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M.D Pediatrics
Ph.D Pediatric special need and nutrition
consultant Mansoura national Hospital
Insurance H Fever H and Egyptian liver H
•”‫منكم‬ ‫آمنوا‬ ‫الذين‬ ‫ا‬ ‫يرفع‬
.“‫درجات‬ ‫العلم‬ ‫أوتوا‬ ‫والذين‬
•”‫الذين‬ ‫يستوى‬ ‫هل‬ ‫قل‬
.“‫يعلمون‬ ‫ل‬ ‫والذين‬ ‫يعلمون‬
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 40º 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 39º 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 76,117,4212
Libya 20 5,631,5852
Sudan 144 39,148,1622
 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 2nd–3rd 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 10–12hr with aspirin
80–100 mg/kg/24 hr divided
every 6 hr orally until 14th illness
day
CONVALESCENT STAGE
 Aspirin 3–5 mg/kg once daily
orally until 6–8 wk after illness
onset
LONG-TERM THERAPY FOR
THOSE WITH CORONARY
ABNORMALITIES
 Aspirin 3–5 mg/kg once daily
orally ± dipyridamole 4–6
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
Kawasakii

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Kawasakii

  • 1. M.D Pediatrics Ph.D Pediatric special need and nutrition consultant Mansoura national Hospital Insurance H Fever H and Egyptian liver H
  • 2. •”‫منكم‬ ‫آمنوا‬ ‫الذين‬ ‫ا‬ ‫يرفع‬ .“‫درجات‬ ‫العلم‬ ‫أوتوا‬ ‫والذين‬ •”‫الذين‬ ‫يستوى‬ ‫هل‬ ‫قل‬ .“‫يعلمون‬ ‫ل‬ ‫والذين‬ ‫يعلمون‬
  • 3. M.D Pediatrics Ph.D Pediatric special need child health and nutrition consultant Mansoura national Hospital Insurance H Fever H and Egyptian liver H
  • 4. male patient 13 years old from Mehalet Damna ,he is the first kid of the family and the older brother of two healthy sisters
  • 5.  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 40º C . The fever was altered temporarily by antipyretics .
  • 6. 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.
  • 7.  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.
  • 8.  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.
  • 9.  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
  • 10.  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.
  • 11. 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
  • 12.  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
  • 13.  There is no history of dyspnea or chest pain Or significant chest symptoms  There is no history of other system affection
  • 14.  There is no significant past medical history or family history as well
  • 15.  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
  • 16.  The temperature during examination was 39º 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.
  • 17.  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.
  • 18.
  • 19.  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.
  • 20.  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.
  • 21.  LL examination revealed erythema and skin peeling in the sole of the foot but no oedema , clubbing or arthritis.
  • 22.
  • 23.  Cardiac examination showed an evident 3rd heart sound with protodiastolic gallop but without significant murmurs.  A monocomponent pericardial rub is heared
  • 24.  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.
  • 25.  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
  • 26.  CRP 18 mg / dl  ASOT 100  Normal liver and kiney functions  Urine analysis is free
  • 27.  ANA and RF negative  EBV Ig M negative  CMV Ig M and Ig G –ve  Widal test and blood culture negative
  • 28.  Bone marrow examination is within normal  Microscopic examination of the lymph nodes showed only reactive hyperplasia
  • 29.  Normal CXR initially  Normal initial pelviabdominal ultrasound  Echocardiography revealed moderate pericardial effusion  ECG is low voltage sinus tachycardia t wave inversion in antrolateral leads
  • 30.
  • 31. What is Kawasaki disease?
  • 32.  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.
  • 33.  The diagnosis of classic Kawasaki disease (KD) requires fever of at least 5 days duration and the presence of 4 of the following :
  • 34. 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
  • 35. 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)
  • 36.  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.
  • 37. Is Kawasaki disease present in Egypt?
  • 38.  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.
  • 39. Kawasaki disease in Northern Africa (Extrapolated Statistics) Country/Region Extrapolated Incidence Population Estimated Used Egypt 280 76,117,4212 Libya 20 5,631,5852 Sudan 144 39,148,1622
  • 40.  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 2nd–3rd wk of illness.
  • 41.  Patients with acute Kawasaki disease should be treated with intravenous immunoglobulin (IVIG) and high-dose aspirin as soon as possible after diagnosis
  • 42. ACUTE STAGE  Intravenous immunoglobulin 2 g/kg over 10–12hr with aspirin 80–100 mg/kg/24 hr divided every 6 hr orally until 14th illness day CONVALESCENT STAGE  Aspirin 3–5 mg/kg once daily orally until 6–8 wk after illness onset
  • 43. LONG-TERM THERAPY FOR THOSE WITH CORONARY ABNORMALITIES  Aspirin 3–5 mg/kg once daily orally ± dipyridamole 4–6 mg/kg/24 hr divided in two or three doses orally (most experts add warfarin for those patients at particularly high risk of thrombosis)
  • 44. ACUTE CORONARY THROMBOSIS  Prompt fibrinolytic therapy with tissue plasminogen activator, streptokinase, or urokinase under supervision of a pediatric cardiologist
  • 45.  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
  • 46.  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
  • 47.  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.
  • 48.  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