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Background: LEOPARD syndrome (LS) is a rare genetic disorder presenting various clinical manifestations from childhood, complicating its diagnosis. In this study, we aim to refine the imaging presentation of LS and emphasize the importance of multimodality imaging in enhancing diagnostic accuracy and preventing serious cardiovascular events. Case: A 41-year-old woman was admitted to hospital with a suspected apical tumor detected by a transthoracic echocardiogram (TTE), which was later identified as apical myocardial hypertrophy through cardiac magnetic resonance imaging (CMR). She had abnormal electrocardiograms from the age of 2â years and freckles around the age of 4â years. In recent years, she has been experiencing exertional dyspnea. Supplemental coronary computer tomography angiography (CCTA) revealed diffuse coronary dilatation. Both multimodality imaging and clinical manifestations led to a suspicion of LS, which was confirmed by subsequent genetic testing. The patient declined further treatment. A 3-month follow-up CMR showed no significant change in the lesion. Conclusion: This report elucidates the diagnostic transition from an initial suspicion of an apical tumor by TTE to a definitive diagnosis of left ventricular apical hypertrophy by CMR in a 41-year-old woman with LS. It underscores the value of multimodality imaging (TTE, CCTA, CMR) in unraveling unusual cardiac manifestations in rare genetic disorders such as LS.
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Introduction: To study the clinical presentation, laboratory profile, echocardiographic details, management, and outcomes of children who were diagnosed to have multisystem inflammatory syndrome in children (MIS-C) in the immediate postoperative period after surgery for congenital heart defects (CHDs). Materials and Methods: This is a prospective case-control study that included children diagnosed to have MIS-C in the postoperative period based on clinical signs, rise in inflammatory markers, and echocardiographic features of ventricular dysfunction or coronary involvement. Management included intravenous immunoglobulin (IVIG), steroids, and antiplatelet medications in addition to routine postoperative care. Out of the 461 children who underwent surgery for CHD between April 1st, 2021, and November 30th, 2021, 18 children were diagnosed with MIS-C. After the initial routine postoperative course, all 18 children had sudden worsening in clinical and laboratory parameters. Other causes such as bacterial infection were ruled out. All of these children had features of MIS-C with ventricular dilatation and dysfunction, coronary artery involvement, and reactive COVID-19 immunoglobulin G antibody. There was a significant improvement in coronary artery dimensions after IVIG administration (P = 0.001). The involvement of the left main coronary artery was associated with significantly increased length of intensive care unit (ICU) and hospital stay (P = 0.019). Mean ICU and hospital stay was prolonged in the MIS-C group. There were two deaths in this group due to severe left ventricular dysfunction. Conclusions: During the pandemic, a proportion of patients undergoing elective cardiac surgery may develop unexpected worsening in clinical status due to MIS-C. A high index of suspicion and prompt treatment with IVIG and steroids may be helpful in improving outcomes.
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Kawasaki disease shock syndrome (KDSS) is a severe form of Kawasaki disease (KD). The hemodynamic instability and atypical manifestations of this syndrome delay its correct diagnosis and timely treatment. We report here an eight-year-old girl who presented with appendicitis. Her fever persisted after appendectomy, accompanied by hemodynamic instability. The girl was diagnosed with KDSS. Intravenous immunoglobulin (IVIG) and corticosteroids were administered. Her symptoms resolved. She had left coronary artery dilatation, which resolved three months later. We also reviewed two other possible cases identified as KDSS with appendicitis. These cases have a more atypical clinical course, prolonged treatment, and a higher rate of IVIG resistance. Better awareness of KDSS is needed for early diagnosis and treatment in children experiencing prolonged fever after appendectomy.
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OBJECTIVES: This study aims to develop a new algorithm for predicting intravenous immunoglobulin (IVIG) resistance and coronary artery involvement in Kawasaki disease (KD) through decision tree models. METHODS: Medical records of children hospitalized for KD were analysed retrospectively. We compared the clinical characteristics, and the laboratory data in the groups with IVIG resistance and coronary artery dilatations (CADs) in KD patients. The decision tree models were developed to predict IVIG resistance and CADs. RESULTS: A total 896 patients (511 males and 385 females; 1 month-12 years) were eligible. IVIG resistance was identified in 111 (12.3%) patients, and CADs were found in 156 (17.4%). Total bilirubin and nitrogen terminal- pro-brain natriuretic peptide (NT-proBNP) were significantly higher in IVIG resistant group than in IVIG responsive group (0.62 ± 0.8 mg/dL vs 1.38 ± 1.4 mg/dL and 1231 ± 2136 pg/mL vs 2425 ± 4459 mL, respectively, P < 0.01). Also, CADs were more developed in the resistant group (39/111; 14.9% vs. 117/785; 35.1%, P < 0.01). The decision tree for predicting IVIG resistance was classified based on total bilirubin (0.7 mg/mL, 1.46 mg/dL) and NT-proBNP (1561 pg/mL), consisting of two layers and four nodes, with 86.2% training accuracy and 90.5% evaluation accuracy. The Receiver Operating Characteristic (ROC) evaluated the predictive ability of the decision tree, and the area under the curve (AUC) (0.834; 95% confidence interval, 0.675-0.973; P < 0.05) showed relatively higher accuracy. The group with CADs had significantly higher total bilirubin and NT-proBNP levels than the control group (0.64 ± 0.82 mg/dL vs 1.04 ± 1.14 mg/dL and 1192 ± 2049 pg/mL vs 2268 ± 4136 pg/mL, respectively, P < 0.01). The decision trees for predicting CADs were classified into two nodes based on NT-proBNP (789 pg/mL) alone, with 83.5% training accuracy and 90.3% evaluation accuracy. CONCLUSION: A new algorithm decision tree model presents for predicting IVIG resistance and CADs in KD, confirming the usefulness of NT-proBNP as a predictor of KD.
Subject(s)
Coronary Artery Disease , Mucocutaneous Lymph Node Syndrome , Bilirubin , Child , Coronary Artery Disease/etiology , Coronary Vessels , Decision Trees , Female , Humans , Immunoglobulins, Intravenous/therapeutic use , Infant , Male , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/drug therapy , Retrospective StudiesABSTRACT
Coronary artery dilatation has been observed with coronavirus disease 2019 (COVID-19)-associated multisystem inflammatory syndrome in children (MIS-C), which is more common in those with Kawasaki-like disease. MIS-C is a clinical syndrome in children and adolescents; its signs and symptoms, as well as cardiac manifestations, are similar to Kawasaki diseases, such as coronary artery dilation, coronary aneurysms, and ventricular dysfunction. The occurrence of coronary artery dilatation in asymptomatic pediatric patients following COVID-19 infection has not been well documented in the literature. Thus, in this article, we present four cases of coronary artery dilation in children with a past history of COVID-19 infection who had very few or no symptoms and were referred to us for vague chest pain and palpitation. As a result, a high index of suspicion is required, and any patient complaining of chest pain and palpitation with a history of COVID-19 exposure should not be ignored and be given proper coronary artery evaluation. This article also raises the question of whether every child infected with COVID-19 should have an echocardiogram.
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Introduction@#Kawasaki disease (KD) is the leading cause of acquired heart disease in childhood, but its diagnosis remains challenging since a significant number of cases do not meet the diagnostic criteria (Incomplete KD). This may delay the diagnosis and initiation of treatment, and increase the risk of morbidity from coronary artery complications.@*Objectives@#This study compared the clinical profile and treatment outcomes of children with complete and incomplete KD. @*Methods@#This is a cross-sectional, retrospective study of pediatric patients diagnosed with KD and admitted in a tertiary hospital from January 1, 2010 to December 31, 2020. Demographics, clinical manifestations, laboratories, 2D echocardiography (2DE) findings and treatment outcomes were obtained by review of medical records and analyzed using descriptive statistics. @*Results@#Among 135 patients studied, 71% were classified as Incomplete Kawasaki Disease. Majority (89%) were children more than 1 year old and predominantly male (55%). Five classic features, other than fever, were more frequent in complete KD – bilateral bulbar conjunctivitis, mucosal changes in the lip and oral cavity, polymorphous exanthem, changes in extremities, and cervical lymphadenopathy. Fever (100%), conjunctivitis (100%), rashes (97%) and oral changes (90%) were the most common findings in complete KD, while fever (100%), rashes (56%), conjunctivitis (46%) and oral changes (35%) were noted in incomplete KD. Higher CRP (167 mg/L vs. 100 mg/L) and lower albumin levels (30 g/L vs. 38 g/L) were seen in complete KD. Coronary artery dilatation (56% vs. 48%) was frequently detected in both complete and incomplete KD. Majority (96%) of cases received only one dose of IVIG and 4% needed additional treatment with methylprednisone. @*Conclusion@#The five principal features of KD other than fever, elevated CRP and lower albumin levels were significantly more common in complete cases. No significant differences in the demographics and 2DE findings of children with complete and incomplete KD were observed.
Subject(s)
Mucocutaneous Lymph Node SyndromeABSTRACT
This case report details the clinical course of a 7-year-old patient with an initial presentation of acute appendicitis, who developed symptoms highly concerning multisystem inflammatory syndrome in children (MIS-C) after appendectomy. Despite appropriate management, the patient went on to develop left main coronary artery dilatation. Given the spectrum of clinical presentations and absence of pathognomonic findings or diagnostic tests for MIS-C, it is essential to maintain a high index of suspicion for MIS-C when pediatric patients first present with nonspecific gastrointestinal symptoms and recent exposure to coronavirus disease 2019 (COVID-19). Importantly, this case illustrates that the diagnosis of MIS-C can be missed in three different ways: 1) if the patient has an absence of classic symptoms such as rash, conjunctivitis, edema, or evidence of mucocutaneous involvement on initial presentation; 2) if the patient initially has leukocytosis, instead of leukopenia (which is more prevalent in MIS-C cases), and a normal platelet count early on in the disease course; and 3) if providers confuse MIS-C for other more common postoperative causes of fever, such as atelectasis. Finally, MIS-C should still be considered part of the differential even if abdominal computer tomography (CT) findings are unremarkable for systemic inflammation. Given the potential for a rapid clinical decline in patients with MIS-C, appropriate workup should be completed in a timely manner.
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A giant coronary artery (CA) aneurysm is a potentially fatal cardiac complication resulting from Kawasaki disease (KD). We aimed to identify epidemiologic characteristics and potential risk factors associated with giant CA aneurysms identified after acute KD. We analyzed 90,252 patients diagnosed with KD from 2011 to 2018, using data obtained in nationwide KD surveys conducted in Japan. Multivariable logistic regression analyses were performed to evaluate potential risk factors associated with subsequent giant CA aneurysm complications (defined as lumen size ≥ 8 mm), adjusting for all potential factors. Giant CA aneurysms were identified in 144 patients (0.16%) after acute KD. The annual prevalence ranged from 0.07 to 0.20% during the study period. In the multivariate analyses, male sex (adjusted odds ratio 2.09 [95% confidence interval 1.41-3.11], recurrent KD (1.90 [1.09-3.33]), IVIG administration at 1-4 days of illness (1.49 [1.04-2.15]) and ≥ 8 days after KD onset (2.52 [1.38-4.60]; reference, 5-7 days), detection of CA dilatations and aneurysms at initial echocardiography (4.17 [1.85-5.41] and 46.5 [28.8-74.8], respectively), and resistance to IVIG treatment (6.09 [4.23-8.75]) were significantly associated with giant CA aneurysm complications identified after acute KD. The annual prevalence of giant CA aneurysms identified after acute KD did not increase during the study period. Patients with larger CA abnormalities detected at initial echocardiography were independently associated with progression to giant CA aneurysm complications after acute KD regardless of the number of days from onset at treatment initiation.
Subject(s)
Coronary Aneurysm/epidemiology , Coronary Aneurysm/etiology , Mucocutaneous Lymph Node Syndrome/complications , Child, Preschool , Coronary Aneurysm/diagnosis , Coronary Artery Disease/diagnosis , Coronary Artery Disease/epidemiology , Coronary Artery Disease/etiology , Dilatation, Pathologic/diagnosis , Echocardiography/methods , Female , Humans , Immunoglobulins, Intravenous/adverse effects , Immunoglobulins, Intravenous/therapeutic use , Infant , Japan/epidemiology , Logistic Models , Male , Mucocutaneous Lymph Node Syndrome/therapy , Odds Ratio , Retrospective Studies , Risk Factors , Severity of Illness Index , Surveys and QuestionnairesABSTRACT
BACKGROUND: X-linked lymphoproliferative disease (XLP) is a rare inherited X-linked primary immunodeficiency diseases (PID). One such disease, X-linked inhibitor of apoptosis protein (XIAP) deficiency, is characterized by Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH). However, EBV-HLH with coronary artery dilation and acute renal injury (AKI) in children is unusual. CASE PRESENTATION: We report the case of a young boy aged 17 months with a novel XIAP variant. He was initially diagnosed with EBV-HLH based on the HLH-2004 diagnostic criteria and the condition was accompanied by coronary artery dilation and acute renal injury. The comprehensive genetic analysis of peripheral blood-derived DNA revealed a hemizygous variant of the XIAP gene [c.116G > C(p.G39A)], which was inherited from his mother (heterozygous condition). After combined treatment with rituximab, intravenous immunoglobulin, corticosteroids, antiviral drugs, and mycophenolate mofetil (MMF) in addition to supportive therapy, his clinical manifestations and laboratory indexes were improved. The patient achieved complete remission with MMF treatment in the 8-month follow-up. CONCLUSIONS: We report the [c.116G > C(p.G39A)] variant in the XIAP gene for the first time in a case of XLP-2 associated with EBV-HLH. For male patients with severe EBV-HLH, the possibility of XLP should be considered and molecular genetic testing should be used early in auxiliary diagnosis. Reports of EBV-HLH with coronary artery dilation and AKI in children are rare. In the patients with EBV-HLH, color Doppler echocardiography and urine tests should be monitored regularly. If necessary, renal biopsy can be performed to clarify the pathology. Treatment with rituximab, immunosuppressors and supportive therapy achieved a good effect, but long-term follow-up is required.
Subject(s)
Acute Kidney Injury , Epstein-Barr Virus Infections , Lymphohistiocytosis, Hemophagocytic , Acute Kidney Injury/diagnosis , Acute Kidney Injury/etiology , Child , Coronary Vessels , Dilatation , Epstein-Barr Virus Infections/complications , Epstein-Barr Virus Infections/diagnosis , Herpesvirus 4, Human/genetics , Humans , Infant , Lymphohistiocytosis, Hemophagocytic/complications , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/genetics , Male , X-Linked Inhibitor of Apoptosis Protein/geneticsABSTRACT
Kawasaki disease (KD) is the leading cause of acquired pediatric heart disease in the developed world as 25-30% of untreated patients and at least 5% of treated patients will develop irreversible coronary artery lesions (CAL). Pentraxin-3 (PTX-3) has been well-studied in inflammatory diseases, particularly in cardiovascular diseases associated with vascular endothelial dysfunction. We hypothesized that PTX-3 plays an important role in the development of KD-associated CAL and investigated the circulating levels of PTX-3 in the serum of KD patients. Children with acute KD were followed from diagnosis through normalization of the clinical parameters of inflammation (convalescent phase). Serum samples were obtained and echocardiograms were conducted at several phases of the illness: acute [prior to intravenous immunoglobulin (IVIG) treatment], sub-acute (5-10 days after IVIG treatment), and convalescent (1-4 months after KD diagnosis). Seventy children were included in the final cohort of the study, of whom 26 (37%) presented with CAL and 18 (26%) developed IVIG resistance. The patients included in this study came from diverse ethnic backgrounds, mostly with mixed ancestry/ ethnicity. Significantly increased PTX-3 levels were observed during the acute phase of KD compared to the sub-acute and the convalescent phases. The PTX-3 levels during acute KD were significantly higher among KD patients with CAL compared to patients with normal coronary arteries (NCA). Also, the PTX-3 levels were significantly higher in patients with IVIG resistance. Furthermore, the PTX-3 levels were significantly higher in IVIG-resistant KD patients with CAL as compared to the NCA group. Moreover, the PTX-3 levels were significantly correlated to coronary artery z-score during acute KD and to neutrophil counts throughout KD progression regardless of coronary artery z-score. Elevated PTX-3 levels correlated to elevated neutrophil counts, a known source of PTX-3 in acute inflammation and an important player in the development of KD vasculitis. We, therefore, suggest PTX-3 as a novel factor in the development of KD-associated CAL and propose neutrophil-derived PTX-3 as contributing to KD vascular dysfunction.
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A number of microorganisms were hypothesised as an aetiology of the Kawasaki disease. Unfortunately, no specific agent that provides reproducible evidence has yet been reported. We report two cases of extremely rare Kawasaki disease with tsutsugamushi disease. These case reports suggest that Kawasaki disease can rarely occur concurrently or immediately after a rickettsial illness such as tsutsugamushi disease.
Subject(s)
Mucocutaneous Lymph Node Syndrome/etiology , Scrub Typhus/diagnosis , Child , Child, Preschool , Echocardiography , Humans , Male , Mucocutaneous Lymph Node Syndrome/microbiology , Orientia tsutsugamushi/isolation & purificationABSTRACT
BACKGROUND: Coronary artery dilatation (CAD) is a great concern with Kawasaki disease (KD). This study aimed to investigate the relationship between serum N-terminal pro-brain natriuretic peptide (NT-proBNP) levels and CAD in patients with the hyperacute phase (≤4â¯days of fever) of KD. METHODS: Serum NT-proBNP levels were compared between patients with and those without CAD, who underwent transthoracic echocardiography (TTE) within 24â¯h of the hyperacute phase of KD in the pediatric emergency department (PED). Electronic medical records of patients aged 1â¯month to 15â¯years who visited the PED were retrospectively assessed from January 2010 to December 31, 2014. RESULTS: One hundred nine patients were enrolled in the study. Twenty-three of those patients had CAD within 24â¯h of TTE. Median serum NT-proBNP levels were significantly higher in patients with CAD (824.1â¯pg/ml; interquartile range [IQR], 515.4-1570.0184.8-767.8â¯pg/ml) than in patients without CAD (396.4â¯pg/ml; IQR, 184.8-767.8â¯pg/ml) (pâ¯≤â¯0.001). The cutoff value of serum NT-proBNP, which predicted CAD during the hyperacute phase of KD, was 515.4â¯pg/ml, which yielded sensitivity of 78.26% and specificity of 61.63%. The area under the curve for NT-proBNP for predicting CAD during hyperacute KD was 0.749 (95% CI, 0.642-0.856). CONCLUSION: Serum NT-proBNP might be an additional laboratory marker for detecting early CAD during the hyperacute phase of KD in the PED.
Subject(s)
Coronary Vessels/pathology , Mucocutaneous Lymph Node Syndrome/complications , Natriuretic Peptide, Brain/blood , Peptide Fragments/blood , Vasodilation , Adolescent , Adult , Biomarkers/blood , Echocardiography , Female , Humans , Logistic Models , Male , Middle Aged , Multivariate Analysis , ROC Curve , Retrospective Studies , Sensitivity and Specificity , Young AdultABSTRACT
Introduction In Kawasaki disease, although coronary dilatation is attributed to vasculitis, the effect of myocardial inflammation is underestimated. Coronary dilatations are determined by Z-scores, which do not take into account dominance. The aim of the present study was to describe the impact of coronary dominance on dilatation in Kawasaki disease. METHODS: We performed a retrospective analysis of coronary dilatations according to angiography categorisation of dominance. RESULTS: Of 28 patients (2.6 [0.2-10.1] years), right dominance was present in 15 patients and left in 13. Early dilatation was present in all patients, of whom 11 were ipsilateral to the dominant segment and 17 contralateral. Ipsilateral dilatations were present at diagnosis (9/11 versus 6/17, p=0.02) compared with contralateral dilatations, which developed 2 weeks after diagnosis (9/11 versus 16/17, p=0.29). Coronary artery Z-scores of patients with contralateral dilatation increased at 2 weeks, before returning to baseline values (2.0±2.2 at diagnosis, 4.1±1.8 at 2 weeks, 1.8±1.2 at 3-6 months, p=0.001), compared with patients with ipsilateral dilatation in whom Z-scores were maximal at diagnosis and remained stable (3.0±0.9, 2.7±1.1 and 2.6±1.5, respectively, p=0.13). Dominant coronary artery Z-scores were higher compared with non-dominant segments at diagnosis (3.0±0.9 versus 1.0±0.8, p<0.001) and at late follow-up (2.6±1.5 versus 0.4±1.4, p=0.002) in patients with ipsilateral dilatation. CONCLUSION: Progression of coronary dilatation after diagnosis may be a sign of dilatation secondary to vasculitis, as opposed to regression of Z-scores in ipsilateral dilatations, probably related to physiological vasodilatation in response to carditis. This needs to be validated in larger studies against vasculitic and myocardial inflammatory markers.
Subject(s)
Coronary Aneurysm/diagnostic imaging , Coronary Aneurysm/physiopathology , Coronary Vessels/diagnostic imaging , Mucocutaneous Lymph Node Syndrome/complications , Biomarkers , Child , Child, Preschool , Coronary Angiography , Dilatation, Pathologic/diagnostic imaging , Echocardiography , Female , Humans , Infant , Male , Quebec , Regression Analysis , Retrospective StudiesABSTRACT
BACKGROUND: Kawasaki disease is a systemic vasculitis predominately affecting coronary arteries. Hemophagocytic lymphohistiocytosis can complicate the course of Kawasaki disease. Rare cases of secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of Kawasaki disease have been reported. CASE REPORT: We report here a 4 month-old girl with diffuse coronary ectasia and secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of incomplete Kawasaki disease. CONCLUSION: Due to the large overlap in clinical symptoms, the presence of atypical findings for Kawasaki disease should suggest the possible diagnosis of hemophagocytic lymphohistiocytosis in these patients.
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INTRODUCTION: The aim of this study was to investigate the serum exosome proteome profile of coronary artery dilatation (CAD) caused by Kawasaki disease (KD). METHODS: Two-dimensional electrophoresis was implemented on proteins of serum exosomes obtained from children with CAD caused by KD and from healthy controls. Differentially expressed proteins were identified by matrix-assisted laser desorption/ionization time-of-flight/time-of-flight mass spectrometry analysis. RESULTS: We identified 38 differentially expressed proteins (13 up-regulated and 25 down-regulated) from serum exosomes of patients with CAD caused by KD compared with healthy controls. Expression levels of three differentially expressed proteins (leucine-rich alpha-2-glycoprotein, sex hormone-binding globulin, and serotransferrin) were validated using western blot analysis. Classification and protein-protein network analysis showed that they are associated with multiple functional groups involved in the acute inflammatory response, defense response, complement activation, humoral immune response, and response to wounding. The majority of the proteins are involved in the inflammation and coagulation cascades. CONCLUSIONS: These findings establish a comprehensive proteome profile of CAD caused by KD and increase our knowledge of scientific insight into its mechanisms.
Subject(s)
Coronary Artery Disease/etiology , Exosomes , Mucocutaneous Lymph Node Syndrome/genetics , Proteomics , Coronary Artery Disease/genetics , Coronary Vessels , Dilatation , Electrophoresis, Gel, Two-Dimensional , Humans , Mucocutaneous Lymph Node Syndrome/complications , ProteomeABSTRACT
In this case, we describe a newborn that presented on the first day of life with diffuse, bilateral coronary artery dilatation, in the absence of intrauterine hypoxia or other identifiable causes of coronary artery ectasia. The infant's symptoms followed an acute course before spontaneously recovering. Kawasaki disease, though relatively rare in neonates, may present in this population in the absence of classical criteria. If untreated, the cardiac sequelae of this disease can be serious. Through this case, where spontaneously remitting coronary dilatation is the paramount finding, we entertain the possibility that this may represent the earliest known presentation of Kawasaki disease.
Subject(s)
Coronary Vessels/physiopathology , Mucocutaneous Lymph Node Syndrome/diagnosis , Dilatation, Pathologic , Echocardiography , Humans , Infant, Newborn , Male , Remission, SpontaneousABSTRACT
PURPOSE: This study investigated the epidemiology of Kawasaki disease (KD) in infants ≤3-month-old. METHODS: To study the epidemiology of KD in Korea, data for 27,851 KD patients were collected on a 3-year basis between 2000 and 2008 in a retrospective survey. From this, data for 609 KD patients ≤3-month-old were analyzed and compared with the data for KD patients >3-month-old. RESULTS: The 609 KD patients ≤3-month-old (385 males and 224 females) constituted 2.2% of the KD patients. They included 25 infants <1-month-old, 198 infants ≤1- to 2-month-old, and 386 infants >2- and 3-months-old. The ratio of males to females was 1.72:1. The incidence of coronary artery (CA) dilatation (19.9% vs. 18.7%) and CA aneurysms (3.4% vs. 2.6%) detected by echocardiography did not differ significantly between patients with KD younger and older than 3-month-old. CONCLUSION: Compared with the data for the KD patients >3-month-old, the data for the 609 patients ≤3-month-old did not show a significantly higher incidence of CA dilatation or CA aneurysms.
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PURPOSE: This study investigated the epidemiology of Kawasaki disease (KD) in infants 3-month-old. RESULTS: The 609 KD patients 2- and 3-months-old. The ratio of males to females was 1.72:1. The incidence of coronary artery (CA) dilatation (19.9% vs. 18.7%) and CA aneurysms (3.4% vs. 2.6%) detected by echocardiography did not differ significantly between patients with KD younger and older than 3-month-old. CONCLUSION: Compared with the data for the KD patients >3-month-old, the data for the 609 patients < or =3-month-old did not show a significantly higher incidence of CA dilatation or CA aneurysms.
Subject(s)
Female , Humans , Infant , Male , Aneurysm , Coronary Aneurysm , Coronary Vessels , Dilatation , Echocardiography , Incidence , Korea , Mucocutaneous Lymph Node Syndrome , Retrospective StudiesABSTRACT
PURPOSE: Kawasaki disease is an acute febrile vasculitis that may cause coronary artery dilatation after the acute phase. We assessed the risk factors of coronary artery dilatation in patients diagnosed with Kawasaki disease. METHODS: From January 1995 through April 2003, a total of 362 patients with Kawasaki disease were enrolled. After dividing these patients into two groups by presence of coronary artery dilatation, we analyzed and compared the early symptoms and laboratory findings. RESULTS: A total of 362 patients were divided into two groups: Group 1 consisted of 65 patients(18.0%) with coronary artery dilatation, while Group 2 consisted of 297 patients(82.0%) with normal coronary arteries. Group 1 exhibited longer duration of fever during the early symptoms and showed elevated levels of CRP and cardiac Troponin-I(cTnI). WBC count, hemoglobin, hematocrit, platelet count, erythrocyte sedimentation rate, hepatic enzymes, serum albumin, CK-MB, total eosinophil count, age and sex were not significantly different between the two groups. CONCLUSION: As the severity of early inflammatory reaction increased, the risk of coronary artery dilatation increased accordingly. Therefore it is essential to establish a treatment to reduce the early inflammatory reaction in order to decrease coronary artery complications.