Back to Basics: Kawaski Disease

Mon, 07/02/2018 - 5:00am
Author: 
Louis Argentine MD
Editor: 
Alexis Pelletier-Bui, MD
Catherine Ginty, MD

Back to Basics: Kawasaki Disease

Lou Argentine MD

 

BACKGROUND:

  • AKA “mucocutaneous lymph node syndrome”

  • No definitive cause, immune mediated disorder

  • Leading cause of acquired heart disease in children

  • Occurs in all ethnic groups, however incidence highest in East Asia and children of Asian descent

  • 80% of patients are <5 years old (peak between 18 and 24 months old)

CLINICAL:

  • No specific tests, diagnosis is based on clinical criteria

  • Clinical Criteria (Fever for 5 or more days PLUS 4 or more of following):

      • Skin rash - polymorphic, nonvesicular, commonly seen on trunk and extremities

      • Mucus membrane changes - strawberry tongue, erythematous/fissured lips, erythema of buccal mucosa/pharynx

      • Conjunctivitis - bilateral bulbar involvement, nonexudative

      • Changes in distal extremities - erythema/swelling of palms or soles, desquamation/peeling of fingers and toes

      • Cervical lymphadenopathy - usually unilateral, at least one node 1.5 cm or larger, non purulent

  • Serious complication: coronary artery aneurysm which can lead thrombosis

  • Incomplete Kawasaki Disease:  KD with incomplete clinical manifestations (lack sufficient signs to fulfill the diagnosis of KD), more common in young infants

WORK-UP:

  • Labs:

        • CBC : normal to elevated WBC count, normocytic anemia, thrombocytosis

        • ESR and CRP : elevated

        • Liver enzymes : elevated with hypoalbuminemia

        • Urinalysis : sterile pyuria and proteinuria

  • Imaging:

        • EKG : look for evidence of myocarditis/ pericarditis/ arrhythmia

        • CXR : look for cardiomegaly

DISPO / TREATMENT:

  • Admit patients with clinical diagnosis of KD or incomplete KD for continuous cardiac monitoring and treatment / echocardiogram

  • Treatment: IVIG and ASA

        • Administration of IVIG in the acute phase (within 10 days of onset of fever) significantly reduces the prevalence of coronary artery dilation and aneurysms

REFERENCES:

 

Shah, BR, et al. Atlas of Pediatric Emergency Medicine. 2nd ed. New York: NY: McGraw-Hill Education, 2013.

 

Stone, CK, et al. Current Diagnosis and Treatment: Pediatric Emergency Medicine. New York: NY: McGraw-Hill Education, 2014.

 

Tintinalli, JE. Tintinalli's Emergency Medicine: A Comprehensive Study Guide. 8th ed.                     New York, NY: McGraw-Hill Education LLC, 2016.