Beckwith-Wiedemann sydrome is a multisystemic pattern of congenital anomalies with overgrowth. This syndrome is first described independently by Beckwith in 1963 and by Wiedemann in 1964. There is wide spectrum of clinical manifestations, including prenatal or postnatal overgrowth, neonatal hypoglycemia, macroglossia, visceromegaly, omphalocele, hemihypertrophy and a predisposition for embryonal tumors, most frequently Wilms' tumor. We experienced a case of Beckwith-Wiedemann syndrome who developed left adrenal cortical neoplasm of indeterminate malignant potential.
Beckwith-Wiedemann Syndrome ; Hernia, Umbilical ; Hypoglycemia ; Macroglossia ; Wilms Tumor

PURPOSE: The aim of this study was to evaluate the effectiveness of a community-based intervention for prehypertensive adults, to enhance stroke risk awareness and to adopt a preventive lifestyle for primary stroke prevention. METHODS: This was a single-blinded, repeated measures quasi-experimental study with 47 participants (23 in the experimental group and 24 in the control group) recruited through convenience sampling from two urban areas. The stroke risk self-management intervention consisted of three weekly, 2-hour, face-to-face sessions and two booster telephone sessions, utilizing strategies to enhance motivation for behavioral changes based on the Self-Determination Theory. All participants completed a pretest, a 1- month and a 3-month post test of stroke risk awareness and preventive lifestyle including blood pressure self-monitoring, healthy diet, and regular physical activity. Data were analyzed using descriptive statistics, chi-square test, two sample t test, repeated measures analysis of variance, and Friedman test with PASW Statistics 18.0. RESULTS: After the intervention, significant improvements were found in the experimental group for stroke risk awareness, blood pressure self-monitoring and regular physical activity, and were sustained over time. CONCLUSIONS: Our preliminary results indicate that the stroke risk self-management intervention is feasible and associated with improvement in self-management of stroke risk factors for primary stroke prevention among a prehypertensive population.
Adult ; Behavior Therapy/*education ; Blood Pressure Determination/*methods ; Female ; Humans ; Male ; Middle Aged ; Motivation ; *Patient Education as Topic ; Prehypertension/*nursing ; Primary Prevention/*methods ; Republic of Korea ; Risk Factors ; Self Care/*methods ; Stroke/*prevention & control ; Urban Population/statistics & numerical data

PURPOSE: In this study a system dynamics methodology was used to identify correlation and nonlinear feedback structure among factors affecting unplanned extubation (UE) of ICU patients and to construct and verify a simulation model. METHODS: Factors affecting UE were identified through a theoretical background established by reviewing literature and preceding studies and referencing various statistical data. Related variables were decided through verification of content validity by an expert group. A causal loop diagram (CLD) was made based on the variables. Stock & Flow modeling using Vensim PLE Plus Version 6.0b was performed to establish a model for UE. RESULTS: Based on the literature review and expert verification, 18 variables associated with UE were identified and CLD was prepared. From the prepared CLD, a model was developed by converting to the Stock & Flow Diagram. Results of the simulation showed that patient stress, patient in an agitated state, restraint application, patient movability, and individual intensive nursing were variables giving the greatest effect to UE probability. To verify agreement of the UE model with real situations, simulation with 5 cases was performed. Equation check and sensitivity analysis on TIME STEP were executed to validate model integrity. CONCLUSION: Results show that identification of a proper model enables prediction of UE probability. This prediction allows for adjustment of related factors, and provides basic data do develop nursing interventions to decrease UE.
Adult ; Airway Extubation/*psychology ; Female ; Humans ; Intensive Care Units ; Intubation, Intratracheal ; Male ; Middle Aged ; *Models, Theoretical ; Nonlinear Dynamics ; Risk Factors

PURPOSE: Bone involvement is known to develop in 40-70Yo of pediatric acute leukemia. We aimed to analyze the clinical course and result of therapy in pediatric acute leukemia with bone involvement. METHODS: Twenty-seven patients diagnosed as pediatric acute leukemia at Dong San Medical Center from Jan. 1996 to Aug. 1998 were evaluated. According to bone X-ray and whole body bone scan, the patients were divided into two groups. RESULTS: Twenty-seven patients were enrolled in this study with 14 patients(52Yo) showing definite bone involvement on simple X-ray or bone scan. Mean age of patients with bone involvement was 5.5 years. Regarding the type of leukemia, 9 patients(64%) were acute lymphocytic leukemia. Ten patients(71%) out of 14 with bone involvement complained of bone pain at the involved bony site. Site of involvement was most frequent in the lower extremity. On simple X-ray, osteolytic lesion was found in 7 patients(50%), diffuse osteopenia in 2 patients(14%) and pathologic fracture in 2 patients(14%). In bone scan, radioactivity was increased in whole cases of patients with bone involvement. Thirteen patients(93%) were completely remitted by chemo-therapy, but, one AML patient died due to induction failure. CONCLUSION: Bone involvement occured in 52% of pediatric acute leukemia. Bone involvement was more frequent in male patients in the lower extremity, and osteolytic lesion was the most frequent finding on simple X-ray. There was no relevence between bone involvement and prognosis. Further study will be needed to evaluate long-term survival and prognosis. (J Korean Pediatr Soc 2000;43:806-813)
Bone Diseases, Metabolic ; Fractures, Spontaneous ; Humans ; Leukemia* ; Lower Extremity ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Radioactivity

PURPOSE: This study was undertaken to explore sex differences in the risk markers of cardiovascular disease (CVD) and psychosocial factors and the relationships among these variables for the community-residing adults. METHODS: A cross-sectional study was conducted with a total of 114 participants consisting of 65 men aged 42.85 years old and 49 women aged 46.86 years old. Participants were selected by a convenient sampling of at community cultural centers and churches. They completed a structured questionnaire including demographic characteristics, physiologic risk markers of CVD; lifestyle factors; and depression, social support, and stress. RESULTS: The male participants showed significantly higher systolic and diastolic blood pressures than their counterparts. Significantly higher percent of women were currently non-smokers, and practiced moderate alcohol intake, physical activity, and low fat diet. Among women, the higher the scores of depression and stress were and the lower that of the social support was, the higher blood pressure, waist circumference and BMI were. Among men, those who were less depressed and with higher scores of social support had more physical activity. CONCLUSION: The findings suggest further studies to explore sex differences of CVD risk markers and psychosocial factors, and their relationships in a larger sample, and to find the mechanism underlying these differences with a prospective design.
Adult* ; Blood Pressure ; Cardiovascular Diseases ; Cross-Sectional Studies ; Depression ; Diet ; Female ; Humans ; Life Style ; Male ; Motor Activity ; Prospective Studies ; Psychology* ; Risk Factors ; Sex Characteristics* ; Waist Circumference

Purpose: This study investigated the differences in physical function, self-efficacy (SE), and health-related quality of life (HRQoL) categorized by disease severity in community-dwelling patients with chronic obstructive pulmonary disease (COPD). Methods: This cross-sectional study included 182 patients with COPD selected from the pulmonology outpatient department of a tertiary hospital. Disease severity was measured using forced expiratory volume in 1 second (FEV1). Physical function, SE, and HRQoL were measured with the six-minute walking distance, pulmonary rehabilitation self-efficacy (PRAISE), and St. George's Respiratory Questionnaire (SGRQ). Disease duration, FEV1, and 12-month history of exacerbations were obtained from medical records. Patients were categorized by Global Initiative for Chronic Obstructive Lung Disease (GOLD) category. Data were analyzed using the two-sample t-test, χ2 test, and one-way ANOVA. Results: Most of the participants were male and nonsmokers. The disease duration was 10.76 ± 10.03 years, the mean FEV1% was 62.13 ± 22.80, and 70.3% of the participants were in GOLD category 2 (moderate) or milder. Half of the participants reported modified Medical Research Council scores ≥ 2. Patients in GOLD categories 1 and 3 (mild and severe) exhibited significantly higher PRAISE scores than those in the other groups (F = 8.23, p < .001). The total SGRQ scores were highest in GOLD 4 (very severe), indicating the lowest HRQoL. Significant differences were identified among GOLD 1, GOLD 2 and 3, and GOLD 4 (F = 9.92, p < .001). Conclusion We identified potentially useful variables to comprehensively assess disease severity and tailor management strategies, including airflow limitation, and to determine the consequences of COPD from patients’ perspectives.

OBJECTIVE: To investigate the association of the muscle contraction with gating of the sensory input at central and peripheral levels according to the intensity of muscle contraction and location of the muscles, somatosensory evoked potentials (SSEPs) studies were evaluated at different levels of isometric contraction in the different muscles. METHOD: Median nerve SSEPs were recorded at Erb's point and scalp in the ten healthy adult subjects with isometric contraction of ipsilateral abductor pollicis brevis (APB), ipsilateral abductor digiti minimi (ADM) and contralateral APB. Median nerve SSEPs were recorded in each of these conditions during precontraction, weak contraction, strong contraction and 4 minutes after contraction. RESULTS: 1) N9 amplitudes of median SSEPs recorded at Erb's point were augumented during weak contraction and these amplitude augumentations were statistically significant in the ipsilateral APB contraction (p<0.05). 2) N20 amplitudes recorded at scalp were inhibited during strong isometric contraction and these amplitude inhibitions were statistically significant in the ipsilateral APB contraction (p<0.05). 3) The latencies of N9 and N20 potentials were not significantly changed during isometric contraction. CONCLUSION: Therefore peripheral nervous system as well as central nervous system is responsible for gating, so the subject should be asked for the best relaxation possible for higher reliability of SSEPs.
Adult ; Central Nervous System ; Evoked Potentials, Somatosensory* ; Humans ; Isometric Contraction ; Median Nerve ; Muscle Contraction* ; Muscles ; Peripheral Nervous System ; Relaxation ; Scalp

PURPOSE: The purpose of this research was to compare the intra-operative and post-operative measured angle in total knee arthroplasty (TKA) and to evaluate the postoperative improvement in the mechanical axis. MATERIALS AND METHODS: Forty TKAs were performed using CAOS between February and May 2004. We measured the mechanical axis in the preoperative orthograms. After inserting the implant, we measured the femoral component angle (FCA), tibial component angle (TCA) and tibial component slope (TCS) using the verification tool in CAOS, on the post-operative orthogram and X-ray of the lateral tibial view, 2 observers measured the FCA, TCA, TCS and mechanical axis. RESULTS: The mean FCA, TCA and TCS using the verification tool in CAOS, were valgus 0.03+/-0.85degrees, valgus 0.49+/-1.02degrees, nd 4.26+/-1.64degrees. The mean FCA, TCA and TCS by observer 1 were valgus 0.67+/-1.24degrees, varus 0.54+/-1.54degrees, and 4.57+/-1.74degrees and those by observer 2 were valgus 0.48+/-1.17degrees, varus 0.52+/-1.44degrees, and 4.24+/-2.83degrees. There was a positive correlation between the measured angle by respective observers and the angle measured by the CAOS (p<0.05). The mechanical axis by observers 1 and 2 improved from varus 13.96degrees to valgus 0.04degrees and from varus 14.12degrees to valgus 0.08degrees respectively. CONCLUSION: CAOS affords the possibility of precisely placing the femoral and tibial components. The angles measured by the respective observers and in the CAOS were significantly correlated.
Arthroplasty* ; Axis, Cervical Vertebra ; Knee*

Objective: We investigated the factors that affect the time from the onset of acute stroke symptoms to arrival at the emergency department (ED). Methods: Between July 2019 and February 2020, patients with acute stroke symptoms who visited a public hospital were evaluated by retrospective analysis using a prospective survey. The data for this study was gathered from baseline interviews with patients enrolled in stroke surveys. The primary outcome was time to arrival at the ED, evaluated as a binary variable with a value of 4.5 hours. Results: Overall, 205 patients were included in the final analysis. Among them, 47% (n=96) of patients with acute stroke symptoms arrived at the ED later than 4.5 hours from the time of onset of acute stroke symptoms. After multivariable logistic regression analyses, it was found that patients with higher National Institutes of Health Stroke Scale (NIHSS) scores, patients who did not live with their family members, and patients who were found by a passerby were associated with early ED arrival post onset of acute stroke symptoms. Among patients diagnosed with acute stroke, those with higher NIHSS scores or underlying malignant diseases were found to present earlier at the ED. Conclusion Approximately half of the patients (47%) with acute stroke symptoms presented to the ED within 4.5 hours. Higher NIHSS scores and the presence of underlying malignancy were identified as the key factors that were associated with an early presentation at the ED from the time of onset of acute stroke.

Plasmapheresis has been used for some conditions during pregnancy and puerperium, such as hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome. We present one case of the HELLP syndrome which was treated with plasmapheresis and also review the indications, complications and guidelines for repetitive plasma exchange.
Blood Platelets ; Female ; HELLP Syndrome* ; Hemolysis ; Liver ; Plasma Exchange ; Plasmapheresis* ; Postpartum Period* ; Pregnancy