eMedicine Specialties > Emergency Medicine > Pediatric

Pediatrics, Kawasaki Disease: Differential Diagnoses & Workup

Author: Steven J Parrillo, DO, FACOEP, FACEP, Associate Professor, Emergency Medicine, Jefferson Medical College and Philadelphia College of Osteopathic Medicine; Medical Director, Department of Emergency Medicine, Einstein Elkins Park; Chair, Emergency Management Committee, Albert Einstein Healthcare Network; Medical Director, Disaster Medicine and Management Masters Program, Philadelphia University
Coauthor(s): Catherine V Parrillo, DO, FACOP, FAAP, Clinical Assistant Professor, Department of Pediatrics, Philadelphia College of Osteopathic Medicine
Contributor Information and Disclosures

Updated: Oct 28, 2009

Differential Diagnoses

Leptospirosis
Tick-Borne Diseases, Rocky Mountain Spotted Fever
Pediatrics, Bacteremia and Sepsis
Toxic Epidermal Necrolysis
Pediatrics, Fever
Toxic Shock Syndrome
Pediatrics, Meningitis and Encephalitis
Toxicity, Mercury
Pediatrics, Pharyngitis
Scarlet Fever
Staphylococcal Scalded Skin Syndrome

Workup

Laboratory Studies

  • No specific laboratory test exists for Kawasaki disease; however, certain abnormalities coincide with various stages.
  • A mild-to-moderate normochromic anemia is observed in the acute stage along with a moderate to alarmingly elevated WBC count with a left shift. 
    • Many of the acute-phase reactant markers, such as the ESR, CRP level, and serum alpha1-antitrypsin level are elevated. Most authors mention only ESR and CRP.
    • Culture results are all negative.
  • During the subacute stage, platelet count elevation is the outstanding marker.
    • It begins to rise in the second week and continues to rise during the third week.
    • Levels as high as 2 million have been observed.
    • The acute reactive markers remain elevated.
  • In the convalescent stage, the levels of platelets and other markers begin to return to values within the reference range. Laboratory values may require 6-8 weeks to normalize.
  • Liver function studies and serum lipase measurement may be indicated in selected cases.

Imaging Studies

  • An echocardiogram is the study of choice in Kawasaki disease to demonstrate coronary artery aneurysms in both fully manifested and suspected incomplete cases.
  • Coronary angiography or percutaneous coronary intervention (PCI) may be required in some with coronary artery aneurysms (CAA).
  • During the acute stage, a baseline echocardiogram is important.
    • The echocardiogram should be repeated in the second or third week and again 1 month after all other laboratory results have normalized.
    • Many centers perform a 1-year echocardiogram, even when the first ones show no aneurysm.
    • If the echocardiogram results are abnormal at any point, the child should be referred to a pediatric cardiologist for a complete cardiac workup and follow-up care.
  • Ultrasonography of the gallbladder may be necessary if any suggestion of liver or gallbladder dysfunction is present.
  • A chest radiograph should be obtained to assess baseline findings and to confirm clinical suspicion of congestive heart failure.

Other Tests

  • An electrocardiogram (ECG) indicates the presence of various conduction abnormalities. Additionally, children with Kawasaki syndrome may suffer acute infarction.
  • Exercise stress testing may play a role later in life.1
  • One study used multislice spiral CT to assess coronary artery abnormalities in 16 adolescents and young adults with Kawasaki disease. Although the numbers were small, CT was 100% sensitive in the detection of coronary artery aneurysms but only 87.5% sensitive for the detection of significant stenosis or occlusion. False-positive results occurred secondary to severe calcification in 5 arteries and cardiac motion artifact in 2. Specificity was therefore 92.5%.17
  • In another small study, electron beam computed tomography (EBCT) was used to determine if coronary artery calcifications could be used as a marker of future coronary artery events. The authors felt that this study may be useful for risk stratification in long-term management of patients with Kawasaki disease.18

Procedures

  • A select group may require cardiac catheterization.
  • Coronary artery bypass grafting may be required.

More on Pediatrics, Kawasaki Disease

Overview: Pediatrics, Kawasaki Disease
Differential Diagnoses & Workup: Pediatrics, Kawasaki Disease
Treatment & Medication: Pediatrics, Kawasaki Disease
Follow-up: Pediatrics, Kawasaki Disease
Multimedia: Pediatrics, Kawasaki Disease
References
[ CLOSE WINDOW ]

References

  1. Pinna GS, Kafetzis DA, Tselkas OI, Skevaki CL. Kawasaki disease: an overview. Curr Opin Infect Dis. Jun 2008;21(3):263-70. [Medline].

  2. Caquard M, Parlier G, Siret D. [Family observation of Kawasaki disease: 2 cases in sister and brother]. Arch Pediatr. May 2006;13(5):453-5. [Medline].

  3. Burns JC, Shimizu C, Gonzalez E, Kulkarni H, Patel S, Shike H. Genetic variations in the receptor-ligand pair CCR5 and CCL3L1 are important determinants of susceptibility to Kawasaki disease. J Infect Dis. Jul 15 2005;192(2):344-9. [Medline].

  4. Treadwell TA, Maddox RA, Holman RC, Belay ED, Shahriari A, Anderson MS, et al. Investigation of Kawasaki syndrome risk factors in Colorado. Pediatr Infect Dis J. Oct 2002;21(10):976-8. [Medline].

  5. Burns JC, Glodé MP. Kawasaki syndrome. Lancet. Aug 7-13 2004;364(9433):533-44. [Medline].

  6. Newburger JW, Fulton DR. Kawasaki disease. Curr Opin Pediatr. Oct 2004;16(5):508-14. [Medline].

  7. Yeo Y, Kim T, Ha K, Jang G, Lee J, Lee K. Incomplete Kawasaki disease in patients younger than 1 year of age: a possible inherent risk factor. Eur J Pediatr. Feb 2009;168(2):157-62. [Medline].

  8. Kim T, Choi W, Woo CW, Choi B, Lee J, Lee K. Predictive risk factors for coronary artery abnormalities in Kawasaki disease. Eur J Pediatr. May 2007;166(5):421-5. [Medline].

  9. Kitamura S, Tsuda E, Kobayashi J, Nakajima H, Yoshikawa Y, Yagihara T. Twenty-five-year outcome of pediatric coronary artery bypass surgery for Kawasaki disease. Circulation. Jul 7 2009;120(1):60-8. [Medline].

  10. Pannaraj PS, Turner CL, Bastian JF, Burns JC. Failure to diagnose Kawasaki disease at the extremes of the pediatric age range. Pediatr Infect Dis J. Aug 2004;23(8):789-91. [Medline].

  11. Manlhiot C, Yeung RS, Clarizia NA, Chahal N, McCrindle BW. Kawasaki Disease at the Extremes of the Age Spectrum. Pediatrics. Aug 24 2009;[Medline].

  12. [Guideline] Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation. Oct 26 2004;110(17):2747-71. [Medline].

  13. Hinze CH, Graham TB, Sutherell JS. Kawasaki disease without fever. Pediatr Infect Dis J. Sep 4 2009;[Medline].

  14. Mine K, Takaya J, Hasui M, et al. A case of Kawasaki disease associated with syndrome of inapproriate secretion of antidiuretic hormone. Acta Paediatr. 2003;22:663-66.

  15. Hua W, Izurieta HS, Slade B, Belay ED, Haber P, Tiernan R, et al. Kawasaki Disease After Vaccination: Reports to the Vaccine Adverse Event Reporting System 1990-2007. Pediatr Infect Dis J. Sep 12 2009;[Medline].

  16. Miron D, Fink D, Hashkes PJ. Kawasaki disease in an infant following immunisation with hepatitis B vaccine. Clin Rheumatol. Dec 2003;22(6):461-3. [Medline].

  17. Kanamaru H, Sato Y, Takayama T, Ayusawa M, Karasawa K, Sumitomo N, et al. Assessment of coronary artery abnormalities by multislice spiral computed tomography in adolescents and young adults with Kawasaki disease. Am J Cardiol. Feb 15 2005;95(4):522-5. [Medline].

  18. Dadlani GH, Gingell RL, Orie JD, Roland JM, Najdzionek J, Lipsitz SR, et al. Coronary artery calcifications in the long-term follow-up of Kawasaki disease. Am Heart J. Nov 2005;150(5):1016. [Medline].

  19. Baumer JH, Love SJ, Gupta A, Haines LC, Maconochie I, Dua JS. Salicylate for the treatment of Kawasaki disease in children. Cochrane Database Syst Rev. 2006;(4):CD004175. [Medline].

  20. Krendel S, Pollack P, Hanly J. Tissue plasminogen activator in pediatric myocardial infarction. Ann Emerg Med. May 2000;35(5):502-5. [Medline].

  21. [Best Evidence] Inoue Y, Okada Y, Shinohara M, Kobayashi T, Kobayashi T, Tomomasa T, et al. A multicenter prospective randomized trial of corticosteroids in primary therapy for Kawasaki disease: clinical course and coronary artery outcome. J Pediatr. Sep 2006;149(3):336-341. [Medline].

  22. Shulman ST. Is there a role for corticosteroids in Kawasaki disease?. J Pediatr. Jun 2003;142(6):601-3. [Medline].

  23. Athappan G, Gale S, Ponniah T. Corticosteroid therapy for primary treatment of Kawasaki disease - weight of evidence: a meta-analysis and systematic review of the literature. Cardiovasc J Afr. Jul-Aug 2009;20(4):233-6. [Medline].

  24. Burns JC, Mason WH, Hauger SB, Janai H, Bastian JF, Wohrley JD, et al. Infliximab treatment for refractory Kawasaki syndrome. J Pediatr. May 2005;146(5):662-7. [Medline].

  25. Burns JC. Revisiting steroids in the primary treatment of acute Kawasaki disease. J Pediatr. Sep 2006;149(3):291-2. [Medline].

  26. Burns JC. The riddle of Kawasaki disease. N Engl J Med. Feb 15 2007;356(7):659-61. [Medline].

  27. Chantepie A, Mauran P, Lusson JR, Vaillant MC, Bozio A. [Cardiovascular complications of Kawasaki syndrome: results of a French multicenter study]. Arch Pediatr. Jul 2001;8(7):713-9. [Medline].

  28. Daniels SR. Steroid treatment for Kawasaki disease. J Pediatrics. Sept 2006;149 (3):336.

  29. Morgan GJ, MacLeod C, Jenkins J, Stewart C, Craig B. IVIG, aspirin, and Kawasaki disease. J Pediatr. Aug 2003;143(2):280-1; author reply 281. [Medline].

  30. Nomura Y, Masuda K, Yoshinaga M, Takei S, Miyata K. Possible relationship between streptococcal pyrogenic exotoxin A and Kawasaki syndrome in patients older than six months of age. Pediatr Infect Dis J. Sep 2003;22(9):794-8. [Medline].

  31. Rowley AH, Shulman ST. Kawasaki syndrome. Pediatr Clin North Am. Apr 1999;46(2):313-29. [Medline].

  32. Saulsbury FT. Comparison of high-dose and low-dose aspirin plus intravenous immunoglobulin in the treatment of Kawasaki syndrome. Clin Pediatr (Phila). Oct 2002;41(8):597-601. [Medline].

  33. Schratz LM, Meyer RA, Schwartz DC. Serial intracoronary ultrasound in children: feasibility, reproducibility, limitations, and safety. J Am Soc Echocardiogr. Aug 2002;15(8):782-90. [Medline].

  34. Selamet Tierney ES, Newburger JW. Are patients with Kawasaki disease at risk for premature atherosclerosis?. J Pediatr. Sep 2007;151(3):225-8. [Medline].

  35. Sundel RP, Baker AL, Fulton DR, Newburger JW. Corticosteroids in the initial treatment of Kawasaki disease: report of a randomized trial. J Pediatr. Jun 2003;142(6):611-6. [Medline].

  36. Weinstein M. Inflammation at a previous inoculation site: an unusual presentation of Kawasaki disease. CMAJ. Feb 14 2006;174(4):459-60. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

Kawasaki disease, Kawasaki disease symptoms, Kawasaki disease treatment, Kawasaki's disease, Kawasaki disease in children, incomplete Kawasaki disease, Kawasaki syndrome, myocardial infarction, myocarditis, acute vasculitic syndrome, coronary artery aneurysms, sudden death

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Steven J Parrillo, DO, FACOEP, FACEP, Associate Professor, Emergency Medicine, Jefferson Medical College and Philadelphia College of Osteopathic Medicine; Medical Director, Department of Emergency Medicine, Einstein Elkins Park; Chair, Emergency Management Committee, Albert Einstein Healthcare Network; Medical Director, Disaster Medicine and Management Masters Program, Philadelphia University
Steven J Parrillo, DO, FACOEP, FACEP is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, American Osteopathic Association, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Catherine V Parrillo, DO, FACOP, FAAP, Clinical Assistant Professor, Department of Pediatrics, Philadelphia College of Osteopathic Medicine
Catherine V Parrillo, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey Glenn Bowman, MD, MS, Consulting Staff, Highfield MRI, Columbus, Ohio
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Grace M Young, MD, Associate Professor, Department of Pediatrics, University of Maryland Medical Center
Grace M Young, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Emergency Physicians
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Richard G Bachur, MD, Associate Professor of Pediatrics, Harvard Medical School; Associate Chief and Fellowship Director, Attending Physician, Division of Emergency Medicine, Children's Hospital of Boston
Richard G Bachur, MD is a member of the following medical societies: American Academy of Pediatrics, Society for Academic Emergency Medicine, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.