Vignette says a 5 year old female child presents to the emergency department with history of high grade fever and red eyes for 6 days; Examination shows red, cracked tongue, red lips, and a faint maculopapular rash initially on the trunk that has now spread to her limbs; Her palms and soles are red and swollen, and she has enlarged bilateral cervical lymph nodes; Laboratory studies show elevated inflammatory markers (i.e. elevated CRP and ESR); Echocardiography shows coronary artery aneurysms; Diagnosis?
Diagnosis is Kawasaki disease.
Kawasaki disease (aka mucocutaneous lymph node syndrome) is an acute, self-limited medium vessel vasculitis of an unknown etiology commonly affecting children.
Fever for at least 5 days with any 4 out of the 5 “CRASH” features.
Conjunctivitis
Rash (polymorphous, non vesicular on trunk and extremities)
Adenopathy (i.e. bilateral non-suppurative cervical lymphadenopathy)
Strawberry tongue
Hands/Feet edema and erythema
Conjunctivitis
Rash (polymorphous, non vesicular on trunk and extremities)
Adenopathy (i.e. bilateral non-suppurative cervical lymphadenopathy)
Strawberry tongue
Hands/Feet edema and erythema
Diagnosis:-
1. CBC shows leukocytosis.
2. Inflammatory markers (e.g. CRP, ESR) are elevated.
3. Echocardiography shows coronary artery aneurysms.
1. CBC shows leukocytosis.
2. Inflammatory markers (e.g. CRP, ESR) are elevated.
3. Echocardiography shows coronary artery aneurysms.
Treatment:- Aspirin + IVIG is the mainstay of treatment and should be initiated within the first 10 days of fever onset; IVIG is given at 2 g/kg in a single infusion and high dose aspirin is given at 80–100 mg/kg/day during the acute phase of the disease; The dose is gradually reduced to 3–5 mg/kg/day when the patient has been afebrile for over 48-72 hours.
Complication:- Coronary artery aneurysms and Myocardial infarction.
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