Multiple cardiac myxomas are rare in children. However, myxomas may be lethal because of their various manifestations such as blood flow obstruction, embolization and constitutional changes. Especially, the cerebral infarction due to tumor fragmentation are more likely to be misdiagnosed of acute disseminated encephalomyelitis. We report a case of multiple cardiac myxoma complicating recurrent right hemiparesis in a 12-year-old child who at first had a wrong diagnosis of acute disseminated encephalomyelitis (ADEM). Consequently, a child who show unrepresentative symptom of ADEM, should be examined rapidly by various tools to rule out the cerebral infarction from cardiogenic cause.
Cerebral Infarction ; Child* ; Diagnosis* ; Encephalomyelitis, Acute Disseminated* ; Humans ; Myxoma* ; Paresis*

Leukocytosis and neutrophilia is common during the acute phase of Kawasaki disease whereas leukopenia is not common and severe neutropenia is rare. Severe neutropenia is defined as absolute neutrophil count less than 500/mm3. There are only few publicatons reporting of atypical Kawasaki disease with severe neutropenia. We report a case of atypical Kawasaki disease with severe neutropenia.
Leukocytosis ; Leukopenia ; Mucocutaneous Lymph Node Syndrome* ; Neutropenia* ; Neutrophils

PURPOSE: Glutathione-S-transferase (GST) is a phase II metabolism enzyme and plays an important role in the detoxification of various chemicals. Recently, the genetic background of Kawasaki disease has been investigated by some researchers and significant results were documented. To demonstrate genetic background of pathogenesis of Kawasaki disease (KD), we examined the genetic polymorphism of Glutathione-S-transferase in KD patients. METHODS: Sixty-seven Korean children with Kawasaki disease and 252 Korean healthy controls were enrolled in this study. The polymorphisms of GSTM1 and GSTT1 gene were analyzed. RESULTS: The distribution of GSTM1 polymorphism between Kawasaki disease patients and controls was not significantly different [2=0.6479, P=0.4209; odds ratios (95% confidence intervals)=0.8004 (0.4651-1.3772)]. However, the distribution of GSTT1 polymorphism was significantly different between two groups [2=18.7898, P<0.0001; odds ratios (95% confidence intervals)=4.1009 (2.0950- 8.0274)]. In the combined analysis of GSTM1 and GSTT1 genes, the frequency of both null type of GSTM1/T1 genes was significantly different from both positive type of GSTM1/T1 genes [2= 8.2528, P=0.0041; odds ratios (95% confidence intervals)=4.1486 (1.4846-11.5927)]. The group of the GSTM1 positive and GSTT null type also showed significantly different from both positive type of GSTM1/T1 genes [2=17.3479, P<0.0001; odds ratios (95% confidence intervals)=6.9143 (2.5387- 18.8314)]. CONCLUSION: These results indicates that the polymorphisms of GSTT1 gene might be a susceptible factor in development of Kawasaki disease in Koreans.
Child* ; Humans ; Metabolism ; Mucocutaneous Lymph Node Syndrome* ; Odds Ratio ; Polymorphism, Genetic

PURPOSE: Recently most of patients with Kawasaki disease (KD) get treatment within several days of illness. But, some patients still suffer from coronary complication (CC) despite early initial treatment with intravenous immune globulin (IVIG) and even the additional therapy. We investigated the risk factors of CC in non-responders to initial therapy who needed additional IVIG infusion among patients with KD. METHODS: Forty five non-responders to initial IVIG who got additional IVIG infusion were reviewed from 1996 to 2007. We devided patients into two groups with CC (group A, n=17) or without CC (group B, n=28). Clinical characteristics, timing of additional IVIG infusion and laboratory results were reviewed. And we investigated the differences between the two groups and tried to find risk factors of CC. RESULTS: In comparison between the two groups, CC, clinical characteristics and timing of IVIG infusion were not different between two groups. But, total febrile days were significantly longer and peak platelets counts were significantly higher in group A (P=0.006, P=0.013). On the logistic regression analysis, total febrile days longer than 10.5 days was the only risk factor of CC in these patients. CONCLUSION: Our results showed that patients with CC inspite of repeated IVIG therapy had longer fever duration. So, additional therapy besides re-treatment with IVIG aiming at shortening total duration of fever seems to be important in refractory KD to prevent CC.
Coronary Aneurysm ; Fever ; Humans ; Immunoglobulins, Intravenous ; Logistic Models ; Mucocutaneous Lymph Node Syndrome* ; Retreatment* ; Risk Factors

PURPOSE: In developed countries, acute rheumatic fever appears to be a vanishing disease. In Korea, the incidence and severity of acute rheumatic fever (ARF) has significantly decreased in recent 30 years. According to this report, Korea sustained low incidence of ARF. METHODS: The medical records of 5 children diagnosed as ARF from January 2000 to September 2006 were reviewed retrospectively about clinical manifestations and laboratory findings. RESULTS: The average incidence of rheumatic fever was 0.17 per annual pediatric in-ward 1,000 patients. During study period, only 1 case had a definite history of preceding infection. Among 5 patients, the incidence of major manifestations were as follows:carditis 5 cases, chorea 1 case, arthritis 1 case and erythema marginatum 2 cases. Clinical findings of carditis were cardiac murmur, cardiomegly, congestive heart failure and pericardial effusion. Significant valvular lesions were mitral and aortic insufficiency. Minor manifestations and other clinical manifestations were fever, arthralgia, dyspnea, coughing, palpitation, weakness and chest pain. Laboratory findings were increased antistreptolysin O titer, positive C reactive protein (CRP) and increased erythrocyte sedimentation rate (ESR). CONCLUSION: The incidence of ARF has reduced but rheumatic carditis varies in severity from moderate to severe cardiac involvement. For many reasons ARF is being diagnosed inappropriately resulting from lack of awareness about the disease due to rarity and secondary prophylaxis. We should be aware of acute rheumatic fever and should pay attention to the treatment of the patients with streptococcal pharyngitis.
Antistreptolysin ; Arthralgia ; Arthritis ; Blood Sedimentation ; C-Reactive Protein ; Chest Pain ; Child ; Chorea ; Cough ; Developed Countries ; Dyspnea ; Erythema ; Fever ; Heart Failure ; Heart Murmurs ; Humans ; Incidence ; Korea ; Medical Records ; Myocarditis ; Pericardial Effusion ; Pharyngitis ; Retrospective Studies ; Rheumatic Fever*

Kawasaki disease (KD) is an acute multisystem vasculitic syndrome of unknown etiology occurring mostly in infants and children younger than 5 years of age. In developed countries, KD is currently the leading cause of acquired heart diseases in children. The incidence of KD is increasing globally. The first generation of patients with KD has now reached reproductive age, and the incidence of 2-generation cases may thus increase in number. Many gene polymorphisms are associated with KD in different populations. There are many individual and racial gene variation. Susceptible gene should be identified in the KD. Individuals with certain genes predisposing to occurrence and severity of KD could be detected in different ethnic populations. This might allow special treatment or other measures to prevent the disease and possibly limit the severity and complications of KD. I reviewed and summarized Korean and international articles about gene studies. Multicenter gene studies are needed to investigate the association between specific genes and coronary artery lesions in KD.
Child ; Coronary Vessels ; Developed Countries ; Heart Diseases ; Humans ; Incidence ; Infant ; Mucocutaneous Lymph Node Syndrome*

Evidence-based medicine is a medical movement combining professional experience with the best scientific research results. Treatment of Kawasaki disease in the acute phase is directed at reducing inflammation in the coronary artery wall and preventing coronary thrombosis, whereas long-term therapy in individuals who develop coronary aneurysms is aimed at preventing myocardial ischemia or infarction. I attempt to summarize the current state of knowledge of the medical management of patients with Kawasaki disease. The recommendations are evidence-based and derived from published data wherever possible.
Coronary Aneurysm ; Coronary Thrombosis ; Coronary Vessels ; Evidence-Based Medicine ; Humans ; Infarction ; Inflammation ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Ischemia

Kawasaki disease (KD) is an acute systemic febrile illness, predominantly affecting children less than the age of 5 years. After its first description in 1967 by Dr Tomisaku Kawasaki, KD has become the leading cause of acquired heart diseases in children in developed nations. KD has been reported in all racial and ethnic groups but the incidence of KD is more common among Asians, and the incidence of the disease varies considerably among different Asian countries. There is a higher rate of KD reported in Asian countries such as Japan and Korea than in other countries. which suggests differences of race-specific susceptibility. In Korea, from 1990s, a nationwide epidemiologic study has been conducted every 3 years by the Korean Pediatric Heart Association to determine the epidemiologic patterns and incidence rate of KD in Korea. So the average annual incidence, 105.0/100,000 children < 5 years in recent study (2003-2005) was the second highest reported rate in the world after Japan.
Asian Continental Ancestry Group ; Child ; Developed Countries ; Epidemiologic Studies ; Epidemiology ; Ethnic Groups ; Heart ; Heart Diseases ; Humans ; Incidence ; Japan ; Korea* ; Mucocutaneous Lymph Node Syndrome* ; Prevalence

No abstract available.
Heart Diseases* ; Heart* ; Humans ; Palivizumab

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), also termed Bland-White-Garland syndrome, is a rare congenital cardiac anomaly characterized by myocardial ischemia which becomes symptomatic during early infancy leading to infarction, left heart failure or even death. More than 90% of patients can be dead within first year of life if untreated, so adult type ALCAPA without surgical correction is quitely rare. We present a case of a 20-year-old man with ALPACA syndrome whose diagnosis took several years to be made because he was mistaken to have organic mitral regurgitation.
Adult* ; Bland White Garland Syndrome ; Camelids, New World ; Coronary Vessels* ; Diagnosis ; Heart Failure ; Humans ; Infarction ; Mitral Valve Insufficiency ; Myocardial Ischemia ; Pulmonary Artery* ; Young Adult