A 3-year old male presents with 3 days of fever (maximal temperature, 105°F), diffuse abdominal pain, and several episodes of nonbilious, nonbloody emesis and loose nonbilious, nonmucousy stools. On day 3 of illness, he was seen at an urgent care clinic where he was diagnosed with acute otitis media and prescribed amoxicillin and ondansetron. He could not tolerate any oral intake and developed red eyes, abdominal pain, and redness of his hands and feet. Later that same night, he presented to the pediatric emergency department (ED) and was admitted to the pediatric ward for management of his fever, abdominal pain, and dehydration.
On examination, the child was notably unhappy and difficult to console. His eyes were crusted and injected bilaterally, and he had scleral icterus. His lips were erythematous and dry, and his tongue and tonsils were injected. He had no tonsillar enlargement or exudate.
His heart rate was increased, and he had a +1/6 systolic ejection murmur. His abdomen was diffusely tender to palpation, without rebound, guarding, organomegaly, or masses. His skin was covered with an erythematous macular papular rash on his entire body, and he showed moderate jaundice. He had no lymphadenopathy.
The boy’s labs were notable for a normal basic metabolic profile, but abnormal liver function testing included an alanine aminotransferase (ALT) of 249 and total bilirubin of 7.4 with a conjugated bilirubin level of 5.9. His total protein was increased at 6.3 but his albumin was low at 2.9. His C-reactive protein (CRP) was significantly elevated at 17.58. His clean-catch urinalysis showed small leukocytes and white blood cells of 50 to 100. His abdominal ultrasound was negative except for bladder wall thickening. Blood, stool, and urine cultures were sent due to his high fever.
Hospital course 1
The patient was resuscitated with intravenous (IV) fluids and started on IV piperacillin and tazobactam empirically for systemic inflammatory response syndrome (SIRS) in the setting of bacteremia or viremia. Given his high fever and gastrointestinal (GI) symptoms of abdominal pain and diarrhea, his differential diagnosis included bacteria (such as Shigella, Salmonella, Yersinia, Escherichia coli, Campylobacter) and viruses (adenovirus, enterovirus, and hepatitis A, B, and C). Syndromes that could explain his high fever, abdominal pain, rash, and ocular symptoms also included autoimmune and vasculitis diseases. The Table provides the working differential diagnosis. The Pediatric Infectious Disease service was consulted.
The boy continued to have fevers up to 104°F, abdominal pain, poor appetite, and multiple loose non-bloody stools. Repeat labs on day 2 of hospitalization revealed an increased CRP to 20.13 with slightly improved total bilirubin level of 6.9 and an even lower albumin level of 2.3. His initial stool testing was positive for enteropathogenic E coli (EPEC) by polymerase chain reaction (PCR), and he was continued on empiric piperacillin and tazobactam for presumed bacterial enteritis.
On day 5 of illness, (corresponding to hospital day 2), the patient’s CRP was decreased from admission but still elevated at 12.54. He met clinical criteria for the diagnosis of Kawasaki disease (KD) and was started on high-dose aspirin and IV immunoglobulin (IVIG). Given the concern for coronary artery dilatation with KD, an echocardiogram (ECHO) was performed but was negative for coronary artery changes.
After 1 dose of IVIG, he defervesced and had improvement in appetite and abdominal pain. His liver function tests showed steady improvement with a discharge total bilirubin level of 1.3. Urine and blood cultures remained sterile. Once he was afebrile for 48 hours, the antibiotics were discontinued, aspirin dose was decreased, and he was discharged home. A follow-up stool culture was negative, so the Pediatric Infectious Disease team determined that the E coli detected on the stool PCR study was a colonizing organism and not a true infection.
Hospital course 2
The day after discharge, the patient again began complaining of abdominal pain. He also developed a fever of 101°F and conjunctival injection, so his family returned to the ED. Laboratories at that time were remarkable for an elevated CRP (from 4.79 on his discharge day to 5.68) but stable liver function tests. He was given a second dose of IVIG and restarted on high-dose aspirin. A repeat ECHO showed a 3-mm dilatation of the left anterior descending artery (Figure).
The next day, his CRP trended down to 4.07. On the third day of this admission, his temperature increased to 100.3°F, so he was treated with a third dose of IVIG. He was observed in the hospital for an additional 72 hours.
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