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    Suspicious fever of unknown origin

     

    Treatment of KD

    High dose IVIG (2 g/kg/d) remains the cornerstone in treating KD, and although IVIG treatment decreases the risk of CCAs from 25% to 5%,3,5,6,7 there is no evidence that acetylsalicylic acid (ASA) decreases this risk.3,4 The AHA guidelines state that medium dose ASA (30-50 mg/kg/d) can be used instead of the traditionally used high dose of 80-100 mg/kg/d as there is no evidence to suggest either dose is more effective.3 Medium to high-dose ASA usually is administered 4 times a day until the patient is afebrile then lowered to antiplatelet low-dose ASA of 3-5 mg/kg once daily.

    Other therapies include steroids, infliximab, and cyclosporine, but these usually are used in refractory cases and not routinely. Consultation with KD experts is always recommended in atypical or refractory cases.3,4,7 (See Table 4: Common pitfalls in diagnosing Kawasaki disease.)

    Patient outcome

    The patient was diagnosed with incomplete KD and received 1 dose of IVIG (2 g/kg) on day 8 of illness. His fever rapidly defervesced within 12 hours. He was started on high-dose aspirin (80 mg/kg/d). An echocardiogram showed normal cardiac structure and function with no coronary artery dilatations or aneurysms. The patient’s irritability resolved, and he was discharged home after 48 hours of being afebrile to follow up with Cardiology as an outpatient.

    A follow-up echocardiogram 6 weeks after discharge revealed ectasia of the left main coronary artery, which confirmed the diagnosis of KD. No aneurysms were seen. The patient was maintained on daily aspirin and he continued to follow up with the Cardiology clinic. Of note, the patient in this case never developed oral changes, conjunctivitis, extremities changes (including skin peeling), or cervical lymphadenopathy.

    Conclusion

    Kawasaki disease in infants aged younger than 6 months requires a high index of suspicion for diagnosis as it can present only with prolonged fever and irritably without any other clinical features. This age group is at higher risk of delayed diagnosis and late treatment resulting in higher risk of CAAs. Intravenous immunoglobulin is an effective treatment and should be administered within 10 days of fever onset. A medium dose of ASA can be used instead of the high dose.

    Next: Ears, nose, and throat, oh my!

    Acknowledgement: The authors would like to thank Paige Triplett, DO, for her final review of this manuscript.

    REFERENCES

    1. Arora R, Mahajan P. Evaluation of child with fever without source: review of literature and update. Pediatr Clin North Am. 2013;60(5):1049-1062.

    2. Antoon JW, Potisek NM, Lohr JA. Pediatric fever of unknown origin. Pediatr Rev. 2015;36(9):380-390.

    3. McCrindle BW, Rowley AH, Newburger JW, et al; American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young; Council on Cardiovascular and Stroke Nursing; Council on Cardiovascular Surgery and Anesthesia; Council on Epidemiology and Prevention. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation. 2017;135(17):e927-e999.

    4. Dietz SM, van Stijn D, Burgner D, et al. Dissecting Kawasaki disease: a state-of-the-art review. Eur J Pediatr. 2017;176(8):995-1009.

    5. Yeom JS, Woo HO, Park JS, Park ES, Seo JH, Youn HS. Kawasaki disease in infants. Korean J Pediatr. 2013;56(9):377-382.

    6. Minich LL, Sleeper LA, Atz AM, et al; Pediatric Heart Network Investigators. Delayed diagnosis of Kawasaki disease: what are the risk factors? Pediatrics. 2007;120(6):e1434-e1440.

    7. Son MB, Newburger JW. Kawasaki disease. Pediatr Rev. 2013;34(4):151-162.

    8. Kawasaki T. Pediatric acute febrile mucocutaneous lymph node syndrome with characteristic desquamation of fingers and toes: my clinical observation of fifty cases. Pediatr Infect Dis J. 2002;21:1-38.

    9. Yoon YM, Yun HW, Kim SH. Clinical characteristics of Kawasaki disease in infants younger than six months: a single-center study. Korean Circ J. 2016;46(4):550-555.

    10. Watanabe T, Abe Y, Sato S, Uehara Y, Ikeno K, Abe T. Sterile pyuria in patients with Kawasaki disease originates from both the urethra and the kidney. Pediatr Nephrol. 2007;22(7):987-991.

    11. Kim DS. Kawasaki disease. Yonsei Med J. 2006;47(6):759-772.

    Lauren Coogle, MD
    Dr Coogle is a pediatric resident, Wright State University, Dayton Children’s Hospital, Dayton, Ohio.
    Shafee Salloum, MD, FAAP
    Dr Salloum is assistant professor of Pediatrics, Boonshoft School of Medicine, Wright State University, and Dayton Children’s Hospital, ...

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