561594
research-article2014
CPJXXX10.1177/0009922814561594Clinical PediatricsUysal et al
Article
Outcomes of Kawasaki Disease: A SingleCenter Experience
Clinical Pediatrics 1–6 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922814561594 cpj.sagepub.com
Fahrettin Uysal, MD1,2,3, Ozlem Mehtap Bostan, MD3,4, Solmaz Celebi, MD4, Berfin Uysal, MD4, Serif Hamitoglu, MD4, and Ergun Cil, MD1,3
Abstract Objectives: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology. Coronary artery lesions (CAL) develop in 15% to 20% of untreated cases. Our objective was to evaluate demographic, clinical, and laboratory features and short-intermediate coronary artery outcomes of children with KD. Patients and Methods: Medical records of patients with KD were retrospectively identified. Clinical information and echocardiography, laboratory, and angiographic results were noted using a standardized form. Results: The study included 44 patients with a mean age of the 29.72 ± 21 months (ranging from 1 month to 9.5 years). There were 28 male and 16 female patients; 20 patients were diagnosed as having had incomplete KD. Four cases with atypical presentation were significantly older than children with complete and incomplete KD; 17 patients (38.6 %) had coronary artery aneurysm (CAA), which declined to 6.8% after intravenous immunoglobulin (IVIG) treatment. Time between fever and diagnosis and abnormal levels of hemoglobin and platelets were all associated with CAA. The children were followed up for a mean of 36.39 ± 19 months (with a maximum of 16 years). Angiographic evolution and regression of CALs have been observed in 14 (82.3%) patients. Three patients in whom CALs persisted did not receive IVIG therapy because of delayed diagnosis. Conclusions: Awareness of KD in children has led to an increase in the number of cases. Utility of IVIG treatment to reduce the coronary artery involvement in patients with delayed diagnoses should be discussed and considered. Long-term results are required to assess whether the KD represents a risk factor for coronary artery diseases seen during adulthood. Keywords Kawasaki disease, outcome, coronary artery aneurysm, children
Introduction
Material and Methods
Kawasaki disease (KD) is an important childhood systemic vasculitis with potentially major cardiovascular sequelae, especially if timely and appropriate treatment is not given. Since the first report of KD, 47 years have passed1 and now it is the leading cause of acquired heart disease in childhood in developed countries in the current era.2 Whereas its cause is still unknown, timely treatment with intravenous immunoglobulins (IVIG) has reduced the incidence of coronary artery lesions (CALs) from about 25% to 5%.3,4 Although CALs does not persist in most of the patients, it is controversial whether a history of KD itself is a risk factor for atherosclerosis.5 Japan has the highest and increasing annual incidence in the world (84:100 000 children), but epidemiological studies of KD in developing countries are inadequate.6 The aim of our study was to evaluate the demographic, clinical, and laboratory features and short-intermediate coronary artery outcomes of children with KD over a 16-year period.
Participants From January 1998 to April 2014, 44 children (28 boys, 16 girls) ranging in age from 1 month to 9 years had a diagnosis of KD determined at the department of Pediatric Cardiology of University of Uludag, a tertiary medical center in Turkey. Databases were collected retrospectively by analyzing the computerized hospital coding system. A diagnosis of KD was made according 1
University of Uludag, School of Medicine, Bursa, Turkey Turkish National Pediatric Society, Ankara, Turkey 3 Turkish Pediatric Cardiology and Cardiovascular Surgery Society, Ankara, Turkey 4 University of Uludag, Bursa, Turkey 2
Corresponding Author: Fahrettin Uysal, Department of Pediatric Cardiology, University of Uludag School of Medicine, Gorukle Campus, Nilufer, Bursa, 16059 Turkey. Email: [email protected]
Downloaded from cpj.sagepub.com at GEORGIAN COURT UNIV on March 21, 2015
2
Clinical Pediatrics
to the American Heart Association guidelines.3 Criteria for the diagnosis included prolonged fever for 5 or more days, plus at least 4 of the 5 principal clinical findings: polymorphous exanthema, bulbar conjunctival injection without exudate, changes in lips and oral cavity, cervical lymphadenopathy (≥1.5 cm diameter), and changes in extremities. Patients who had fewer than 4 of these criteria were labeled as having incomplete KD, and those who had a problem that was generally not seen in KD were described as having atypical KD. Demographic data included age at diagnosis and gender. Symptoms and physical findings at presentation, duration of fever, the time of IVIG treatment, response to treatment, and laboratory findings were noted. Failure to respond was defined as persistent or recrudescent fever ≥36 hours after completion of the initial IVIG infusion.
Echocardiographic Study Routine 2-dimensional echocardiographic evaluation was performed with an ultrasound system provided with pulsed, continuous, and color Doppler (Vivid–6 GE and HP Sonos 5500). Coronary arteries were evaluated in the parasternal short and long axes at the level of great arteries as well as in apical 4-chamber and subcostal views. Coronary artery aneurysms (CAAs) were defined according to criteria established by the Japanese Ministry of Health in 1984.7 When the coronary artery was larger than normal (dilated) and without a segmental aneurysm, the vessel was considered ectasic.3 Echocardiography was repeated within 2 weeks of the onset of illness, during the fourth week, and thereafter depending on the initial findings.
Catheterization and Angiography Aortic angiography was performed under sedoanalgesia by puncturing the femoral artery and administering a contrast agent just above the aortic valve to obtain a precise image of coronary artery anatomy if the patient had complex CALs and if there was any doubt about the CAAs. Selective coronary angiography was performed when required.
Statistical Analysis Statistical analysis was performed using a computerbased program (SPSS for Windows version 21; SPSS, Chicago, IL). Quantitative variables were expressed as means, and qualitative variables were expressed as percentages. Univariate comparison between continuous variables was performed with Student’s t test or the Mann-Whitney U test, and categorical data were compared using the χ2 test or Fisher’s exact test. A P value
Seasonal distribu on of pa ents with Kawasaki Disease (%) 22.7 43.2
22.7 11.4
Spring
Autumn
Winter
Summer
Figure 1. Seasonal distribution of patients with Kawasaki disease.
research-article2014
CPJXXX10.1177/0009922814561594Clinical PediatricsUysal et al
Article
Outcomes of Kawasaki Disease: A SingleCenter Experience
Clinical Pediatrics 1–6 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922814561594 cpj.sagepub.com
Fahrettin Uysal, MD1,2,3, Ozlem Mehtap Bostan, MD3,4, Solmaz Celebi, MD4, Berfin Uysal, MD4, Serif Hamitoglu, MD4, and Ergun Cil, MD1,3
Abstract Objectives: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology. Coronary artery lesions (CAL) develop in 15% to 20% of untreated cases. Our objective was to evaluate demographic, clinical, and laboratory features and short-intermediate coronary artery outcomes of children with KD. Patients and Methods: Medical records of patients with KD were retrospectively identified. Clinical information and echocardiography, laboratory, and angiographic results were noted using a standardized form. Results: The study included 44 patients with a mean age of the 29.72 ± 21 months (ranging from 1 month to 9.5 years). There were 28 male and 16 female patients; 20 patients were diagnosed as having had incomplete KD. Four cases with atypical presentation were significantly older than children with complete and incomplete KD; 17 patients (38.6 %) had coronary artery aneurysm (CAA), which declined to 6.8% after intravenous immunoglobulin (IVIG) treatment. Time between fever and diagnosis and abnormal levels of hemoglobin and platelets were all associated with CAA. The children were followed up for a mean of 36.39 ± 19 months (with a maximum of 16 years). Angiographic evolution and regression of CALs have been observed in 14 (82.3%) patients. Three patients in whom CALs persisted did not receive IVIG therapy because of delayed diagnosis. Conclusions: Awareness of KD in children has led to an increase in the number of cases. Utility of IVIG treatment to reduce the coronary artery involvement in patients with delayed diagnoses should be discussed and considered. Long-term results are required to assess whether the KD represents a risk factor for coronary artery diseases seen during adulthood. Keywords Kawasaki disease, outcome, coronary artery aneurysm, children
Introduction
Material and Methods
Kawasaki disease (KD) is an important childhood systemic vasculitis with potentially major cardiovascular sequelae, especially if timely and appropriate treatment is not given. Since the first report of KD, 47 years have passed1 and now it is the leading cause of acquired heart disease in childhood in developed countries in the current era.2 Whereas its cause is still unknown, timely treatment with intravenous immunoglobulins (IVIG) has reduced the incidence of coronary artery lesions (CALs) from about 25% to 5%.3,4 Although CALs does not persist in most of the patients, it is controversial whether a history of KD itself is a risk factor for atherosclerosis.5 Japan has the highest and increasing annual incidence in the world (84:100 000 children), but epidemiological studies of KD in developing countries are inadequate.6 The aim of our study was to evaluate the demographic, clinical, and laboratory features and short-intermediate coronary artery outcomes of children with KD over a 16-year period.
Participants From January 1998 to April 2014, 44 children (28 boys, 16 girls) ranging in age from 1 month to 9 years had a diagnosis of KD determined at the department of Pediatric Cardiology of University of Uludag, a tertiary medical center in Turkey. Databases were collected retrospectively by analyzing the computerized hospital coding system. A diagnosis of KD was made according 1
University of Uludag, School of Medicine, Bursa, Turkey Turkish National Pediatric Society, Ankara, Turkey 3 Turkish Pediatric Cardiology and Cardiovascular Surgery Society, Ankara, Turkey 4 University of Uludag, Bursa, Turkey 2
Corresponding Author: Fahrettin Uysal, Department of Pediatric Cardiology, University of Uludag School of Medicine, Gorukle Campus, Nilufer, Bursa, 16059 Turkey. Email: [email protected]
Downloaded from cpj.sagepub.com at GEORGIAN COURT UNIV on March 21, 2015
2
Clinical Pediatrics
to the American Heart Association guidelines.3 Criteria for the diagnosis included prolonged fever for 5 or more days, plus at least 4 of the 5 principal clinical findings: polymorphous exanthema, bulbar conjunctival injection without exudate, changes in lips and oral cavity, cervical lymphadenopathy (≥1.5 cm diameter), and changes in extremities. Patients who had fewer than 4 of these criteria were labeled as having incomplete KD, and those who had a problem that was generally not seen in KD were described as having atypical KD. Demographic data included age at diagnosis and gender. Symptoms and physical findings at presentation, duration of fever, the time of IVIG treatment, response to treatment, and laboratory findings were noted. Failure to respond was defined as persistent or recrudescent fever ≥36 hours after completion of the initial IVIG infusion.
Echocardiographic Study Routine 2-dimensional echocardiographic evaluation was performed with an ultrasound system provided with pulsed, continuous, and color Doppler (Vivid–6 GE and HP Sonos 5500). Coronary arteries were evaluated in the parasternal short and long axes at the level of great arteries as well as in apical 4-chamber and subcostal views. Coronary artery aneurysms (CAAs) were defined according to criteria established by the Japanese Ministry of Health in 1984.7 When the coronary artery was larger than normal (dilated) and without a segmental aneurysm, the vessel was considered ectasic.3 Echocardiography was repeated within 2 weeks of the onset of illness, during the fourth week, and thereafter depending on the initial findings.
Catheterization and Angiography Aortic angiography was performed under sedoanalgesia by puncturing the femoral artery and administering a contrast agent just above the aortic valve to obtain a precise image of coronary artery anatomy if the patient had complex CALs and if there was any doubt about the CAAs. Selective coronary angiography was performed when required.
Statistical Analysis Statistical analysis was performed using a computerbased program (SPSS for Windows version 21; SPSS, Chicago, IL). Quantitative variables were expressed as means, and qualitative variables were expressed as percentages. Univariate comparison between continuous variables was performed with Student’s t test or the Mann-Whitney U test, and categorical data were compared using the χ2 test or Fisher’s exact test. A P value
Seasonal distribu on of pa ents with Kawasaki Disease (%) 22.7 43.2
22.7 11.4
Spring
Autumn
Winter
Summer
Figure 1. Seasonal distribution of patients with Kawasaki disease.
