Kawasaki Disease

Question 1

Another term for Kawasaki Disease…

Answer 1

Mucocutaneous Lymph Node

Question 2

What is Kawasaki Disease

Answer 2

An acute onset of a generalized systemic inflammation, which results in a vasculitis (inflammation of blood vessels) of medium sized arteries of the body.

Question 3

A common place for the disease to attack…

Answer 3

Notably, Kawasaki disease attacks the coronary arteries, responsible for delivering oxygen-rich blood to the heart, often resulting in high risk of aneurysm

Question 4

Kawasaki Disease - What is it??

Answer 4

1st diagnosed in 1967
Leading cause of acquired heart disease in children in US
90% of patients affected are less than 5 years old
Boys are affected 50% > than girls
Highest reported rates during the winter and spring
Highest rate of occurrence in children with Asian ancestry
Generally self-limiting over a 6-8 week course
“Diagnosis of Exclusion”

Question 5

Etiology

Answer 5

The exact etiology of KD remains unknown. However, it is believed to be immune regulated in response to either:

(1) Infectious Agents or
(2) Environmental Toxins

Question 6

Acute Febrile

Answer 6

5 - 14 days, fever, and hallmark symptoms

Question 7

Subacute

Answer 7

~25 days post fever, peeling of hands and feet, arthralgic pain

Question 8

Convalescent

Answer 8

~ 6-8 weeks after onset, clinical signs are gone, lab values returning to normal

Question 9

Kawasaki Disease - Hallmark S&S

Answer 9

• Bilateral painless conjunctival injection - limbus sparing, without exudate
• Truncal polymorphous exanthema (maculopapular rash)
• Cervical lymphadenopathy, usually unilateral > 1.5cm
• Edematous and erythematous hands and feet
• Erythematous mouth and mucus membranes, “strawberry tongue” and chapped dry lips

Question 10

Lab Results

Answer 10

Elevated erythrocyte sedimentation rate > 40 mm/h
C-reactive protein level > 3.0 mg/L
WBC count > 15,000 uL
Normochromic, normocytic anemia for age
Sterile pyuria ( >10 WBCs per high-power field)
Serum alanine aminotransferase level >50 U/L
Serum albumin level 450,000/mm after seven days of illness

Question 11

Treatment

Answer 11

• Hospitalized, CICU or PICU
• IV infusion of immunoglobulin (IVIG): to reduce dilation of coronary arteries and prevent aneurysms, and help with fever reduction
• Aspirin: as an antipyretic, anti-inflammatory, and anti platelet agent
• Supportive care: cautious rehydration, comfort, and pain management necessary
• Skin care: cool cloths, application of aquaphor / unseated lotions, & use of light soft cotton clothing

Question 12

Following Up

Answer 12

Aspirin, low doses for 6-8 weeks
No contact sports during aspirin therapy
Healthy diet (+/- Na restriction)
- Pediatric cardiologist follow-up for serial echocardiograms to assess coronary artery status and left ventricular function / contractility @ 2 weeks post diagnosis, 6-8 weeks, 12 months
- Hold Varicella & MMR. No live flu vaccines for several months after IVIG
Cardiac catheterization in extreme cases