No abstract available.
Histiocytosis*

No abstract available.
Neurofibromatoses* ; Peripheral Nerves*

BACKGROUND: The survival rate of children with neuroblastoma has been improved over 20 years, excluding the metastatic disease, in which it does not exceed 20% so far. New treatment modalities have been developed to improve the outcome in metastatic disease. Preoperative chemotherapy reduce the size, the vascularity and the adhesiveness, so increase the resectability of the primary tumor. This retrospective clinical study was designed to review the survival rate in neuroblastoma and to analyze the effect of preoperative chemotherapy in the view point of neoadjuvant therapy on long-term survival in advanced disease. METHODS: One hundred and thirty-four cases were reviewed from 135 patients with neuroblastoma registered at the Department of Pediatrics in Seoul National University Children's Hospital from January, 1985 till December, 1995. The survival rate was reviewed according to the stage. The age, sex of the patients, the stage, anatomical site of the tumor, the level of serum ferritin and neuron-specific enolase were analyzed for the risk factors on survival. RESULTS: Ranges of age at diagnosis were from 1 month to 166 months with the median of 39 months. Five year survival rates and five year disease-free survival rates were 100%, 100% in stage 1(n=5), 90.9%, 90.9% in stage 2(n=13), 43.4%, 40.6% in stage 3(n=19), 27.1%, 19.8% in stage 4(n=95) and 100%, 100% in stage 4S(n=2), respectively. In stage 3, five year survival rate was 52.5% in group receiving neoadjuvant chemotherapy, 28.6% in control group(P=0.02). Five year disease-free survival rate was also noted as 48.6%, 28.6% in each group(P=0.02). In stage 4, five year and ten year survival rates were 27.6%, 23.6% in group receiving neoadjuvant chemotherapy, 26.9%, 0% in control group(P=0.02). Five year and ten year disease-free survival rates were 14.3%, 14.3% in group receiving neoadjuvant chemotherapy, 20%, 0% in control group (P=0.11). In univariate analysis, the age, the stage, and the site of primary tumor appeared to affect the long-term survival. CONCLUSION: Neoadjuvant chemotherapy and delayed primary surgery contribute for advance in survival in advanced neuroblastoma via increasing the resectability of the primary tumor.
Adhesiveness ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy* ; Ferritins ; Humans ; Neoadjuvant Therapy ; Neuroblastoma ; Pediatrics ; Phosphopyruvate Hydratase ; Retrospective Studies ; Risk Factors ; Seoul ; Survival Rate

Isolated pulmonary stenosis, a relatively common congenital anomaly that accounts for about 10 percent of all congenital heart disease, is characterized by stenosis of pulmonary valve itself, infundibulum or both of them. Since cardiac catherterization was applied to man by Cournand and Ranges, pulmonary stenosis had been easily diagnosed and many clinical studies had been investigated. It has a wide clinical spectrum depending on the degree of stenosis. The electrocardiogram, phonocardiogram and chest X-ray have proved useful in estimating the severity of hemodynamic facotrs in individual cases. This series comprises 47 cases in whom the clinical diagnosis of isolated pulmonary stenosis was confirmed by right heart catheterization with cardiac cineangiography at Severance Hospital, Yonsei University. An attempt was made to correlate the electrocardiographic, phonocardiographic, chest X-ray findings, and types of stenosis with the hemodynamic data in these cases. 1. Of 47 patients, 33 were male and 14 female. Their ages ranged from 2 to 42 years: the mean age was 19.1 years. 2. The incidence was 5.9 percent of all 797 catheterized congenital heart disease cases. The pulmonary valvular stenosis was 30 (68.3%), infundibular 7 (14.6%) and combined 10 (21.6%) cases. 3. The correlation between electrocardiogram and hemodynamic data were as follows. i) The regression equation between right ventricular sysytolic pressure (RVSP) and height of R wave in V1 lead (RV1) was RVSP=3.32 RV1+48.2: its correlation coefficient was 0.818 and it was very significant (p=0.000). ii) The higher the RVSP, the more the frontal axis of QRS complex shifted to the right side (r=0.55. p=0.001). iii) The RVSP of the groups with positive ECG findings such as p-pulmonale, right ventricular hypertrophy or right ventricular strain were much higher than the RVSP of the groups without such findings (p=0.032.0.000, 0.000). iv) The group with RV1 higher than 20 mm showed much more elevated mean of RVSP than the group with lower RV1 (p=0.000). v) The groups with the above mentioned positive ECG findings showed good correlation with the severity of RVSP which was arbitrarily classified as 49 or less, 50~100, and 100 mmHg or more (chi-square=8.96, 26.69, 19.06; p=0.011, 0.000, 0.000). 4. The group with late occurrence of the maximum intensity of the ejection systolic murmur showed higher mean of RVSP than the group with early peak of the murmur (p=0.014). 5. The means of RVSP of the groups with chest X-ray findings such as decreased pulmonary vascularity, were much higher than the means of RVSP of the groups without such findings (p=0.000, 0.005, 0.015). The groups with above mentioned positive chest X-ray findings showed good corelations with the severity of RVSP which was classified as above limits (chi-square=7.55, 10.94, 13.36; p=0.022, 0.004, 0.001). 6. Combined pulmonary valvular and infundibular stenosis showed more severe systolic pressure gradient and higher mean of RVSP than the isolated types (p=0.000).
Axis, Cervical Vertebra ; Blood Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Catheters ; Cineangiography ; Constriction, Pathologic ; Diagnosis ; Electrocardiography ; Female ; Heart Defects, Congenital ; Hemodynamics* ; Humans ; Hypertrophy, Right Ventricular ; Incidence ; Male ; Pulmonary Valve ; Pulmonary Valve Stenosis* ; Systolic Murmurs ; Thorax

BACKGROUND: Recent studies revealed that inhalational anesthetics (IA) attenuate NO production. But the hemodynamic changes produced by IA in septic syndrome patient are still sufficient to threaten patient, surgeon and anesthesiologist. So we examined which IA is proper to maintain vascular contractile force and evaluated the effects of NOS inhibitors on contractile force of septic rat aorta under IA. METHODS: Aortic ring preparation was obtained from LPS-treated (1.5 mg/kg, i.p. for 18h) rats. The development of sepsis was confirmed by iNOS activity and iNOS expression using RT-PCR. Contractile responses of aorta to phenylephrine admministation in the presence or absence of halothane, enflurane and isoflurane were evaluated. We also evaluated the effects of NOS inhibitors, one is NG-nitro-L-arginine methyl ester (L-NAME) and the other is aminoguanidine. Statistical significances (p<0.05) were analyzed according to data characteristics by unpaired t-test and paired t-test. RESULTS: The contractile responses to phenylephrine admministration were attenuated in LPS-treated rings. Isoflurane, even at the dose of 2 MAC, didn't affect the contractile response while both halothane and enflurane decreased the contractile response even at the dose of 1 MAC. The potentiation of contractile responses by NOS inhibitors were not affected during administeration of IA. CONCLUSIONS: From these results, it is suggested that isoflurane is the safest inhalational anesthetic and NOS inhibitors, especially L-NAME, may be very useful in the therapy of septic shock patients during general anesthesia.
Anesthesia, General ; Anesthetics* ; Animals ; Aorta ; Enflurane ; Halothane ; Hemodynamics ; Humans ; Isoflurane ; NG-Nitroarginine Methyl Ester ; Nitric Oxide Synthase* ; Nitric Oxide* ; Phenylephrine ; Rats* ; Sepsis ; Shock, Septic

No abstract available.
Cyclosporine* ; Humans ; Vitiligo*

PURPOSE: To evaluate the pre-operative and the intra-operative factors including marginal fibrosis affecting the result of vitrectomy for macular hole. METHODS: The medical records of the patient who had been operated for idiopathic full-thickness macular hole from January 1997 to July 2000 were investigated retrospectively. RESULTS: Hole closure, as the implication of anatomical success, was seen in 28 eyes (84.8%) at 1 month after surgery and 23 eyes (69.7%) at the end of follow-up period. And the improvement of two or more Snellen line, as the implication of functional success, was seen in 17 eyes (51.5%). The smaller size of the hole was well correlated with higher rate of anatomical success (P=0.001). And there was a good correlation of higher anatomic success rate with the lack of marginal fibrosis around the hole (P=0.001). CONCLUSIONS: There must be more efforts and investigations on proper management of marginal fibrosis, which might help to improve the result of macular hole surgery.
Fibrosis* ; Follow-Up Studies ; Humans ; Medical Records ; Retinal Perforations* ; Retrospective Studies ; Vitrectomy

BACKGROUND: Circardian variation in the onset of cardiovascular events includig sudden cardiac death, myocardial infarction and ventricular arrhythmias has been discribed. The frequency of ventricular premature complexes has also been reported to demonstrate a pattern consisting of a daytime peak and nightime nadir. We tried to see if the same circardian pattern is found in dilated cardiomyopathy patients. We have also studed how various modifying factors such as left ventricular ejection fration and ACE inhibitor use may affect the circardian pattern. METHOD: 24-hour ambulatory electrocaridiographic monitorings were performed in 50 dilated cardiomyopathy patients and 20 control subjects. Patients were prospectively divided in 2 groups based on LVEF and ACE inhibitor use. RESULTS: In dilated cardiomyopathy patients, the expected morning increase in VPC frequency is absent and show a peak in evening. This pattern is not correlated with heart rate. Evening peak is more prominent in low LVEF group and ACE inhibitor non-user group. CONCLUSION: In dilated cardiomyopathy patients, VPC frequency show a peak in the evening.
Arrhythmias, Cardiac ; Cardiomyopathy, Dilated* ; Death, Sudden, Cardiac ; Heart Rate ; Humans ; Myocardial Infarction ; Prospective Studies ; Ventricular Premature Complexes*

Stroke is a leading cause of long-term disability in ischemic survivors who are suffering from motor, cognitive, and memory impairment. Previously, we have reported suppressing LPA5 activity with its specific antagonist can attenuate acute brain injuries after ischemic stroke. However, it is unclear whether suppressing LPA5 activity can also attenuate chronic brain injuries after ischemic stroke. Here, we explored whether effects of LPA5 antagonist, TCLPA5, could persist a longer time after brain ischemic stroke using a mouse model challenged with tMCAO. TCLPA5 was administered to mice every day for 3 days, starting from the time immediately after reperfusion. TCLPA5 administration improved neurological function up to 21 days after tMCAO challenge. It also reduced brain tissue loss and cell apoptosis in mice at 21 days after tMCAO challenge. Such long-term neuroprotection of TCLPA5 was associated with enhanced neurogenesis and angiogenesis in post-ischemic brain, along with upregulated expression levels of vascular endothelial growth factor. Collectively, results of the current study indicates that suppressing LPA5 activity can provide long-term neuroprotection to mice with brain ischemic stroke.

BACKGROUND: The purpose of this study was to identify the epidemiologic characteristics of hand tendon injuries in children and to compare these with those of adults. METHODS: This retrospective study was conducted on acute traumatic tendon injuries of the hand treated at our institution from 2005 to 2013, based on medical records and X-ray findings. Age, sex, hand injured, mechanism of injury, tendons and zones injured, number of affected digits, and comorbidities and complications were analyzed. Patients were divided into 2 groups: a pediatric group (≤15 years) and an adult group (>15 years). RESULTS: Over the 9-year study period, 533 patients were surgically treated for acute traumatic tendon injuries of the hand. In the pediatric group (n=76), being male, the right hand, the extensor tendon, complete rupture, the middle finger, and glass injury predominated in hand tendon injuries. In the adult group (n=457), results were similar, but injury to the index finger and knife injury were the most common. An accompanying fracture was more common in the adult group and complication rates were non-significantly different. CONCLUSIONS: This comparative analysis revealed no significant epidemiologic intergroup differences. The belief that pediatric tendon injuries tend to be less severe is misplaced, and careful physical examination and exploration should be conducted in pediatric cases of hand injury.
Adult* ; Child ; Comorbidity ; Epidemiologic Studies ; Fingers ; Glass ; Hand Injuries ; Hand* ; Humans ; Male ; Medical Records ; Physical Examination ; Retrospective Studies ; Rupture ; Tendon Injuries* ; Tendons* ; Wounds and Injuries