Kawasaki Disease Complicated by Salmonella oranienburg Coinf
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A previously healthy, vaccinated 12-month-old male who was born full-term presented to the hospital with two and a half days of fever, rash, red eyes, and non-bloody diarrhea. The rash started behind the ears and then spread to his entire body. He was breast- and bottle-fed, with no history of prior hospitalizations or daycare exposures. He lived at home with his biological parents and three older male siblings. Upon presentation, the patient was febrile up to 102.6F, with a blood pressure of 110/77mmHg and heart rate of 174 beats/min.

Initial assessment revealed an interactive, well-appearing infant with an erythematous tongue, cracked lips, bilateral nonpurulent scleral injection with limbic sparing, hand and foot swelling, and diffuse polymorphous rash sparing the palms and soles. He was noted to have a 1.5cm mobile, non-tender anterior cervical lymph node. Head examination was normocephalic, atraumatic, and revealed a closed anterior fontanelle. Cardiovascular, respiratory, and abdominal examinations were unremarkable.

Initial laboratory data revealed an elevated CRP of 115mg/L, elevated ESR to 55 mm/hour, and no leukocytosis. Chemistry was pertinent for slightly increased ALT to 60 IU/L but normal alkaline phosphatase and AST. Respiratory viral panel PCR was positive for rhino/enterovirus, and his SARS CoV-2 PCR was negative. His presentation was concerning for evolving Kawasaki disease versus a viral illness, so he was admitted for monitoring of fever, as five days of fever along with his clinical findings would meet the diagnostic criteria for KD.

The patient continued to be febrile throughout the next two days with his signs of illness waxing and waning. Blood culture was obtained and was negative. A GI PCR panel was sent due to persistent diarrhea, which was positive for enteropathogenic E. coli and unspecified Salmonella species. A transthoracic echocardiogram was obtained on the fifth day of fever showing no evidence of coronary artery dilation. By the seventh day, his clinical exam was unchanged, and the echocardiogram was repeated due to persistent fever. The proximal right and left main coronary arteries were noted to be at the upper limit of normal with a Z-score of 1.9 and concern for the “beads-on-a-string” appearance of the left main coronary artery. The left anterior descending artery (LAD) was moderately dilated with a Z-score of 3.27, meeting the criteria for a small aneurysm. Treatment with 2g/kg IVIG and 80mg/kg/day of aspirin was started.

48 hours after IVIG, there was a recrudescence of the fever, suggesting resistance to IVIG treatment. This led to the administration of a second dose of IVIG on the ninth day of illness. After this second round of IVIG, a third echo showed that his coronary artery aneurysms had improved. At this time, his clinical symptoms also began improving. However, he continued to have daily fevers ranging from 38–39.5°C. On day 12, the blood culture that was drawn on the tenth day of the illness was noted to be positive for Gram-negative rods, and he was started on IV ceftriaxone. An ultrasound of the abdomen was obtained and found to be unremarkable. At this juncture, pediatric rheumatology was consulted for IVIG-resistant KD and recommended treatment with 8mg/kg/day of anakinra (an IL-1 receptor antagonist). Eventual state laboratory speciation revealed pan-sensitive Salmonella oranienburg. Three days after initiation of antibiotics, the fevers resolved, and repeat blood cultures were negative. He was treated with ceftriaxone for seven days and discharged with oral azithromycin for an additional five days. Upon follow-up, the patient was doing well without further complications or recurrence of symptoms. Follow-up echocardiography showed resolution of the coronary artery aneurysms.