kawasaki i

8/12/2019 Kawasaki i

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M.D Pediatrics

Ph.D Pediatric special need and nutritionconsultant Mansoura national Hospital

Insurance H Fever H and Egyptian liver H


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M.D Pediatrics

Ph.D Pediatric special need child health and nutritionconsultant Mansoura national Hospital Insurance H

Fever H and Egyptian liver H


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male patient 13 years old fromMehalet Damna ,he is the firstkid of the family and the older

brother of two healthy sisters


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The conditon stareted 1.5 months agowhen fever developed and increased

gradually .the child was feverish all theday and fever was more at night butwithout significant diurnal variations

and it was oscillating around 40C . Thefever was altered temporarily byantipyretics .


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The family sought medical

advice and the child received alot of medications includingdifferent classes of antibiotics

without any improvement.Then the child was admitted tohospital and was fully

investigated but yetundiagnosed.


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One week later the childdeveloped oral ulcers which

markedly interfered with oral

feedingThese ulcers were multiple and

persisted the whole duration ofillness.


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Also 2 days later the the child

developed simultaneousevents.

There was edema , erythema ,pain in the hands and feet.thispain was associated with

limitation of movements.


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At the same time the patient

developed multiple neck

swellings bilaterally.

These neck swellings werevariable in size the largest of

them was about lemon

size.They gradually

decreased in size


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One of the cervical lymph

nodes was excised and sent tobiopsy

The child developed redness inboth eyes without marked

discharge which continued thewhole month.


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Thre was no history of otherskin rash

There was no history suggestive

of bleeding tendency orpurpuric eruption .

No history of abdominal

distensionNo history of big joint affection


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No history of jaundice or

changes of the colour of urineor stool

No history of convulsions orabnormal movements or limbweakness.

No history of disturbedconscious level or increasedintracranial tension


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There is no history of dyspneaor chest pain Or significantchest symptoms

There is no history of othersystem affection


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There is no significant pastmedical history or family historyas well


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General Examination

The patient is fully consciouswell oriented for time place

and person , he is co-operativeand of average intelligence

The patient has no special

decubitus


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The temperature during

examination was 39 C .

Pulse was 145 BPM of average

volume , reglar , equal bilaterally

with intact peripheral pulsations

and no special character.

Blood pressure 100/70 mmhg .Respiratory rate 30.


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Head and Neck examination

revealed bilateral non purulentconjuctival congestion ,

multiple mouth ulceration , red

strawberry tongue ,multiplesmall lymph nodes the largest

of them 2 cm in diameter , the

lymph nodes are mobile ,non-

tender and firm in consistency.


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Neck veins are 3 cm above the

clavicle measured at the samelevel to that of Angle of Louis.Neck veins showed normal

pulsations with no inspiratoryfilling.

There was no goitre , complexion

abnormalities or any othersignificant abnormality.


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UL examination revealedbilateral distal skin peeling , noarthritis , no clubbing, no sc

nodules , bilateral epitrochlearlymphadenopathy 1.5 cm ,firm and non tender. There

was no axillary lymph nodes.


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LL examinationrevealed erythema

and skin peeling inthe sole of the footbut no oedema ,clubbing orarthritis.


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Cardiac examination showed anevident 3rd heart sound with

protodiastolic gallop but withoutsignificant murmurs.

A monocomponent pericardialrub is heared


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Abdominal examinationrevealed no abnormities apartfrom mild tender

hepatomegaly ( liver span 11cm in MCL ) with roundedborder and soft consistency .

Chest and neurologicalexamination were normal.


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Laboratory : ESR70 mm in the 1st hour and 110 mm in

the 2nd hour CBC

WBCs 8000/ cmm with normal differentialcont

Hb 11gm/dl Platelets 485.000 / cmm


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CRP18 mg / dl

ASOT100

Normalliver and kineyfunctions

Urine analysisis free


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ANAand RFnegative

EBV Ig Mnegative

CMV Ig Mand Ig Gve

Widal testand blood culture

negative


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Bone marrow examinationiswithin normal

Microscopic examination of thelymph nodesshowed onlyreactive hyperplasia


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Normal CXRinitially

Normal initial pelviabdominalultrasound

Echocardiographyrevealedmoderate pericardial effusion

ECGis low voltage sinus

tachycardia t wave inversion inantrolateral leads


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What is Kawasaki disease?


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Kawasaki disease (KD) (ie,Kawasaki syndrome [KS]) is afebrile illness of childhood. It

is a self-limited acute vasculiticsyndrome of unknownetiology, first described by

Tomisaku Kawasaki in 1967.


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The diagnosis of classic Kawasakidisease (KD) requires fever of atleast 5 days duration and the

presence of 4 of the following :


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1-Changes in extremities (eg, erythema,

edema, desquamation): This maylimit movement and cause the childto refuse to bear weight.Desquamation of the fingers andtoes begins in the periungual region,may involve the palms and soles


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2-Bilateral conjunctivitis (notassociated with exudates)

3- Polymorphous rash (notvesicular)

4- Cervical lymphadenopathy

5- Changes in the lips and oralcavity (eg, pharyngeal erythema,dry/fissured or swollen lips,strawberry tongue)


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Cardiac involvement is themost important manifestationof Kawasaki disease.

Myocarditis manifested bytachycardia and decreasedventricular function occurs in

at least 50% of patients.


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Is Kawasaki disease present inEgypt?


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It is estimated that at least 3,000cases are diagnosed annually in theUnited States. The incidence ofKawasaki disease in Asian childrenis substantially higher than in otherracial groups, but the illness occurs

worldwide in all ethnic groups.


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Kawasaki disease in NorthernAfrica (Extrapolated Statistics)

Country/RegionExtrapolated

Incidence

Population

Estimated UsedEgypt 280 76,117,421

2

Libya 20 5,631,5852

Sudan 144 39,148,1622


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Pericarditis with a smallpericardial effusion is commonduring the acute illness.

Coronary artery aneurysmsgenerally develop in up to 25%of untreated patients during

the 2nd3rd wk of illness.


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Patients with acute Kawasakidisease should be treated with

intravenous immunoglobulin(IVIG) and high-dose aspirin assoon as possible after diagnosis


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ACUTE STAGE

Intravenous immunoglobulin 2g/kg over 1012hr with aspirin80100 mg/kg/24 hr dividedevery 6 hr orally until 14th illnessday

CONVALESCENT STAGE

Aspirin 3

5 mg/kg once dailyorally until 68 wk after illnessonset


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LONG-TERM THERAPY FOR

THOSE WITH CORONARYABNORMALITIES

Aspirin 35 mg/kg once daily

orally dipyridamole 4

6mg/kg/24 hr divided in two orthree doses orally (most experts

add warfarin for those patients atparticularly high risk ofthrombosis)


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ACUTE CORONARY THROMBOSIS

Prompt fibrinolytic therapy with

tissue plasminogen activator,streptokinase, or urokinase undersupervision of a pediatric

cardiologist


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IVIG also should be administered

to

children presenting after the10th day of illness (ie, childreninwhom the diagnosis was missedearlier) if they have eitherpersistent fever without otherexplanation or aneurysms andongoing systemic inflammation,

as manifested by elevated ESR orCRP


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SteroidsAlthough corticosteroids are the treatmentof choice in otherforms of vasculitis, theiruse has been limited in childrenwithKawasaki disease

Corticosteroids also have been used to treatpatients who have failed to respond toinitial therapy for Kawasaki disease


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Abciximab, a platelet glycoprotein IIb/IIIareceptor inhibitor,has been used to treatpatients in the acute or subacute phaseofKawasaki disease who have large coronaryaneurysms.

A new class of agents that may play a role inthe treatment of patients with refractoryKawasaki disease is monoclonal antibodiestovarious proinflammatory cytokines.


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A humanized monoclonalantibody againstTNF -, infliximab, is being studied in a

clinicaltrial of treatment for children whofail to become afebrileafter initial IVIGtreatment.

Cytotoxic agentslike cyclophosphamide, inconjunction with oral steroids, have beensuggested as useful for the treatment ofexceptional patients with particularly

refractory acute Kawasaki disease


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kawasaki i

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