Kawasaki disease by Hassan AL-Qarni

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

THANK YOU