• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.12 no.1
• /
• pp.42-48
• /
• 2012

Pompe disease is a rare lysosomal glycogen storage disorder caused by a total or partial deficiency of the acid ${\alpha}$-glucosidase (GAA) enzyme due to the GAA gene mutations. The classic infantile form of Pompe disease is a rapidly progressive multi-organ disease with hypotonia, generalized muscle weakness, and hypertrophic cardiomyopathy, usually leading to death in the first 2 years of life. Enzyme replacement therapy with recombinant human GAA has been shown to be effective and subsequently yielded promising results. Here, we present clinical and genetic characteristics of three Korean non-classic infantile Pompe patients, and the short term efficacy of enzyme replacement therapy. Considering that enzyme replacement therapy can change the natural course of infantile Pompe disease, early diagnosis and early initiation of treatment is critical to improving patient outcomes.

• Journal of muscle and joint health
• /
• v.25 no.2
• /
• pp.122-134
• /
• 2018

Purpose: The purpose of this study was to develop a 12-week structured exercise program (12-week-SEP) and evaluate its effects on pain, physical function and quality of life in patients with total hip arthroplasty (THA). Methods: A nonequivalent control group non-synchronized design was utilized to examine the effects of the 12-week-SEP on patients' outcomes after THA. A total of 46 adult patients (experimental group=25, control group=21) who had THA were recruited for the study. The 12-week-SEP consisted of education, exercise (muscle strengthening and progressive walking), and feedback. Data were analyzed by SPSS/WIN 21.0 using the $x^2$ test and repeated measures ANOVA. Results: The mean age of the participants was 60.9 years and 25 men (54.5%) and 21 women (45.7%) were included in the study. There were significant intervention effects on pain reduction and increased physical function but no effects on quality of life. Conclusion: The 12-week-SEP provided pain relief and enhanced physical function in patients underwent THA. Clinical application of the 12-week-SEP can improve postoperative nursing practices for patients with THA. Future research with larger sample is of necessity to obtain greater generalizability of the empirical evidences of the 12-week-SEP.

• Tuberculosis and Respiratory Diseases
• /
• v.76 no.2
• /
• pp.66-74
• /
• 2014

Background: The increasing number of outpatients with multidrug-resistant (MDR) pathogens has led to a new category of pneumonia, termed healthcare-associated pneumonia (HCAP). We determined the differences in etiology and outcomes between patients with HCAP and those with community-acquired pneumonia (CAP) to clarify the risk factors for HCAP mortality. Methods: A retrospective study comparing patients with HCAP and CAP at Jeju National University Hospital. The primary outcome was 30-day mortality. Results: A total of 483 patients (208 patients HCAP, 275 patients with CAP) were evaluated. Patients with HCAP were older than those with CAP (median, 74 years; interquartile range [IQR], 65-81 vs. median, 69 years; IQR, 52-78; p<0.0001). Streptococcus pneumoniae was the major pathogen in both groups, and MDR pathogens were isolated more frequently from patients with HCAP than with CAP (18.8% vs. 4.9%, p<0.0001). Initial pneumonia severity was greater in patients with HCAP than with CAP. The total 30-day mortality rate was 9.9% and was higher in patients with HCAP based on univariate analysis (16.3% vs. 5.1%; odds ratio (OR), 3.64; 95% confidence interval (CI), 1.90-6.99; p<0.0001). After adjusting for age, sex, comorbidities, and initial severity, the association between HCAP and 30-day mortality became non-significant (OR, 1.98; 95% CI, 0.94-4.18; p=0.167). Conclusion: HCAP was a common cause of hospital admissions and was associated with a high mortality rate. This increased mortality was related primarily to age and initial clinical vital signs, rather than combination antibiotic therapy or type of pneumonia.

• Journal of Technologic Dentistry
• /
• v.35 no.4
• /
• pp.303-312
• /
• 2013

Purpose: In this research, non-linear three dimensional finite element models with contact elements were constructed. For the investigations of the distributions of contact stresses, 3 units fixed partial dentures model were studied, especially on the interface of the gold screw and cylinder, abutment screw. Methods: 3 types of models were constructed ; the basic fixed partial denture in molar region with 3 units and 3 implants, the intermediate pontic fixed partial denture model with 3 units and 2 implants, and the extension pontic fixed partial denture model with 3 units and 2 implants. For all types, the external loading due to chewing was simulated by applying $45^{\circ}$ linguo-buccal loading of 300 N to the medial crown. For the simulation of the clamping force which clinically occurs due to the torque, thermal expansion was provided to the cylinder as a preload. Results: Under 300 N concentrated loading to the medial crown, the maximum contact stress between abutment screw and gold screw was 86.85~175.86MPa without preload, while the maximum contact stress on the same area was 25.59~57.84MPa with preload. Conclusion: The preloading affected the outcomes of the finite element stress analysis. Reflecting the clinical conditions, the preloading conditions should be considered for other practical study utilizing FEA. For the study of the contact stresses and related motions, various conditions, such as frictional coefficient changes, gap between contact surfaces, were also varied and analyzed.

• Journal of Trauma and Injury
• /
• v.32 no.2
• /
• pp.86-92
• /
• 2019

Purpose: There is increasing interest in intra-abdominal pressure (IAP) and intra-abdominal hypertension (IAH) in critically ill patients. This study investigated the effects and outcomes of elevated IAP in a trauma intensive care unit (ICU) population. Methods: Eleven consecutive critically ill patients admitted to the trauma ICU at Pusan National University Hospital Regional Trauma Center were included in this study. IAP was measured every 8-12 hours (intermittently) for 72 hours. IAP was registered as mean and maximal values per day throughout the study period. IAH was defined as $IAP{\geq}12mmHg$. Abdominal compartment syndrome was defined as $IAP{\geq}20mmHg$ plus ${\geq}1$ new organ failure. The main outcome measure was in-hospital mortality. Results: According to maximal and mean IAP values, 10 (90.9%) of the patients developed IAH during the study period. The Sequential Organ Failure Assessment (SOFA) score was significantly higher in patients with $IAP{\geq}20mmHg$ than in those with IAP <20 mmHg (16 vs. 5, p=0.049). The hospital mortality rate was 27.3%. Patients with a maximum $IAP{\geq}20mmHg$ exhibited significantly higher hospital mortality rates (p=0.006). Non-survivors had higher maximum and mean IAP values. Conclusions: Our results suggest that an elevated IAP may be associated with a poor prognosis in critically ill trauma patients.

• Korean Journal of Head & Neck Oncology
• /
• v.34 no.2
• /
• pp.17-21
• /
• 2018

Background/Objectives: Cancers of the abdominal or pelvic organ rarely metastasize to the cervical lymph nodes. Although it indicates distant metastasis, perceivable prolongation of survival or cure may be possible in selected cases. We sought to identify patients with cervical metastasis from cancers below the diaphragm and identify patients who may benefit from aggressive treatment. Materials & Methods: From 2009 to 2017, patients with pathologically confirmed metastatic cervical lymph nodes originating from below the diaphragm were included for analysis. Patient demographics, cancer characteristics, treatment course, and clinical outcomes were analyzed. Results: 208 patients were identified. Left supraclavicular node (Virchow's node) was the most frequently involved. Irrespective of treatment, survival for uterine cervical and ovarian cancers was significantly longer than that of other primaries. Patients with isolated cervical metastasis (oligometastasis) had significantly longer median survival compared to patients with concomitant bone, lung, brain, and mediastinal metastases. Conclusion: Although cervical metastasis from cancers of the abdominal and pelvic organ represent distant metastasis, patients with uterine cervix and ovary primary and oligometastatic lesions may benefit from aggressive treatment. Prudent patient selection and further investigation is warranted.

• The Korean Journal of Pain
• /
• v.32 no.2
• /
• pp.113-119
• /
• 2019

Background: Discogenic pain is a common cause of disability and is assumed to be a major cause of non-specific low back pain. Various treatment methods have been used for the treatment of discogenic pain. This study was conducted to compare the therapeutic success of radiofrequency (an intradiscal procedure) and laser annuloplasty (both an intradiscal and extradiscal procedure). Methods: This single-center study included 80 patients and followed them for 6 months. Transforaminal laser annuloplasty (TFLA, 37 patients) or intradiscal radiofrequency annuloplasty (IDRA, 43 patients) was performed. The main outcomes included pain scores, determined by the numeric rating scale (NRS), and Oswestry disability index (ODI), at pre-treatment and at post-treatment months 1 and 6. Results: The patients were grouped according to procedure. In all procedures, NRS and ODI scores were significantly decreased over time. Mean post-treatment pain scores at months 1 and 6 were significantly lower (P < 0.01) in both groups, and between-group differences were not significant. The ODI score was also significantly decreased compared with baseline. Among patients undergoing TFLA, 70.3% (n = 26) reported pain relief (NRS scores < 50% of baseline) at post-treatment 6 months, vs. 58.1% (n = 25) of those undergoing IDRA. There were no statistically significant differences between the groups in ODI reduction of > 40%. Conclusions: Our results indicate that annuloplasty is a reasonable treatment option for carefully selected patients with lower back and radicular pain of discogenic origin, and TFLA might be superior to IDRA in patients with discogenic low back pain.

• Korean Circulation Journal
• /
• v.48 no.12
• /
• pp.1148-1156
• /
• 2018

Background and Objectives: It is controversial that decreased left ventricular function could predict poststroke outcomes. The purpose of this study is to elucidate whether left ventricular ejection fraction (LVEF) can predict cardiovascular events and mortality in acute ischemic stroke (AIS) without atrial fibrillation (AF) and coronary heart disease (CHD). Methods: Transthoracic echocardiography was conducted consecutively in patients with AIS or transient ischemic attack at Soonchunhyang University Hospital between January 2008 and July 2016. The clinical data and echocardiographic LVEF of 1,465 patients were reviewed after excluding AF and CHD. Poststroke disability, major adverse cardiac events (MACE; nonfatal stroke, nonfatal myocardial infarction, and cardiovascular death) and all-cause mortality during 1 year after index stroke were prospectively captured. Cox proportional hazards regressions analysis were applied adjusting traditional risk factors and potential determinants. Results: The mean follow-up time was $259.9{\pm}148.8days$ with a total of 29 non-fatal strokes, 3 myocardial infarctions, 33 cardiovascular deaths, and 53 all-cause mortality. The cumulative incidence of MACE and all-cause mortality were significantly higher in the lowest LVEF (<55) group compared with the others (p=0.022 and 0.009). In prediction models, LVEF (per 10%) had hazards ratios of 0.54 (95% confidence interval [CI], 0.36-0.80, p=0.002) for MACE and 0.61 (95% CI, 0.39-0.97, p=0.037) for all-cause mortality. Conclusions: LVEF could be an independent predictor of cardiovascular events and mortality after AIS in the absence of AF and CHD.

• The Korean Journal of Pain
• /
• v.32 no.4
• /
• pp.292-300
• /
• 2019

Background: The aim of this study was to investigate the clinical characteristics of complex regional pain syndrome (CRPS) in young male patients in South Korea, especially focusing on the association with military service. Methods: From January 2007 to May 2017, we investigated the electronic medical records of 430 consecutive patients, aged 18 to 30 years, who visited Seoul National University Hospital Pain Center, with a suspected diagnosis of CRPS at the initial visit. The following patient details were available for analysis: demographic and disease-related variables, relevance to military service, medications, and the treatment modalities received. Results: Out of 430 patients, 245 (57.0%) were diagnosed with CRPS, of which, 200 were male patients and 45 were female patients. Of the male patients, 95 (47.5%) developed CRPS during military service. CRPS during military service was more likely to result from sprain/strain, and the incidence of CRPS was significantly higher in the lower extremities in patients from the military service group than in those from the non-military service group. During the follow-up period, 37.9% of male CRPS patients (n = 61/161) were treated successfully. Patients with moderate to severe initial pain intensity, and diagnosed during their military service, showed better outcomes. Conclusions: Our results demonstrated that manifestation of CRPS in the young Korean population was more common in male and among those male CRPS patients, about half the cases developed during the military service period.

• Journal of Korean Academy of Nursing
• /
• v.50 no.6
• /
• pp.863-875
• /
• 2020

Purpose: This study was conducted to develop and test the effects of a program for practice application of intravenous infusion evidence based nursing practice (EBP) guidelines in small and medium-sized hospitals. Methods: A mixed method research design was used, combining non-equivalent control group pre-post test design with qualitative study analysis. The subjects consisted of 55 nurses. The practice application program was developed based on the Advancing Research and Clinical practice through close Collaboration (ARCC) model. Data were collected for analysis in the following areas: nurses' EBP organizational culture and readiness, EBP beliefs, EBP implementation, importance about intravenous infusion, and performance about intravenous infusion, with data assessed using valid and reliable instruments. Patient outcomes were collected from the hospital's medical records. Data were analyzed using t-test, χ2-test, and Shapiro-Wilk test, with qualitative content analysis used for interview data. Results: Following the intervention, nurses' EBP organizational culture and readiness, EBP beliefs, EBP implementation, and performance of intravenous infusion and perceptions of its importance showed significant improvement in the experimental group. Phlebitis rates decreased in the experimental group compared to the control group. Conclusion: This program is effective to improve nurse's perception and practice of evidence based nursing. Therefore we recommend to use this program at same levels of hospitals.