6. History
HajarYassin Mohammed is 3 year old female pt,
admitted at 8/4/2014 with history of fever, skin
rash ,mouth ulcres ,red eyes with discharge since
4 days prior to admission
No history of cough , vomiting and diarrhea
No family history of URTI
Diagnosed as URTI with stomatitis and query
skin allergy
7. On examination
VS:
Temp: 39.9 PR: 135
RR: 40 SPO2: 95% W/0 O2
General examination:
Conscious, febrile ,looks ill
Erythematous lips with vesicles and ulcers
Congested throat , normal tongue
Maculopapular rash with scratch marks on upper & lower
limbs (eczema like rash)
Conjunctvitis with bilateral mucupurulent disharge
NO significant lymph node enlargement
8. On examination
Systemic examination:
Chest : bilateral air entry w/0 added sounds
CVS: s1+s2+0, NO murmur , NO gallop
Abdomen : soft ,lax . NO organomegally
CNS: conscious
Case seen by dermatologist , diagnosed as:
(herpes simplex infection vs erythema multiform) ?
Treated with acyclovir , Cefrixone
Case seen by ophthalmologist, diagnosed as :
mucupurluent conjunctivitis
10. Management
After 2 days, as fever not subsided and pt
developed erythema , swelling of both hand
& feet , became irritable
Diagnosed as (query Kawasaki disease) :
IVIG started as 2g/kg
Aspirin 80 mg /kg
On 6th day of admission ,pt developed peeling
of skin, cracked lips.
11. Management
On 7th day of admission , fever was on & off (refractory )
Another dose of IVIG given 2g/kg
Aspirin continued as 80 mg/kg
On 10th day of admission
NO fever for more than 1 day
Pt signed discharge against medical advice(DAMA)
Given aspirin 80 mg/kg ( high dose) to complete 14 days
aspirin Prophylaxis 60 mg OD to be started after 14 days
Follow up
Pediatric Cardiology clinic
Consultant pediatric clinic
13. Definition
Kawasaki disease (KD) is an acute febrile
vasculitic syndrome of early childhood
previously called :
Mucocutaneous lymph node syndrome
Most common vasculitides of childhood
Typically a self-limited condition, with fever and
other acute inflammatory manifestations lasting
for an average of 12 days
14. EPIDEMIOLOGY
Greatest in children who lives in East Asia (eg, Japan,
Korea,Taiwan) or are of Asian ancestry living in other
parts of the world
In japan :
134 cases per 100,000 children younger than 5 years
10 - 20 times higher than in Western countries
Other risk factors include:
Male gender
Age between 6 months - 5years
Family history of KD
15. EPIDEMIOLOGY
In Saudi Arabia :
CITY:
Madinah region, Kingdom of Saudi Arabia (KSA).
METHODS
retrospective
Maternity and Children Hospital, Madinah
January 2007 to January 2010.
51 patients suspected cases of Kawasaki disease
RESULTS
24 patients diagnosed as Kawasaki ( 47 %)
M:F = 1.7 : 1
CONCLUSION:
High index of suspicion is mandatory for early diagnosis of Kawasaki
disease
Delayed diagnosis may lead to coronary lesions
Kawasaki disease in western Saudi Arabia
Khalid Alharbi
SMJ 2010
17. Etiology
Immunologic response:
Affects medium-sized arteries
Inflammatory cell infiltration into KD vascular
tissue vascular damage
Stimulus for this inflammatory infiltration has
not been identified
18. Etiology
Infectious etiology:
Similarities between KD and other pediatric infectious
conditions suggest that KD is caused by a transmissible agent
include:
Febrile exanthem with lymphadenitis and mucositis
Seasonal increase in disease incidence in the winter and
summer
No studies have convincingly identified a specific virus,
bacteria or bacterial toxin, or other pathogen associated with
KD
19. Etiology
Genetic factors:
Increased frequency of the disease in Asian and
Asian-American populations and among family
members
21. CLINICAL MANIFESTATIONS
Conjunctivitis
Bilateral nonexudative conjunctivitis is present in
more than 90 % of patients
Courtesy of Robert Sundel, MD.
Graphic 78898Version 2.0
23. CLINICAL MANIFESTATIONS
Rash
Polymorphous
Begins as perineal erythema and desquamation,
followed by macular, morbilliform, or targetoid
skin lesions of the trunk and extremities
24. CLINICAL MANIFESTATIONS
Extremity changes
last manifestation to appear
Indurated edema of the dorsum of their hands and
feet
Diffuse erythema of their palms and soles.
26. CLINICAL MANIFESTATIONS
Cardiovascular findings :
During the first week to 10 days of illness include:
Tachycardia out of proportion
Gallop sounds
Muffled heart tones
Fusiform aneurysms of the brachial arteries that are easily palpable or
visible in the axillae .
Young infants may have cold, pale, or cyanotic digits of the hands and
feet due to reduced blood perfusion
29. Investigation
CBC :
Leukocytosis, and a left-shift in the white blood cell
count
Thrombocytosis: may reach to 1,000,000/mm3
Normocytic, normochromic anemia
Increased of acute phase reactants [CRP,ESR]
Urinary microscopy: white blood cells (Pyuria ) is often of
urethral origin
Abnormal liver function test because of intrahepatic
congestion
Echocardiography : study of choice to evaluate for coronary
artery aneurysms
ECG
30. Treatment
Intravenous immune globulin (IVIG)
Single dose of (IVIG) (2 g/kg) administered over 8 to 12 hours
Aspirin
high-dose : (80 - 100 mg/kg/day)
Untill resolution of fever
Or 14 days of fever
Prophylaxis (3 -5 mg/kg /day)
48 hours after the resolution of fever.
continued until laboratory markers of acute inflammation (eg, platelet count and
ESR) return to normal
unless coronary artery (CA) abnormalities are detected by echocardiography
31. Treatment of refractory
Kawasaki disease
INCIDENCE :
( 10% - 20%)
Significantly increased risk of developing
coronary artery aneurysms
Manifested as persistent fever 36 hours
after completion of initial therapy