DNase B
Nose and throat swab, and
stool sample for culture (superantigen toxin typing if Staphylococcus aureus and/or ß haemolytic
streptococci detected)
Renal and liver function tests
Coagulation profile
(antinuclear antibodies; extractable nuclear antibodies; rheumatoid factor; antineutrophil
cytoplasmic antibodies)
Serology (IgG and IgM) for mycoplasma pneumoniae, enterovirus, adenovirus, measles,
parvovirus, Epstein–Barr virus, cytomegalovirus
Urine microscopy and culture
Dip test of urine for blood and protein
Electrocardiogram and echocardiogram (see fig 1)
Consider serology for rickettsiae and leptospirosis if history suggestive
Consider chest x ray
Blood
A)WBC:
Leukocytosis
Neutrophilia-lymphocytosis
50% patients WBC>15,000/cm
Leukopenia is rare
B)Anaemia:
Mild- normocytic, normochromic
Normal RBC indices
More –prolonged illness
Hemolytic anaemia-IVIG complication
C) Thrombocytosis
2-3 weeks
500,000-1 million/cm
Peaks at 3rd week
Normalises by 4-8 weeks
Thrombocytopenia-risk factor for CA
Acute phase reactants
CRP & ESR –Elevation is nearly universal
May show a discripancy
Increased ESR-C/O IVIG t/t
Both should be considered together
LFT
Increased Transaminases
Increased GGT
Mild Hyperbilirubinemia
Hypoalbuminemia
Plasma lipids
Acute: Cholesterol,HDL,Apolipoproteins
Urinanalysis
Sterile pyuria
SPA-no pyuria
LP - Aseptic meningitis
Limitations
Stenosis/thrombosis
Decreased visualisation of CA with increased body size
Other Investigations
Transesophageal ECHO/Stress ECHO
Angiography
Intravascular US(IVUS)
MRA/MRI
Ultrafast CT
Cardiac Stress Testing—Nuclear Perfusion Scan
Cardiac Catherisation/Angiography
Consider
Reye syndrome
Varicella/influenza
Temporary ommission/another antiplatelet
Annual influenza vaccination
IVIG
Before 10th day
Can be used after 10th day
Preferably not before 5th day
Decreases CA incidence to 2 %
Transient coronary artery dilatation----5%
Giant aneurysm----1%
Repeat dose
IVIG
Dose
Before infusion
Infusion
Monitoring
Reactions
Fluid overload-can consider divided dose
Documentation
Steroids
Initial study----Prednisolone—detrimental
effect
High dose pulse Methylprednisolone+IVIG
Methylprednisolone----Refractory to IVIG
Religious cause
Needs further study
Pentoxifylline
TNF-inhibitor
Oral prep promising
Uncertain
Initial t/t failure
IVIG
IVIG +steroids
Methylprednisolone—30mg/kg—over 3 hrs
OD for 3 days
Other t/t
Plasma exchange: Refractory to IVIG
Ulinastatin: human urine, trypsin inhibitor
refractory to IVIG
Abciximab: Platelet glycoprotrein receptor
inhibitor, large CA
Monoclonal antibodies
Cytotoxic agents: Cyclophosphamide, Cyclosporin
Antithrombotic treatment
Low dose Aspirin
Dipyridamole
Dipyridamol +Clopidogrel
MC:Aspirin+Warfarin(INR:2 to 2.5)
LMW Heparin---SC inj BD
T/T of Thrombosis
Cardiac Transplantation
Long term follow-up
Risk stratification
Take home
High index of suspicion
C/F+ Lab findings-be considered together
ECHO+IVIG------liberal use (IKD)
Follow-up
Search for specific inv & treatment
Mystery continues----
Acknowledgement