Paediatric Acute care Guidelines PMH Emergency Department

Background

  • Kawasaki disease is a common vasculitis of childhood especially in < 5 year olds
  • Aetiology is unknown
  • Is a self limiting condition, with fever and manifestations of acute inflammation lasting an average 12 days without therapy, however, early treatment is necessary to prevent complications

Complications

  • Coronary artery aneurysms
    • Infants under 12 months at increased risk of coronary artery aneurysm
    • Delay of treatment (after 10 days) increases risk of coronary artery aneurysm by five times
  • Depressed myocardial contractility & heart failure
  • Myocardial infarction
  • Arrhythmias
  • Peripheral arterial occlusion 

Assessment

Diagnostic Criteria

Kawasaki Disease is a clinical diagnosis with no diagnostic laboratory test.

Presence of prolonged unexplained fever ≥ 5 days (fever > 38.5°C) with at least 4 of the following criteria: 

  1. Bilateral non-exudative conjunctivitis
  2. Polymorphous rash
  3. Cervical lymphadenopathy (at least 1 lymph node >1.5cm in diameter)
  4. Mucositis – cracked red lips, injected pharynx or strawberry tongue
  5. Extremity changes – erythema of palms/soles, oedema of hands/feet (acute phase), and periungual desquamation (convalescent phase) 

Associated non-specific symptoms

  • Diarrhoea, vomiting, or abdominal pain 
  • Irritability 
  • Cough or rhinorrhea 
  • Joint pain  
  • Weakness

Incomplete (Atypical) Kawasaki Disease

  • Diagnostic criteria not completely fulfilled (< 4 signs of mucocutaneous inflammation) but otherwise similar clinical picture to that of “classic” Kawasaki Disease
  • More likely with children < 12 months old and > 5 years old
  • Atypical Kawasaki Disease patients are still at risk of cardiovascular complications
    • If prolonged unexplained fever discuss with ED Consultant/on call General Paediatric Consultant  

Laboratory findings (not diagnostic but supportive) 

  • Elevated acute phase reactants (CRP, ESR)
  • Elevated WCC with predominant neutrophilia
  • Elevated platelets (after 1 week) 
  • Normocyctic, normochromic anaemia
  • Sterile pyuria (need clean voided specimen) 

Investigations

No diagnostic lab tests for Kawasaki Disease but can be supportive or used to exclude other causes of fever.

  • CPR, ESR, FBC, ALT, Albumin
  • ASOT/AntiDNAse B
  • Urinalysis – clean catch or in-out catheter
  • Blood culture 

Differential diagnoses

  • Measles
  • Adenovirus
  • Epstein Barr Virus
  • Scarlet fever
  • Toxic Shock Syndrome
  • Steven-Johnson Syndrome

Management

All suspected cases should be discussed with the ED Admitting Registrar/Consultant for admission under the on call General Paediatric Consultant

Initial Management

  • IV Immunoglobulin (IVIG) 2 grams/kg over 8-12 hours
  • Low dose aspirin at 3-5mg/kg daily

Further Management 

  • Second dose IVIG may be given if incomplete treatment response, particularly in high risk age group and “atypical” Kawasaki Disease 
  • General Paediatric Team will refer the patient to Cardiology for echocardiogram only after the diagnosis of Kawasaki Disease is made/confirmed and treatment instituted 
  • Many patients (especially if < 3 years old) will require sedation to perform the echocardiogram as the irritability (commonly seen) precludes performing adequate echocardiogram in the acute phase
  • Echocardiogram is required at/after initial diagnosis and repeated at 4-8 weeks post treatment
  • Echocardiogram plays no role in the diagnosis of Kawasaki Disease
  • Referral to the Infectious Disease Consultant is at the discretion of the General Paediatric Consultant

Discharge Treatment

  • Continue aspirin (low dose) at 3-5mg/kg daily until the repeat echocardiogram at 4-8 weeks confirms absence of coronary involvement

Nursing

Routine nursing care.

References

  1. Sundal R (2014) Kawasaki Disease: Clinical Features and Diagnosis. UpToDate. Accessed at www.uptodate.com
  2. Sundal R (2014) Kawasaki Disease: Initial Treatment and Prognosis. UpToDate. Accessed at www.uptodate.com
  3. AMH Children’s Dosing Companion (online). Adelaide: Australian Medicines Handbook Pty Ltd; 2015 January. Available from: https://childrens-amh-net-au.pklibresources.health.wa.gov.au

We want your feedback!

Help us provide guidelines that are useful to you, the clinician.

Give feedback here