Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder characterized by uncontrolled terminal complement activation. Eculizumab, a monoclonal antibody C5 inhibitor was introduced in Korea in 2009 and has been the standard treatment option for PNH. Methods: This study assessed the long-term efficacy/safety of eculizumab in PNH using real-world data from the Korean Health Insurance Review and Assessment Service. Eighty patients who initiated eculizumab from 2009–2020 were enrolled. Results: At eculizumab initiation, the median age was 51.5 years, lactate dehydrogenase (LDH) 6.8 × upper limit of normal, and granulocyte clone size 93.0%. All patients had at least one PNH-related complication before eculizumab initiation, including renal failure (n = 36), smooth muscle spasm (n = 24), thromboembolism (n = 20), and pulmonary hypertension (n = 15). The median (range) duration of eculizumab treatment was 52.7 (1.0, 127.3) months (338.6 total treated patient-years). Despite high disease activity in the study population before treatment initiation, overall survival was 96.2% and LDH levels were stabilized in most patients during treatment. PNH-related complications at treatment initiation were resolved in 44.4% of patients with renal failure, 95.8% with smooth muscle spasm, 70.0% with thromboembolism, and 26.7% with pulmonary hypertension. Extravascular hemolysis occurred in 28.8% of patients (n = 23; 0.09 per patient-year) and breakthrough hemolysis in 18.8% (n = 15; 0.06 per patient-year). No treatment discontinuation cases related to eculizumab were observed. Conclusion These data provided evidence for the long-term efficacy and safety of eculizumab in Korean PNH patients with high disease burdens.

Objectives: . Thyroid cancer is the most common endocrine tumor, with rapidly increasing incidence worldwide. However, its transcriptomic characteristics associated with immunological signatures, driver fusions, and recurrence markers remain unclear. We aimed to investigate the transcriptomic characteristics of advanced papillary thyroid cancer. Methods: . This study included 282 papillary thyroid cancer tumor samples and 155 normal samples from Chungnam National University Hospital and Seoul National University Hospital. Transcriptomic quantification was determined by high-throughput RNA sequencing. We investigated the associations of clinical parameters and molecular signatures using RNA sequencing. We validated predictive biomarkers using the Cancer Genome Atlas database. Results: . Through a comparison of differentially expressed genes, gene sets, and pathways in papillary thyroid cancer compared to normal tumor-adjacent tissue, we found increased immune signaling associated with cytokines or T cells and decreased thyroid hormone synthetic pathways. In addition, patients with recurrence presented increased CD8+ T-cell and Th1-cell signatures. Interestingly, we found differentially overexpressed genes related to immune-escape signaling such as CTLA4, IDO1, LAG3, and PDCD1 in advanced papillary thyroid cancer with a low thyroid differentiation score. Fusion analysis showed that the PI3K and mitogen-activated protein kinase (MAPK) signaling pathways were regulated differently according to the RET fusion partner genes (CCDC6 or NCOA4). Finally, we identified HOXD9 as a novel molecular biomarker that predicts the recurrence of thyroid cancer in addition to known risk factors (tumor size, lymph node metastasis, and extrathyroidal extension). Conclusion . We identified a high association with immune-escape signaling in the immune-hot group with aggressive clinical characteristics among Korean thyroid cancer patients. Moreover, RET fusion differentially regulated PI3K and MAPK signaling depending on the partner gene of RET, and HOXD9 was found to be a recurrence marker for advanced papillary thyroid cancer.

Background: Inbred mice have several advantages, including genetic similarity to humans, a well-established gene manipulation system, and strong tolerance to inbreeding. However, inbred mice derived from a limited genetic pool have a small genetic diversity. Thus, the development of new inbred strains from wild mice is needed to overcome this limitation. Hence, in this study, we used a new strain of inbred mice called KWM/Hym. We sequenced the Mx1 gene to elucidate the genetic diversities of KWM/Hym mice and observed the biological alterations of the Mx1 protein upon influenza A infection. Results: The Mx1 gene in KWM/Hym mice had 2, 4, and 38 nucleotide substitutions compared to those in the Mx1 gene in A2G, CAST/EiJ, and Mus spretus mice, respectively. Moreover, the Mx1 protein in KWM/Hym mice had 2 and 25 amino acid substitutions compared to those in the Mx1 protein in CAST/EiJ and M. spretus mice, respectively. To elucidate the function of the Mx1 protein, we inoculated the influenza A virus (A/WSN/1933) in KWM/Hym mice. Nine days after infection, all infected KWM/Hym mice survived without any weight loss. Four days after infection, the lungs of the infected KWM/Hym mice showed mild alveolitis and loss of bronchiolar epithelium; however, the pulmonary viral titers of the infected KWM/Hym mice were significantly lower than that in the infected BALB/c mice (2.17 × plaqueforming units mL −1 ). Conclusions Our results demonstrate that the KWM/Hym mice are resistant to influenza A virus infection. Further, these mice can be used as a model organism to understand the mechanism of influenza A virus susceptibility.

Purpose: This study is a pseudo-experimental study of design before and after the non-equivalent control group, which was attempted to verify that the application of the ukulele to the elderly has the effects of reducing depression, improving self-efficacy, strengthening social bonding, and improving cognitive function. Methods: 46 (23 in the experimental group and 23 in the control group) participants were selected. The experimental group was provided with three sessions of a music program using eight Ukuleles for the elderly, while the control group was provided with three sessions for the elderly. IBM SPSS 25.0 was used for data analysis, and the independent t-test, x2 -test, and Fisher's exact probability test were performed to verify the homogeneity of the subject's general characteristics.The effect verification after the experimental treatment was analyzed by Fisher's exact probability test, Friedman test, and Mann-Whitney U test. Results: Depression showed a statistically significant difference between the two groups (F=39.88, p<.001), self-efficacy showed a statistically significant difference between the two groups (z=-4.96, p<.001), social bonding showed a statistically significant difference between the two groups (z=-5.19, p<.001), and cognitive function showed a statistically significant difference between the two groups (z=-3.98, p<.001). Conclusion It was found that the ‘Music Program using the Ukulele’ was effective in reducing depression of the elderly, improving self-efficacy, reinforcing social bonding, and improving cognitive function. We hope that the Music Program using the Ukulele can be used in the elderly nursing curriculum in the future, and we suggest it should be applied as a nursing intervention to those who are experiencing cognitive decline.

Objective: High-quality intensive care, including targeted temperature management (TTM) for patients with postcardiac arrest syndrome, is a key element for improving outcomes after out-of-hospital cardiac arrest (OHCA). We aimed to assess the status of postcardiac arrest syndrome care, including TTM and 6-month survival with neurologically favorable outcomes, after adult OHCA patients were treated with TTM, using data from the Korean Hypothermia Network prospective registry. Methods: We used the Korean Hypothermia Network prospective registry, a web-based multicenter registry that includes data from 22 participating hospitals throughout the Republic of Korea. Adult comatose OHCA survivors treated with TTM between October 2015 and December 2018 were included. The primary outcome was neurological outcome at 6 months. Results: Of the 1,354 registered OHCA survivors treated with TTM, 550 (40.6%) survived 6 months, and 413 (30.5%) had good neurological outcomes. We identified 839 (62.0%) patients with preClinsumed cardiac etiology. A total of 937 (69.2%) collapses were witnessed, shockable rhythms were demonstrated in 482 (35.6%) patients, and 421 (31.1%) patients arrived at the emergency department with prehospital return of spontaneous circulation. The most common target temperature was 33°C, and the most common target duration was 24 hours. Conclusion The survival and good neurologic outcome rates of this prospective registry show great improvements compared with those of an earlier registry. While the optimal target temperature and duration are still unknown, the most common target temperature was 33°C, and the most common target duration was 24 hours.

Purpose: The purpose of the present study is to examine the factors influencing intention of dementia prevention behavior in adults with hypertension or diabetes. Methods: A total of 113 community-dwelling adults with hypertension or diabetes agreed to participate in this study. Data were analyzed using descriptive statistics, t-test, ANOVA, correlation, and multiple regression. Results: Self-efficacy and cues to action toward dementia prevention behavior were factors influencing intention of dementia prevention behavior of participations. Self-efficacy toward dementia prevention behavior was the most influential factor. These factors accounted for 22% of intention of dementia prevention behavior. Conclusion These results suggest a need for strengthening self-efficacy to increase intention of dementia preventive behaviors. And dementia prevention programs should be developed in consideration of strategies to reinforce cues to action.

Korean Society of Thyroid-Head and Neck Surgery appointed a Task Force to provide guidance on the implementation of a surgical treatment of oral cancer. MEDLINE databases were searched for articles on subjects related to “surgical management of oral cancer” published in English. Results were restricted to systematic reviews, randomized control trials/controlled clinical trials, and observational studies. The quality of evidence was rated with use RoBANS (Risk of Bias Assessment Tool for Nonrandomized Studies) and AMSTAR (A Measurement Tool to Assess the Methodological Quality of Systematic Reviews). Evidence-based recommendations for practice were ranked according to the American College of Physicians grading system. Additional directives are provided as expert opinions and Delphi questionnaire when insufficient evidence existed. The Committee developed 68 evidence-based recommendations in 34 categories intended to assist clinicians and patients and counselors, and health policy-makers. Proper surgical treatment selection for oral cancer, which is directed by patient- and subsite-specific factors, remains the greatest predictor of successful treatment outcomes. These guidelines are intended for use in conjunction with the individual patient's treatment goals.
Advisory Committees ; Bias (Epidemiology) ; Carcinoma, Squamous Cell ; Counseling ; Expert Testimony ; Humans ; Mouth Neoplasms ; Neck ; Republic of Korea

PURPOSE: This descriptive study was conducted to examine factors that affect hospital workers in their decision to withdraw from life-sustaining treatment, such as knowledge, attitude, and perception of organ donation, transplantation, death and hospice palliative care. METHODS: A questionnaire was completed by 228 workers of a tertiary general hospital, and data were analyzed using t-test, ANOVA, and Pearson's correlation by using SPSS 21.0. RESULTS: The subjects' knowledge of biomedical ethics awareness differed by age, education level, occupation, affiliated department, and biomedical ethics education. Their knowledge of brain death, organ donation and transplantation was positively correlated with attitudes toward tissue donation and transplantation, knowledge of hospice palliative care, and perception of hospice palliative care. Their attitudes toward tissue donation and transplantation were significantly correlated with knowledge of hospice palliative care, perception of hospice palliative care, and withdrawal of life-sustaining treatment. Their awareness of death was significantly correlated with knowledge of hospice palliative care, perception of hospice palliative care and withdrawal of life-sustaining treatment. The perception of hospice palliative care was significantly correlated with withdrawal of life-sustaining treatment. Factors associated with their withdrawal of life-sustaining treatment were work at the hospice ward (32.5%), attitudes toward tissue donation and transplantation and perception of hospice palliative care. CONCLUSION: This study has shown that work at the hospice ward, attitudes toward tissue donation and transplantation and perception of hospice palliative care were related to attitudes toward withdrawal of life-sustaining treatment. More research is needed to further develop various curriculums based on biomedical methods.
Bioethics ; Brain Death ; Curriculum ; Education ; Hospice Care ; Hospices* ; Hospitals, General* ; Occupations ; Palliative Care ; Tissue and Organ Procurement* ; Transplantation

PURPOSE: This descriptive study is aimed at identifying how nursing hospital workers' performance of end-of-life care is influenced by their knowledge and awareness of hospice palliative care, attitude towards end-of-life care, performance, importance, awareness of death and the factors. METHODS: A self-reported questionnaire was used to collect data from 113 workers at an accredited nursing hospital in K province. Variables were their knowledge and awareness of hospice palliative care, attitude towards end-of-life care, end-of-life care performance and importance and awareness of death. An analysis was performed with the frequency, percentage, mean, standard deviation, t-test, ANOVA, Scheffe's test, Pearson's correlation coefficient and multiple regression using IBM SPSS 21.0. RESULTS: The factors affecting the nursing hospital workers' end-of-life care performance were the importance of end-of-life care and their marital status, which showed an explanatory power of 38.2%. CONCLUSION: In order to improve the nursing hospital workers' end-of-life care performance, a training on the importance of end-of-life care should be provided. Therefore, we would like to propose establishing administrative measures such as 1) efficient staffing to help the caregivers better perform what they think is important, 2) development of a training program that can improve their performance of end-of-life nursing care and 3) a study to verify the effectiveness of the program.
Caregivers ; Education ; Hospices ; Humans ; Marital Status ; Nursing Care ; Nursing* ; Palliative Care

PURPOSE: The purpose of this study was to construct and test a structural equation modeling on the reproductive health behavior of single women with sexual experiences. This study employed Ajzen's Theory of Planned Behavior (TPB). METHODS: The data were collected after receipt of consent from 250 single women with sexual experiences, and analyzed using SPSS 18.0 and AMOS 18.0. RESULTS: Model fit indices for the hypothetical model were suitable for the recommended level: x²=362.407, RMR=0.065, RMSEA=0.070, GFI=0.867. TLI=0.927, CFI=0.938, IFI=0.939, and x²/dF=2.237. Intention showed direct effect with the biggest effect being on reproductive health behavior. Attitude, subjective norm and perceived behavioral control were found to have a direct effect on intention. Among them, perceived behavioral control revealed the largest influence. CONCLUSION: This study suggests that the TPB is a suitable model in explaining the reproductive health behavior of single women with sexual experience. Strategic plans for educational and intervention programs should be aimed to encourage single women to engage in reproductive health behavior.
Female ; Humans ; Intention ; Reproductive Health* ; Single Person*