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HASSAN AL-QARNI
MEDICAL INTERNTAIF UNIVERSTY
Kawasaki Disease
Outcomes
Case presentation
Definition
Etiology & Epidemiology
Clinical manifestation
Diagnosis
Treatment
Complication
Case presentation
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
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
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
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
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.
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
Tomisaku Kawasaki (centre right) at the 8th International Kawasaki Disease Symposium, 2005
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
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
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 ArabiaKhalid AlharbiSMJ 2010
Etiology
Unknown
Theories : Immunologic response
Infectious etiology
Genetic factors
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
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
Etiology
Genetic factors:
Increased frequency of the disease in Asian and Asian-American populations and among family members
CLINICAL MANIFESTATIONS
Fever :
Most consistent manifestation of KD
above 38.5ºC during most of the illness
CLINICAL MANIFESTATIONS
Conjunctivitis
Bilateral nonexudative conjunctivitis is present in more than 90 % of patients
Courtesy of Robert Sundel, MD.Graphic 78898 Version 2.0
CLINICAL MANIFESTATIONS
Mucositis
Mucositis often becomes evident as KD progresses.
Cracked, red lips and a strawberry tongue
CLINICAL MANIFESTATIONS
Rash
Polymorphous
Begins as perineal erythema and desquamation, followed by macular, morbilliform, or targetoidskin lesions of the trunk and extremities
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.
CLINICAL MANIFESTATIONS
Lymphadenopathy :
Involve primarily the anterior cervical nodes overlying the sternocleidomastoid muscles
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
Investigation
No laboratory test specific for KD
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 intrahepaticcongestion
Echocardiography : study of choice to evaluate for coronary artery aneurysms
ECG
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 feverOr 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
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
Complication
References
Uptodate.com
Emedicine.com
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