Kawasaki disease

3. HASSAN AL-QARNI
MEDICAL INTERN
TAIF UNIVERSTY
Kawasaki Disease

4. Outcomes
 Case presentation
 Definition
 Etiology & Epidemiology
 Clinical manifestation
 Diagnosis
 Treatment
 Complication

5. Case presentation

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

9. Investigation
 CBC:
 WBC: 7000 – 12000 (3 days) – 13000 - 15000
 Platelet : 257000- 59700,000 (3 days) -700,000 – 815000
(after 1 wk)
 Lymphocyte : 55%
 Neutrophil : 38%
 ESR : 95 mm/hr – 124 mm/hr
 CRP: +ve
 ASO ( Antistreptolysin O ) titer : -ve
 LFT , RFT & Chemistry : normal
 ECG & Echocardiography : normal
 Blood & urine culture : -ve

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

12. Tomisaku Kawasaki (centre right) at the 8th
International Kawasaki Disease Symposium, 2005

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

16. Etiology
 Unknown
 Theories :
 Immunologic response
 Infectious etiology
 Genetic factors

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

20. CLINICAL MANIFESTATIONS
 Fever :
 Most consistent manifestation of KD
 above 38.5ºC during most of the illness

21. CLINICAL MANIFESTATIONS
 Conjunctivitis
 Bilateral nonexudative conjunctivitis is present in
more than 90 % of patients
Courtesy of Robert Sundel, MD.
Graphic 78898Version 2.0

22. CLINICAL MANIFESTATIONS
 Mucositis
 Mucositis often becomes evident as KD
progresses.
 Cracked, red lips and a strawberry tongue

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.

25. CLINICAL MANIFESTATIONS
 Lymphadenopathy :
 Involve primarily the anterior cervical nodes
overlying the sternocleidomastoid muscles

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

28. Investigation
 No laboratory test specific for KD

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

33. Complication

35. References
 Uptodate.com
 Emedicine.com

36. THANK YOU 