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*

A markedly enlarged right heat with a normal outflow tract was detected by fetal echocardiography in a fetus at 28 week's gestation. Follow-up scan at 32 week's gestation also showed asymmetric ventricles. The neonate were normal after delivery. Marked discrepancy between the sizes of the right and left ventricle generally indicates structural heart anomaly, such as coactation of aorta, hypoplastic left heat syndrome, or right ventricular outflow tract obstruction. But we experienced a case of marked fetal ventricular size discrepancy on 4 chamber view and turned out to be normal heart postnatally.
Aorta ; Echocardiography* ; Fetus ; Follow-Up Studies ; Heart ; Heart Ventricles ; Hot Temperature ; Humans ; Infant, Newborn ; Pregnancy

Coarctation of aorta(CoA) has been recognized to be cured by corrective operation, so many clinicians have discontinued follow-up early. However, high incidences of cardiovascular morbidity and mortality, of which causes have been known as persistent resting hypertension and exercise-induced hypertension after corrective operation, have been reported during long-term follow-up. And left ventricular mass increase associated with persistent resting hypertension and exercise-induced hypertension has been known as an independent risk factor of cardiovascular disease. So, even for successfully operated CoA patients, increased left ventricular mass as well as resting hypertension and exercise-induced hypertension must be detected early and normalized through persistent observation to adulthood.
Aortic Coarctation ; Cardiovascular Diseases ; Follow-Up Studies ; Heart Ventricles* ; Humans ; Hypertension ; Hypertrophy, Left Ventricular ; Incidence ; Mortality ; Risk Factors

PURPOSE: Prenatal diagnosis of congenital heart disease has been made by fetal echocardiography and its clinical impact on the outcome of CHD cases has been analysed. METHODS: A prospective study was performed for the fetal diagnosis of CHD for the standard risk pregnancy, confirmed postnatally or at second study and/or at autopsy and/or follow up at CHA hospital in 2001. Incidence of CHD has been calculated and compared to the incidence of CHD of the prescreening period(1992.3-1995.2). RESULTS: There were 66 cases of CHD prenatally during the study period. CHD consisted of 7 cases of ventricular septal defects(VSD), 7 cases of tetralogy of Fallot(TOF), 5 cases of heterotaxy, 5 cases of double outlet right ventricle(DORV), 5 cases of severe pulmonary stenosis(PS), 3 cases of hypoplastic left heart syndrome(HLHS), 3 cases of coarctation, 3 cases of critical aortic stenosis(AS), 3 cases of pulmonary atresia with intact ventricular septum(PA IVS). Among 66 fetal CHD cases, 30 cases of CHD has been terminated, 1 case died in utero and 23 cases has been delivered at cardiac center through planned delivery. The most common factors of termination were extracardiac and chromosomal anomaly. 17 cases of false negative diagnosis were small VSD and mild PS that couldn't be diagnosed in fetal stage. There was no false positive diagnosis. There were 30 cases of CHDs born in 2001 at CHA hospital. 28 cases were diagnosed postnatally. Among 30 cases of CHDs, there were 18 cases of VSD(17 small VSD), 6 cases of mild PS, 4 ASD, 1 PDA. Incidence rate of CHD in 2001 and period 1992.3-1995.2 were 5.95 and 10.15/1,000 livebirth respectively. CONCLUSION: The data suggested that all of the significant CHDs could be diagnosed prenatally accurately by fetal echocardiography. And the incidence rate of CHD has been decreased by 41.4%. And the most of the complex CHD has been transferred to the cardiac center for planned delivery or has been terminated. The rate of termination was 45.5%.
Autopsy ; Diagnosis* ; Echocardiography* ; Follow-Up Studies ; Heart ; Heart Defects, Congenital* ; Incidence ; Pregnancy ; Prenatal Diagnosis ; Prospective Studies ; Pulmonary Atresia

PURPOSE: This study was undertaken to determine the incidence of chromosome 22q11 deletion in patients with infundibular ventricular septal defect(VSD). METHODS: Sixty-two children with infundibular VSD were included in this study from January 1999 to December 2000. Chromosome 22q11 deletion was confirmed by FISH, using LSI DiGeorge/VCFS region dual color probe(Vysis, USA). RESULTS: Thirty-two patients had conotruncal cardiac defects:tetralogy of Fallot (TOF) in 15; TOF with absent pulmonary valve in 1; VSD with pulmonary atresia in 7; truncus arteriosus in 3; double outlet right ventricle in 2; interrupted aortic arch in 2; transposition of the great arteries in 2. Thirty patients had isolated infundibular VSD without conotruncal cardiac defect:perimembranous infundibular VSD in 15; subarterial infundibular VSD in 9; muscular infundibular VSD in 6. Chromosome 22q11 deletion was observed in 8 patients(male 5, female 3):TOF 2; VSD with pulmonary atresia 4; truncus arteriosus 1; perimembranous infundibular VSD 1. All of the patients with chromosome 21q11 deletion showed typical facial appearance. Low incidence was found of chromosome 22q11 deletion in patients with infundibular VSD without conotruncal cardiac defect than in those with conotruncal cardiac defect(3.3% vs 21.9%). CONCLUSION: These data indicate that a small proportion of isolated infundibular VSD is pathogenetically related to deletion of chromosome region 22q11.
Aorta, Thoracic ; Arteries ; Child ; Double Outlet Right Ventricle ; Female ; Heart Septal Defects, Ventricular* ; Humans ; Incidence ; Pulmonary Atresia ; Pulmonary Valve ; Truncus Arteriosus

No Abstract available.
Heart Transplantation* ; Heart*