1. Kawasaki disease
By: Brittni McClellan

2. Kawasaki disease - description
An idiopathic, multisystem disease of young children
Characterized by vasculitis of the small- and medium-sized blood vessels.
The acute phase is self-limited;.
Signs and symptoms evolve over the 1st 10 days, then resolve spontaneously.
20–25% of untreated patients will develop coronary artery aneurysms.
Etiology: Unknown

3. Kawasaki disease – H&P Patient History: 3 Phases:
Acute Phase (1-2 Weeks from Onset)
Febrile: >39 degrees C
Oral Changes
Rash – Prone to Perineal Area
Edema/Erythema of Feet
Painful, Limits Ambulation
Subacute Phase (2-8 Weeks from Onset)
Gradual Improvement, Decrease in Fever
Desquamation of the Perineal Area, Palms, Soles, and Periungal Areas
Coronary Artery Aneurysms/MI
Arthritis/Arthralgias
Convalescent Phase (Months-Years from Onset)
Resolution of Remaining Symptoms/Normal Labs
Aneurysms may resolve or be persistent.

4. Kawasaki disease – H&P Physical Exam:
High, Unremitting Fever for 1-2 Weeks
Unilateral, Cervical
Rash: Erythematous and Polymorphous
Aseptic Meningitis
Prominent on Trunk, Perineum
Pancarditis
Maculopapular, May Coalesce
Tachycardia, Gallop Rhythms
Conjunctivitis: Bilateral
Muffled Heart Sounds, Signs of CHF, or Murmurs
Oral Changes
Abdominal Changes
Erythema, Fissures, Crusting of Lips
RUQ Mass Abdominal Pain
Diffuse Oropharyngeal Erythema
Diarrhea
Strawberry Tongue
Hydrops of Gallbladder
Extremity Changes
Hepatosplenomegaly/Jaundice
Metatitis/Vulvitis
Erythema/Desquamation of the Palms/Soles
Beau Lines (Transverse Grooves Across Fingernails)
Arthritis/Anthralgias
Adenopathy
Uveitis

5. Kawasaki disease – signs and symptoms

6. Kawasaki disease - Diagnosis
Fever for >45 Days
>4 of the Criteria:
Bilateral nonexudative conjunctival injection
Polymorphous, nonvesicular rash
Mucosal involvement of the upper respiratory tract that may include erythema, fissured lips, crusting of the lips and mouth, strawberry tongue, or injected, nonexudative pharyngitis.
Edema or erythema of the hands and feet
Cervical adenopathy (>1.5 cm diameter), which is often unilateral

7. Kawasaki disease - Diagnosis
CBC:
WBC usually increased with a left shift; >15,000
Anemia for age
Elevated ESR and C-reactive protein
Other laboratory abnormalities:
Sterile pyuria (70%)
Mild increases in hepatic transaminases
A CSF pleocytosis with a normal protein/glucose
Mild hypoalbuminemia
Imaging
Chest x-ray: Dilated heart during acute phase
Echocardiography:
Decreased shortening fraction and effusion
Coronary ectasia, dilation, or aneurysms may be detected as early as 6 days into the illness; peak onset is between 3 and 4 weeks.

8. Kawasaki disease – treatment
Medication
IV Immunoglobulin
Aspirin
Complications:
Aneurysms:
Noted days after onset of disease
May Thrombose, or Rupture
Reticulocyte count increases in 3-4 days

9. Question 1
A 9 month old boy is brought to the clinic because he has had temperatures up to 104°F. He has been fussy, had been eating less, and his mother explains that the fever began 5 days ago. The child does not have any close sick contacts. His temperature is currently 103.5°F. A physical exam reveals the following findings: cracked fissured lips, redness of oral mucosa, redness of conjunctiva, enlarged cervical lymph node. There is also a maculopapular rash over the trunk and dorsal edema of the hands. The patient’s hematocrit is 32%, leukocyte count is 15,000/mm³, and platelet count is 560,000/mm³. What is the most feared complication of this disease?
Hepatosplenomegaly and Jaundice
Erythematous, Maculopapular Rash, covering over 90% of the body surface area.
Aneurysms, especially 3-4 weeks post- disease.
Anaphylaxis from the IVIG used to treat the disease.
Aneurysms, especially during the first week of the disease.
c) Aneurysms, especially 3-4 weeks post-disease.