Virtual Pediatric Patients
Donna M. D'Alessandro M.D., Tamra E. Takle M2
Peer Review Status: Internally Peer Reviewed
The Problem / Clinical Presentation
"A few days ago, I noticed Tanner's right jaw was a little bit swollen. I thought he might have a problem with his teeth, because the jaw seemed to be a little bit sore. He also seemed a little warm when I touched him, so I gave him some children's Tylenol and he seemed to feel a little bit better. The next day, I was at the hospital with my wife because she was in labor with our daughter who was just born. So Tanner spent the day with the babysitter. She thought he was a little bit cranky and a little bit warm too, so she gave him some more Tylenol. He hadn't been eating as much the last few days and he's seemed kind of tired."
"The following morning when we got up to go to the hospital, his neck was more swollen - this was about 10 AM. He also had a little rash on his bottom, but we thought it was maybe heat rash since he'd been playing outside. We went to see my wife and by lunchtime the rash had spread and he was getting pretty irritable. I took him to the emergency room and he had a fever. They gave him some antibiotics but that didn't seem to help. We saw a pediatrician who was worried about him having his swollen neck, fever, and rash. That's when we were sent to the University of Iowa."
Clinical Physical Exam
Tanner was alert and able to answer questions but was not extremely active. His temperature was 37 C, pulse 132, BP 98/45. His height was at the 75th percentile for age, and weight >95th percentile. HEENT examination revealed bilateral bright red injected conjunctiva. His nose had nasal crusting, he had dry erythematous, cracked lips, slightly enlarged tonsils that were erythematous and he had elevation of the tongue papillae.
His neck was supple but had a 4 cm right anterior cervical node which was tender. He had no other adenopathy. Heart exam showed a regular rate and rhythm. There was a normal S1 and S2 without murmur. His skin examination revealed an erythematous maculopapular confluent rash over his entire body, but especially in the upper extremities and groin area. Extremity examination revealed somewhat edematous hands and feet with no scratches or bites.
Clinical Differential Diagnosis
His CBC showed Hgb=13 g/dl, Hct=37%, platelet count=333 K/mm3, WBC=18.1 K/mm3 with 88% PMNs, 2% bands, and 5% lymphocytes. Urinalysis showed specific gravity=1.005, pH=6.0 and a positive leukocyte esterase. Microscopic urinalysis showed 15-19 WBC/hpf and no RBCs. A general screen showed normal electrolytes, BUN and Creatinine but a slightly elevated GGT, ALT and total bilirubin. A blood culture and urine culture showed no growth after 7 days. An EKG was normal.
Laboratory Differential Diagnosis
Elevated platelet count, sterile pyuria and elevated liver enzymes with a negative blood culture is consistent with Kawasaki disease.
Pediatric Cardiology's echocardiogram showed normal coronary arteries and normal atrial and ventricular function.
Imaging Differential Diagnosis
No operative intervention was performed
Treatment Course, Prognosis and Follow-up
Tanner was given intravenous immunoglobulin (IVIG), aspirin and will follow up with his local physician for a physical exam, CBC and differential. He will return to cardiology clinic in 4-6 weeks for a follow-up echocardiogram. He was also given anti-pyretics.
"Tanner's been a little quieter than usual, he doesn't like to talk much, which is not like Tanner. He's usually running around yelling. He's never been sick before except for the usual childhood things. He hasn't minded being in the hospital, but he's starting to get a little combative and tells everyone to 'go away'. We had to keep the room pretty dark because his eyes were bothering him. We were really scared when the doctors told us he had Kawasaki Disease and they didn't know what causes it, especially because it can cause heart problems. But Tanner's heart test was normal and the doctors said with the treatment he will probably be okay. He does have to take the aspirin and come back for another heart test though."
A fever is an elevation of body temperature above normal. The exact elevation is debated but generally is accepted as greater than 100.5 F or 38.0 C.
Fever is a common complaint and can be challenging because of the emotional attributes given this symptom. Families become upset if the fever is "high" or has occurred "for a long time," both of which are specific for an individual. Fevers that persist beyond a few days often produce emotional responses from caregivers.
The potential implications of the fever depend on the child's age and presenting symptoms. A serious bacterial infection or occult bacteremia is always a concern and is more likely in a young infant or child and with "high" fevers (>103.0 F or 39.5 C). The differential diagnosis is exceedingly long as almost any disease process may have fever associated with it. These are some of the more "common" causes:
If rash is a prominent symptom also consider:
History and Physical
History should include time of day, height of fever, duration, and associated symptoms such as cough, rhinorrhea, pain, emesis, diarrhea, rashes and irritability or other behavioral changes. It should also include an exposure history including contacts with ill persons, medications, travel history and occupational or leisure activities of the patient and family including animals. Family history should include any rheumatologic and gastrointestinal problems.
Physical examination should document the fever and proceed with a careful examination for subtle signs of underlying infection, and other disease processes such as skin changes, lymphadenopathy, organomegaly, and changes in cardiac and respiratory status.
The laboratory evaluation needs to be guided by history, physical examination and differential diagnosis. Initial evaluation may include:
Consider Infectious Disease
Most children have a self-limited, localized infectious disease process such as otitis media or upper respiratory tract infection. These should be treated appropriately. Suspected meningitis needs to be evaluated quickly and antibiotics begun as soon as possible. Guidelines exist for the evaluation and treatment of children less than 36 months without localizing signs. Empiric antibiotics should be considered for these children because of the risk of occult bacteremia. If the fever is the heralding sign of a systemic disease such as juvenile arthritis or malignancy, treatment should be appropriately initiated.
Kawasaki disease was first described by Japanese allergist Dr. Tomisaku Kawasaki in 1967.
Presentation in the acute febrile period (the first 7-14 days of the illness) may include the following symptoms: fever, conjunctival injection, mouth and lip changes, swelling and erythema of the hands and feet, rash, lymphadenopathy, aseptic meningitis, diarrhea or hepatic dysfunction. Symptoms in the subacute phase (days 10-24 of the illness) may include irritability, anorexia, continued conjunctival injection and peeling of the fingers.
The American Heart Association has published information concerning the diagnostic criteria for Kawasaki Disease (1990), therapeutic recommendations (1993) and long term follow-up of patients (1994). Diagnostic criteria for Kawasaki Disease in the U.S. include:
Extreme irritability is often associated with this disease.
It is the leading cause of acquired heart disease in the United States. Eighty percent of patients are under age 5, and there is a male-to-female ratio of approximately 1.5: 1. The etiology is unknown. An infectious cause has been suggested, but no consistent causative agent has been found.
Common findings associated with Kawasaki Disease are leukocytosis with neutrophils and elevation of the erythrocyte sedimentation rate (ESR). The platelet count may elevate in the second week. A sterile pyuria may also be seen.
The echocardiogram is the primary tool for recognition and follow-up of coronary artery aneurysms. Coronary artery aneurysms occur mainly in the proximal portion of the coronary artery. Echocardiography can be up to 100% sensitive in detection of coronary artery aneurysms. Giant aneurysms are 8 mm or greater in diameter and impact on long term prognosis. These aneurysms may calcify, narrow the entry into the aneurysm and stenosis may result. Spontaneous resolution of giant aneurysms without complications is extremely rare.
During the acute phase, Kawasaki Disease causes medium and large vessel arteritis. Therefore, baseline echocardiography is needed. Kawasaki Disease causes arterial aneurysms (not limited to the coronary arteries) valvulitis, and myocarditis. Of particular concern are the coronary artery aneurysms which may predispose to stasis of blood, subsequent thrombosis, and overtime potential evolution into segmental thrombosis in the chronic phase. Coronary artery aneurysms are so unique to Kawasaki that when they are identified, strict adherence to fulfilling 4 or 5 of the diagnostic criteria is not done.
Acute management of Kawasaki Disease includes the use of IVIG and anticoagulants such as aspirin and/or Coumadin.
The American Heart Association committee guidelines for long term follow-up and management are based upon the degree of coronary artery involvement. Management includes anticoagulants, especially anti-platelet medications such as aspirin. It is recommended to discontinue the use of aspirin with influenza illness and with Varicella vaccine administration because of the risk of Reye's Syndrome. Influenza Vaccine should also be considered for these patients. Testing may include repeated echocardiography, electrocardiogram, stress testing and angiography.
Intravenous gammaglobulin (IVIG) given before the 10 day of the illness has reduced the morbidity of Kawasaki Disease and the coronary artery aneurysms from 25% to 5% (6-8 weeks after the initiation of therapy). In most patients, coronary artery aneurysms regress within two years of the illness. Approximately 1% of patients who recover from Kawasaki Disease or who develop giant aneurysms go on to have coronary artery destruction. A rare complication is peripheral ischemia, which can cause gangrene.
Shulman ST, De Inocerio J, Hirsch R. Kawasaki Disease. Pediatric Clinics of North America 1995: 42(5), 1205-1222.
Tolan, RW, Kleiman, MB. When Fever Won't Go Away. Contemporary Pediatrics. 1992. pp. 25-54.
Berkowitz, Carol D, Pediatrics A Primary Care Approach. W.B. Saunders and Co. Philadelphia, PA, 1996. pp. 127-132.
Jaskiewicz, JA. et al. Febrile infants at low risk for serious bacterial infection-an appraisal of the Rochester Criteria and implications for management. Pediatrics 1994. 94: 390-396.
Baraff LJ. Bass JW. Fleisher GR. Klein JO. McCracken GH Jr. Powell KR. Schriger DL. Practice guideline for the management of infants and children 0 to 36 months of age with fever without source. Annals of Emergency Medicine. 1993. 22(7): 1198-210.
. Anonymous. Guidelines for long-term management of patients with
Kawasaki disease. Committee on Rheumatic Fever, Endocarditis, and
Kawasaki Disease, Council on Cardiovascular Disease in the Young,
American Heart Association. Heart Disease & Stroke. 1994.
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