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Author Anonymous (Edited by Ayokunle Olubaniyi and Alison Leibowitz)
Exam Type USMLE Step 2 CK
Main Category Pediatrics
Sub Category Allergy/Immunology
Prompt A 13-year-old boy is brought to the clinic with a fever of 5 days duration accompanied by a diffuse rash. His mother states that the fever has been as high as 103.0°F (39.4°C) leading her to keep him home from school. Observed upon physical examination is a diffuse morbilliform rash over his trunk and extremities, which also becomes confluent in the groin. Both sclerae are infected and his tongue is red and bumpy on appearance. Notable lymphadenopathy is present in the anterior and posterior cervical chains.

What is the next step in management of this patient?

Answer A High-dose aspirin
Answer A Explanation [[AnswerAExp::Aspirin therapy is started at high doses until the fever subsides, and then it is continued at a low dose when the patient returns home. The use of aspirin is generally contraindicated in children due to its association with Reye's syndrome.]]
Answer B [[AnswerB::High-dose acetaminophen]]
Answer B Explanation [[AnswerBExp::This is not indicated in the management of Kawasaki disease.]]
Answer C Obtain complete blood count (CBC) and erythrocyte sedimentation rate (ESR)
Answer C Explanation The diagnosis of Kawasaki disease is mainly clinical. Although investigations such as CBC, ESR, C-reactive protein, and liver function tests may aid in the diagnosis, the initiation of high dose aspirin therapy is paramount whenever there is a high suspicion of the disease based on symptoms and physical examination.
Answer D Admit to the hospital and start IV steroids.
Answer D Explanation [[AnswerDExp::IV corticosteroids have also been used, especially when other treatments fail or symptoms recur, but it is still uncertain whether the addition of steroids to the combination of IVIG and aspirin improves clinical outcome.]]
Answer E [[AnswerE::Start a course of amoxicillin and arrange follow-up in 2 days.]]
Answer E Explanation [[AnswerEExp::The use of antibiotics is not indicated for Kawasaki disease.]]
Right Answer A
Explanation [[Explanation::The patient described in this vignette has Kawasaki disease, also known as lymph node syndrome, mucocutaneous node disease, infantile polyarteritis and Kawasaki syndrome. It is a poorly understood self-limited vasculitis that affects many organs, including the skin and mucous membranes, lymph nodes, blood vessel walls, and the heart. It is characterized by fever of 5 or more days duration, together with the presence of at least 4 of the following 5 clinical signs:
  • Rash
  • Cervical lymphadenopathy (at least 1.5 cm in diameter)
  • Bilateral conjuctival injection
  • Oral mucosal changes
  • Peripheral extremity changes.

Treatment of Kawasaki disease consists of high-dose aspirin with possible addition of intravenous immunoglobulin (IVIG) as well as admission to the hospital for diagnostic workup, including electrocardiogram/echocardiogram and management of any complications such as coronary aneurysm, e.t.c
Educational Objective: Kawasaki disease is a form of vasculitis with multisystemic involvement, usually affecting the skin and mucous membranes, lymph nodes, blood vessel walls, and the heart. Intravenous immunoglobulin ([IVIG) is the standard treatment for Kawasaki disease and is administered in high doses with marked improvement usually noted within 24 hours. Aspirin therapy is started at high doses until the fever subsides.
References: http://www.wikidoc.org/index.php/Kawasaki_disease]]

Approved No
Keyword Kawasaki disease, kasasaki syndrome, Vasculitis, mucocutaneous node disease, high dose aspirin
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