Podcast
Questions and Answers
What laboratory finding may indicate the need for further evaluation in a patient with a prolonged fever?
What laboratory finding may indicate the need for further evaluation in a patient with a prolonged fever?
In assessing coronary artery abnormalities through echocardiography, a Z-score of which value indicates a small aneurysm?
In assessing coronary artery abnormalities through echocardiography, a Z-score of which value indicates a small aneurysm?
Which of the following treatments is considered first-line therapy for Kawasaki Disease during the initial phase?
Which of the following treatments is considered first-line therapy for Kawasaki Disease during the initial phase?
When should aspirin therapy be discontinued in patients with Kawasaki Disease?
When should aspirin therapy be discontinued in patients with Kawasaki Disease?
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What is the follow-up recommendation for patients diagnosed with giant coronary artery aneurysms?
What is the follow-up recommendation for patients diagnosed with giant coronary artery aneurysms?
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Which of the following factors contributes the most to the long-term risk in patients with Kawasaki Disease?
Which of the following factors contributes the most to the long-term risk in patients with Kawasaki Disease?
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What can be used in patients who do not respond to IVIG treatment for Kawasaki Disease?
What can be used in patients who do not respond to IVIG treatment for Kawasaki Disease?
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What does a Z-score of less than 2 indicate in echocardiographic assessments of coronary arteries?
What does a Z-score of less than 2 indicate in echocardiographic assessments of coronary arteries?
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What is the primary demographic most affected by Kawasaki Disease?
What is the primary demographic most affected by Kawasaki Disease?
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What is a key characteristic of the acute febrile stage of Kawasaki Disease?
What is a key characteristic of the acute febrile stage of Kawasaki Disease?
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Which of the following is NOT one of the five clinical findings required for a Kawasaki Disease diagnosis?
Which of the following is NOT one of the five clinical findings required for a Kawasaki Disease diagnosis?
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What is a significant complication that can arise during the subacute stage of Kawasaki Disease?
What is a significant complication that can arise during the subacute stage of Kawasaki Disease?
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What is presumed to trigger Kawasaki Disease in genetically predisposed individuals?
What is presumed to trigger Kawasaki Disease in genetically predisposed individuals?
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Which of the following is a mistaken belief about the chronic stage of Kawasaki Disease?
Which of the following is a mistaken belief about the chronic stage of Kawasaki Disease?
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What is a common misunderstanding regarding the ethnic incidence of Kawasaki Disease?
What is a common misunderstanding regarding the ethnic incidence of Kawasaki Disease?
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What role do inflammatory cytokines play in Kawasaki Disease?
What role do inflammatory cytokines play in Kawasaki Disease?
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Study Notes
Kawasaki Disease
- Kawasaki Disease is a systemic vasculitis affecting small and medium-sized arteries, especially coronary arteries.
- It predominantly affects children between 1 and 5 years old with a higher incidence in males and those of Asian ethnicity.
- Japanese children have a 10-fold higher incidence compared to Caucasian children.
- Kawasaki Disease is the leading cause of acquired heart disease in children in developed countries.
Pathogenesis
- The exact cause of Kawasaki Disease is unknown, but it is thought to be triggered by an infectious agent in a genetically predisposed individual.
- The infection likely enters the bloodstream and then the tissues, leading to an aberrant immune response.
- The immune response involves a switch from B lymphocytes to IgA plasma cells, suggesting stimulation by an intracellular pathogen.
- Coronary artery aneurysm formation is attributed to inflammatory cytokines and enzymes released by infiltrating neutrophils and mononuclear cells, which destroy collagen and elastin fibers.
Clinical Manifestations
- Kawasaki Disease presents in stages:
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Acute Febrile Stage:
- Fever lasting at least 5 days, typically above 39°C.
- Conjunctivitis (non-exudative, bilateral) in over 90% of cases.
- Polymorphous rash, particularly on the groin and trunk.
- Edema or erythema of the hands and feet.
- Cervical lymphadenopathy (above 1.5 cm diameter, often unilateral).
- Mucosal involvement including strawberry tongue, dry, fissured lips, pharyngeal or lip mucosa injection.
- Myocarditis or pericarditis may also be present.
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Subacute Stage: Fever, rash, and lymphadenopathy resolve.
- Desquamation (peeling) of the skin occurs, particularly around the nails.
- Coronary artery aneurysms typically develop in this stage.
-
Convalescent Stage: Signs and symptoms of inflammation recede.
- Acute phase markers normalize.
- Coronary artery aneurysms may enlarge.
- Chronic Stage: Aneurysms may resolve, but sometimes persist into adulthood.
-
Acute Febrile Stage:
Diagnosis
- Kawasaki Disease is a clinical diagnosis requiring fever and at least 4 out of 5 of the following clinical findings:
- Conjunctivitis
- Rash
- Edema or erythema of hands and feet
- Cervical lymphadenopathy
- Mucosal involvement
- Incomplete or atypical Kawasaki Disease diagnosis is made if only 2 or 3 criteria are present.
- Laboratory values can aid in diagnosis:
- Anemia
- Platelet count above 450,000/µL after 7 days of fever
- Hypoalbuminemia
- Elevated alanine aminotransferase
- White blood cell count above 15,000/µL
- Urine with more than 10 white blood cells/high-power field.
- Echocardiography is the primary imaging modality to assess coronary artery abnormalities.
Echocardiographic Findings
- Z-scores are used to describe the diameter of the coronary artery relative to the average diameter in a child of the same body surface area.
- Positive for coronary abnormalities if:
- Z-score of 2.5 or more in the left anterior descending or right coronary arteries.
- Visible coronary artery aneurysm.
- Three or more suggestive features like decreased left ventricular function, mitral regurgitation, pericardial effusion, or a z-score of 2 to 2.5 in the left anterior descending or right coronary arteries.
- Z-score interpretation:
- Less than 2: Normal
- 2 to 2.5: Dilatation only
- 2.5 to 5: Small Aneurysm
- 5 to 10: Medium Aneurysm
- More than 10: Large or Giant Aneurysm
Treatment
- Intravenous immunoglobulins (IVIG) are the mainstay of treatment, indicated if:
- Patient is within 10 days of fever onset.
- Patient is beyond 10 days of fever with ongoing fever or elevated inflammatory markers.
- Aspirin is often used concurrently with IVIG, continued for at least 4 to 6 weeks or longer if the z-score hasn't returned to normal.
- Steroids or anti-TNF-alpha agents (e.g., infliximab) can be used in patients not responding to IVIG.
Follow-Up
- Follow-up depends on the z-score.
- Patients with a z-score below 2 at all times discontinue aspirin therapy after 4 to 6 weeks and undergo cardiovascular risk assessment at that time and again at one year.
- Patients with giant aneurysms receive:
- Aspirin therapy
- Anticoagulation medication (e.g., warfarin or low molecular weight heparin)
- Regular assessments throughout the first year and every 3-6 months thereafter
- Stress echocardiography every 6-12 months
- Consideration for angiography during the first year and every 1-5 years afterwards
- Avoidance of high-intensity activities
- Some patients may also require dual antiplatelet therapy (aspirin and clopidogrel).
- Beta-blockers may be initiated in some high-risk cases.
- Treatment is scaled down based on aneurysm regression.
Prognosis
- The overall prognosis for patients with Kawasaki Disease is excellent with treatment.
- While coronary artery aneurysms can be a significant complication leading to myocardial infarction and sudden cardiac death, most patients recover well.
- The development of coronary artery aneurysms is the greatest long-term risk factor.
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Description
This quiz covers Kawasaki Disease, a systemic vasculitis affecting children, particularly those aged 1 to 5 years. It explores the pathogenesis, with emphasis on the immune response and its impact on coronary arteries. Test your knowledge on the causes, incidence, and outcomes of this significant pediatric condition.
