WBR0011

Author	PageAuthor::Anonymous (edited by Ayokunle Olubaniyi)
Exam Type	ExamType::USMLE Step 2 CK
Main Category	MainCategory::Pediatrics
Sub Category	SubCategory::Allergy/Immunology
Prompt	[[Prompt::A 13-year-old boy is brought to the clinic for a gradually worsening fever of 10 days duration accompanied by a diffuse rash. His mother states that the fever has been as high as 103.0°F (39.4°C) and she has kept him out of school because of this. The physical exam is notable for a diffuse morbilliform rash over his trunk and extremities, which also becomes confluent in the groin. Both scleras 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 commonest and the most life-threatening complication of the disease in this patient?]]
Answer A	AnswerA::Myocarditis
Answer A Explanation	AnswerAExp::This is also a common complication of Kawasaki disease which usually responds well to intravenous immunoglobulin (IVIG) therapy. Other cardiac involvement include: cardiomyopathy and left ventricular dysfunction.
Answer B	AnswerB::Hearing loss
Answer B Explanation	AnswerBExp::Sensorineural hearing loss is a rare and transient complication. It responds promptly to intravenous immunoglobulin (IVIG) therapy.
Answer C	AnswerC::Interstitial nephritis
Answer C Explanation	AnswerCExp::Acute interstitial nephritis is a rare complication. Other urinary abnormalities associated with Kawasaki disease include: mild proteinuria, acute renal failure, hemolytic-uremic syndrome, and immune complex-mediated glomerulonephritis.
Answer D	AnswerD::Pneumonitis
Answer D Explanation	AnswerDExp::This is rare; it responds promptly to intravenous immunoglobulin (IVIG) therapy.
Answer E	AnswerE::Involvement of the coronary artery
Answer E Explanation	AnswerEExp::This is the most life-threatening complication.
Right Answer	RightAnswer::E
Explanation	[[Explanation::The patient in this vignette has a condition known as Kawasaki disease. Kawasaki disease is an acute febrile vasculitis affecting many systems, including the skin, nervous system, gastrointestinal tract, musculo-skeletal system, kidneys, lungs, eyes and the hematologic system. The most common and the most life-threatening complication of Kawasaki syndrome is aneurysms which is usually confined to the medium-sized vessels and classically those located in the heart. Coronary aneurysms may be small (<5 mm) or giant (>8 mm). The latter is associated with profound morbidity and mortality. High risk patients of coronary artery aneurysms include: Children without intravenous immunoglobulin (IVIG) within 10 days from the onset of fever Patients who have persistent fever despite IVIG treatment Patients with laboratory findings suggestive of persistent inflammation (increased ESR, C-reactive protein (CRP) or both); Young children (<6 months old) or older children (>8 years old) Male sex A careful diagnostic workup must be initiated upon identification of disease with follow-up exams 6-8 weeks after resolution of disease, coupled with a timely referral to a cardiologist. Educational Objective: Cardiac involvement, especially, the involvement of the coronary artery, leading to the development of coronary aneurysms is the most feared complication of Kawasaki disease. The likelihood of this complication reduces with a prompt initiation of intravenous immunoglobulin (IVIG) therapy within the first 10 days of onset of symptoms. References: Mori M, Imagawa T, Yasui K, et al. Predictors of coronary artery lesions after intravenous gamma-globulin treatment in Kawasaki disease. J Pediatr. 2000;137:177-180]]
Approved	Approved::No
Keyword	WBRKeyword::Kawasaki disease, WBRKeyword::complications of kawasaki disease
Linked Question	Linked::
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