Infantile hypertrophic pyloric stenosis (IHPS) is the most common condition requiring abdominal surgery in early infancy, and is caused by hypertrophied pyloric muscle. The development of successful surgical treatment in the early 1900s by Fredet and Ramstedt made it possible for infants worldwide to survive. Modern pediatric anesthetic techniques have virtually eliminated mortality from surgical management. Atropine sulfate is a cholinergic blocking agent with potent antimuscarinic activity that decreases peristaltic contractions by relaxing smooth muscles. We treated two cases of IHPS with incomplete pyloromyotomy in 3-month-old and 5-month-old male infants by administering atropine sulfate intravenously. They were free from vomiting after 5 days of intravenous atropine sulfate treatment. In these rare cases of persistent vomiting or refractory emesis following incomplete pyloromyotomy, there may be a role for atropine sulfate.
Atropine* ; Constriction, Pathologic* ; Humans ; Infant ; Male ; Mortality ; Muscle, Smooth ; Pyloric Stenosis, Hypertrophic ; Vomiting*

No abstract available.
Cyclosporine* ; Dermatomyositis*

No abstract available.

The pulmonary sarcomatoid carcinoma is a rare malignant tumor, which is composed of an admixture of carcinomatous and sarcomatous components, and accounts for 0.3% of all pulmonary neoplasms. Clinicopathological features are often related to anatomical location: central endobronchial type and peripheral parenchymal type. Noninvasive diagnostic test had a low yield in detection sarcomatoid carcinoma. Metastasis to the regional lymph nodes and to distant organ is common. The prognosis is poor and the median survival is about 15 months. We report two cases of pulmonary sarcomatoid carcinoma with review of literatures.
Diagnostic Tests, Routine ; Lung Neoplasms ; Lung* ; Lymph Nodes ; Neoplasm Metastasis ; Prognosis ; Sarcoma

PURPOSE: To investigate the clinical effects of a single high dose intravenous immunoglobulin (IVIG) combined with initial dexamethasone as a primary treatment on Kawasaki disease (KD). MATERIALS AND METHODS: Between January 2008 and December 2010, we reviewed the medical records of 216 patients with complete KD patients that were admitted to a single medical center. 106 patients were treated with a single high dose of IVIG (2 g/kg) alone and 110 patients received IVIG and dexamethasone (0.3 mg/kg per day for three days). RESULTS: The combined IVIG plus dexamethasone patient group had a significantly shorter febrile period and duration of hospital stay (1.4+/-0.7 days vs. 2.0+/-1.2 days, p<0.001; 5.8+/-1.7 days vs. 6.9+/-2.5 days, p<0.001, respectively) than the IVIG alone group. The combined IVIG plus dexamethasone group required IVIG retreatment significantly less than the IVIG only group (12.7% vs. 32%, p=0.003). After completion of the initial IVIG, C-reactive protein levels in the combined IVIG plus dexamethasone group were significantly lower than those in the IVIG only group (2.7+/-4.0 mg/dL vs. 4.6+/-8.7 mg/dL, p=0.03). In the combined IVIG plus dexamethasone group, the incidence of coronary artery lesions tended to be lower without worse outcomes at admission after initial infusion of IVIG and in follow-up at two months; however, the differences were not significant (8.2% vs. 11.3%, p=0.22; 0.9% vs. 2.8%, p=0.29). CONCLUSION: Initial combined therapy with dexamethasone and a single high-dose of IVIG resulted in an improved clinical course, in particular a shorter febrile period, less IVIG retreatment, and shorter hospital stay without worse coronary outcomes.
Child, Preschool ; Dexamethasone/administration & dosage/adverse effects/*therapeutic use ; Female ; Fever/drug therapy ; Glucocorticoids/administration & dosage/adverse effects/*therapeutic use ; Humans ; Immunoglobulins, Intravenous/administration & dosage/adverse effects/therapeutic use ; Immunologic Factors/administration & dosage/adverse effects/*therapeutic use ; Infant ; Length of Stay ; Male ; Mucocutaneous Lymph Node Syndrome/*drug therapy ; Treatment Outcome

No Abstract available.
Intensive Care Units* ; Critical Care* ; Tachycardia*

PURPOSE: This study was designed to define normal values for a nongeometric MPI in children and evaluate the utility of m%cardial performance index(MPI) in congenital heart disease with distorted ventricular geometry. METHODS: The study population consisted of 44 normal patients and 28 patients with atrial septal defect(ASD) with dilated right ventricle and paradoxical septal motion. Right ventricular(RV) and left ventricular(LV) isovolumic contraction time, isovolumic relaxation time, ejection time, ejection fraction and pre-ejection period/ejection time(PEP/ET) were measured using conventional echo- Doppler methods. The MPI measures the ratio of total time spent in isovolumic activity(isovolumic contraction time and isovolumic relaxation time) to ejection time. RESULTS: In normal children, the RV MPI was 0.33+/-0.09 and the LV MPI was 0.36+/-0.04. In the ASD group, LV function seemed grossly normal but LV EF was significantly lower than normal children(57+/-3% vs 67+/-4%, P<0.05), but both LV and RV MPI had no statistically significant difference. After correction of ASD, RV MPI was increased and LV EF(57+/-3% vs 64+/-3%, P<0.05) as wll as the RV PEP/ET(0.27+/-0.05 vs 0.38+/-0.06, P<0.05) were significantly greater than the normal or the preoperation group. CONCLUSION: Components of the MPI are easily measured with conventional Doppler technique using standard echocardiographic examination. These results suggest that the MPI is useful as a means of quantitative assessment of ventricular performance in patients with complex ventricular geometry, particularly RV.
Child ; Echocardiography ; Heart Defects, Congenital ; Heart Septal Defects, Atrial* ; Heart Ventricles ; Humans ; Reference Values ; Relaxation ; Ventricular Function*

BACKGROUND: The prostaglandin E1(PGE1) is a well known protent dilator of arteriosus. Maintaining of the patency of ductus arteriosus is crucial for the survival of patients suffering from ductus-dependent cyanotic congenital heart disease. We aimed to analyse the efficacy and the influencing factors upon PGE1 in patients suffering from this disease. METHODS: Between May 1991 and April 1993, 26 neonates and infants with ductus- dependent cyanotic congenital heart disease received on intravenous infusion of PGE1 in the Division of Pediatric Cardiology. Yonsei Cardiovascular Center. The result was a dramatic improvement in systemic arterial oxygen tension and oxygen saturation during infusion of PGE1with a dependency on the infusion of PGE1. We evaluated the arterial blood gas analysis both at the immediate pre-infusion stage and 2 hours after infusion. We aimed to analyse the factors which may influence the intravenous of PGE1to infant suffers of ducts-dependent cyanotic congenital heart disease, such as pulmonary atresia(n=14), severe pulmonary stenosis(n=7) or complete transposition of the great arteries(n=5). RESULTS: 1) There was a significant increase in PaO2 and Oxygen saturation 2 hours after the infusion of PGE1. This appeared to be unrelated to the different forms of the disease when compared with the pre-infusion values. 2) The infants' responsiveness of the ductus arteriosus appeared to be age related with significant differences emerging between the 2 group(p<.05). In infants younger than 9 hours old, the differences in PaO2 changes between pre-infusion and post-infusion of PGE1 were 16.3+/-3.7mmHg compared to just 10.4+/-0.4mmHg in infants older than 96 hours. 3) No significant difference emerged between an increase in PaO2or oxygen saturation relating to the shape of ductus arteriosus ; or the level of PaO2prior to the infusion. 4) The side effects of PGE1were as follows ; fever(84.6%),loose stool(61.5%), apnea(30.8%) and hypotension(15.4%), etc.. CONCLUSION: PGE1provides excellent medical palliation for infants suffering from ductus-dependent cyanotic congenital heart disease until the pulmonary arteries are large enough for a modified Blalock-Taussig shunt ; or until corrective surgery is possible.
Alprostadil ; Blalock-Taussig Procedure ; Blood Gas Analysis ; Cardiology ; Ductus Arteriosus ; Heart Defects, Congenital* ; Humans ; Infant* ; Infant, Newborn ; Infusions, Intravenous ; Oxygen ; Pulmonary Artery

With further development of transcatheter techniques and improved occlusion devices, percutaneous closure has become the treatment of choice for secundum ASD. The Amplatzer septal occluder device is frequently used for ASD occlusion due to its straightforward implant technique and efficacy in occluding a wide range of ASD sizes. TEE is vital in the recognition of morphologic variations of the ASD and patient selection. It allows clear visualization of the defect and the device during the procedure, precise measurements of stretch diameters, guiding of deployment, and stable positioning of the device. This is especially important in patients with large ASDs or multiple ASDs and those with atrial septal aneurysm. With TEE, incorrect positioning of the device can be detected while it is still screwed to the delivery cable, which allows its early redeployment, before any complications occur. Compared with the patients undertaken surgical repair, the left ventricular function using strain rate imaging was preserved after the device closure. Echocardiographic evaluation for ASD device closure is essential in patient selection, during procedure, after occlusion, and long term follow-up.
Aneurysm ; Echocardiography* ; Follow-Up Studies ; Heart Septal Defects, Atrial* ; Humans ; Patient Selection ; Septal Occluder Device ; Ventricular Function, Left

A complete history and physical examination for heart diseases in children, especially neonates and infants, enables the pediatrician to compile an appropriate differential diagnosis, order tests such as chest radiography and electrocardiogram in a suitable manner, and efficiently care for the patients. The ability to obtain an accurate history and to perform an excellent physical examination is very important and provides the basis for best management of children with heart diseases. As such, we are reviewed the components of the history and the physical examination that are critical in assessing the cardiovascular system.
Cardiovascular System ; Child* ; Diagnosis, Differential ; Electrocardiography ; Heart Diseases* ; Heart* ; Humans ; Infant ; Infant, Newborn ; Physical Examination* ; Radiography ; Thorax