No abstract available.
Anemia, Iron-Deficiency* ; Erythrocyte Indices* ; Iron*

No abstract available.

No abstract available.
Heart Atria*

PURPOSE: Deletion of chromosome 22q11 is associated with DiGeorge syndrome, velocardiofacial syndrome, and conotruncal anomaly face syndrome. This study was performed to determine the criteria of clinical phenotype as recognizable syndrome and to research the loss of heterozygosity in CATCH 22 patients and their family. METHODS: An evaluation of the clinical and genetic profiles of 30 persons of CATCH 22 syndrome or their family referred with a diagnosis of either congenital heart disease or cleft palate was undertaken. The deletions of 22q11 were analyzed using the fluorescences in situ hybridization(N25, Oncor) and short tandem-repeat polymorphic makers(STRP, D22S941). RESULTS: The dysmorphic features of CATCH 22 showed considerable overlap and intrafamilial difference was common. The familial cases of CATCH 22 were transmitted maternally as autosomal dominant. The target gene study using the STRP maker(D22S941) in these series showed good clinico-genetic correlation but some heterogeneity. CONCLUSION: Although 22q11 deletion was large in size and high variable in polymorphic markers, extensive evaluation clinically as well as genetically will be necessary for subgrouping of CATCH 22 syndrome due to good clinicogenetic correlation. Furthermore, we also suggest the development of new polymorphic markers to research the unknown characteristics of polymorphic markers in Korean patients with CATCH 22 syndrome.
Cleft Palate ; Diagnosis ; DiGeorge Syndrome ; Heart Defects, Congenital ; Humans ; Loss of Heterozygosity ; Phenotype ; Population Characteristics

No abstract available.
Aconitum* ; Arrhythmias, Cardiac* ; Cardiopulmonary Resuscitation*

PURPOSE: The aim of this study is to assess the cardiac effect of long-term steroid therapy in nephrotic syndrome and the validity of LV functional parameters as an early predictor of subclinical cardiac dysfunction. METHODS: The study group was composed of 21 patients diagnosed as minimal change nephrotic syndrome(NS), being managed with prednisone over 6 months or within 6 weeks after stopping medication. The control group was composed of 23 healthy children without cardiopulmonary dysfunction. The functional parameters of the left ventricle, including systolic and diastolic indices were measured using 2D-doppler echocardiography. RESULTS: There was a significant increase of left ventricular mass index in the study group(P value<0.05). Isovolumetric relaxation time of the left ventricle was prolonged significantly in the study group(P value<0.05). The systolic phase area and the ratio of systolic and diastolic phase area of pulmonary vein flow profile were increased significantly in the study group(P value<0.05). CONCLUSION: In NS patients managed with long-term steroid therapy, left ventricular diastolic functional abnormality may be present even though functional limitation is not yet evident. In the future, the longitudinal study will be needed conderning the cardiac effect of long-term steroid treatment.
Child ; Echocardiography ; Heart Ventricles ; Humans ; Longitudinal Studies ; Nephrotic Syndrome* ; Prednisone ; Pulmonary Veins ; Relaxation

PURPOSE: The aim of this study is to assess the cardiac effect of long-term steroid therapy in nephrotic syndrome and the validity of LV functional parameters as an early predictor of subclinical cardiac dysfunction. METHODS: The study group was composed of 21 patients diagnosed as minimal change nephrotic syndrome(NS), being managed with prednisone over 6 months or within 6 weeks after stopping medication. The control group was composed of 23 healthy children without cardiopulmonary dysfunction. The functional parameters of the left ventricle, including systolic and diastolic indices were measured using 2D-doppler echocardiography. RESULTS: There was a significant increase of left ventricular mass index in the study group(P value<0.05). Isovolumetric relaxation time of the left ventricle was prolonged significantly in the study group(P value<0.05). The systolic phase area and the ratio of systolic and diastolic phase area of pulmonary vein flow profile were increased significantly in the study group(P value<0.05). CONCLUSION: In NS patients managed with long-term steroid therapy, left ventricular diastolic functional abnormality may be present even though functional limitation is not yet evident. In the future, the longitudinal study will be needed conderning the cardiac effect of long-term steroid treatment.
Child ; Echocardiography ; Heart Ventricles ; Humans ; Longitudinal Studies ; Nephrotic Syndrome* ; Prednisone ; Pulmonary Veins ; Relaxation

PURPOSE: Toxicity caused by acetaminophen and its toxic mechanisms in the liver have been widely studied, including effects involving metabolism and oxidative stress. However, its adverse effects on heart have not been sufficiently investigated. This study evaluated the cardiac influence and molecular events occurring within the myocardium in rats treated with a dose of acetaminophen large enough to induce conventional liver damage. MATERIALS AND METHODS: Male rats were orally administered a single dose of acetaminophen at 1,000 mg/kg-body weight, and subsequently examined for conventional toxicological parameters and for gene expression alterations to both the heart and liver 24 hours after administration. RESULTS: Following treatment, serum biochemical parameters including aspartate aminotransferase and alanine aminotransferase were elevated. Histopathological alterations of necrosis were observed in the liver, but not in the heart. However, alterations in gene expression were observed in both the liver and heart 24 hours after dosing. Transcriptional profiling revealed that acetaminophen changed the expression of genes implicated in oxidative stress, inflammatory processes, and apoptosis in the heart as well as in the liver. The numbers of up-regulated and down-regulated genes in the heart were 271 and 81, respectively, based on a two-fold criterion. CONCLUSION: The induced expression of genes implicated in oxidative stress and inflammatory processes in the myocardium reflects molecular levels of injury caused by acetaminophen (APAP), which could not be identified by conventional histopathology.
Acetaminophen/*toxicity ; Administration, Oral ; Analgesics, Non-Narcotic/*toxicity ; Animals ; Drug-Induced Liver Injury/pathology/*physiopathology ; Gene Expression Profiling ; Heart/*physiology ; Liver/pathology/physiology ; Male ; Myocardium/pathology ; Rats ; Transcriptome/*drug effects

No abstract available.
Catheters* ; Cystic Adenomatoid Malformation of Lung, Congenital* ; Fetal Therapies* ; Lung*

PURPOSE: Treatment of Kawasaki disease with intravenous gamma globulin(IVGG), together with aspirin, has been dernonstrated to be safe and effective in preventing coronary artery lesion and systemic inflarnmation, but optimal IVGG dosage and administration method are still controversial. We compared the therapeutic efficacy and clinical response of single IVGG 1g/kg to that of IVGG lg/kg for comparable risk group of Kawasaki disease. METHODS: We conducted a prospective study involving 63 children with Kawasaki disease requiring IVGG treatment(Harada score> or =4) at Chungnam National University Hospital from February 1996 to January 1999. The children were assigned to receive IVGG either as a single infusion of 1g/kg(A group, 32 person) or 2g/kg(B group, 31 person) and aspirn(100mg/kg/day through acute phase, then 3 to 5mg/kg/day for 8 weeks of duration). RESULTS: There were no significant difference between the two groups according to clinical and laboratry data, including coronary artery lesions(group A, 31.3% and group B, 29.0%) before treatment. After IVGG treatment ratio of complication with coronary artery lesion(group A 1/32=3.1% and group B, 2/31=6.5%) and that of retreatment(group A, 4/32=12.5%, group B, 2/31=6.5%), duration of fever(group A, 1.3+/-1.6 days and group B, 0.7+/-1.4 days), hospital stay(group A, 7.0+/-1.4 days and group B, 6.5+/-2.0 days), laboratory finding and side effects of IVGG were not significantly different(P>0.05). The total dosage of IVGG was significantly lower in group A than group B(group A, 1.16+/-0.37g/kg, 375,421+/-207,351won and group B, 2.10+/-0.40g/kg, 641,498+/-274,750won (P<0.05). CONCLUSION: The therapeutic efficacy and clinical response of single 1g/kg therapy are comparable to that of single 2g/kg therapy.
Aspirin ; Child ; Chungcheongnam-do ; Coronary Vessels ; gamma-Globulins* ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Prospective Studies