Increased Detection Rate of Kawasaki Disease Using New Diagnostic Algorithm, Including Early Use of Echocardiography

doi:10.1016/j.jpeds.2009.04.058

The Journal of Pediatrics

Volume 155, Issue 5, November 2009, Pages 695-699.e1

https://doi.org/10.1016/j.jpeds.2009.04.058 Get rights and content

Objective

To determine the incidence of Kawasaki disease (KD) in Northern France by using new American Heart Association (AHA) criteria.

Study design

A 1-year prospective multicenter cohort study was performed in all pediatric departments. Patients <18 years old, who were admitted for prolonged but initially unexplained fever or suspected KD were included. All patients received the standard treatment considered appropriate by their physicians. A descriptive analysis and comparison of patients with complete and incomplete forms of KD were performed. The incidence of confirmed cases of KD (complete and incomplete forms) was calculated.

Results

Seventy-seven children were included (39 in whom KD was diagnosed). Of the patients with KD, 26 (67%) met the classic AHA case definition, and 7 (18%) had incomplete KD. Cardiac ultrasound scanning was helpful in the diagnosis of 6 of 7 patients with incomplete KD (86%). The final incidence of confirmed KD was 9 of 100 000 children <5 years of age. In 6 children (15%) the diagnosis of KD was uncertain, but they were successfully treated for it. Coronary disease was identified in 48% of patients with confirmed KD.

Conclusion

The incidence of KD is higher than previously reported, in part because earlier reports did not include incomplete forms. New AHA criteria (laboratory tests and early echocardiography) were helpful for the diagnosis of incomplete forms of KD.

Section snippets

Methods

We conducted a descriptive, prospective multicenter cohort study in a 1-year period (Sep 1, 2005-Aug 31, 2006) that involved all pediatric departments of the 18 hospitals in Northern France (1 million children <18 years old). All departments agreed to participate in this study. The local coordinating investigator at each center was a pediatrician belonging to the Hospital Network for Evaluating the Management of Common Childhood Diseases. Each patient <18 years old admitted for a prolonged but

Results

During the study period, 77 children met the inclusion criteria. Confirmed or uncertain KD was diagnosed in 39 children. The mean age of the population with KD was 37 months (range, 3-161 months), with 30 of these patients (77%) <5 years of age. The overall ethnic distribution was 69% Caucasian, 5% North African, 3% African, 0% Asian or American, and 23% unknown. The male- to-female ratio was 2.9. The mean length of fever was 6.3 days (median, 6; range, 3-15). Echocardiography was performed in

Discussion

Of the 39 patients identified prospectively as having confirmed or uncertain KD, 33 had confirmed KD, for an incidence of 9 in 100 000 children <5 years old. Seven of these 33 cases (21%) had incomplete KD, and all except 1 case were confirmed with echocardiographic abnormalities.

The failure to include incomplete KD may result in an underestimation of its incidence in Europe. Earlier reports from France, for example, estimated the incidence of KD at 5 in 100 000 children <5 years of age,9, 10

References (31)

K.A. Taubert
Epidemiology of Kawasaki disease in the United States and worldwide
Prog Pediatr Cardiol
(1997)
J.C. Burns et al.
Kawasaki syndrome
Lancet
(2004)
E.A. Rosenfeld et al.
Kawasaki disease in infants less than one year of age
J Pediatr
(1995)
A.H. Rowley et al.
Shulman ST l. Incomplete Kawasaki disease with coronary artery involvement
J Pediatr
(1987)
J.W. Newburger 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
Pediatrics
(2004)
Y. Nakamura et al.
Epidemiologic features of Kawasaki disease in Japan: results from the nationwide survey in 2005-2006
J Epidemiol
(2008)
A.S. Kao et al.
Spatial and temporal clustering of Kawasaki syndrome cases
Pediatr Infect Dis J
(2008)
C.L. Wang et al.
Kawasaki disease: infection, immunity and genetics
Pediatr Infect Dis J
(2005)
E. Salo
Kawasaki disease in Finland in 1982-1992
Scand J Infect Dis
(1993)
B. Schiller et al.
Kawasaki disease in Sweden: incidence and clinical features
Acta Paediatr
(1995)

A. Harnden et al.

Rising incidence of Kawasaki disease in England: analysis of hospital admission data

BMJ

(2002)

J.C. Borderon et al.

Le syndrome de Kawasaki en France. Enquête prospective sur 1 an

Med Mal Infect

(1998)

J. Gaudelus et al.

Paries J et le groupe de Pathologie Infectieuse Pédiatrique. Le syndrome de Kawasaki en France. Enquête rétrospective sur 5 ans

Med Mal Infect

(1998)

M.T. Witt et al.

Kawasaki disease: more patients are being diagnosed who do not meet American Heart Association criteria

Pediatrics

(2005)

A.H. Rowley

Incomplete (atypical) Kawasaki disease

Pediatr Infect Dis J

(2002)

Cited by (63)

French national diagnostic and care protocol for Kawasaki disease
2023, Revue de Medecine Interne
Kawasaki disease (KD) is an acute vasculitis with a particular tropism for the coronary arteries. KD mainly affects male children between 6 months and 5 years of age. The diagnosis is clinical, based on the international American Heart Association criteria. It should be systematically considered in children with a fever, either of 5 days or more, or of 3 days if all other criteria are present. It is important to note that most children present with marked irritability and may have digestive signs. Although the biological inflammatory response is not specific, it is of great value for the diagnosis. Because of the difficulty of recognising incomplete or atypical forms of KD, and the need for urgent treatment, the child should be referred to a paediatric hospital as soon as the diagnosis is suspected. In the event of signs of heart failure (pallor, tachycardia, polypnea, sweating, hepatomegaly, unstable blood pressure), medical transfer to an intensive care unit (ICU) is essential. The standard treatment is an infusion of IVIG combined with aspirin (before 10 days of fever, and for a minimum of 6 weeks), which reduces the risk of coronary aneurysms. In case of coronary involvement, antiplatelet therapy can be maintained for life. In case of a giant aneurysm, anticoagulant treatment is added to the antiplatelet agent. The prognosis of KD is generally good and most children recover without sequelae. The prognosis in children with initial coronary involvement depends on the progression of the cardiac anomalies, which are monitored during careful specialised cardiological follow-up.
La maladie de Kawasaki (MK) est une vascularite aiguë ayant un tropisme particulier pour les artères coronaires. La MK touche préférentiellement les enfants âgés de 6 mois à 5 ans, avec une prédominance masculine. Le diagnostic est clinique, basé sur les critères internationaux de l’American Heart Association, et doit être posé systématiquement chez les enfants présentant une fièvre depuis 5 jours ou plus, ou depuis 3 jours si tous les critères sont présents. Il est important de noter que la plupart des enfants présentent une irritabilité marquée et parfois des signes digestifs. Une inflammation biologique marquée, bien que non spécifique, est d’une grande valeur pour le diagnostic. En raison des difficultés à reconnaître la MK dans les formes incomplètes ou atypiques, et de l’urgence du traitement, l’enfant doit être référé à un hôpital pédiatrique dès que le diagnostic est simplement suspecté. En cas de signes d’insuffisance cardiaque (pâleur, tachycardie, polypnée, sueurs, hépatomégalie, instabilité tensionnelle), un transfert en soins intensifs est indispensable. Le traitement de référence repose sur les IgIV associée à l’aspirine, (avant 10 jours de fièvre, et pour une durée minimale de 6 semaines), ce qui réduit le risque d’anévrisme coronarien. En cas d’atteinte coronarienne, les antiagrégants peuvent être maintenus à vie. En cas d’anévrisme géant, les anticoagulants sont ajoutés aux antiagrégants. Le pronostic de la MK est généralement bon, la majorité des enfants se rétablissant sans séquelles. En cas d’atteinte coronarienne initiale, le pronostic dépend de l’évolution de l’atteinte cardiaque telle qu’elle est définie au cours d’un suivi cardiologique rapproché.
Postnatal Risk of Acquiring Kawasaki Disease: A Nationwide Birth Cohort Database Study
2017, Journal of Pediatrics
Citation Excerpt :
In addition, Kawasaki disease onset in adolescence (age 10-14 years) was a critical risk factor for coronary complications (OR, 5.939; 95% CI, 1.453-24.28). Because Kawasaki disease occurs predominantly in children aged <5 years, its epidemiologic data are commonly reported as the incidence in children aged <5 years.4-19 In concordance with those reports, 93.9% of the patients in the present study had onset of Kawasaki disease before age 5 years.
To investigate the postnatal risk of Kawasaki disease and coronary complications from a nationwide birth cohort in Taiwan, a country with the third-highest incidence of Kawasaki disease worldwide.
We enrolled children born between 2000 and 2009 with complete postnatal medical care records for 2000-2014 in the Taiwan national database.
Out of a total of 2 150 590 live births, we identified 6690 (62.6% boys) patients with Kawasaki disease. The onset was mostly (93.9%) within the first 5 years of life (median, 16 months; 38% during infancy), but was rare within the first 3 months of life. The overall cumulative incidence of Kawasaki disease by age 5 years was 2.78‰ (3.33‰ for boys and 2.17‰ for girls; P < .001) and exhibited an increasing trend with birth year (from 2.28‰ for 2000 to 3.67‰ for 2009). The incidence ratio was 1.535 in boys and 1.055 in each increasing year. Kawasaki disease recurred more often in younger patients (cumulative incidence, 2.3% in infants vs 1.7% in children aged 1-4 years). Coronary complications occurred in 16.2% of the patients, including 4 cases of acute myocardial infarction (3 occuring during the acute stage and 1 occurring 5 years later). The probability of a major cardiac event (infarction, undergoing percutaneous coronary intervention or coronary artery bypass grafting, or death) by adolescence was 1.9%.
The postnatal risk of Kawasaki disease was 3‰-4‰ and increased with every birth year. Patients with Kawasaki disease are at substantial risk for a major cardiac events during childhood.
Coronary artery lesions and the increasing incidence of Kawasaki disease resistant to initial immunoglobulin
2016, International Journal of Cardiology
Citation Excerpt :
The number and proportion of both HLA-DR+ CD4+ T cells and HLA-DR+ CD8+ T cells at diagnosis are higher in initial IVIG-non-responders than that in the responders [34]. Newly diagnosed KD patients are increasing in number worldwide [9,18,21,35–38]. On the other hand, the incidence of KD resistant to IVIG varied among pediatric hospitals and treatment patterns [39].
Kawasaki disease (KD) is a systemic vasculitis of childhood involving coronary arteries. Treatment for intractable cases at a higher risk of cardiac sequelae remains controversial.
Clinical outcomes of KD patients diagnosed in Yamaguchi prefecture, Japan between 2003 and 2014 were analyzed using the medical records from all 14 hospitals covering the prefecture. The study included 1487 patients (male:female, 873:614; median age at diagnosis, 24 months).
The proportion of initial intravenous immunoglobulin (IVIG)-resistant patients increased from 7% to 23% during this decade, although no patients died. Twenty-four patients developed coronary artery lesions (CALs) over one month after the KD onset. The incidence of CAL in patients who received corticosteroid during the disease course (10/37; 27.0%) was higher than that in those who did not (14/1450; 0.97%, p = 2.0 × 10^− 35). Nine patients who responded to initial IVIG plus corticosteroids had no CAL. Conversely, IVIG-resistant patients with alternate corticosteroid therapy more frequently developed CAL than those without it (10/28; 35.7% vs. 5/194; 2.6%, p = 8.9 × 10^− 10). Multivariate analyses indicated corticosteroid therapy (p < 0.0001), hyperbilirubinemia (p = 0.0010), and a longer number of days before treatment (p = 0.0005) as risk factors associated with CAL over a month after onset. The odds ratio of corticosteroid use increased from 18.3 to 43.5 if the cases were limited to initial IVIG non-responders and corticosteroid free-IVIG responders.
IVIG-failure has recently increased. The incidence of CAL increased in intractable cases with prolonged corticosteroid use. Corticosteroid may not be alternate choice for IVIG-failure to reduce the risk of cardiac sequelae.
Pediatric Systemic Lupus Erythematosus, Juvenile Dermatomyositis, Scleroderma, and Vasculitis
2016, Kelley and Firestein's Textbook of Rheumatology: Volumes 1-2, Tenth Edition
Differential protein analysis of serum exosomes post-intravenous immunoglobulin therapy in patients with Kawasaki disease
2017, Cardiology in the Young
Rising Kawasaki disease incidence in New Zealand: Analysis of national population incidence and outcomes 2000-2017
2023, Archives of Disease in Childhood

View all citing articles on Scopus

^∗: A list of members of the Hospital Network for Evaluating the Management of Common Childhood Diseases is available at www.jpeds.com (Appendix). The authors declare no conflicts of interest.

View full text

Original ArticleIncreased Detection Rate of Kawasaki Disease Using New Diagnostic Algorithm, Including Early Use of Echocardiography

Objective

Study design

Results

Conclusion

Section snippets

Methods

Results

Discussion

Prog Pediatr Cardiol

Lancet

J Pediatr

J Pediatr

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

Pediatrics

Epidemiologic features of Kawasaki disease in Japan: results from the nationwide survey in 2005-2006

J Epidemiol

Spatial and temporal clustering of Kawasaki syndrome cases

Pediatr Infect Dis J

Kawasaki disease: infection, immunity and genetics

Pediatr Infect Dis J

Kawasaki disease in Finland in 1982-1992

Scand J Infect Dis

Kawasaki disease in Sweden: incidence and clinical features

Acta Paediatr

Rising incidence of Kawasaki disease in England: analysis of hospital admission data

BMJ

Le syndrome de Kawasaki en France. Enquête prospective sur 1 an

Med Mal Infect

Paries J et le groupe de Pathologie Infectieuse Pédiatrique. Le syndrome de Kawasaki en France. Enquête rétrospective sur 5 ans

Med Mal Infect

Kawasaki disease: more patients are being diagnosed who do not meet American Heart Association criteria

Pediatrics

Incomplete (atypical) Kawasaki disease

Pediatr Infect Dis J

Original Article
Increased Detection Rate of Kawasaki Disease Using New Diagnostic Algorithm, Including Early Use of Echocardiography