Kawasaki Disease
Description
Case History:
A 3-year-old boy's parents took him to the paediatric emergency room after he had a high-grade fever, agitation, and poor oral intake for seven days. He first experienced eye redness without discharge on day three of his sickness, which was followed by a widespread erythematous rash that covered his torso and groin. His parents also saw a "strawberry tongue" and red, cracked lips. His hands and feet have been swollen and red for the past two days, and the skin around his nails has been peeling. He showed bilateral non-exudative conjunctival injection, cervical lymphadenopathy (2.5 cm, left side), mucosal erythema with split lips, oedema of the hands and feet, and a fever (39.5°C) when examined. A tachycardia without murmurs was discovered during the cardiovascular examination.
ESR (95 mm/hr), CRP (12 mg/dL), leukocytosis (WBC 16,000/mm³ with neutrophilia), normocytic anaemia, and thrombocytosis (platelets 550,000/mm³) were all increased in the first lab results. The left coronary artery was shown to be dilated on echocardiography.
Classic Kawasaki illness was diagnosed based on supporting tests and clinical criteria. After starting IVIG (2 g/kg single infusion) and high-dose aspirin, the child's temperature and symptoms quickly subsided.
Overview
Mucocutaneous lymph node syndrome, another name for Kawasaki disease (KD), is a self-limited, acute systemic vasculitis of medium-sized arteries that primarily affects children under five. In wealthy nations, it is the primary cause of acquired heart disease in children. The most serious side effect is the development of coronary artery aneurysms, which can cause myocardial infarction, ischaemia, or unexpected death.
Etiology
- Unknown exact cause; thought to be complex.
- Proposed mechanisms.:
Infectious triggers (viral, bacterial superantigens).
Genetic predisposition (polymorphisms in the ITPKC, CASP3, and BLK genes; greater frequency in Asian children).
environmental variables (late winter and early spring seasonal clustering).
- According to available data, infants who are genetically predisposed may have aberrant immune responses following exposure to environmental or viral stimuli.
Epidemiology
- Age: Children under the age of five account for 80–90% of cases. peak between 18 and 24 months.
Sex: 1.5–1.7:1 male to female ratio
- Geographic: 50–250 per 100,000 children under the age of five in Japan.
10–20 per 100,000 children under five in the U.S. and Canada.
- Seasonality: Late winter and early spring see a higher occurrence.
There is a 2-3% chance of recurrence.
Clinical Features:
Stages of Disease
- Acute Phase (1–2 weeks)
high-grade fever lasting longer than five days.
non-exudative conjunctivitis on both sides.
Oral mucosal alterations (strawberry tongue; red, cracked lips).Rashes that are polymorphous (trunk, groyne, extremities).
Extremity changes (palm/sole oedema, erythema).lymphadenopathy of the cervical region (≥1 node >1.5 cm).
- Subacute Phase (2–4 weeks)
The fever goes away. PeriungualdesQuamation (peel)ithrombocytosis, nail ringing, and coronary arterAneurysms mosttprobable occurrence.
- Convalescence Phase (6–8 weeks)
Clinical recovery.
Labs normalize.
Abnormalities of the heartties might continue.
Diagnostic Criteria:
- Classic Kawasaki Disease (American Heart Association)
Fever lasting more than five days (and at least four of the five features)
Oral mucosal changes.
Polymorphous rash.
Extremity changes.
Cervical lymphadenopathy ≥1.5 cm.
- Incomplete (Atypical) Kawasaki Disease
Fever ≥5 days + 2–3 criteria + supportive labs/imaging.
Common in infants <6 months and older children.
Investigations
Blood tests: CBC: thrombocytosis (in the subacute phase), leukocytosis, and anaemia.
CRP and ESR: high.
LFTs: transaminitis that is mild.
Urinalysis: sterile pyuria.
Imaging:
Echocardiogram: myocarditis, pericardial effusion, coronary artery dilatation/aneurysms.
MRI if the echo is not conclusive.
Measles, scarlet fever, adenovirus, toxic shock syndrome, and medication responses are examples of differential diagnoses.
Protocol
Management:
IVIG in the first line (2 g/kg given once over 10–12 hours).
Aspirin: high dosage till afebrile (30–50 mg/kg/day in 4 doses).
Then, for 6–8 weeks (or longer if coronary involvement is present), take a modest dosage (3–5 mg/kg/day).
KD Refractory
IVIG should be repeated.
corticosteroids.
biologics (Anakinra, Infliximab) in situations of resistance.
intravenous support fluids.
antipyretics.
anticoagulants (LMWH, warfarin) in the event of large aneurysms.
Nursing interventions:
- Temperature and Inflammation Issue:
A kid with systemic vasculitis and a persistently high temperature is susceptible to consequences, including dehydration and irritability.
- Medical Administration:
To lower inflammation and lower the risk of coronary artery aneurysms, provide intravenous immunoglobulin (IVIG) at a dose of 2 g/kg over a period of 10–12 hours, preferably within 7–10 days after the commencement of the fever.
During the acute phase, the kid was given high-dose aspirin (80–100 mg/kg/day) every six hours. After 48 hours, the child was switched to low-dose aspirin.
- Interventions in Nursing:
Regularly check your temperature and vital signs to see how well your treatment is working.
As directed, provide IVIG and aspirin while keeping a look out for any negative side effects, such as hypersensitivity, haemolysis, or Reye syndrome.
To avoid dehydration, promote oral fluid intake or, if required, give IV fluids.
Offer comfort measures such as cold compresses and a peaceful setting.
Risk of Coronary Artery Aneurysms
- Issue: Medium-vessel vasculitis with a high risk of aneurysm development and coronary artery involvement.
- Medical Management: Echocardiography is used to track heart function during the acute phase, two weeks, and eight weeks after diagnosis; if coronary aneurysms are found, this may be done more regularly.
If the fever doesn't go away 36–48 hours after the first dose, repeat IVIG.
Antiplatelet or anticoagulant drugs, such as clopidogrel or warfarin, for big aneurysms; low-dose aspirin for 6–8 weeks or longer if cardiac involvement continues.
- Interventions in Nursing:
Check for symptoms of myocardial ischaemia, such as pallor, tachycardia, diaphoresis, and chest discomfort.
Keep an eye on the following test results: liver function, platelet counts, cardiac indicators, CBC, CRP, and ESR.
Inform carers on the symptoms of heart problems and the need of cardiac follow-up.
Mucocutaneous Changes and Skin Issues
- Problem: Rash, edema, strawberry tongue, cracked lips, and later desquamation may cause discomfort and risk of infection.
- Medical Management: Symptomatic relief.
- Nursing Interventions:
Administer mouth and skin care to prevent secondary infections (e.g., apply lip balm, keep skin clean and dry).
Use soft toothbrushes and encourage gentle oral hygiene.
Advise against scratching to reduce infection risk.
Assess for peeling and secondary skin infections, and escalate care if wounds or infection signs develop
Notes
For more information visit https://www.ncbi.nlm.nih.gov/books/NBK537163/