Kawasaki diseaseMANAGEMENT

PEDIATRIC-SPECIFIC PRESENTATIONS · 12 SECTIONS

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324 items

☷ Legend

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Cardiovascular Presentations66▾

Respiratory Presentations47▾

Neurological Presentations77▾

Gastrointestinal Presentations63▾

Genitourinary and Reproductive Presentations44▾

Infectious Disease Presentations146▾

Trauma Presentations88▾

Toxicologic Presentations67▾

Psychiatric Presentations25▾

Musculoskeletal and Rheumatologic Presentations89▾

Dermatology Presentations21▾

Environmental and Exposure Presentations26▾

Hematologic and Oncologic Presentations30▾

Pediatric-Specific Presentations13▾

Bronchiolitis Croup Emergency delivery Febrile seizure Intussusception Kawasaki disease Neonatal jaundice Neonatal sepsis Nursemaid's elbow Pediatric fever 0 to 28 days Pediatric fever 29 to 60 days Pediatric fever 61 to 90 days Pyloric stenosis

Endocrine and Metabolic Presentations42▾

ENT and Maxillofacial Presentations21▾

Ophthalmologic Presentations15▾

Obstetric Presentations17▾

Systemic and Miscellaneous Presentations15▾

01Approach to the Critical Patient/attcp29

Time critical priorities

›Stabilization priorities

›Airway compromise from severe cervical lymphadenopathy

›Shock physiology from myocarditis or KD shock syndrome

›Respiratory failure from pulmonary edema

›Early senior pediatrics involvement for ill appearance

›Early pediatric cardiology involvement for suspected KD

›If hypotension or poor perfusion, resuscitation bay escalation

›IV access x2 or IO

›Isotonic crystalloid 10-20 mL/kg bolus with reassessment

›If persistent shock, vasoactive support and ICU

›Point of care ultrasound for ventricular function and effusion

High risk features

›High risk clinical states

›Age under 6 months

›Prolonged fever 7 days or more

›Incomplete KD features

›Toxic appearance

›Refractory fever after IVIG

›Suspected macrophage activation syndrome

›Significant myocarditis

›Tachycardia out of proportion to fever

›Gallop rhythm

›Elevated troponin

›Depressed ventricular function on echocardiography

Hemodynamic and monitoring targets

›Monitoring and targets

›Continuous cardiac monitoring

›Frequent blood pressure measurement

›Strict intake and output

›If shock or myocarditis, ICU level monitoring

›If vasoactive infusions, arterial line consideration

02History/hx41

Presentation pattern

›Typical illness pattern

›Fever 5 days or more

›Fever often high and persistent

›Poor response to antipyretics

›Irritability and poor feeding

›Timing of day of illness for treatment window

Classic clinical features

›Principal diagnostic features

›Bilateral nonexudative conjunctival injection

›Oral mucosal changes

›Erythematous cracked lips

›Strawberry tongue

›Diffuse oropharyngeal erythema

›Polymorphous rash

›Extremity changes

›Erythema and edema of hands or feet

›Periungual desquamation in subacute phase

›Cervical lymphadenopathy

›Often unilateral

›Node size 1.5 cm or more

Incomplete and atypical clues

›Incomplete KD indicators

›Prolonged fever with 2 to 3 principal features

›Infants under 6 months with prolonged fever and few signs

›Early sterile pyuria

›Early hepatitis or cholestasis

›Aseptic meningitis symptoms

›Abdominal pain or hydrops of gallbladder

›Arthralgia or arthritis

Exposure and alternative diagnosis screen

›Mimic screen

›Sick contacts with viral conjunctivitis

›Exudative pharyngitis history

›Recent antibiotic exposure with drug eruption

›Recent SARS CoV 2 infection or exposure for MIS C

›Travel and tick exposure for rickettsial illness

›Vaccination history

Cardiac symptom screen

›Cardiac and perfusion clues

›Chest pain

›Dyspnea

›Syncope

›Palpitations

›Decreased urine output

›Exercise intolerance in older child

03Physical Exam/exam28

Core findings

›Principal signs

›Temperature pattern and toxic appearance

›Conjunctival injection without exudate

›Oral mucosal erythema

›Cracked lips

›Strawberry tongue

›Polymorphous rash distribution

›Hand and foot edema

›Palmar or plantar erythema

›Cervical lymphadenopathy

Cardiovascular and perfusion

›Cardiac assessment

›Heart rate relative to temperature

›Capillary refill time

›Peripheral pulses

›New murmur

›Gallop rhythm

›Hepatomegaly

›Peripheral edema beyond extremities

Subacute phase findings

›Later signs

›Periungual desquamation

›Beau lines

›Arthritis or limited range of motion

PITFALLS

›Common pitfalls

›Exudative conjunctivitis suggesting adenovirus rather than KD

›Exudative pharyngitis suggesting streptococcal infection

›Vesicular rash suggesting varicella or enterovirus

›Hypotension and multisystem involvement suggesting MIS C or toxic shock

›Early normal platelet count not excluding KD

04Differential Diagnosis/ddx21

Life threatening and time sensitive

›Critical alternatives

›Toxic shock syndrome ICD 10 A48.3

›Staphylococcal scalded skin syndrome ICD 10 L00

›Meningococcemia ICD 10 A39.2

›Stevens Johnson syndrome ICD 10 L51.1

›MIS C ICD 10 M35.81

Common mimics

›Infectious mimics

›Adenovirus infection

›Scarlet fever ICD 10 A38

›Measles ICD 10 B05

›EBV infectious mononucleosis ICD 10 B27.90

›Enteroviral exanthem

›Parvovirus B19

›Inflammatory mimics

›Systemic juvenile idiopathic arthritis ICD 10 M08.2

›Systemic lupus erythematosus ICD 10 M32.9

›Polyarteritis nodosa ICD 10 M30.0

›Serum sickness like reaction

KD coding targets

›Kawasaki disease coding

›Kawasaki disease ICD 10 M30.3

›Mucocutaneous lymph node syndrome SNOMED CT concept

05Laboratory Tests/lt33

Baseline inflammatory and hematology

›Core laboratory set

›C reactive protein for incomplete algorithm support

›Threshold 30 mg/L or more as supportive criterion in incomplete algorithm

›Erythrocyte sedimentation rate

›Threshold 40 mm/h or more as supportive criterion in incomplete algorithm

›Complete blood count with differential

›Leukocytosis common in acute phase

›Normocytic anemia of inflammation

›Thrombocytosis typical after day 7

›Comprehensive metabolic panel

›Sodium mmol/L

›Albumin g/L

›ALT and AST

›Bilirubin

›Creatinine

›Urinalysis

›Sterile pyuria support

Incomplete KD supplemental laboratory criteria

›Supplemental criteria set

›Albumin 30 g/L or less

›Anemia for age

›ALT elevation

›Platelets 450 x10^9/L or more after day 7

›White blood cells 15 x10^9/L or more

›Urine white blood cells 10 per high power field or more

Myocarditis and shock evaluation

›Cardiac and shock labs

›High sensitivity troponin if myocarditis concern

›BNP or NT proBNP if heart failure concern

›Venous blood gas if shock

›Lactate mmol/L

›Blood culture if sepsis alternative plausible

Treatment safety monitoring

›Medication safety labs

›Baseline AST and ALT before prolonged aspirin

›Baseline hemoglobin and platelets before anticoagulation escalation

06Diagnostic Tests/dt51

Scoring Systems

›Diagnostic frameworks

›Classic KD criteria

›Fever 5 days or more

›Four or more principal clinical features

›Exception for earlier diagnosis with strong clinical suspicion

›Incomplete KD algorithm

›Fever 5 days or more with 2 to 3 principal features

›CRP 30 mg/L or more or ESR 40 mm/h or more

›Supplemental laboratory criteria count

›Echocardiography integration into decision pathway

›IVIG resistance risk tools

›Kobayashi score

›Egami score

›Sano score

›Limited performance outside Japanese cohorts

›Coronary artery involvement definitions

›Z score based classification

›Small aneurysm Z 2.5 to under 5

›Medium aneurysm Z 5 to under 10 and absolute dimension under 8 mm

›Large or giant aneurysm Z 10 or more or absolute dimension 8 mm or more

MRI

›Cardiac MRI roles

›Myocarditis assessment when diagnosis uncertain

›Ventricular function and myocardial edema evaluation

›Coronary artery imaging when echocardiography limited in older child

›Sedation considerations in young children

›Contrast considerations with renal function

›CT applications

›Coronary CT angiography for poor echocardiographic windows

›Coronary aneurysm and thrombosis assessment in selected cases

›Radiation minimization strategies

›Contrast allergy and renal function considerations

Ultrasound

›Echocardiography and bedside ultrasound

›Transthoracic echocardiography key elements

›Coronary artery dimensions with Z scores

›Ventricular systolic function

›Mitral regurgitation

›Pericardial effusion

›Timing principles

›Echocardiography at diagnosis without delaying IVIG

›Repeat echocardiography 1 to 2 weeks after onset

›Repeat echocardiography 4 to 6 weeks after onset

›Point of care ultrasound adjuncts

›Global LV function estimation

›Pericardial effusion assessment

›IVC assessment in shock with clinical correlation

›ECG

›Baseline rhythm and conduction assessment

›Myocarditis or ischemia patterns

›Chest radiograph

›Pulmonary edema in myocarditis

›Alternative pneumonia diagnosis support

07Disposition/dispo14

Level of care

›Admission decision set

›All suspected KD for inpatient management

›Incomplete KD meeting algorithm criteria for inpatient management

›Transfer to pediatric center if echocardiography not available

›ICU indications

›Shock requiring vasoactive support

›Significant ventricular dysfunction

›Arrhythmia requiring intervention

›Respiratory failure

›Giant coronary aneurysm or coronary thrombosis

Follow up structure

›Follow up planning

›Pediatric cardiology follow up for all confirmed KD

›Echocardiography schedule alignment with coronary findings

›Long term thromboprophylaxis plan for aneurysm risk tiers

08Treatment/t48

First line therapy

›Standard acute therapy

›IVIG 2 g/kg single infusion

›Initiate as early as possible within first 10 days of illness

›If day 10 or later with ongoing inflammation, IVIG still indicated

›AHA Class I recommendation for IVIG in acute KD

›Aspirin anti inflammatory phase

›Moderate dose 30 to 50 mg/kg/day divided dosing

›High dose 80 to 100 mg/kg/day alternative by local protocol

›Uncertain added benefit of high dose aspirin for coronary outcomes

›Aspirin antiplatelet phase

›Low dose 3 to 5 mg/kg/day once daily

›Continue until 6 to 8 weeks and normal echocardiography

Adjunctive primary therapy for high risk

›High risk intensification

›Corticosteroids with IVIG for selected high risk patients

›AHA Class IIa recommendation for selected high risk features

›Prednisolone 2 mg/kg/day divided dosing with taper per protocol

›Methylprednisolone 30 mg/kg IV single daily dose option

IVIG resistant KD

›Persistent or recrudescent fever 36 hours or more after IVIG

›Second IVIG 2 g/kg infusion option

›Infliximab 5 mg/kg IV single dose option

›Corticosteroid escalation option

›Cyclosporine option in selected cases

›Anakinra option in refractory disease with specialist input

Antithrombotic escalation for coronary aneurysm

›Thromboprophylaxis by coronary risk tier

›No aneurysm or transient dilation

›Low dose aspirin only

›Small aneurysm

›Low dose aspirin

›Medium aneurysm

›Low dose aspirin

›Consider additional antiplatelet therapy per cardiology

›Large or giant aneurysm

›Low dose aspirin

›Systemic anticoagulation per cardiology

›Warfarin INR target per cardiology protocol

›Low molecular weight heparin weight based dosing per local protocol

Supportive care and safety

›Supportive care

›Hydration support with heart failure awareness

›Antipyresis for comfort

›Avoid ibuprofen with aspirin antiplatelet therapy

›IVIG precautions

›Monitor for infusion reactions

›Hemolysis risk awareness in high dose IVIG

›Aseptic meningitis risk counseling

›Aspirin precautions

›Bleeding risk monitoring

›Reye syndrome counseling for viral illness

›Influenza vaccination prioritization

09Special Populations/sp18

Pregnancy

›Pregnancy considerations

›Adult KD rare but possible

›Cardiology involvement for maternal coronary risk

›Aspirin dose selection coordinated with obstetrics

›Anticoagulation decisions coordinated with maternal fetal medicine

Geriatric

›Older adult considerations

›KD extremely rare

›Alternate vasculitides more likely

›Coronary imaging thresholds individualized

›Higher baseline cardiovascular risk affecting outcomes

Pediatrics

›Pediatric specific management

›Peak incidence in children under 5 years

›Infants under 6 months higher incomplete presentation risk

›Weight based dosing for IVIG and aspirin

›Early echocardiography and repeat schedule

›MIS C differentiation in school age children and adolescents

›Hypotension and GI symptoms more prominent in MIS C

›Cardiac dysfunction common in both

10Background/b15

Epidemiology

›Epidemiology facts

›Most common cause of acquired heart disease in children in high income countries

›Highest incidence in children under 5 years

›Male predominance

›Seasonal clustering in some regions

Pathophysiology

›Disease mechanism

›Medium vessel vasculitis with coronary artery predilection

›Endothelial inflammation leading to aneurysm formation

›Myocarditis in acute phase contributing to shock

›Thrombosis risk in large aneurysms

Therapeutic Considerations

›Treatment rationale

›IVIG reduces coronary artery aneurysm risk when given early

›Aspirin used for anti inflammatory and antiplatelet effects

›Early identification of high risk patients for intensified therapy focus in 2024 AHA update

›Coronary Z score based risk stratification guides follow up and thromboprophylaxis

11Patient Discharge Instructions/pdi17

copy discharge instructions

›Home care and follow up plan

›Aspirin exactly as prescribed

›Avoid ibuprofen unless cardiology approves

›Cardiology follow up appointment attendance

›Echocardiography follow up attendance

›Live vaccines delayed after IVIG per immunization guidance

›Fever diary for recurrence after discharge

›Return to emergency criteria

›Fever recurrence

›Breathing difficulty

›Chest pain

›Fainting

›New severe abdominal pain

›Signs of dehydration

›Bleeding or black stools while on aspirin

›New swelling of hands or feet

›Extreme sleepiness or confusion

12References/r9

Clinical guidelines and key sources

›Guideline sources

›American Heart Association scientific statement update on Kawasaki disease 2024

›American Heart Association statement diagnosis treatment and long term management 2017

›ACC summary of AHA 2017 ten points to remember

›Royal Childrens Hospital Melbourne clinical practice guideline Kawasaki disease

Evidence summaries and recent studies

›Evidence sources

›JAMA Network Open 2025 aspirin dosing and outcomes in Kawasaki disease

›Cochrane style evidence review IVIG and aspirin regimens 2023

›File reference for formatting and structure

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Reviewed by SymptomDx Medical Team · Last reviewed June 16, 2026

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