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May 22, 1991, Vol 265, No. 20 >
Clinical Cardiology | May 22, 1991

Diagnosis and Management of Kawasaki Disease FREE

Welton M. Gersony, MD
[+] Author Affiliations

This article is one of a series sponsored by the American Heart Association.

Reprint requests to Division of Pediatric Cardiology, College of Physicians and Surgeons of Columbia University, Babies Hospital, 3959 Broadway, Room 102 S, New York, NY 10032 (Dr Gersony).


From the Division of Pediatric Cardiology, College of Physicians and Surgeons of Columbia University, New York, NY.


JAMA. 1991;265(20):2699-2703. doi:10.1001/jama.1991.03460200079040
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References

Kawasaki disease is an acute vasculitis characterized by mucosal inflammation, rash, cervical adenopathy, indurative edema of the hands and feet, and late membranous desquamation of the fingertips. Early cardiac effects include myocarditis (occasionally with congestive heart failure), pericardial inflammation, and, rarely, valve involvement. Coronary artery aneurysms are a long-term concern because coronary thrombosis with myocardial infarction can be a late manifestation. The origin of Kawasaki disease is unknown, but an infectious agent is most likely. Management consists of aspirin for control of fever and inflammatory manifestations and intravenous gamma globulin for the prevention of coronary aneurysm formation. Careful late follow-up is required, especially for patients with persistent coronary abnormalities. Giant aneurysms (>8 mm) are more likely to progress to coronary obstructive disease, and coronary bypass grafts have been required for some patients. Late coronary artery manifestations in patients with mild early coronary dilatation have not been described. However, since long-term epidemiologic studies have not yet been performed, it is prudent to consider childhood Kawasaki disease to be a potential risk factor for coronary disease, especially in atherosclerosis-prone Western societies.

(JAMA. 1991;265:2699-2703)

REFERENCES

1 +
Kawasaki T.  Acute febrile mucocutaneous lymph node syndrome: clinical observations of 50 cases . Jpn J Allergol. 1967;;16:178.
2 +
Kawasaki T, Kosaki F, Okawa S, et al.  A new infantile febrile mucocutaneous lymph node syndrome prevailing in Japan . Pediatrics . 1974;;54:271.
3 +
Newburger JW, Burns JC.  Kawasaki syndrome: update in pediatric cardiology . Cardiol Clin. 1989;;7:453-465.
4 +
Bell DM, BrinkEW, Nitzkin JL, et al.  Kawasaki syndrome: description of two outbreaks in the United States . N Engl J Med. 1981;;304:1568-1575.
5 +
Yanagawa H, Kawasaki T, Shigematsu I.  Nationwide survey on Kawasaki disease in Japan . Pediatrics . 1987;;80:58-62.
6 +
Cook DH, Antia A, Attie F, et al.  Results from an international survey of Kawasaki disease in 1979-82 . Can J Cardiol. 1989;;5:389-394.
7 +
Nakamura Y, Fujita Y, Nagai M, et al.  Epidemiology of Kawasaki disease in Japan (1988) . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2,1988 ; Tokyo, Japan. Pages 23-26.
8 +
Kato H, Inoue O, Akagi T.  Kawasaki disease: cardiac problems and management . Pediatr Rev. 1988;;9:209-217.
9 +
Melish ME, Hicks RM, Larson EJ.  Mucocutaneous lymph node syndrome in the United States . AJDC. 1976;;130:599.
10 +
Rauch AM.  Kawasaki syndrome: critical review of U.S. epidemiology . Prog Clin Biol Res. 1987;;250:33-44.
11 +
Taubert KA, Rowley AH, Shulman ST.  A U.S. nationwide hospital survey of Kawasaki disease (KD) and acute rheumatic fever (ARF) . J Pediatr. In press.
12 +
Leung DY, Collins T, Lapierre LA, et al.  Immunoglobulin M antibodies present in the acute phase of Kawasaki syndrome: lyse cultured vascular endothelial cells stimulated by gamma interferon . J Clin Invest. 1986;;77:1428-1435.
13 +
Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease. Diagnostic Guidelines for Kawasaki Disease . Dallas, Tex: American Heart Association; 1989;.
14 +
Kato H, Ichinose E, Yoshioka F, et al.  Fate of coronary aneurysms in Kawasaki disease: serial coronary angiography and long-term follow-up study . Am J Cardiol. 1982;;49:1758-1766.
15 +
Nakano H, Ueda K, Saito A, et al.  Scoring method for identifying patients with Kawasaki disease at high risk of coronary artery aneurysms . Am J Cardiol. 1986;;58:739.
16 +
Gidding SS.  Risk factors for coronary aneurysms in Kawasaki disease . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2, 1988 ; Tokyo, Japan. Pages 229-232.
17 +
ArjunanK, Daniels SR, MeyerRA, et al.  Coronary artery caliber in normal children and patients with Kawasaki disease but without aneurysms: an echocardiographic and angiographic study . J Am Coll Cardiol. 1986;;8:1119-1124.
18 +
Capannari TE, Daniels SR, Meyer RA, et al.  Sensitivity, specificity and predictive value of two-dimensional echocardiography in detecting coronary artery aneurysms in patients with Kawasaki disease . J Am Coll Cardiol. 1986;;7:355-360.
19 +
Bierman FZ, Gersony WM.  Kawasaki disease: clinical perspective . J Pediatr. 1987;;11:789-793.
20 +
Inoue O, Akagi T, Kato H.  Fate of giant coronary artery aneurysms in Kawasaki disease: long-term follow-up study . Circulation . 1989;;80( (suppl II) ):II-262.
21 +
Takahashi M, Mason W, Lewis AB.  Regression of coronary aneurysms in patients with Kawasaki syndrome . Circulation . 1987;;75:387-394.
22 +
Takahashi K, Tanaka K, Naoe S, Masuda H, Tanaka N.  Morphological aspects of sequelae of Kawasaki disease . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2, 1988 ; Tokyo, Japan. Pages 175-177.
23 +
Cremer H, Rieger C.  Considerations on treatment in Kawasaki syndrome (KS) . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2,1988 ; Tokyo, Japan. Pages 297-300.
24 +
Newburger J, Takahashi M, Burns JC, et al.  The treatment of Kawasaki syndrome with intravenous gamma globulin . N Engl J Med. 1986;;315:341-347.
25 +
Furusho K, Kamiya T, Nakano H, et al.  High-dose intravenous gamma globulin for Kawasaki disease . Lancet . 1984;;2:1055.
26 +
Furusho K, Kamiya T, Nakano H, et al.  Japanese gamma globulin trials for Kawasaki disease . Prog Clin Biol Res. 1987;;250:425-432.
27 +
Nagashima M, Matsushima M, Matsuoka H, et al.  High-dose gamma globulin therapy for Kawasaki disease . J Pediatr. 1987;;110:710-712.
28 +
Rowley AH, Duffy CE, Shulman ST.  Prevention of giant coronary artery aneurysms in Kawasaki disease by intravenous gamma globulin therapy . J Pediatr. 1988;;113:290-294.
29 +
Leung DY, Burns JC, Newburger JW, et al.  Reversal of lymphocytic activation in vivo in the Kawasaki syndrome by intravenous gamma globulin . J Clin Invest. 1987;;79:468-472.
30 +
Newburger JW, Sanders SP, Burns JC, Parness IA, Beiser AS, Colan SD.  Left ventricular contractility and function in Kawasaki syndrome: effect of intravenous γ-globulin . Circulation . 1989;;76:1237-1245.
31 +
Newburger JW.  Preliminary results of multicenter trial on IVGG treatment of Kawasaki disease with single-infusion vs four-infusion regimen . Pediatr Res. 1990;;27:22A.
32 +
 Intravenous immunoglobulin: prevention and treatment of disease . JAMA. 1990;;264:3189-3193. NIH Consensus Conference.
33 +
Chung KJ.  Incidence and prognosis of giant coronary artery aneurysms in Kawasaki disease . Circulation . 1989;;80( (suppl II) ):II-282.
34 +
Hirose H, Kawashima Y, Nakano S, et al.  Long-term results in surgical treatment of children 4 years old or younger with coronary involvement due to Kawasaki disease . Circulation . 1986;;74:177-181.
35 +
Kawashima Y, Suma K, Naito Y, et al.  Surgical treatment for Kawasaki disease in Japan . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2,1988 ; Tokyo, Japan. Pages 455-458.
36 +
Echigo S.  Percutaneous transluminal coronary angioplasty for the children with coronary artery lesion due to Kawasaki disease . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2, 1988 ; Tokyo, Japan. Pages 351-353.
37 +
Kato H, Ichinose E, Inoue O, et al.  Intracoronary thrombolytic therapy in Kawasaki disease: treatment and prevention of acute myocardial infarction . Prog Clin Biol Res. 1987;;250:445-454.
38 +
Burtt DM, Pollack P, Bianco JA.  Intravenous streptokinase in an infant with Kawasaki disease complicated by acute myocardial infarction . Pediatr Cardiol. 1986;;6:307-311.
39 +
Suzuki A, Kamiya T, Ono Y, et al.  Indication of aorto-coronary bypass for coronary arterial obstruction due to Kawasaki disease: follow-up study by cardiac catheterization and coronary angiography . Pediatr Cardiol. 1988;;9:1-5.
40 +
Paridon SM, Ross RD, Kuhns LR, Pinsky WM.  Myocardial performance and perfusion during exercise in patients with coronary artery disease caused by Kawasaki disease . J Pediatr. 1990;;116:52-56.
41 +
Pahl E, Ettedgui J, Neches WH, Park SC.  The value of angiography in the follow-up of coronary involvement in Kawasaki disease . J Am Coll Cardiol. 1989;;14:1318-1325.
42 +
 Management of Kawasaki syndrome: a consensus statement prepared by North American participants of the Third International Kawasaki Disease Symposium, Tokyo, Japan, December 1988 . Pediatr Infect Dis J. 1989;;8:663-665.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

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1 +
Kawasaki T.  Acute febrile mucocutaneous lymph node syndrome: clinical observations of 50 cases . Jpn J Allergol. 1967;;16:178.
2 +
Kawasaki T, Kosaki F, Okawa S, et al.  A new infantile febrile mucocutaneous lymph node syndrome prevailing in Japan . Pediatrics . 1974;;54:271.
3 +
Newburger JW, Burns JC.  Kawasaki syndrome: update in pediatric cardiology . Cardiol Clin. 1989;;7:453-465.
4 +
Bell DM, BrinkEW, Nitzkin JL, et al.  Kawasaki syndrome: description of two outbreaks in the United States . N Engl J Med. 1981;;304:1568-1575.
5 +
Yanagawa H, Kawasaki T, Shigematsu I.  Nationwide survey on Kawasaki disease in Japan . Pediatrics . 1987;;80:58-62.
6 +
Cook DH, Antia A, Attie F, et al.  Results from an international survey of Kawasaki disease in 1979-82 . Can J Cardiol. 1989;;5:389-394.
7 +
Nakamura Y, Fujita Y, Nagai M, et al.  Epidemiology of Kawasaki disease in Japan (1988) . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2,1988 ; Tokyo, Japan. Pages 23-26.
8 +
Kato H, Inoue O, Akagi T.  Kawasaki disease: cardiac problems and management . Pediatr Rev. 1988;;9:209-217.
9 +
Melish ME, Hicks RM, Larson EJ.  Mucocutaneous lymph node syndrome in the United States . AJDC. 1976;;130:599.
10 +
Rauch AM.  Kawasaki syndrome: critical review of U.S. epidemiology . Prog Clin Biol Res. 1987;;250:33-44.
11 +
Taubert KA, Rowley AH, Shulman ST.  A U.S. nationwide hospital survey of Kawasaki disease (KD) and acute rheumatic fever (ARF) . J Pediatr. In press.
12 +
Leung DY, Collins T, Lapierre LA, et al.  Immunoglobulin M antibodies present in the acute phase of Kawasaki syndrome: lyse cultured vascular endothelial cells stimulated by gamma interferon . J Clin Invest. 1986;;77:1428-1435.
13 +
Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease. Diagnostic Guidelines for Kawasaki Disease . Dallas, Tex: American Heart Association; 1989;.
14 +
Kato H, Ichinose E, Yoshioka F, et al.  Fate of coronary aneurysms in Kawasaki disease: serial coronary angiography and long-term follow-up study . Am J Cardiol. 1982;;49:1758-1766.
15 +
Nakano H, Ueda K, Saito A, et al.  Scoring method for identifying patients with Kawasaki disease at high risk of coronary artery aneurysms . Am J Cardiol. 1986;;58:739.
16 +
Gidding SS.  Risk factors for coronary aneurysms in Kawasaki disease . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2, 1988 ; Tokyo, Japan. Pages 229-232.
17 +
ArjunanK, Daniels SR, MeyerRA, et al.  Coronary artery caliber in normal children and patients with Kawasaki disease but without aneurysms: an echocardiographic and angiographic study . J Am Coll Cardiol. 1986;;8:1119-1124.
18 +
Capannari TE, Daniels SR, Meyer RA, et al.  Sensitivity, specificity and predictive value of two-dimensional echocardiography in detecting coronary artery aneurysms in patients with Kawasaki disease . J Am Coll Cardiol. 1986;;7:355-360.
19 +
Bierman FZ, Gersony WM.  Kawasaki disease: clinical perspective . J Pediatr. 1987;;11:789-793.
20 +
Inoue O, Akagi T, Kato H.  Fate of giant coronary artery aneurysms in Kawasaki disease: long-term follow-up study . Circulation . 1989;;80( (suppl II) ):II-262.
21 +
Takahashi M, Mason W, Lewis AB.  Regression of coronary aneurysms in patients with Kawasaki syndrome . Circulation . 1987;;75:387-394.
22 +
Takahashi K, Tanaka K, Naoe S, Masuda H, Tanaka N.  Morphological aspects of sequelae of Kawasaki disease . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2, 1988 ; Tokyo, Japan. Pages 175-177.
23 +
Cremer H, Rieger C.  Considerations on treatment in Kawasaki syndrome (KS) . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2,1988 ; Tokyo, Japan. Pages 297-300.
24 +
Newburger J, Takahashi M, Burns JC, et al.  The treatment of Kawasaki syndrome with intravenous gamma globulin . N Engl J Med. 1986;;315:341-347.
25 +
Furusho K, Kamiya T, Nakano H, et al.  High-dose intravenous gamma globulin for Kawasaki disease . Lancet . 1984;;2:1055.
26 +
Furusho K, Kamiya T, Nakano H, et al.  Japanese gamma globulin trials for Kawasaki disease . Prog Clin Biol Res. 1987;;250:425-432.
27 +
Nagashima M, Matsushima M, Matsuoka H, et al.  High-dose gamma globulin therapy for Kawasaki disease . J Pediatr. 1987;;110:710-712.
28 +
Rowley AH, Duffy CE, Shulman ST.  Prevention of giant coronary artery aneurysms in Kawasaki disease by intravenous gamma globulin therapy . J Pediatr. 1988;;113:290-294.
29 +
Leung DY, Burns JC, Newburger JW, et al.  Reversal of lymphocytic activation in vivo in the Kawasaki syndrome by intravenous gamma globulin . J Clin Invest. 1987;;79:468-472.
30 +
Newburger JW, Sanders SP, Burns JC, Parness IA, Beiser AS, Colan SD.  Left ventricular contractility and function in Kawasaki syndrome: effect of intravenous γ-globulin . Circulation . 1989;;76:1237-1245.
31 +
Newburger JW.  Preliminary results of multicenter trial on IVGG treatment of Kawasaki disease with single-infusion vs four-infusion regimen . Pediatr Res. 1990;;27:22A.
32 +
 Intravenous immunoglobulin: prevention and treatment of disease . JAMA. 1990;;264:3189-3193. NIH Consensus Conference.
33 +
Chung KJ.  Incidence and prognosis of giant coronary artery aneurysms in Kawasaki disease . Circulation . 1989;;80( (suppl II) ):II-282.
34 +
Hirose H, Kawashima Y, Nakano S, et al.  Long-term results in surgical treatment of children 4 years old or younger with coronary involvement due to Kawasaki disease . Circulation . 1986;;74:177-181.
35 +
Kawashima Y, Suma K, Naito Y, et al.  Surgical treatment for Kawasaki disease in Japan . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2,1988 ; Tokyo, Japan. Pages 455-458.
36 +
Echigo S.  Percutaneous transluminal coronary angioplasty for the children with coronary artery lesion due to Kawasaki disease . In: Program and abstracts of the Third International Kawasaki Disease Symposium; November 29-December 2, 1988 ; Tokyo, Japan. Pages 351-353.
37 +
Kato H, Ichinose E, Inoue O, et al.  Intracoronary thrombolytic therapy in Kawasaki disease: treatment and prevention of acute myocardial infarction . Prog Clin Biol Res. 1987;;250:445-454.
38 +
Burtt DM, Pollack P, Bianco JA.  Intravenous streptokinase in an infant with Kawasaki disease complicated by acute myocardial infarction . Pediatr Cardiol. 1986;;6:307-311.
39 +
Suzuki A, Kamiya T, Ono Y, et al.  Indication of aorto-coronary bypass for coronary arterial obstruction due to Kawasaki disease: follow-up study by cardiac catheterization and coronary angiography . Pediatr Cardiol. 1988;;9:1-5.
40 +
Paridon SM, Ross RD, Kuhns LR, Pinsky WM.  Myocardial performance and perfusion during exercise in patients with coronary artery disease caused by Kawasaki disease . J Pediatr. 1990;;116:52-56.
41 +
Pahl E, Ettedgui J, Neches WH, Park SC.  The value of angiography in the follow-up of coronary involvement in Kawasaki disease . J Am Coll Cardiol. 1989;;14:1318-1325.
42 +
 Management of Kawasaki syndrome: a consensus statement prepared by North American participants of the Third International Kawasaki Disease Symposium, Tokyo, Japan, December 1988 . Pediatr Infect Dis J. 1989;;8:663-665.

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