Your browser doesn't support javascript.
loading
節目: 20 | 50 | 100
结果 1 - 20 de 1.678
过滤器
1.
文章 在 英语 | WPRIM | ID: wpr-1043236

摘要

Background@#and Purpose Unlike other immune-mediated neuropathies, anti-myelin-associated glycoprotein (MAG) neuropathy is often refractory to immunotherapy. It is necessary to compare the relative efficacies of various immunotherapies and develop objective biomarkers in order to optimize its clinical management. @*Methods@#This study recruited 91 patients with high anti-MAG antibody titers from 7 tertiary hospitals in South Korea. We analyzed the baseline characteristics, therapeutic outcomes, and nerve conduction study (NCS) findings of 68 patients and excluded 23 false positive cases. @*Results@#The rate of positive responses to treatment was highest using zanubrutinib (50%) and rituximab (36.4%), followed by corticosteroids (16.7%), immunosuppressants (9.5%), intravenous immunoglobulin (5%), and plasma exchange (0%). Disability and weakness were significantly associated with multiple NCS parameters at the time of diagnosis, especially distal compound muscle action potential (CMAP) amplitudes. Moreover, the longitudinal trajectory of the average CMAP amplitudes paralleled the clinical courses, with a 16.2 percentile decrease as an optimal cutoff for predicting a clinical exacerbation (area under the receiver operating characteristic curve=0.792). @*Conclusions@#Our study supports the use of NCS as an objective marker for estimating disease burden and tracking clinical changes in patients with anti-MAG neuropathy. We have described the beneficial effects of rituximab and a new drug, zanubrutinib, compared with conventional immunotherapies.

2.
文章 在 英语 | WPRIM | ID: wpr-1043279

摘要

Human reflexes are simple motor responses that are automatically elicited by various sensory inputs. These reflexes can provide valuable insights into the functioning of the nervous system, particularly the brainstem and spinal cord. Reflexes involving the brainstem, such as the blink reflex, laryngeal adductor reflex, trigeminal hypoglossal reflex, and masseter H reflex, offer immediate information about the cranial-nerve functionality and the overall state of the brainstem. Similarly, spinal reflexes such as the H reflex of the soleus muscle, posterior root muscle reflexes, and sacral reflexes provide crucial information about the functionality of the spinal cord and peripheral nerves. One of the critical benefits of reflex monitoring is that it can provide continuous feedback without disrupting the surgical process due to no movement being induced in the surgical field. These reflexes can be monitored in real time during surgical procedures to assess the integrity of the nervous system and detect potential neurological damage.It is particularly noteworthy that the reflexes provide motor and sensory information on the functional integrity of nerve fibers and nuclei. This article describes the current techniques used for monitoring various human reflexes and their clinical significance in surgery. We also address important methodological considerations and their impact on surgical safety and patient outcomes. Utilizing these methodologies has the potential to advance or even revolutionize the field of intraoperative continuous monitoring, ultimately leading to improved surgical outcomes and enhanced patient care.

3.
文章 在 英语 | WPRIM | ID: wpr-1043548

摘要

Background@#The US Food and Drug Administration (FDA) and European Medicines Agency (EMA) approved empagliflozin for reducing cardiovascular mortality and heart failure (HF) hospitalization in patients with both HF with reduced ejection fraction (HFrEF) and HF with preserved ejection fraction (HFpEF). However, limited data are available on the generalizability of empagliflozin to clinical practice. Therefore, we evaluated real-world eligibility and potential cost-effectiveness based on a nationwide prospective HF registry. @*Methods@#A total of 3,108 HFrEF and 2,070 HFpEF patients from the Korean Acute Heart Failure (KorAHF) registry were analyzed. Eligibility was estimated by inclusion and exclusion criteria of EMPagliflozin outcomE tRial in Patients With chrOnic heaRt Failure With Reduced Ejection Fraction (EMPEROR-Reduced) and EMPagliflozin outcomE tRial in Patients With chrOnic heaRt Failure With Preserved Ejection Fraction (EMPEROR-Preserved) trials and by FDA & EMA label criteria. The cost-utility analysis was done using a Markov model to project the lifetime medical cost and quality-adjusted life year (QALY). @*Results@#Among the KorAHF patients, 91.4% met FDA & EMA label criteria, while 44.7% met the clinical trial criteria. The incremental cost-effectiveness ratio of empagliflozin was calculated at US$6,764 per QALY in the overall population, which is far below a threshold of US$18,182 per QALY. The cost-effectiveness benefit was more evident in patients with HFrEF (US$5,012 per QALY) than HFpEF (US$8,971 per QALY). @*Conclusion@#There is a large discrepancy in real-world eligibility for empagliflozin between FDA & EMA labels and clinical trial criteria. Empagliflozin is cost-effective in HF patients regardless of ejection fraction in South Korea health care setting. The efficacy and safety of empagliflozin in real-world HF patients should be further investigated for a broader range of clinical applications.

4.
文章 在 英语 | WPRIM | ID: wpr-1045259

摘要

The worldwide incidence of precocious puberty, which is associated with negative health outcomes, is increasing. Several studies have suggested that environmental factors contribute to the development of precocious puberty alongside genetic factors. Some epidemiological studies have provided limited evidence suggesting an association between exposure to air pollution and changes in pubertal development. This systematic review aimed to summarize existing evidence on the association between air pollution exposure and precocious puberty. Following the Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines, we searched two databases (PubMed and Web of Science) until August 2023. The included studies assessed the association between air pollutant exposure and the risk of precocious puberty, early menarche, or pubertal development. Two authors independently performed study selection and data extraction. A meta-analysis and analysis of the risk of bias were infeasible due to the limited number of studies and the heterogeneity among them. The literature search resulted in 184 studies, from which we included six studies with sample sizes ranging from 437 to 4,074 participants. The studies reported heterogeneous outcomes. Four studies found that increased exposure to air pollution was related to earlier pubertal onset. One study was inconclusive, and another suggested that air pollutant exposure may delay the onset of thelarche. Most studies suggest that exposure to air pollutants accelerates pubertal development; however, the results from the available studies are inconsistent. More extensive and well-designed longitudinal studies are required for a comprehensive understanding of the association between air pollution and precocious puberty.

5.
文章 在 英语 | WPRIM | ID: wpr-1041183

摘要

Purpose@#This study is descriptive survey research on the analysis of nurses' perception of the 4th industrial revolution and the importance and performance of future core nursing competencies in a tertiary hospital located in Seoul. @*Methods@#Data were collected from 149 nurses with more than a year of work experience and analyzed using descriptive statistics, t-test, one-way ANOVA, and Importance Performance Analysis(IPA) with the IBM SPSS/WIN 25.0 program. @*Results@#The nurses’ perception of the 4th industrial revolution was 3.23±0.71 out of 5 points. The importance of future core nursing competencies was 4.31±0.48, and the performance of it was 3.47±0.54. The analysis results of IPA showed that A (area of continuous maintenance) included critical thinking, problem-solving skills, teamwork and collaboration, evidence-based practice, communication, quality improvement and safety, professionalism, self-regulation and self-management, and personal literacy. The specific competencies were not included in B (area of priority improvement). Creativity, informatics, healthcare policy, leadership, research ability, and continuing education were included in C (area of progressive improvement).Knowledge and patient-centered care, ability to manage resources as well as professional, legal, and ethical responsibility were included in D (area of overinvestment). @*Conclusion@#The nurses seemed not to be fully prepared for the 4th industrial revolution. However, they were well aware of the importance of the future core nursing competencies. Therefore, if nurses increase the performance of core competencies in order of priority according to the IPA results, they will be able to independently lead the changing nursing field.

6.
文章 在 英语 | WPRIM | ID: wpr-1041203

摘要

Purpose@#This study aimed to investigate the impact of a nurse shift improvement pilot project on nurses' shift adjustment, work-life balance, and turnover intention, as well as nurses' experiences of participating in the pilot project. @*Methods@#An explanatory sequential mixed-methods study was performed. Survey data was collected from a total of 1,051 nurses both in participant and non-participant groups. For the qualitative study, focus group interviews were conducted with 22 nurses who participated in the shift improvement pilot, including 12 staff nurses, 7 nurse managers, and 3 substitute nurses. @*Results@#There were significant positive effects on the key variables of nurses’ shift adjustment, work-life balance, and turnover intention in the group that participated in the nurse shift improvement pilot project. Based on the qualitative study, participants in the shift improvement pilot project indicated that it enabled nurses to recover their circadian rhythm, and achieve a better work-life balance, and they wanted to continue working as a nurse, and hoped expanded application of the pilot project. @*Conclusion@#In order for expanded implementation of the pilot project and continued improvement in the shift system, we recommend establishing compensation standard, systematizing the use of substitute nurses, developing guidelines for shift schedules, and expanding the shift improvement pilot project to other departments such as the emergency room.

7.
文章 在 英语 | WPRIM | ID: wpr-1041331

摘要

Obesity is a major risk factor for type 2 diabetes mellitus (T2DM). Bariatric surgery is the most effective means of inducing weight loss, and can ameliorate or induce the remission of obesity-related metabolic comorbidities, including T2DM. The guidelines for the management of T2DM emphasize weight management and recommend metabolic surgery for the treatment of T2DM accompanied by obesity. However, despite the clear beneficial effects of metabolic surgery, only 20–50% of patients who experience remission will stay in remission over the long term. Moreover, the beneficial effects of metabolic surgery tend to diminish with time, and a subset of patients experience a relapse of their diabetes. Therefore, in the present review, we discuss potential strategies for the maintenance of diabetic remission following metabolic surgery.

8.
文章 在 英语 | WPRIM | ID: wpr-999689

摘要

Bile pigment, bilirubin, and biliverdin concentrations may change as a results of biliary tract cancer (BTC) altering the mechanisms of radical oxidation and heme breakdown. We explored whether changes in bile pigment components could help distinguish BTC from benign biliary illness by evaluating alterations in patients with BTC. We collected bile fluid from 15 patients with a common bile duct stone (CBD group) and 63 individuals with BTC (BTC group). We examined the bile fluid’s bilirubin, biliverdin reductase (BVR), heme oxygenase (HO-1), and bacterial taxonomic abundance. Serum bilirubin levels had no impact on the amounts of bile HO-1, BVR, or bilirubin. In comparison to the control group, the BTC group had considerably higher amounts of HO-1, BVR, and bilirubin in the bile. The areas under the curve for the receiver operating characteristic curve analyses of the BVR and HO-1 were 0.832 (p<0.001) and 0.891 (p<0.001), respectively. Firmicutes was the most prevalent phylum in both CBD and BTC, according to a taxonomic abundance analysis, however the Firmicutes/Bacteroidetes ratio was substantially greater in the BTC group than in the CBD group. The findings of this study showed that, regardless of the existence of obstructive jaundice, biliary carcinogenesis impacts heme degradation and bile pigmentation, and that the bile pigment components HO-1, BVR, and bilirubin in bile fluid have a diagnostic significance in BTC. In tissue biopsies for the diagnosis of BTC, particularly for distinguishing BTC from benign biliary strictures, bile pigment components can be used as additional biomarkers.

9.
文章 在 英语 | WPRIM | ID: wpr-1000258

摘要

Background@#To investigate the efficacy and safety of moderate-intensity rosuvastatin/ezetimibe combination compared to highintensity rosuvastatin in high atherosclerotic cardiovascular disease (ASCVD) risk patients with type 2 diabetes mellitus (T2DM). @*Methods@#This study was a randomized, multicenter, open, parallel phase 4 study, and enrolled T2DM subjects with an estimated 10-year ASCVD risk ≥7.5%. The primary endpoint was the low-density lipoprotein cholesterol (LDL-C) change rate after 24-week rosuvastatin 10 mg/ezetimibe 10 mg treatment was non-inferior to that of rosuvastatin 20 mg. The achievement proportion of 10-year ASCVD risk <7.5% or comprehensive lipid target (LDL-C <70 mg/dL, non-high-density lipoprotein cholesterol <100 mg/dL, and apolipoprotein B <80 mg/dL) without discontinuation, and several metabolic parameters were explored as secondary endpoints. @*Results@#A hundred and six participants were assigned to each group. Both groups showed significant reduction in % change of LDL-C from baseline at week 24 (–63.90±6.89 vs. –55.44±6.85, combination vs. monotherapy, p=0.0378; respectively), but the combination treatment was superior to high-intensity monotherapy in LDL-C change (%) from baseline (least square [LS] mean difference, –8.47; 95% confidence interval, –16.44 to –0.49; p=0.0378). The combination treatment showed a higher proportion of achieved comprehensive lipid targets rather than monotherapy (85.36% vs. 62.22% in monotherapy, p=0.015). The ezetimibe combination significantly improved homeostasis model assessment of β-cell function even without A1c changes (LS mean difference, 17.13; p=0.0185). @*Conclusion@#In high ASCVD risk patients with T2DM, the combination of moderate-intensity rosuvastatin and ezetimibe was not only non-inferior but also superior to improving dyslipidemia with additional benefits compared to high-intensity rosuvastatin monotherapy.

10.
Experimental Neurobiology ; : 170-180, 2023.
文章 在 英语 | WPRIM | ID: wpr-1000341

摘要

Stroke destroys neurons and their connections leading to focal neurological deficits. Although limited, many patients exhibit a certain degree of spontaneous functional recovery. Structural remodeling of the intracortical axonal connections is implicated in the reorganization of cortical motor representation maps, which is considered to be an underlying mechanism of the improvement in motor function. Therefore, an accurate assessment of intracortical axonal plasticity would be necessary to develop strategies to facilitate functional recovery following a stroke. The present study developed a machine learning-assisted image analysis tool based on multi-voxel pattern analysis in fMRI imaging. Intracortical axons originating from the rostral forelimb area (RFA) were anterogradely traced using biotinylated dextran amine (BDA) following a photothrombotic stroke in the mouse motor cortex. BDA-traced axons were visualized in tangentially sectioned cortical tissues, digitally marked, and converted to pixelated axon density maps. Application of the machine learning algorithm enabled sensitive comparison of the quantitative differences and the precise spatial mapping of the post-stroke axonal reorganization even in the regions with dense axonal projections. Using this method, we observed a substantial extent of the axonal sprouting from the RFA to the premotor cortex and the peri-infarct region caudal to the RFA. Therefore, the machine learningassisted quantitative axonal mapping developed in this study can be utilized to discover intracortical axonal plasticity that may mediate functional restoration following stroke.

11.
Gut and Liver ; : 456-465, 2023.
文章 在 英语 | WPRIM | ID: wpr-1000388

摘要

Background/Aims@#We explored whether high sodium intake, assessed by urinary excretion, determines the risk of sarcopenia and nonalcoholic fatty liver disease (NAFLD). @*Methods@#We analyzed 10,036 adult participants with normal kidney function from the Korea National Health and Nutrition Examination Survey (2008–2011). NAFLD was identified using the fatty liver index, and the muscle mass was evaluated using dual X-ray absorptiometry. The dietary sodium intake was estimated using Tanaka’s equation. @*Results@#The mean 24-hour urinary sodium excretion was 144.2±36.1 mmol/day (corresponding to 3.3 g/day Na) in the total population. The 24-hour urinary sodium excretion showed moderate accuracy in predicting NAFLD (area under the receiver operating characteristic, 0.702; 95% confidence interval [CI], 0.692 to 0.712). A cutoff value of 99.96 mmol/day (corresponding to 2.30 g/day Na) for urinary sodium excretion in predicting NAFLD showed 76.1% sensitivity and 56.1% specificity. The results of multiple adjusted models indicated that the participants with the highest urinary sodium excretion had a significantly higher risk of NAFLD (odds ratio, 1.46; 95% CI, 1.27 to 1.66; p<0.001) and sarcopenia (odds ratio, 1.49; 95% CI, 1.28 to 1.73; p<0.001) than those with the lowest urinary sodium excretion. The association between a higher 24-hour urinary sodium excretion and NAFLD was independent of sarcopenia. @*Conclusions@#Participants with a high sodium intake, as assessed by sodium excretion, had a substantial risk of NAFLD and sarcopenia

12.
文章 在 英语 | WPRIM | ID: wpr-937426

摘要

Background@#Diabetes is a leading cause of death that is responsible for 1.6 million annual deaths worldwide. However, the life expectancy and age at death of people with diabetes have been a matter of debate. @*Methods@#The National Health Insurance Service claims database, merged with death records from the National Statistical Information Service in Korea from 2006 to 2018, was analyzed. @*Results@#In total, 1,432,567 deaths were collected. The overall age at death increased by 0.44 and 0.26 year/year in the diabetes and control populations, respectively. The disparity in the mean age at death between the diabetes and control populations narrowed from 5.2 years in 2006 to 3.0 years in 2018 (p<0.001). In a subgroup analysis according to the presence of comorbid diseases, the number and proportion of deaths remained steady in the group with diabetes only, but steadily increased in the groups with diabetes combined with dyslipidemia and/or hypertension. Compared to the control population, the increase in the mean death age was higher in the population with diabetes. This trend was more prominent in the groups with dyslipidemia and/or hypertension than in the diabetes only group. Deaths from vascular disease and diabetes decreased, whereas deaths from cancer and pneumonia increased. The decline in the proportion of deaths from vascular disease was greater in the diabetes groups with hypertension and/or dyslipidemia than in the control population. @*Conclusion@#The age at death in the population with diabetes increased more steeply and reached a comparable level to those without diabetes.

13.
文章 在 英语 | WPRIM | ID: wpr-937821

摘要

Microvascular decompression (MVD) is a widely used surgical intervention to relieve the abnormal compression of a facial nerve caused by an artery or vein that results in hemifacial spasm (HFS). Various intraoperative neurophysiologic monitoring (ION) and mapping methodologies have been used since the 1980s, including brainstem auditory evoked potentials, lateral-spread responses, Z-L responses, facial corticobulbar motor evoked potentials, and blink reflexes. These methods have been applied to detect neuronal damage, to optimize the successful decompression of a facial nerve, to predict clinical outcomes, and to identify changes in the excitability of a facial nerve and its nucleus during MVD. This has resulted in multiple studies continuously investigating the clinical application of ION during MVD in patients with HFS. In this study we aimed to review the specific advances in methodologies and clinical research related to ION techniques used in MVD surgery for HFS over the last decade. These advances have enabled clinicians to improve the efficacy and surgical outcomes of MVD, and they provide deeper insight into the pathophysiology of the disease.

14.
文章 在 英语 | WPRIM | ID: wpr-938047

摘要

Background@#As the coronavirus disease 2019 (COVID-19) pandemic continues, there are concerns regarding waning immunity and the emergence of viral variants. The immunogenicity of Ad26.COV2.S against wild-type (WT) and variants of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) needs to be evaluated.Method: This prospective cohort study was conducted between June 2021 and January 2022 at two university hospitals in South Korea. Healthy adults who were scheduled to be vaccinated with Ad26.COV2.S were enrolled in this study. The main outcomes included anti-spike (S) IgG antibody and neutralizing antibody responses, S-specific T-cell responses (interferon-γ enzyme-linked immunospot assay), solicited adverse events (AEs), and serious AEs. @*Results@#Fifty participants aged ≥ 19 years were included in the study. Geometric mean titers (GMTs) of anti-S IgG were 0.4 U/mL at baseline, 5.2 ± 3.0 U/mL at 3–4 weeks, 55.7 ± 2.4 U/mL at 5–8 weeks, and 81.3 ± 2.5 U/mL at 10–12 weeks after vaccination. GMTs of 50% neutralizing dilution (ND50) against WT SARS-CoV-2 were 164.6 ± 4.6 at 3-4 weeks, 313.9 ± 3.6 at 5–8 weeks, and 124.4 ± 2.6 at 10–12 weeks after vaccination. As for the S-specific T-cell responses, the median number of spot-forming units/10 6 peripheral blood mononuclear cell was 25.0 (5.0–29.2) at baseline, 60.0 (23.3–178.3) at 5-8 weeks, and 35.0 (13.3–71.7) at 10–12 weeks after vaccination. Compared to WT SARS-CoV-2, ND50 against Delta and Omicron variants was attenuated by 3.6-fold and 8.2-fold, respectively. The most frequent AE was injection site pain (82%), followed by myalgia (80%), fatigue (70%), and fever (50%). Most AEs were grade 1–2, and resolved within two days. @*Conclusion@#Single-dose Ad26.COV2.S was safe and immunogenic. NAb titer and S-specific T-cell immunity peak at 5–8 weeks and rather decrease at 10–12 weeks after vaccination.Cross-reactive neutralizing activity against the Omicron variant was negligible.

15.
文章 在 英语 | WPRIM | ID: wpr-925480

摘要

Purpose@#Pediatric diabetes is a common health burden worldwide. This study aimed to investigate the prevalence of fasting hyperglycemia in Korean youth aged 10–18 years and to evaluate its association with metabolic indicators. @*Methods@#To assess the risk of diabetes in domestic children and adolescents, the prevalence of fasting hyperglycemia was calculated, a trend was evaluated using multi-year Korea National Health and Nutrition Examination Survey (KNHANES) data, and multivariate analysis was performed to evaluate the relationships between hyperglycemia and metabolic factors. @*Results@#The prevalence of fasting hyperglycemia, defined as impaired fasting glucose (fasting glucose level > 100 mg/dL and < 125 mg/dL), or diabetes mellitus (fasting glucose ≥ 126 mg/dL) was estimated in Korean teenagers. The prevalence increased from the fourth (2007–2009) to the fifth (2010–2012), sixth (2013–2015), and seventh (2016–2018) KNHANES surveys, from 5.39 to 4.79, 10.03, and 11.66 per 100 persons, respectively. In multivariate analysis, systolic blood pressure and serum triglycerides were higher in the fasting hyperglycemia group; systolic blood pressures were 109.83 mmHg and 112.64 mmHg and serum triglycerides were 81.59 mg/dL and 89.60 mg/dL in the normal blood glucose and fasting hyperglycemia groups, respectively. @*Conclusion@#The prevalence of fasting hyperglycemia among children and adolescents has increased over the past decade, and this increase is potentially associated with metabolic abnormalities such as hypertension and hypertriglyceridemia. Effort is urgently required to reduce this chronic medical burden in adolescence.

16.
Gut and Liver ; : 290-299, 2022.
文章 在 英语 | WPRIM | ID: wpr-925008

摘要

Background/Aims@#Nonalcoholic fatty liver disease (NAFLD) and obesity are independently associated with an increased risk for atherosclerotic cardiovascular disease (ASCVD), the leading cause of mortality in patients with NAFLD. Many NAFLD patients are lean, but their ASCVD risk compared to obese subjects with NAFLD is unclear. @*Methods@#Data from the 2008 to 2011 Korea National Health and Nutrition Examination Surveysdatabase were analyzed (n=4,786). NAFLD was defined as a comprehensive NAFLD score ≥40 or a liver fat score ≥–0.640. ASCVD risk was evaluated using the American College of Cardiol-ogy/American Heart Association guidelines. @*Results@#The frequency of subjects without NAFLD, with obese NAFLD, and with lean NAFLD was 62.4% (n=2,987), 26.6% (n=1,274), and 11.0% (n=525), respectively. Subjects with lean NAFLD had a significantly higher ASCVD score and prevalence of a high ASCVD risk (mean 15.6±14.0, 51.6%) than those with obese NAFLD and without NAFLD (mean 11.2±11.4, 39.8%; mean 7.9±10.9, 25.5%; all p<0.001). Subjects with lean NAFLD and significant liver fibrosis showed a significantly higher odds ratio for a high risk for ASCVD than those with obese NAFLD with or without significant liver fibrosis (odds ratio, 2.60 vs 1.93; p=0.023). @*Conclusions@#Subjects with lean NAFLD had a significantly higher ASCVD score and prevalence of high risk for ASCVD than those with obese NAFLD. Similarly, lean subjects with significant liver fibrosis had a higher probability of ASCVD than obese subjects in the subpopulation with NAFLD.

17.
文章 在 英语 | WPRIM | ID: wpr-925231

摘要

Background@#and Purpose Hereditary spastic paraplegia (HSP) progresses over time and is associated with locomotive dysfunction. Understanding the factors affecting disease severity and locomotive function is important in HSP. This study investigated the factors influencing disease severity and ambulation status of HSP. @*Methods@#We consecutively enrolled 109 Korean patients (64 males, and 45 females)from 84 families with a clinical diagnosis of HSP. HSP was primarily diagnosed based on clinical criteria including clinical findings, family history, and supported by genetic studies. Epidemiological and clinical features of the patients were analyzed, and the Spastic Paraplegia Rating Scale (SPRS) score and ambulatory status were used to evaluate disease severity. @*Results@#Ninety-two (84.4%) patients had pure HSP, and 55 (50.4%) had a dominant family history. Thirty-one (28.4%) patients required a mobility aid for locomotion. A Kaplan-Meier analysis showed that HSP patients lost their independent gait ability after a median disease duration of 34 years. Those with an age at onset of ≤18 years had a longer median independent walking time. Pure HSP is characterized by predominant bilateral lower extremity weakness and spasticity, whereas complicated HSP presents more complex neurological findings such as ocular and bulbar symptoms, ataxia, and cognitive impairment. Complicated HSP was significantly correlated with the SPRS mobility score (β=3.70, 95% confidence interval=0.45–6.94). The age at onset and disease duration were significantly correlated with disease severity, and they were significant predictors of the use of a mobility aid (p<0.05). @*Conclusions@#These findings suggest that a later age at onset and longer disease duration are significant factors affecting the disease severity and ambulatory function in patients with HSP. These findings can help clinicians to identify subjects at risk of locomotive impairment.

18.
文章 在 韩国 | WPRIM | ID: wpr-968469

摘要

Purpose@#Triage tools play a vital role in classifying the severity of children in emergency departments (EDs). We investigated the association between the Korean Triage and Acuity Scale (KTAS) and severity of dyspnea in the ED. @*Methods@#We conducted a retrospective study of children aged 3-14 years with dyspnea who visited the ED from January 2015 through December 2021. They were divided into severe (KTAS level 1-3) and non-severe (KTAS level 4-5) groups. Between the groups, we compared the clinical characteristics, including age, sex, associated symptoms, vital signs, route of visit, treatment at ED, and outcomes. @*Results@#Among a total of 468 children with dyspnea, 267 and 201 were assigned to the severe and non-severe groups, respectively. The severe group had higher frequencies of fever (21.7% vs. 13.9%; P = 0.031), cough (53.2% vs. 43.3%; P = 0.034), systemic steroids (42.3% vs. 25.9%; P < 0.001), intravenous fluids (47.6% vs. 25.4%; P < 0.001), oxygen therapy (16.5% vs. 6.5%; P = 0.001), inotropics (4.1% vs. 1.0%; P = 0.042), and hospitalization (24.7% vs. 11.9%; P = 0.002). The severe group also showed a higher mean heart rate, respiratory rate, and temperature, and lower mean oxygen saturation (all Ps < 0.001). Among these findings, fever, heart rate, respiratory rate, temperature, intravenous fluids, oxygen therapy, inotropics, and hospitalization remained significantly different between the groups after defining the severe group as a KTAS level 1-2. @*Conclusion@#This study shows the association between KTAS and severity of dyspnea in the ED. Therefore, KTAS may reflect not only the initial clinical conditions but also emergency measures and outcomes in children with dyspnea who visit EDs.

19.
文章 在 英语 | WPRIM | ID: wpr-914928

摘要

Intentional replantation has been used for alternative treatment for conventional root canal therapy. This case report describes that the intentional replantation with application of extrusive orhodontic force for 6 weeks, on which tooth of previous root canal the-rapy. As preapplicatory orthodontic force, tooth moblity was increased so that extraction of the designated tooth was more easily accomplished and augmented volume of periodontal ligament is expected to acceleated gingival reattachment.

20.
文章 在 英语 | WPRIM | ID: wpr-915023

摘要

Maturity-onset diabetes of the young (MODY) is caused by autosomal dominant pathogenic variants in one of 14 currently known monogenic genes. Characteristics of patients with MODY include early-onset clinical disease with a family history of diabetes and negative autoantibodies and may present with heterogeneous phenotypes according to the different subtypes. Here, we report a patient with early-onset diabetes who presented asymptomatic mild fasting hyperglycemia with the absence of autoantibodies. She was diagnosed with glucokinase (GCK)-MODY caused by a GCK variant, c.1289T>C (p.L430P), identified by targeted gene-panel testing, and the affected father had the same variant. We interpreted this rare missense variant as a likely pathogenic variant and then she stopped taking oral medication. This case highlights the usefulness of genepanel testing for accurate diagnosis and appropriate management of MODY. We also note the importance of familial genetic testing and genetic counseling for the proper interpretation of MODY variants.

搜索明细