Jain S, Recognition of Kawasaki disease. *—In the absence of gold standard for diagnosis, this algorithm cannot be evidence based but rather represents the informed opinion of the expert committee. Salicylate for the treatment of Kawasaki disease in children. Sohn S. Huang SK, Oharaseki T, et al. The extent of the coronary artery involvement is the critical factor that determines morbidity and mortality. 10. Lee G, Belay ED. History & Physical, Clinical Criteria. Tremoulet AH, Infliximab for intravenous immunoglobulin resistance in Kawasaki disease: a retrospective study. Viral infections – fever and rash are common is young children with viral infections (examples – adenovirus, enterovirus, Epstein Barr virus, measles) 2. This material may not otherwise be downloaded, copied, printed, stored, transmitted or reproduced in any medium, whether now known or later invented, except as authorized in writing by the AAFP. et al. 1. Uehara R, Cox JR, Three-dimensional echocardiography has been used to localize coronary anomalies such as thrombi, although they are more difficult to perform in smaller children with higher heart rates. J Rheumatol. ECG = electrocardiography; INR = international normalized ratio, Reprinted with permission from Newburger JW, Takahashi M, Gerber MA, et al. et al. Sallis RE. PLoS One. J Pediatr. / Don't miss a single issue. J Cardiol. Efficacy and limitation of infliximab treatment for children with Kawasaki disease intractable to intravenous immunoglobulin therapy: report of an open-label case series. Pediatrics. Gauvreau K, Yin Y, Classic (typical) Kawasaki disease is diagnosed based on the presence of a fever lasting five or more days, acc… Some patients with Kawasaki Disease do not meet all the diagnostic criteria. et al. 2006;(4):CD004175. Tobayama H, Kawasaki disease is an acute, systemic vasculitis that predominantly affects patients younger than five years. 26. Daniels LB, Yim D, Hong YM, Is high-dose aspirin necessary in the acute phase of Kawasaki disease? 27. Nishizawa T, 15. BACKGROUND: Kawasaki disease is an acute vasculitis of childhood ... classic clinical criteria are incomplete. **—If the echocardiogram is positive, treatment should be given to children within 10 d of fever onset and those beyond day 10 with clinical and laboratory signs (CRP, ESR) of ongoing inflammation. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. Mori M, Endothelial dysfunction in children within 5 years after onset of Kawasaki disease. The AHA guideline states that the relative roles of repeated use of IVIG and other adjunctive therapies (e.g., corticosteroids, TNF-α antagonists, plasma exchange, cyclophosphamide) are uncertain,7 although additional evidence has emerged since the last update. Fabri O, This approach reduces development of coronary artery abnormalities from approximately 25% to less than 5%; development of giant aneurysms is reduced to 1%.7,13 The mechanism of action of IVIG is unknown, but effects may be from its modulation of cytokine production, influence on T-cell activity, and suppression of antibody synthesis.7,17, Acute disease is also marked by inflammation and platelet activation; aspirin is believed to modify the inflammatory state and prevent thrombosis, but it does not appear to impact the development of coronary aneurysms.7,13–15 The AHA guideline recommends high-dose aspirin, 80 to 100 mg per kg per day in four divided doses, until the patient is afebrile for 48 to 72 hours (some recommend continuing until the 14th day of illness has also passed).7 Afterward, low-dose aspirin at 3 to 5 mg per kg per day is given as a single dose until six to eight weeks after disease onset; if coronary abnormalities develop or persist, aspirin may be needed indefinitely.7,13–15 Because of concern for Reye syndrome, patients on long-term aspirin should receive the influenza vaccine, and varicella vaccination status should be checked and cautions given against potential exposure.7, Approximately 10% of patients have refractory disease that does not respond to initial therapy (i.e., fever persists or recurs 36 hours after initial IVIG dose). IVIG has a dose-dependent effect, with higher doses given in a single infusion having the greatest effect in preventing coronary abnormalities. Terai M, Heart. Guidelines for diagnosis and management of cardiovascular sequelae in Kawasaki disease (JCS 2008)—digest version. Early treatment with intravenous immunoglobulin (IVIg) has been shown to reduce morbidity and mortality. All patients who have had Kawasaki disease should have, at a minimum, periodic cardiovascular risk assessment; those with persistent aneurysms should have more intensive screening. Transthoracic echocardiography is the diagnostic imaging modality of choice to screen for coronary aneurysms, although other techniques are being evaluated for diagnosis and management. ; Zaroff JG. Maconochie I, Kawasaki disease, also known as mucocutaneous lymph node syndrome, is an acute, systemic vasculitis of small- and medium-sized arteries that predominantly affects patients younger than five years. Atherosclerosis in survivors of Kawasaki disease. et al. ††—Typical peeling begins under nail bed of fingers and then toes. 25. Lancet. 2014;383(9930):1731–1738. Krucoff MW. Cardiovascular biomarkers in acute Kawasaki disease. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. 24. J Pediatr. Heparin and warfarin (Coumadin) are reserved for treating larger aneurysms, and coronary thrombosis is treated with thrombolytic agents in conjunction with aspirin and heparin.7. §—Supplemental laboratory criteria include albumin ≤ 3.0 g/dL, anemia for age, elevation of alanine aminotransferase, platelets after 7 d ≥ 450,000/mm3, white blood cell count ≥ 15,000/mm3, and urine ≥ 10 white blood cells/high-power field. 2004;110(17):2748. http://circ.ahajournals.org/content/110/17/2747.full. Update on etio and immunopathogenesis of Kawasaki disease. Hara R, It represents the most prominent cause of acquired coronary artery disease in childhood. An open-label trial demonstrated resolution of inflammatory markers and symptoms in 18 of 20 patients given infliximab (Remicade) after IVIG was ineffective.19 A retrospective cohort study found that patients given infliximab (n = 20) had faster resolution of fever and similar coronary outcomes compared with IVIG retreatment (n = 86).20 A phase 3 randomized controlled study of infliximab for the primary treatment of Kawasaki disease (n = 196) found that although it decreased fever duration and some inflammatory markers, it did not improve treatment response over IVIG and aspirin alone.21, A case series of 125 patients with Kawasaki disease refractory to IVIG who were later treated with plasma exchange found that patients without coronary artery abnormalities at the start of therapy remained lesion free during follow-up, whereas 12 of 14 patients with coronary dilatation and two of six patients with aneurysms at the start of exchange experienced symptom resolution.22, Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). 2013;99(2):76–82. et al. Circulation. Patel A, Herzog L, For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort. Corticosteroids may be helpful as adjunctive therapy to IVIG for preventing coronary abnormalities. 17. et al. 2013;61(5):330–335. Tabnak F, Cox JR, SCOTT GROGAN, MD, MBA, is the director of faculty development and research at the Dwight D. Eisenhower Army Medical Center Family Medicine Residency Program. Love SJ, Incomplete or atypical disease is suspected with fever of five days or more with two or three of the features. 6(March 15, 2015) 6. Meadows A, 2014;133(2):e305–e311. 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. Characteristics suggesting disease other than Kawasaki disease include exudative conjunctivitis, exudative pharyngitis, discrete intraoral lesions, bullous or vesicular rash, or generalized adenopathy. Author disclosure: No relevant financial affiliations. Takahashi K, Kawasaki Disease Global Climate Consortium. https://familydoctor.org/familydoctor/en/diseases-conditions/kawasaki-disease.html, http://circ.ahajournals.org/content/110/17/2747.full, https://www.pediatrics.ucsd.edu/research/Research_Centers/Kawasaki-Disease/Pages/default.aspx, What to Do at Well-Child Visits: The AAFP’s Perspective, Common Questions About Infectious Mononucleosis. Infants < 6 months toxic shock 4 MB, Gauvreau K, Fukunaga H, et al SE... Days with < 4 clinical criteria at any time during current febrile illness ; Laboratory testing, placebo-controlled trial the!, Gruber D, Abraham SS, et al Hara R, et al for! Onouchi Y. 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