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*

Myocarditis is inflammation, necrosis and myocytolysis of myocardium by many infectious, connective tissue, granulomatous, toxic, or idiopathic process. Viruses are probably the most important cause of myocarditis. But the exact cause remains unknown in the majority of patients with presumed myocarditis. We describe a case of myocarditis by measles virus in a 11-year-old girl with a brief review of related literatures.
Child ; Connective Tissue ; Female ; Humans ; Inflammation ; Measles virus* ; Measles* ; Myocarditis* ; Myocardium ; Necrosis

Congenital complete atrioventricular heart block(CCAVB) is a rare disease of the newborn that carries significant mortality and has a heterogenous etiology. It may occur as a result of the presence of maternal autoantibodies that are transferred to the fetus and affect the fetal heart or be associated with a congenital structural abnormality of the heart. Infants with CCAVB are at risk of diminished cardiac output and the subsequent development of congestive heart failure. We report two cases of CCAVB in newborns treated with pacemaker implantation after birth. The first case revealed CCAVB with patent ductus arteriosus and anti- Ro(SS-A) antibody in both of the mother and the newborn. The second case was accompanied with mitral regurgitation and tricuspid regurgitation, but anti-Ro(SS-A) antibody was absent in both of the mother and the newborn. Pacemaker implantation was performed for both cases and the result was favorable. We concluded that our experience supports that the pacemaker implantation in the newborn is feasible and beneficial in the treatment of CCAVB.
Atrioventricular Block* ; Autoantibodies ; Cardiac Output ; Ductus Arteriosus, Patent ; Fetal Heart ; Fetus ; Heart ; Heart Failure ; Humans ; Infant ; Infant, Newborn ; Mitral Valve Insufficiency ; Mortality ; Mothers ; Parturition ; Rare Diseases ; Tricuspid Valve Insufficiency

PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) can present as heart failure or arrhythmias in a child. It is a rare disorder, characterized by prominent trabecular meshwork and deep intertrabecular recesses. We still know little about the diagnosis, symptoms, and clinical outcomes of INVM. METHODS: We included in our study 6 patients who showed ventricular noncompaction on echocardiography. Patients were diagnosed as INVM were excessively prominent trabeculations with deep intertrabecular recesses were found on echocardiography. Patients who had other complex heart lesions such as pulmonary atresia with intact ventricular septum in addition to ventricular noncompaction, were excluded. RESULTS: Age at presentation ranged from 1 day 7 years, with follow up being as long as 6 years. Symptoms at initial presentation were heart murmur, paroxysmal supraventricular tachycardia, cyanosis, feeding intolerance, ventricular tachycardia, and cardiomegaly at fetal screening. Prominent trabeculations and intertrabecular recesses were observed at left ventricular apex in all six patients. All patients were alive at last follow-up. One patient showed WPW syndrome on electrocardiography. Echocardiography revealed decreased systolic function in 4 patients, and decreased systolic and diastolic function in 1 patient. One patient is currently asymptomatic. CONCLUSION: Six patients were diagnosed with INVM with various symptoms at initial presentation. Echocardiography is the most important tool in the diagnosis of INVM due to its morphological characteristics. INVM can rarely be the cause of long term systolic dysfunction, and early detection by echocardiographic screening may be beneficial.
Arrhythmias, Cardiac ; Cardiomegaly ; Child ; Cyanosis ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart ; Heart Failure ; Heart Murmurs ; Humans ; Isolated Noncompaction of the Ventricular Myocardium* ; Mass Screening ; Pulmonary Atresia ; Tachycardia, Supraventricular ; Tachycardia, Ventricular ; Trabecular Meshwork ; Ventricular Septum ; Wolff-Parkinson-White Syndrome

PURPOSE: Dexamethasone is a well-known treatment for preterm infants with bronchopulmonary dysplasia. However, serious side effects have been identified, including hypertension, and cardiac hypertrophy. This study was undertaken to examine whether dexamethasone induced cardiac hypertrophy in preterm infants with bronchopulmonary dysplasia. METHODS: We retrospectively reviewed 12 infants with bronchopulmonary dysplasia treated with dexamethasone at Korea University Hospital from August 1995 to February 1999. Serial two-dimensional and M-mode echocardiographic measurements were taken before treatment and at 1,2,3,4,5 weeks after the start of dexamethasone therapy. RESULTS: Patients receiving dexamethasone had a significantly increase in interventricular septal thickness and left ventricular posterior wall thickness. These effects were transient, reached their maximal degree by the third week of treatment, and approached pretreatment conditions by the fifth week of treatment. Heart rate was increased but statistically not significant. The fractional shortening, systolic and diastolic arterial pressure were transiently increased during dexamethasone therapy. CONCLUSION: We conclude that a transient myocardial hypertrophy is associated with dexamethasone therapy in infants with bronchopulmonary dysplasia. Follow up of careful echocardiac monitoring should be considered in infants with dexamethasone therapy.
Arterial Pressure ; Bronchopulmonary Dysplasia ; Cardiomegaly* ; Cardiomyopathy, Hypertrophic* ; Dexamethasone* ; Echocardiography ; Follow-Up Studies ; Heart Rate ; Humans ; Hypertension ; Hypertrophy* ; Infant ; Infant, Newborn ; Infant, Premature* ; Korea ; Retrospective Studies

No Abstract available.
Double Outlet Right Ventricle*