Hearing loss is the most prevalent disabling disease. Cochlear implantation（CI） serves as the primary intervention for severe to profound hearing loss. This consensus systematically explores the value of genetic diagnosis in the pre-operative assessment and efficacy prognosis for CI. Drawing upon domestic and international research and clinical experience, it proposes an evidence-based medicine three-tiered prognostic classification system（Favorable, Marginal, Poor）. The consensus focuses on common hereditary non-syndromic hearing loss（such as that caused by mutations in genes like GJB2, SLC26A4, OTOF, LOXHD1） and syndromic hereditary hearing loss（such as Jervell & Lange-Nielsen syndrome and Waardenburg syndrome）, which are closely associated with congenital hearing loss, analyzing the impact of their pathological mechanisms on CI outcomes. The consensus provides recommendations based on multiple round of expert discussion and voting. It emphasizes that genetic diagnosis can optimize patient selection, predict prognosis, guide post-operative rehabilitation, offer stratified management strategies for patients with different genotypes, and advance the application of precision medicine in the field of CI.
Humans ; Cochlear Implantation ; Prognosis ; Hearing Loss/surgery* ; Consensus ; Connexin 26 ; Mutation ; Sulfate Transporters ; Connexins/genetics*

Invasive fungal infections (IFIs) have become prominent global health threats, escalating the burden on public health systems. The increasing occurrence of invasive fungal infections is due primarily to the extensive application of chemotherapy, immunosuppressive therapies, and broad-spectrum antifungal agents. At present, therapeutic practices utilize multiple categories of antifungal agents, such as azoles, polyenes, echinocandins, and pyrimidine analogs. Nevertheless, the clinical effectiveness of these treatments is progressively weakened by the emergence of drug resistance, thereby substantially restricting their therapeutic utility. Consequently, there is an imperative need to expedite the discovery of novel antifungal agents. This review seeks to present an exhaustive synthesis of novel antifungal drugs and candidate agents that are either under current clinical investigation or anticipated to progress into clinical evaluation. These emerging compounds exhibit unique benefits concerning their modes of action, antimicrobial spectra, and pharmacokinetic characteristics, potentially leading to improved therapeutic outcomes relative to conventional antifungal regimens. It is anticipated that these novel therapeutic agents will furnish innovative treatment modalities and enhance clinical outcomes in managing invasive fungal infections.

Objective:This study aimed to provide basic data for the prevention, diagnosis and treatment of pediatric viral myocarditis (VMC) in China through analyzing the epidemic characteristics and disease burden of pediatric inpatients with VMC from 2016 to 2021.Methods:We performed a descriptive statistical analysis to the age, genders, seasons, regions and hospitalization cost and days of pediatric VMC inpatients and the death. All of the information was obtained from 27 Children′s hospitals or Maternal and Child Health hospitals of 23 provinces of China from 2016 to 2021.Results:A total of 7 647 599 cases including 1 646 VMC inpatients were admitted into our study. The annual numbers of hospitalizations were 173, 227, 313, 301, 295 and 337, with the hospitalized constituent ratios being 14.9/100 000, 17.9/100 000, 23.0/100 000, 20.5/100 000, 26.5/100 000 and 26.4/100 000 from 2016 to 2021. In recent 6 years, the proportion of VMC hospitalizations had increased yearly ( P<0.001), and had associated with the onset age ( P<0.001). Aged 12-≤18 years owned the highest hospitalized constituent ratio. The Northeast of China owned the largest number of VMC inpatients, and the East second to it. Among the 1 646 VMC children, there were 68 deaths, with the hospitalized case fatality rate of 4.13%. There were no significant differences between genders, age, seasons, years and fatality rate of VMC inpatients. For the diseases burden, the median of hospitalization days of all VMC inpatients was 10 days (IQR 6, 21), and the median of hospitalization cost was 1 1 842.3 RMB (IQR 6 969.22, 19 714.78). The median of hospitalization days of deceased VMC children was only 1 day (IQR 1, 3), the median cost could be 8 874.03 RMB (IQR 5 277.94, 5 6 151.59). Conclusions:In this study, we found that proportion of hospitalization of VMC children increased year by year, adolescence might be a risk factor of VMC. The fatality of VMC inpatients could be up to 4.13%, and the death led to a huge economic burden of society, family and individuals.

Objective The scope of related research on ethical conflicts in the interprofessional collaboration in nursing was reviewed,so as to summarize the obstacles and coping strategies that lead to ethical conflicts in interprofessional collaboration and provide references for promoting nurse engagement in interprofessional collaboration.Methods Based on the methodological framework of the scope review,we systematically searched domestic and foreign databases including the Cochrane Library,CINAHL,Embase,PubMed,Web of Science,CNKI,VIP,Wanfang and SinoMed.The time limit for database retrieval was from the establishment of each database to March 2023.The selected studies were summarized and analyzed.Results Totally 13 articles published between 1991 and 2022 were included.The obstacle factors that lead to ethical conflicts in nurse engagement of interprofessional collaboration can be divided into 3 categories,including personal factors(insufficient professional knowledge,differences in professional values),interprofessional factors(imbalanced power between professions,lack of respect and collaboration between cooperators)and environmental factors(insufficient resources and staffing,high-intensity workload,and high-pressure work environment).At the same time,in view of these 3 aspects,it puts forward some countermeasures,such as enhancing professional knowledge,strengthening interprofessional education,improving understanding and cooperation among medical workers in different wards,providing medical practitioners with a team-based,ethical work environment that facilitates interprofessional collaborations and open dialogues.Conclusion It is of great significance for nurses to get involved in interprofessional collaborations,in order to ensure high-quality nursing services.Further studies are needed for the development of education and the core competency framework construction for interprofessional collaborations in nursing.More attention should be paid to develop the organizational atmosphere at the same time,so as to continuously improve nurse'capabilities in interprofessional collaborations.

Objectives: . Branchio-oto syndrome (BOS) primarily manifests as hearing loss, preauricular pits, and branchial defects. EYA1 is the most common pathogenic gene, and splicing mutations account for a substantial proportion of cases. However, few studies have addressed the structural changes in the protein caused by splicing mutations and potential pathogenic factors, and several studies have shown that middle-ear surgery has limited effectiveness in improving hearing in these patients. BOS has also been relatively infrequently reported in the Chinese population. This study explored the genetic etiology in the family of a proband with BOS and provided clinical treatment to improve the patient’s hearing. Methods: . We collected detailed clinical features and peripheral blood samples from the patients and unaffected individuals within the family. Pathogenic mutations were identified by whole-exome sequencing and cosegregation analysis and classified according to the American College of Medical Genetics and Genomics guidelines. Alternative splicing was verified through a minigene assay. The predicted three-dimensional protein structure and biochemical experiments were used to investigate the pathogenicity of the mutation. The proband underwent middle-ear surgery and was followed up at 1 month and 6 months postoperatively to monitor auditory improvement. Results: . A novel heterozygous EYA1 splicing variant (c.1050+4 A>C) was identified and classified as pathogenic (PVS1(RNA), PM2, PP1). Skipping of exon 11 of the EYA1 pre-mRNA was confirmed using a minigene assay. This mutation may impair EYA1-SIX1 interactions, as shown by an immunoprecipitation assay. The EYA1-Mut protein exhibited cellular mislocalization and decreased protein expression in cytological experiments. Middle-ear surgery significantly improved hearing loss caused by bone-conduction abnormalities in the proband. Conclusion . We reported a novel splicing variant of EYA1 in a Chinese family with BOS and revealed the potential molecular pathogenic mechanism. The significant hearing improvement observed in the proband after middle-ear surgery provides a reference for auditory rehabilitation in similar patients.

Hip fracture is considered as the most severe osteoporotic fracture characterized by high disability and mortality in the elderly. Improved surgical techniques and multidisciplinary team play an active role in alleviating prognosis, which places higher demands on perioperative nursing. Dysfunction, complications, and secondary impact of anaesthesia and surgery add more difficulties to clinical nursing. Besides, there still lack clinical practices in perioperative nursing for elderly patients with hip fracture in China. In this context, led by the Orthopedic Nursing Committee of Chinese Nursing Association, the Expert consensus on clinical practice in perioperative nursing for elderly patients with hip fracture ( version 2023) is developed based on the evidence-based medicine. This consensus provides 11 recommendations on elderly patients with hip fracture from aspects of perioperative health education, condition monitoring and inspection, complication risk assessment and prevention, and rehabilitation, in order to provide guiding advices for clinical practice, improve the quality of nursing and ameliorate the prognosis of elderly patients with hip fracture.

Hip fractures are among the most common fractures in the elderly, presenting to be a leading cause of disability and mortality. Surgical treatment is currently the main treatment method for hip fractures. The incidence of perioperative malnutrition is increased after hip fractures in the elderly due to the comorbidities, decreased basal metabolic rate, accelerated protein breakdown, weakened anabolism and surgical stress. However, malnutrition not only increases the incidence of postoperative complications, but also leads to increased mortality, indicating an important role of perioperative nursing management of nutrition for the elderly patients with hip fractures. At present, there still lacks scientific guidance and application standards on perioperative nursing management of nutrition for the elderly patients with hip fractures. Therefore, the Orthopedic Nursing Committee of Chinese Nursing Association and the Editorial Board of Chinese Journal of Trauma organized relevant experts to formulate the Expert consensus on perioperative nursing management of nutrition for elderly patients with hip fractures ( version 2023) according to evidence-based medical evidences and their clinical experiences. Fourteen recommendations were made from aspects of nutrition screening, nutrition assessment, nutrition diagnosis, nutrition intervention and nutrition monitoring to provide guidance for perioperative nursing management of nutrition in elderly patients with hip fractures.

To summarize the nursing experience of a child with pulmonary rejection after hematopoietic stem cell transplantation who underwent double lung transplantation and was complicated with bronchiolitis obliterans after lung transplantation and underwent second lung transplantation.The key points of nursing included:the implementation of step-wise lateral position and prone position alternate non-invasive mechanical ventilation to improve lung function;sequential airway clearance was used to control pulmonary infection;precise volume management to prevent pulmonary edema;to strengthen the observation and medication management,prevent hospital infection and rejection;to provide goal-oriented personalized nutritional support to improve nutritional status;psychological nursing measures were implemented to improve the treatment confidence and compliance of children;health education and follow-up management should be strengthened to improve the long-term quality of life.After active treatment and careful nursing,the child recovered and was discharged 26 days after surgery.

Background China is facing enormous challenges of occupational disease prevention and control and high incidences of occupational diseases. Occupational disease diagnosis is an important part of occupational disease prevention and control. However, the current situation of occupational disease diagnosis service system in China is not optimistic. Relevant national laws, regulations, policies, and plans require to improve the current technical support system of occupational disease diagnosis and treatment, and to establish a sound occupational disease diagnosis, treatment, and rehabilitation network on the foundation of existing medical and health institutions. Objective To analyze the development status and existing problems of occupational disease diagnosis institutions in China, and propose countermeasures and suggestions. Methods All occupational disease diagnosis institutions in China were surveyed by questionnaire using comprehensive census. The Occupational Health Center of the National Health Commission organized the preparation of the questionnaire and the participation of all relevant institutions in the survey. All data collected in the survey were for the year 2020 and available as of December 31, 2020. The questionnaire covered the overall situation, service provided, staffing of certified physicians for providing occupational disease diagnosis, and instruments in selected categories of occupational disease diagnosis institutions. Results As of December 2020, there were 587 occupational disease diagnosis institutions in China; the highest average number of enterprises served by an occupational disease diagnosis institution was 39000, and that of employees served by an occupational disease diagnosis institution was 2.15 million in Chongqing. There were a total of 5809 physicians certified to diagnose occupational diseases in China, with 20.4 doctors per specified occupational disease prevention and treatment hospital, 9.5 doctors per general hospital, and 8.3 doctors per disease control and prevention center. Only 87.7% of the institutions were equipped with digital radiography (DR), 58.1% with computed tomography (CT), and 96.4% with pulmonary function meters; the equipment rate of computed radiography (CR) was only 6.5%, and that of CT was only 14.6% in all-level disease control and prevention centers; 45 disease control and prevention centers were not equipped with any common x-ray machine, CR, DR, or CT. Conclusion In the face of the still serious situation of prevention and treatment of occupational diseases, the overall distribution of institutions is uneven around China, the number of institutions in some regions is relatively insufficient, and the comprehensive capacity of centers for disease control and prevention at all levels needs to be improved. Strengthening comprehensive capacity building of occupational disease diagnosis institutions and training of relevant physicians would be helpful to ensure the accuracy and scientificity of diagnosis. At the national level, further increasing policy guidance and financial input would help occupational disease diagnosis institutions upgrade their techniques and service capacity, and protect workers' occupational health rights.

Objective:To investigate the clinical characteristics of central nervous system tuberculosis in adults and the possible factors affecting the mortality and disability of the patients.Methods:The clinical data of patients diagnosed as "tuberculous meningitis" "tuberculous meningoencephalitis" "tuberculous cerebrospinal meningitis" or "tuberculous brain ubscess" in Huashan Hospital, Fudan University and Jing′an Branch, Huashan Hospital, Fudan University in Shanghai from January 1, 2010 to December 31, 2017 were collected, and a retrospective cohort was established. The clinical characteristics were analyzed, Medical Research Council (MRC) grade system was used to assess the severity of meningitis, and the modified Rankin Scale was used to assess the impairment of self-care. Survival rate and disability rate of the cohort were analyzed. Binary logistic regression was used for multivariate analysis. Kaplan-Meier survival curve was used for survival analysis.Results:A total of 161 patients with central nervous system tuberculosis were enrolled. Among the 161 patients, 55 cases (34.2%) were confirmed, 72 cases (44.7%) were highly suspected and 34 cases (21.1%) were suspected diagnosis. There were 56 cases (34.8%) with MRC grade Ⅰ, 76 cases (47.2%) with MRC grade Ⅱ and 29 cases (18.0%) patients with MRC grade Ⅲ before treatment. Up to January 1, 2019, ten (6.2%) patients died, 32 (19.9%) patients lost to follow-up, 119 (73.9%) patients survived. The five-year survival rate was 92.83%. There were 72 patients with no impact on life, 34 patients with moderate impact and 13 patients with severe impact. The disability rate was 39.5% (47/119). Binary logistic regression analysis showed that increasing age (odds ratio ( OR)=1.06, 95%confidence interval ( CI) 1.00 to 1.13, P=0.032) and deterioration of MRC grade during anti-tuberculosis treatment ( OR=89.00, 95% CI4.46 to 1 779.00, P=0.003) were independent risk factors for death. When severe disability and death were used as adverse outcomes, logistic regression analysis showed increasing age ( OR=1.07, 95% CI 1.01 to 1.13, P=0.035) and deterioration of MRC grade during anti-tuberculosis treatment ( OR=77.17, 95% CI4.45 to 1 337.00, P=0.003) were still independent risk factors for adverse outcomes. Conclusions:The mortality of central nervous system tuberculosis in adults in this cohort is relatively low, but the disability rate is still high. Increasing age and deterioration of MRC grade during anti-tuberculosis treatment are independent risk factors for death and disability.