Kawasaki Disease

The Mission Hospital

Durgapur
 Investigations

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

Troponin Assay-Increased troponin 1

Controversial
Not routine
 ECG changes
Tachycardia-Gallop rhythm
Decreased R wave voltage
ST depression
T wave flattening & inversion
Prolonged PR/QT interval
 ECHOCARDIOGRAPHY
Aneurysms-----min 10 days
Before: Perivascular brightness
Ectasia
Lack of tappering
Decreased LV contractility
MR/AR
Pericardial effusion
 ECHO
First: As soon as the diag is made
Baseline image
Uncomplicated:
At Diag
2 wks
6-8 wks
1 yr
Complicated: More frequent
 Considerations

2D/highest freq transducer, dynamic video

Internal vessel diam
Z-score
Small/medium/giant

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

For complex coronary artery lesions

Follow-up: Evidence of myoc ischaemia
For distal coronary arteries
After surgical revascularisation
 Incomplete/Atypical KD

Preferable name ? (AHA)

Atypical KD----KD with renal impairement
Conventional Diagnostic Criteria
Conventional Lab findings
C/F+ Lab findings+ ECHO (be liberal)
 Incomplete KD
ECHO-To consider

Infants <6 mths with

>7 days of fever
Lab evidence of systemic inflammation
No other explanation for febrile illness
Common Pitfalls in Diagnosis
Fever: Common C/F, Antipyretic use
Rash, mucosal changes: Reaction to
antibiotics
Sterile Pyuria -partially treated UTI
LP—Aseptic meningitis
Acute abdomen-------surgical condition
 Risk factors for predicting CA
Harada score
Beiser score (North America)

WBC Platelet count

CRP Haematocrit
Hb Albumin
Age Male

Positive predictive value---Less satisfactory

Imperfect performance
 Treatment of KD
Criteria (US and Japanese experts)

4/5 classic criteria:4 days of fever to start t/t

Coronary arteries: z score
IKD--algorithm—not evidence based
 Initial treatment
Aspirin: Anti-inflammatory/Antiplatelets
80-100mg/kg----4 times daily
2days/2wks of onset
3-5mg/kg ----once----6-8 wks
3-5mg/kg----once----indefinitely
30 mg/kg----devided doses---ADC
Dipyridamol----can be used together
Ibuprofen----can not be used together
 Aspirin

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

Streptokinase, Urokinase,tPA:varying success

rate
Surgery
Excision /plication of CA
Int mammary graft/saphenous graft

Interventional Cardiac Catheterisation

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

American Heart Association

North American Journal Paediatrics
Archives of disease in childhood
Royal Children Hospital, Sydney
Kawasaki Disease Foundation
Kawasaki Support Group, UK
Questions
Thank you