Kawasaki CPD Online 2014

8/17/2019 Kawasaki CPD Online 2014

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Dr dr Najib Advani SpA(K),MMed(Paed)

• 1979 : Doctor, University of Indonesia

• 1989 : Pediatrician, University of Indonesia

• 1997 : Cardiology fellow, SCH, Rotterdam, Holland

• 1997- 98 : Cardiology fellow, RCH, Melbourne, Australia

• 1999 : Master of Medicine in Paediatrics, University of Melbourne

• 1999 : Consultant in Pediatric Cardiology

• 1994 - present: teaching staff at Department of Child Health University of Indonesia

• 2013 : PhD in Kawasaki Disease

Kawasaki disease:

• (Head) ASEAN countries Kawasaki disease Registry

• Member of International KD Genetic Studies

• Member of International KD Climate Studies

• International Faculty for International KD Symposium IX, Taipei, 2008

• International Faculty for International KD Symposium X, Kyoto 2012


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Kawasaki disease

what should we know ?

Dr dr Najib Advani SpA(K), MMed (Paed) Dept of Childhealth University of Indonesia

Jakarta

[email protected]

HP 0813 15 15 9500

mailto:[email protected]:[email protected]


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• First described by Tomisaku Kawasaki in 1967 in

Japan.

• Complication : coronary artery aneurysm in 20

– 40% of patients

• Etiology : unknown• Systemic vasculitis


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Kawasaki Disease


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EPIDEMIOLOGY

The commonest acquired heart disease inchildren in developed countries

Japan : highest in the world, > 200,000 children Worldwide: estimated 1000,000 Kawasaki cases

Asia esp Japan and Korea : 100-200/ yearper 100,000 children below 5 yr Male to female : 1.6 : 1 (Indonesia, Advani 2014) Indonesia

- Estimated incidence 5000 per year, diagnosed100/yr (Advani 2006, Advani et al 2008)

- 667 cases reported 2003-2013 (Advani 2014)- 71 % below 5 yr, youngest 33 days

old, oldest 16 yr (Advani 2014)


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Distribusi pasien PK menurut usia

n=667 (2003-2013). Advani 2014


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CLINICAL MANIFESTATIONS

ACUTE PHASE (First 10 days)

Conjunctivitis, bilateral, non exudative

Changes in mouth and lips : strawberry tongue, red oral

cavity, erythema and cracked lipsChanges in the hands and feet : erythema and edema

Polymorphous exanthem

Fever (remittent), not responsive to antibiotics, may

persist for 1-2 / 3-4 wks

Cervical lymphadenopathy, unilateral (>1.5 cm)


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Other associated findings (acute phase)

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


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My HEART

Clinical manifestations of KD

M ucosal changes : erythema

H and and foot changes: erythema,

edema

E ye changes : conjunctivitis

A denopathy : unilateral

R ash : polymorph exanthem

T emperature : remittent


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Cardiovascular findings during acute phase

Tachycardia

Murmur / gallop

Cardiomegaly

Pericardial effusion

LV dysfunction

ECG changes : PR interval >, low QRS voltage

ST depression/elevation


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BCG


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Subacute phase (day 11-25)

Desquamation: tips of fingers and toes

Rash, fever, lymphadenopathy disappearSignificant cardiovascular changes : coronary

aneurysm, pericardial eff, myocard infarct

Thrombocytosis, peaking at 2 weeks />


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Convalescent phase (day > 25)

Lasts till ESR and platelet count return to

normal. Deep transverse grooves (Beau’s

line) : finger nails and toenails


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DIAGNOSTIC CRITERIA FOR KD


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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 and cracking oflips.

4. Changes in the hands and feet : erythema and edema

5. Polymorphous exanthem

6. Unilateral cervical lymphadenopathy (>1.5 cm)


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Diagnostic criteria

• Fever plus 4 of the 5 other criteria allows for

diagnosis

• Fever plus fewer than 4 of 5 other criteria can

be diagnosed as KD if coronary artery disease

is detected (incomplete KD)

• Other possible diagnoses should be excluded


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Incomplete KD should be suspected in all

children with unexplained fever ≥ 5 days + 2-3

diagnostic criteria

The risk of coronary aneurysm is the same either

in complete or incomplete KD


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Not all of the clinical features may present at a

single point in time -> watchful waiting is

sometimes necessary before a diagnosis can be

made


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KD should be considered in DD/ of every child

with fever of at least several days’ duration,

rash, and nonpurulent conjunctivitis


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Laboratory test : not pathognomonic

• Leukocytosis with a shift to the left

• Mild to moderate anemia

• CRP, ESR during acute phase• Thrombocytosis : subacute phase may

> 1,000,000 sometimes 2,000,000/mm3

• Pyuria (due to urethritis)

• Liver enzyme increase, hypoalbuminemia


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ECG

• Low voltage QRS

• ST elevation/depression

•

QTc >• Wide and deep Q wave : myocard infarct


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Echocardiography

• Mandatory

• 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 : Z score


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Normal LAD

Giant aneurysm of LAD

Normal vs giant aneurysm of Left Coronary Artery (LCA, LAD)


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Normal RCA

Giant aneurysm of RCA

Normal vs giant aneurysm of Right Coronary Artery (RCA)


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Catheterization

• Selective

•

Large or multiple aneurysm• Sign of ischemia clinically or in ECG

• Suggest stenosis


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DIFFERENTIAL DIAGNOSIS

• Measles

• Stevens Johnson syndrome

• Staphylococcal scalded skin syndrome

• Drug reaction

• Scarlet fever

•Roseola infantum


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Should avoid :

• Over diagnosis

Actually not Kawasaki but diagnosed as Kawasaki

•

Under diagnosisActually Kawasaki but undiagnosed

Need to have a good knowledge on DD/ of KD


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Management

All KD patients should be hospitalized, consult pediatric cardiologist if possible

IGIV 2 g/kgBW single dose within 10-12 hours

Aspirin 80-100 mg/kgBW/day, orally divided into 4 doses till 2-3 days after fever

subsides, then

3-5 mg/kgBW/day single dose untill no aneurysms detected by echocardiography ,

at least for 6 weeks

Fever persists 36-48 hours after completion of IVIG -> repeat IVIG if necessary

(reevaluate diagnosis, no other source of fever)

Newburger JW, dkk. Pediatrics. 2004;114:1708-33.Dummer KB, dkk. Pediatr Cardiol. 2004;19:129-35.


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Management: acute/subacute stage

• Even when treated with high dose IVIG regimens within the

first 10 days of illness, around 5 % of children with KD

develop at least transient coronary artery dilation and 1 %

develop giant aneurysms.(Dajani et al. Circulation 1994;89:916-22.Durongpisitkul K et al Pediatrics 1995;96:1057-

61.Terai et al J Pediatr 1997;131:888-93)


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Invasive treatment

• Coronary Artery Bypass Graft (CABG) for

obstructive lesion

• Baloon angioplasty : not successful

• Stent placement : in older children

• Cardiac transplantation : last choice


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Without coronary aneurysms

With coronary aneurysms

Total recovery

Outcome depends on diameter of aneurysms

Belay ED, Pediatr Infect Dis J. 2006;25:245-249.Kato H, dkk. Circulat ion. 1996;94:1379-85.Rowley AH, dkk. Nelson Textbook of Pediatrics; edisi ke-18. 2007;1036-42.

Outcome

Myocarditis, pericardial effusion

Improve within 1 month


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Small aneurysm (< 5 mm)

Mostly regress within 2 years

Moderate aneurysms (5-8 mm)Mostly regress within 5 years

Giant aneurysms (>8mm)

Unlikely to regress thrombosis or stenosis may follow

years later

Regressed aneurysms intimal thickening and endothelial dysfunction

atherosclerotic lesion long-term follow up needed?

Outcome

Belay ED, Pediatr Infect Dis J. 2006;25:245-249.Kato H, dkk. Circulation. 1996;94:1379-85.Rowley AH, dkk. Nelson Textbook of Pediatrics;18th ed. 2007;1036-42.


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Course and complications

• Self limiting

•

IVIG : clinical improvement within 24 hours and reduceincidence of Coronary Aneurysm (C.A)

• Arterial remodeling or revascularization may occur

• C. A infarct

•

Mortality 1-5 %

decreasingAdvani 2013 : mortality 0% (667 cases observed for max 126months)

• Persisting C.A ischaemic heart disease at young adult age

• Regressed C.A intimal thickening & endothel dysfunction

premature atherosclerosis

• Lifelong monitoring needed ?


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Conclusion

KD is a vasculitis of unknown etiologyDiagnosis of KD is based on clinical findings, lab tests are

not specific but may support the diagnosisKD should be considered in DD/ of every child with fever

of at least several days’ duration, rash, and nonpurulent

conjunctivitisAll patients with KD must be hospitalized and consulted

to a pediatric cardiologist who is familiar with KD IVIG 2 g/kg BW for 10-12 hours is the treatment of

choice, best given on day 5-7 to 10.

Coronary aneurysm occurs in 15-25% of untreated casesOutcome depends on the severity of coronary aneurysms

Kawasaki CPD Online 2014

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