Kawasaki disease

Background

• Mucocutaneous lymph node syndrome
• Vasculitis of unknown etiology
• Peaks at 18-24 months
  • Rare in <4mo, >5yr
• Leading cause of acquired heart disease in children

Classic Kawasaki Disease	Incomplete Kawasaki Disease
Fever for 5 d or more plus four of the following symptoms	Fever for 5 d and two to three clinical criteria of classic Kawasaki disease
1. Bilateral nonexudative conjunctivitis	plus
2. Mucous membrane changes (erythema, peeling, cracking of lips, "strawberry tongue," or diffuse oropharyngeal mucosae)	C-reactive protein [[Image:]]3.0 milligrams/L and/or erythrocyte sedimentation rate [[Image:]]40 mm/h plus three or more of the following supplemental labs or positive echo
3. Changes of the extremities (erythema or swelling of hands/feet, peeling of finger tips/toes in the convalescent stage)	1. Albumin <3 grams/dL
	2. Anemia for age
	3. Elevated alanine aminotransferase
	4. Platelets >450,000/mm3 after 7 d of fever onset
4. Rash
5. Cervical adenopathy (more than one node >1.5 cm unusually unilateral anterior cervical)	5. White blood cell count >12,000/mm3
	6. Presence of pyuria

Diagnosis

CDC Definition

• Fever >5d and 4 of the following:

1. Rash
2. Cervical LAD (>1.5cm diam, usually unilat)
3. Bilateral nonexudative conjunctivitis
4. Oral mucosal changes
5. Extremity edema/erythema/desquamation

Presentation

• Fever that is high, abrupt
• Rash often seen in perineum; accompanies onset of fever
• Cardiac complications develop early on
  • Coronary artery aneurysm development most prevalent as fever lessens

Associated Sx

1. Cardiac
   1. Coronary aneurysm
      1. Most develop during 3-4th week of illness
      2. May lead to MI (leading cause of death)
   2. Myo/pericarditis
   3. Pericardial effusion
   4. LV dysnfunction
   5. Valvular dysfunction
   6. Dysrhythmias
2. Labs
   1. Elevated ESR/WBC/LFTs/Plts
3. Aseptic meningitis
4. Urethritis, Anemia
5. RUQ pain, large GB (hydrops)

Work-Up

1. CBC/Diff/SPA/ALT/TBili
2. Blood Cx and UA
3. ECG
4. Echo (Coronaries, LV, Valves)
5. Red Top "Kawasaki Serum to CBR"

Treatment

1. Immunoglobulin
   1. IVIG 2gm/kg over 12hr
2. ASA 20mg/kg/dose q6h

Disposition

1. Cardiology f/u
2. Cont ASA at high dose
   1. Switch to ASA 3-5mg/kg/day once afebrile x48h

Source

Tintinalli