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    Kawasaki Disease Diagnostic Criteria

    Diagnoses Kawasaki Disease.
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    Children with several days of unexplained fever associated with any of the principal clinical features of Kawasaki Disease (KD).

    • The Kawasaki Disease Criteria are widely used for the diagnosis of Kawasaki Disease, also known as mucocutaneous lymph node syndrome, adopted by the American Heart Association (AHA) and endorsed by the American Academy of Pediatrics (APA). (Newburger 2004)
    • There is no diagnostic laboratory test. The diagnosis is clinical.
    • Clinical findings do not commonly manifest simultaneously and there is no typical order of appearance. Repeated examinations and close history-taking are important.
    • Can be confused with other infectious exanthema of childhood, and concurrent viral infections are common. Non-specific symptoms commonly occur in children, including arthritis, vomiting, diarrhea, abdominal pain, irritability, cough, rhinorrhea and decreased oral intake. (Baker 2009)
    • Although there is no diagnostic laboratory test, elevated WBC and platelet counts, transaminases, and acute phase reactants, as well as anemia and pyuria, can be suggestive of KD.

    Points to keep in mind:

    • It is suspected that at least 10% of patients who develop coronary artery (CA) abnormalities fail to meet the criteria for KD. (Sundel 2002). The criteria may fail to diagnose a substantial number of patients.
    • Patients suspected of having KD who do not fulfill the diagnostic criteria may have incomplete (atypical) KD. Children with incomplete KD, whose diagnosis is delayed, are more likely to develop CA abnormalities. The AHA and AAP have developed criteria to help diagnose and guide treatment in incomplete KD. (Newburger 2004)

    The most common and dangerous long-term sequelae of KD are CA abnormalities (aneurysms or ectasia) that develop in up to 25% of untreated children and may lead to sudden death or ischemic heart disease. (Newburger 2004)

    Fever for ≥5 days and 4+ additional criteria required for positive diagnosis (see Evidence for exceptions)

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    About the Creator
    Dr. Tomisaku Kawasaki
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      • Joyce Brown, MD
      • Reviewed by Angela Cavanna, DO

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      Advice

      • If the diagnosis of KD is made in the acute phase, treatment should be initiated to prevent CA abnormalities.
      • If the patient does not meet the criteria for KD, consider incomplete KD.

      Management

      • Prompt treatment in the acute phase with intravenous immune globulin (IVIG) and aspirin reduces the prevalence of the development of CA abnormalities and subsequent mortality and morbidity. The two appear to have an additive effect. (Newburger 2004)
      • Treatment should be instituted within the first 10 days of the illness and if possible within 7 days of illness.
      • IVIG
        • The mechanism is unknown, but believed to be linked to the anti-inflammatory effect.
        • Different dosing regimens exist. A single, higher dose is most effective. (Newburger 1991)
        • Based on timing of development of the CA abnormalities, therapy should be instituted within the first 10 days of illness and, if possible, within 7 days of illness.
        • IVIG should be considered in children presenting after the 10th day of illness if they have persistent fever without other explanation or aneurysms and ongoing systemic inflammation, as manifested by elevated ESR or CRP. (Newburger 2004)
      • High dose aspirin
        • Administered with IVIG.
        • Has anti-inflammatory effects, but does not appear to lower the frequency of development of CA abnormalities. (Newburger 2004)
        • Dosing regimens vary.
        • The risk of Reye Syndrome must be considered and weighed against the benefits of therapy.
        • Annual influenza vaccination is recommended in patients taking aspirin long term.
        • The risks and benefits of varicella vaccination in children receiving long term aspirin therapy must be considered.
        • Ibuprofen should be avoided because it antagonizes the irreversible platelet inhibition that is induced by aspirin.
      • Steroids may be considered as adjuvant treatment to help decrease the rate of CA abnormalities, although they are not routinely used.
      • Management algorithms exist for the treatment of incomplete and refractory KD.

      Critical Actions

      • Suspect KD in any patient (particularly children <5 years old) presenting with several days of otherwise unexplained fever.
      • Early diagnosis and treatment during the acute phase is essential in preventing CA abnormalities.

      Formula

      Classic clinical features for diagnosis:

      • Fever persisting at least 5 days
      • At least 4 of the following 5 principal features:
        1. Changes in extremities
          • Acute: Erythema of palms and soles; edema of hands and feet
          • Subacute: Periungual peeling of fingers and toes in weeks 2 and 3
        2. Polymorphous exanthem
        3. Bilateral bulbar conjunctival injection without exudate
        4. Changes in lips and oral cavity: Erythema, lips cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosae
        5. Cervical lymphadenopathy (>1.5-cm diameter), usually unilateral
      • Exclusion of other diseases with similar findings

      Additionally, the diagnosis is made if any of the following is true:

      • Fever for ≥5 days and at least 4 of the 5 principal features.
      • Fever for ≥5 days and <4 principal features when coronary artery disease is detected by 2D echocardiography or coronary angiography.
      • Day 4 of illness and ≥4 principal features.

      Evidence Appraisal

      • The criteria for diagnosis of Kawasaki Disease were developed by expert consensus and have not yet been validated. There is no gold standard for diagnosis for Kawasaki Disease.
      • A new classification tool has been developed and is currently being evaluated to help differentiate KD from other pediatric febrile illnesses at the point of care. The results are promising, but more evaluation is needed. It is hoped that this tool will increase the ability of the clinician to diagnose KD and subsequently decrease the incidence of CA abnormalities due to delayed or missed diagnosis. (Hao 2016)

      Literature

      Other References

      Research PaperDajani A, et al. Diagnosis and therapy of Kawasaki disease in children. Circulation. 1993; 87, 1776-1780.Research PaperBaker AL, et al. Associated Symptoms in the Ten Days Before Diagnosis of Kawasaki Disease. The Journal of Pediatrics. 2009; 154(4), 592. doi:10.1016/j.jpeds.2008.10.006Research PaperSundel RP. Update on the treatment of kawasaki disease in childhood. Current Rheumatology Reports. 2002; 4(6), 474-482. doi:10.1007/s11926-002-0053-6Research PaperKawasaki T. Pediatric acute febrile mucocutaneous lymph node syndrome with characteristic desquamation of fingers and toes: My clinical observation of fifty cases. Pediatr Infect Dis J. 2002; 21, 1-38.Research PaperNewburger JW, et al. A Single Intravenous Infusion of Gamma Globulin as Compared with Four Infusions in the Treatment of Acute Kawasaki Syndrome. The New England Journal of Medicine. 1991; 24, 1633-1639.Research PaperHao S, et al. A Classification Tool for Differentiation of Kawasaki Disease from Other Febrile Illnesses. The Journal of Pediatrics. 2016; 176, 114-120.
      Dr. Tomisaku Kawasaki

      About the Creator

      Tomisaku Kawasaki, MD is a pediatrician and formerly served as the director of the department of pediatrics at the Red Cross Hospital. He was also the director of the Japan Kawasaki Disease Research Center, chairman of the Kawasaki Disease Research Committee and the director of the Kawasaki Disease Research Center in Tokyo. He has won several awards, including the first Japanese Pediatric Society Prize.


      To view Dr. Tomisaku Kawasaki's publications, visit PubMed

      Content Contributors
      • Joyce Brown, MD
      • Reviewed by Angela Cavanna, DO
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