By Elizabeth Thorpe, MD
Pediatric Emergency Medicine Attending
A 3 yo male presents with 6 days of fever > 101.5, red eyes, cervical lymphadenopathy, a red tongue, cracked lips, macular rash on the back and trunk and fussiness. His po intake has been poor. No vomiting, no diarrhea, no cough. Based on the history and the child’s clinical appearance (see picture below), you are suspicious of specific diagnosis. What lab profile would confirm your diagnosis?
Pediatric Emergency Medicine Attending
A 3 yo male presents with 6 days of fever > 101.5, red eyes, cervical lymphadenopathy, a red tongue, cracked lips, macular rash on the back and trunk and fussiness. His po intake has been poor. No vomiting, no diarrhea, no cough. Based on the history and the child’s clinical appearance (see picture below), you are suspicious of specific diagnosis. What lab profile would confirm your diagnosis?
Image freely available online from the American College of Osteopathic Dermatologists at: http://www.aocd.org/?page=KawasakisDisease
ANSWER
1. This patient has Kawasaki disease (KD). KD is one of the most common vasculitides of childhood and occurs rarely in adults. It is typically a self-limited condition. However, complications such as coronary artery aneurysms, depressed myocardial contractility and heart failure, myocardial infarction, and arrhythmias may develop and lead to significant morbidity and mortality making timely diagnosis and treatment paramount.
The diagnosis of KD requires the presence of fever for > 5 days without any other explanation (i.e. AOM, PNA, UTI) combined with at least 4 of the following 5 criteria:
If the patient meets the clinical characteristics, laboratory tests are sent to confirm the diagnosis. Expected laboratory findings are
Morbidity and morality are greatly decreased with timely use of IVIG and high dose aspirin therapy. Patients should be admitted to the hospital and have either cardiology or infectious disease consultations. Timely echocardiogram to diagnose the presence of cardiac abnormalities is important.
PEARL: Put KD on your differential for any child with fever > 5 days and no other explanation.
ARTICLE: This is a good review article in Lancet
Kawasaki syndrome.
Burns JC, Glodé MP
Lancet. 2004;364(9433):533.
Kawasaki syndrome is an acute, self-limited vasculitis that occurs in children of all ages and presents a challenge for the clinician: the disorder can be difficult to recognize; there is no diagnostic laboratory test; there is an extremely effective therapy; and there is a 25% chance of serious cardiovascular damage if the treatment is not given early in the course of the disease. This review includes discussion of the history of the syndrome, the diagnostic challenges, epidemiology, etiology, pathology, immunopathogenesis, therapy, genetic influences, and the long-term cardiovascular sequelae.
1. This patient has Kawasaki disease (KD). KD is one of the most common vasculitides of childhood and occurs rarely in adults. It is typically a self-limited condition. However, complications such as coronary artery aneurysms, depressed myocardial contractility and heart failure, myocardial infarction, and arrhythmias may develop and lead to significant morbidity and mortality making timely diagnosis and treatment paramount.
The diagnosis of KD requires the presence of fever for > 5 days without any other explanation (i.e. AOM, PNA, UTI) combined with at least 4 of the following 5 criteria:
- Bilateral conjunctival injection (non-exudative)
- Oral mucous membrane changes, red tongue
- Peripheral extremity changes – edema or erythema in acute phase and desquamation in convalescent phase
- Polymorphous rash
- Cervical LAN with at least one node > 1.5cm
If the patient meets the clinical characteristics, laboratory tests are sent to confirm the diagnosis. Expected laboratory findings are
- Signs of inflammation and elevated acute phase reactant: elevated CRP, ESR and Platelets
- Sterile pyuria
- Mild transaminitis
- Elevated triglycerides
- Hyponatremia
Morbidity and morality are greatly decreased with timely use of IVIG and high dose aspirin therapy. Patients should be admitted to the hospital and have either cardiology or infectious disease consultations. Timely echocardiogram to diagnose the presence of cardiac abnormalities is important.
PEARL: Put KD on your differential for any child with fever > 5 days and no other explanation.
ARTICLE: This is a good review article in Lancet
Kawasaki syndrome.
Burns JC, Glodé MP
Lancet. 2004;364(9433):533.
Kawasaki syndrome is an acute, self-limited vasculitis that occurs in children of all ages and presents a challenge for the clinician: the disorder can be difficult to recognize; there is no diagnostic laboratory test; there is an extremely effective therapy; and there is a 25% chance of serious cardiovascular damage if the treatment is not given early in the course of the disease. This review includes discussion of the history of the syndrome, the diagnostic challenges, epidemiology, etiology, pathology, immunopathogenesis, therapy, genetic influences, and the long-term cardiovascular sequelae.
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