No Abstract available.
Heart Defects, Congenital* ; Hemodynamics*

No abstract available.
Heart Defects, Congenital*

The clinical data of 27 patiets with hypophosphatemic rickets treated with phosphate and 1alpha-hydroxyvitamin D were analysed retrospectively. The median age at diagnosis was 4 years, and the main clinical manifestations were bowleg and short stature. Among total 24 families, 5 families (21%) had X-linked dominant mode of inheritance, 1 family (4%) had autosomal dominant mode and 17 families (71%) had no family history, The serum phosphorus concentration rose from initial value of 2.7+/-0.13mg/dl to 3.5+/-0.19mg/dl. The serum alkaline phosphatase was reduced from 871+/-63IU/L to 393+/-41IU/L. Healing of rickets was demonstrated by radiography. Patients treated for at least two years before the onset of puberty had an increase in the mean height SD score from -1.58 to -0.79. Orthopedic surgeries for severe lower extremity deformity were performed in 11 patients, of whom 8(73%) were dignosed over 5 years of age. Complications of therapy were as follows; 12 patients (44%) developed more than one episode of hypercalciuria, 5 patients (19%) developed more than one episode of hypercalcemia, and nephrocalcinosis was noted in 5(33%) out of 15 patients by renal ultrasound. The group with nephrocalcinosis had a higher incidence of hypercalemic episodes than the group without nephrocalcinosis. In conclusion, treatment of hypophosphatemic rickets results in healing of rickets and acceleration of growth, and we must evaluate complications of therapy such as hypercalcemia and nephrocalcinosis.
Acceleration ; Adolescent ; Alkaline Phosphatase ; Congenital Abnormalities ; Diagnosis ; Humans ; Hypercalcemia ; Hypercalciuria ; Incidence ; Lower Extremity ; Nephrocalcinosis ; Orthopedics ; Phosphorus ; Puberty ; Radiography ; Retrospective Studies ; Rickets ; Rickets, Hypophosphatemic* ; Ultrasonography ; Wills

BACKGROUND: It has been reported that sudden cardiac death can occur in postoperative tetralogy of Fallot (TOF) and this episode is related to the sustained ventricular tachyarrhythmia. However, the connecting mechanism of various clinical features and sudden cardiac death is not defined yet. Based on the reports that sudden cardiac death is related to the alteration of heart rate dynamics in some diseases, investigation of heart rate dynamics in postoperative TOF seems important to reveal the mechanism of sudden cardiac death. This study was done as a part of the project to investigate the alteration of heart rate variability and to identify the factors related to the sudden cardiac death in postoperative TOF. METHODS: Among the children with postoperative TOF, 13 asymptomatic children without significant hemodynamic disturbances (Doppler velocity for tricuspid regurgitation 2.5m/sec and mild pulmonary regurgitation) and ventricular arrhythmia were selected (10 boys, 3 girls; mean age, 9 year 3 months; mean duration after operation, 7 years). We evaluated the complexity and periodic dynamics of heart rate (HR) throughout 24 hours in these children and control group (mean age, 8 year 10 months). After examining each ECG data on 24-hour Holter ECG segmented into 1-hour length, we analyzed each HR time series and quantified the overall complexity of each HR time series by its correlation dimension. In addition, the power spectrum of HR and obtained low-frequency component (0.08-0.15Hz) and high-frequency component (0.15-0.4Hz) were calculated. RESULTS: This study showed that TOF group had lower correlation dimension and a nonlinear characteristics of HR time series than control group (4.055+/-0.4134 vs. 4.9310+/-0.2054, p<0.05). However, the difference was not significant in the low- (0.9864+/-0.5598 vs. 1.5560+/-0.8325, p<0.05) and high- (1.1168+/-0.1.1448 vs. 0.9271+/-0.6528, p<0.05) frequency components. CONCLUSIONS: It can be concluded that HR time series are more regular in postoperative TOF. The result was significant in correlation dimension analysis and not in the frequency domain analysis. Although the meaning of this finding in postoperative TOF is not clear at present, this may suggest the phenomenon related to the sudden cardiac death.
Arrhythmias, Cardiac ; Child* ; Death, Sudden, Cardiac ; Electrocardiography ; Female ; Heart Rate* ; Heart* ; Hemodynamics ; Humans ; Tachycardia ; Tetralogy of Fallot* ; Tricuspid Valve Insufficiency

BACKGROUND: It has been reported that sudden cardiac death can occur in postoperative tetralogy of Fallot (TOF) and this episode is related to the sustained ventricular tachyarrhythmia. However, the connecting mechanism of various clinical features and sudden cardiac death is not defined yet. Based on the reports that sudden cardiac death is related to the alteration of heart rate dynamics in some diseases, investigation of heart rate dynamics in postoperative TOF seems important to reveal the mechanism of sudden cardiac death. This study was done as a part of the project to investigate the alteration of heart rate variability and to identify the factors related to the sudden cardiac death in postoperative TOF. METHODS: Among the children with postoperative TOF, 13 asymptomatic children without significant hemodynamic disturbances (Doppler velocity for tricuspid regurgitation 2.5m/sec and mild pulmonary regurgitation) and ventricular arrhythmia were selected (10 boys, 3 girls; mean age, 9 year 3 months; mean duration after operation, 7 years). We evaluated the complexity and periodic dynamics of heart rate (HR) throughout 24 hours in these children and control group (mean age, 8 year 10 months). After examining each ECG data on 24-hour Holter ECG segmented into 1-hour length, we analyzed each HR time series and quantified the overall complexity of each HR time series by its correlation dimension. In addition, the power spectrum of HR and obtained low-frequency component (0.08-0.15Hz) and high-frequency component (0.15-0.4Hz) were calculated. RESULTS: This study showed that TOF group had lower correlation dimension and a nonlinear characteristics of HR time series than control group (4.055+/-0.4134 vs. 4.9310+/-0.2054, p<0.05). However, the difference was not significant in the low- (0.9864+/-0.5598 vs. 1.5560+/-0.8325, p<0.05) and high- (1.1168+/-0.1.1448 vs. 0.9271+/-0.6528, p<0.05) frequency components. CONCLUSIONS: It can be concluded that HR time series are more regular in postoperative TOF. The result was significant in correlation dimension analysis and not in the frequency domain analysis. Although the meaning of this finding in postoperative TOF is not clear at present, this may suggest the phenomenon related to the sudden cardiac death.
Arrhythmias, Cardiac ; Child* ; Death, Sudden, Cardiac ; Electrocardiography ; Female ; Heart Rate* ; Heart* ; Hemodynamics ; Humans ; Tachycardia ; Tetralogy of Fallot* ; Tricuspid Valve Insufficiency

c-erbB-2 oncogene is a normal cellular proto-oncogene coding transmembrane glycoprotein structurally similar to the epidermal growth factor receptor. Amplification of this oncogene in a variety of human adenocarcinomas has been reported and is particularly well documented in breast carcinoma. It has been suggested that amplification of this oncogene is indicative of poor prognosis and is valuable only second to the lymph node status. Using immunohistochemical staining for the c-erbB-2 protein, overexpression of this protein was analysed in 228 primary breast cancer specimens and the frequency of overexpression and the relationship between overexpression and the other established prognostic variables are evaluated. Ninty three cases out of 228 cases(40.8%) show postive oncoprotein overexpression and using the chi-squared test for a trend, a significant correlation was found between c-erbB-2 protein staining and the histological grade, lymph node status, and estrogen receptor status(P<0.05). No significant association was found between staining and the patient's age and tumor size. Most of the tumors with histological types known to have good prognosis showed negative expression. Above findings strongly suggest that expression of c-erbB-2 oncogene is another independent indicator of poor prognosis in breast carcinoma.
Humans ; Adenocarcinoma ; Breast Neoplasms

Serum alkaline phosphatase (AP) activity is elevated in hepatobiliary disease, bone disease, pregnancy and certain neoplasms. Recently we experienced marked elevation of serum AP activity after administration of albumin preparation in nephrotic patients who suffered from hypovolemic symptoms. So serum AP activity and the isoenzymes in the albumin preparations & patient's serum after the administration of albumin preparation were studied. Serum AP activity was significantly higher after administration of albumin preparation (318+/-101 IU/L) then before (123+/-43 IU/L). The predominant isoenzyme after administration of albumin was placental type, while liver and bone type was predominant before administration. AP activity in albumin preparation was high (2,133+/-1,410 IU/L) and the isoenzyme was mostly placental type. So we concluded that marked elevation of serum AP activity after administration of albumin was traced to the placental type AP isoenzyme in some albumin preparations which was manufactured from the plasma of placental origin. Elevated serum AP activity like these may lead to erroneous interpretation. Manufactures should notify alkaline phosphatase activity in albumin preparations of placental origin.
Administration, Intravenous* ; Alkaline Phosphatase* ; Bone Diseases ; Humans ; Hypovolemia ; Isoenzymes ; Liver ; Plasma ; Pregnancy

The use of recombinant DNA technology to produce human growth hormone has resulted in a marked increase in availability of Growth Hormone(GH) to treat short stature due to GH deficiency and other conditions, such as Turner syndrome, familial short stature, chronic renal insufficiency and intrauterine growth retardation (IUGR). But, the GH therapy may result in the adverse events such as sodium and water retention, pseudotumor cerebri, slipped capital femoral epiphysis, growth of nevi, recurrence of tumor. We experienced a case of severe hypertension associated with GH therapy in a 14-year-old male who presented high blood pressure up to 190/100 mmHg and normalized at 2-3 weeks after discontinuation of GH. Therefore, we think that the blood pressure should be carefully monitored during GH therapy.
Adolescent ; Blood Pressure ; DNA, Recombinant ; Fetal Growth Retardation ; Growth Hormone* ; Human Growth Hormone ; Humans ; Hypertension* ; Male ; Nevus ; Noonan Syndrome ; Pseudotumor Cerebri ; Recurrence ; Renal Insufficiency, Chronic ; Slipped Capital Femoral Epiphyses ; Sodium

Patients with congenital heart diseases (CHD) are confronted with early- and late-onset complications, such as conduction disorders, arrhythmias, myocardial dysfunction, altered coronary flow, and ischemia, throughout their lifetime despite successful hemodynamic and/or anatomical correction. Rhythm disturbance is a well-known and increasingly frequent cause of morbidity and mortality in patients with CHD. Predisposing factors to rhythm disturbances include underlying cardiac defects, hemodynamic changes as part of the natural history, surgical repair and related scarring, and residual hemodynamic abnormalities. Acquired factors such as aging, hypertension, diabetes, obesity, and others may also contribute to arrhythmogenesis in CHD. The first step in evaluating arrhythmias in CHD is to understand the complex anatomy and to find predisposing factors and hemodynamic abnormalities. A practical stepwise approach can lead to diagnosis and prompt appropriate interventions. Electrophysiological assessment and management should be done with integrated care of the underlying heart defects and hemodynamic abnormalities. Catheter ablation and arrhythmia surgery have been increasingly applied, showing increasing success rates with technological advancement despite complicated arrhythmia circuits in complex anatomy and the difficulty of access. Correction of residual hemodynamic abnormalities may be critical in the treatment of arrhythmia in patients with CHD.
Aging ; Arrhythmias, Cardiac ; Catheter Ablation ; Cicatrix ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Hemodynamics ; Humans ; Hypertension ; Ischemia ; Natural History ; Obesity ; Resin Cements ; Risk Factors

We investigated the interrelations between surface electrocardiographic changes and clinical outcomes in children with idiopathic dilated cardiomyopathy (DCMP). 33 patients (19 boys, 14 girls) were classified into two groups; group I (15) who were in poor clinical status or dead; and group II (18) who showed good clinical status. Group I had larger LV dimensions compared to group II (Gr I vs. Gr II; LVEDD, 52 +/-11 vs. 42+/-7 (mm); LVESD, 43+/-12 vs. 30+/-5 (mm); p<0.05). QRS duration was prolonged in Gr I compared to Gr II and normal (Gr I, 84+/-28; Gr II, 66+/-12; normal control, 67+/-9). The QRS duration was correlated with the dimensions of left ventricle (LV). Corrected QT and JT interval and dispersions of QT in the DCMP group showed a significant difference compared to the normal control, however there was no significant difference between Gr I and II. In conclusion, QRS duration was correlated with ventricular dimension and clinical outcome in children with idiopathic dilated cardiomyopathy. Irrespective of increased ventricular inhomogeneity, QT dispersion could not be used to predict long-term prognosis.
Cardiomyopathy, Dilated/*diagnosis/*mortality ; Child ; Child, Preschool ; *Electrocardiography ; Electrodes ; Female ; Humans ; Infant ; Male ; Predictive Value of Tests ; Prognosis ; Retrospective Studies