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*

No abstract available.
Fontan Procedure* ; Heart*

No abstract available.
Fontan Procedure*

PURPOSE: Despite extracardiac conduit Fontan had many advantages, long-term results related to longevity of conduit, and anticoagulation were not proven. This study was to evaluate the long-term outcome of hospital survivors with extracardiac Fontan circulation. METHODS: Between 1996 and 2006, 200 patients underwent extracardiac conduit Fontan operation. Median age at the Fontan operation was 3.4 years (range:16 months-35.7 years). All patients (89.5 %) except 21 patients of one stage Fontan operation underwent bi-directional cavopulmonary shunt. Fenestration was required in 85 patients (42.5%). RESULTS: There has been 6 hospital mortalities (3.0%), and 7 late mortalities (3.6%) at a mean follow-up of 52.4+/-32.2 months (range; 18days-120 months). Overall 10-years survival was 92.4 2.1%. Multivariate analysis identified severe infection at early postoperative periods (hazard ratio =12.439, P=0.001), and high pulmonary arterial pressure at preoperative period (hazard ratio=3.445, P=0.038) as risk factors for mortality. Reoperation was performed in 24 patients (12.0%), and freedom from reoperation was 82.4+/-4.1% at 10 years. Arrhythmia occurred in 32 patients (16.0%) after Fontan operation, and freedom from arrhythmia was 85.14.4% at 10 years. Risk factors for arrhythmia were heterotaxy syndrome (P=0.001), Follow up duration (P=0.027) and the age at Fontan operation (P=0.001). Freedom from thromboembolism was 92.91.9% at 10 years. The conduit cross- sectional area decreased by 14%, and the extent of decrease of the conduit cross-sectional remained stable irrespective of the follow-up duration. 95.2% of patients had New York Heart Association class I. CONCLUSIONS: After 10 years of follow-up, the overall survival, and the functional status of survivors of the extracardiac Fontan procedure are satisfactory. Fenestration has a beneficial effect on the results of high-risk Fontan patients. The incidence of late deaths, reoperations, obstructions of the cavopulmonary pathway, arrhythmias and thromboembolisms is low.
Arrhythmias, Cardiac ; Arterial Pressure ; Follow-Up Studies ; Fontan Procedure* ; Freedom ; Heart ; Heart Bypass, Right ; Heterotaxy Syndrome ; Hospital Mortality ; Humans ; Incidence ; Korea* ; Longevity ; Mortality ; Multivariate Analysis ; Postoperative Period ; Preoperative Period ; Reoperation ; Risk Factors ; Survivors ; Thromboembolism

No abstract available.
Prognosis* ; Tetralogy of Fallot*

No abstract available.
Asian Continental Ancestry Group* ; Humans ; Mortality* ; Tetralogy of Fallot*