M.

D Pediatrics
Ph.D Pediatric special need and nutrition
consultant Mansoura national Hospital
Insurance H Fever H and Egyptian liver H

M.D Pediatrics
Ph.D Pediatric special need child health and nutrition
consultant Mansoura national Hospital Insurance H
Fever H and Egyptian liver H
male patient 13 years old from
Mehalet Damna ,he is the first
kid of the family and the older
brother of two healthy sisters
The conditon stareted 1.5 months ago
when fever developed and increased
gradually .the child was feverish all the
day and fever was more at night but
without significant diurnal variations
and it was oscillating around 40 C . The
fever was altered temporarily by
antipyretics .
The family sought medical
advice and the child received a
lot of medications including
different classes of antibiotics
without any improvement.
Then the child was admitted to
hospital and was fully
investigated but yet
undiagnosed.

One week later the child
developed oral ulcers which
markedly interfered with oral
feeding
These ulcers were multiple and
persisted the whole duration of
illness.


Also 2 days later the the child
developed simultaneous
events.
There was edema , erythema ,
pain in the hands and feet.this
pain was associated with
limitation of movements.
At the same time the patient
developed multiple neck
swellings bilaterally.
These neck swellings were
variable in size the largest of
them was about lemon
size.They gradually
decreased in size
One of the cervical lymph
nodes was excised and sent to
biopsy

The child developed redness in
both eyes without marked
discharge which continued the
whole month.
Thre was no history of other
skin rash
There was no history suggestive
of bleeding tendency or
purpuric eruption .
No history of abdominal
distension
No history of big joint affection
No history of jaundice or
changes of the colour of urine
or stool
No history of convulsions or
abnormal movements or limb
weakness.
No history of disturbed
conscious level or increased
intracranial tension
There is no history of dyspnea
or chest pain Or significant
chest symptoms
There is no history of other
system affection


There is no significant past
medical history or family history
as well
General Examination
The patient is fully conscious
well oriented for time place
and person , he is co-operative
and of average intelligence
The patient has no special
decubitus
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.
Head and Neck examination
revealed bilateral non purulent
conjuctival congestion ,
multiple mouth ulceration , red
strawberry tongue ,multiple
small lymph nodes the largest
of them 2 cm in diameter , the
lymph nodes are mobile ,non-
tender and firm in consistency.
Neck veins are 3 cm above the
clavicle measured at the same
level to that of Angle of Louis
.Neck veins showed normal
pulsations with no inspiratory
filling.
There was no goitre , complexion
abnormalities or any other
significant abnormality.
UL examination revealed
bilateral distal skin peeling , no
arthritis , no clubbing, no sc
nodules , bilateral epitrochlear
lymphadenopathy 1.5 cm ,
firm and non tender. There
was no axillary lymph nodes.
LL examination
revealed erythema
and skin peeling in
the sole of the foot
but no oedema ,
clubbing or
arthritis.

Cardiac examination showed an
evident 3rd heart sound with
protodiastolic gallop but without
significant murmurs.
A monocomponent pericardial
rub is heared
Abdominal examination
revealed no abnormities apart
from mild tender
hepatomegaly ( liver span 11
cm in MCL ) with rounded
border and soft consistency .
Chest and neurological
examination were normal.
Laboratory :
ESR 70 mm in the 1st hour and 110 mm in
the 2nd hour
CBC
WBCs 8000/ cmm with normal differential
cont
Hb 11gm/dl
Platelets 485.000 / cmm

CRP 18 mg / dl
ASOT 100
Normal liver and kiney
functions
Urine analysis is free
ANA and RF negative
EBV Ig M negative
CMV Ig M and Ig G ve
Widal test and blood culture
negative
Bone marrow examination is
within normal
Microscopic examination of the
lymph nodes showed only
reactive hyperplasia
Normal CXR initially
Normal initial pelviabdominal
ultrasound
Echocardiography revealed
moderate pericardial effusion
ECG is low voltage sinus
tachycardia t wave inversion in
antrolateral leads
What is Kawasaki disease?
Kawasaki disease (KD) (ie,
Kawasaki syndrome [KS]) is a
febrile illness of childhood. It
is a self-limited acute vasculitic
syndrome of unknown
etiology, first described by
Tomisaku Kawasaki in 1967.
The diagnosis of classic Kawasaki
disease (KD) requires fever of at
least 5 days duration and the
presence of 4 of the following :
1-Changes in extremities (eg, erythema,
edema, desquamation): This may
limit movement and cause the child
to refuse to bear weight.
Desquamation of the fingers and
toes begins in the periungual region,
may involve the palms and soles

2-Bilateral conjunctivitis (not
associated with exudates)
3- Polymorphous rash (not
vesicular)
4- Cervical lymphadenopathy
5- Changes in the lips and oral
cavity (eg, pharyngeal erythema,
dry/fissured or swollen lips,
strawberry tongue)

Cardiac involvement is the
most important manifestation
of Kawasaki disease.
Myocarditis manifested by
tachycardia and decreased
ventricular function occurs in
at least 50% of patients.
Is Kawasaki disease present in
Egypt?
It is estimated that at least 3,000
cases are diagnosed annually in the
United States. The incidence of
Kawasaki disease in Asian children
is substantially higher than in other
racial groups, but the illness occurs
worldwide in all ethnic groups.
Kawasaki disease in Northern
Africa (Extrapolated Statistics)
Country/Region
Extrapolated
Incidence
Population
Estimated Used
Egypt 280 76,117,421
2

Libya 20 5,631,585
2

Sudan 144 39,148,162
2


Pericarditis with a small
pericardial effusion is common
during the acute illness.
Coronary artery aneurysms
generally develop in up to 25%
of untreated patients during
the 2nd3rd wk of illness.

Patients with acute Kawasaki
disease should be treated with
intravenous immunoglobulin
(IVIG) and high-dose aspirin as
soon as possible after diagnosis
ACUTE STAGE
Intravenous immunoglobulin 2
g/kg over 1012hr with aspirin
80100 mg/kg/24 hr divided
every 6 hr orally until 14th illness
day
CONVALESCENT STAGE
Aspirin 35 mg/kg once daily
orally until 68 wk after illness
onset


LONG-TERM THERAPY FOR
THOSE WITH CORONARY
ABNORMALITIES
Aspirin 35 mg/kg once daily
orally dipyridamole 46
mg/kg/24 hr divided in two or
three doses orally (most experts
add warfarin for those patients at
particularly high risk of
thrombosis)


ACUTE CORONARY THROMBOSIS
Prompt fibrinolytic therapy with
tissue plasminogen activator,
streptokinase, or urokinase under
supervision of a pediatric
cardiologist

IVIG also should be administered
to children presenting after the
10th day of illness (ie, children in
whom the diagnosis was missed
earlier) if they have either
persistent fever without other
explanation or aneurysms and
ongoing systemic inflammation,
as manifested by elevated ESR or
CRP
Steroids
Although corticosteroids are the treatment
of choice in other forms of vasculitis, their
use has been limited in children with
Kawasaki disease
Corticosteroids also have been used to treat
patients who have failed to respond to
initial therapy for Kawasaki disease
Abciximab, a platelet glycoprotein IIb/IIIa
receptor inhibitor ,has been used to treat
patients in the acute or subacute phase of
Kawasaki disease who have large coronary
aneurysms.
A new class of agents that may play a role in
the treatment of patients with refractory
Kawasaki disease is monoclonal antibodies to
various proinflammatory cytokines.
A humanized monoclonal antibody against
TNF - , infliximab, is being studied in a
clinical trial of treatment for children who
fail to become afebrile after initial IVIG
treatment.
Cytotoxic agents like cyclophosphamide, in
conjunction with oral steroids, have been
suggested as useful for the treatment of
exceptional patients with particularly
refractory acute Kawasaki disease