There is a substantial unmet need for treatments in the field of pediatric rare diseases, and investigator initiated trial(IIT) provide a critical pathway for testing and developing new drugs or treatment strategies. However, healthcare institutions, when conducting such research, must address compliance risks related to project approval, contract management, data protection, and conflict of interest management. This study aims to analyze the particularities and challenges of IIT in pediatric rare diseases, review relevant regulations and regulatory requirements, and provide healthcare institutions with a reference framework for compliance risk management to maximize the benefits of IIT. Based on literature review, analysis of laws and regulations, practical work experience, and frameworks from other institutions, we summarize the unique aspects of pediatric rare disease IIT in terms of participant characteristics, innovative technologies, and organizational structures.On this basis, targeted compliance management recommendations are proposed, which include establishing a risk rating and full-cycle risk monitoring mechanism, a consent and ethical review mechanism tailored to pediatric participants, a robust contract management mechanism, a comprehensive data security management mechanism, and a multidisciplinary team and multi-channel compensation mechanism. The study concludes that healthcare institutions, funders, and other collaborating entities should implement compliance management in line with the characteristics of IIT to ensure the safety and effectiveness of research and facilitate innovation and development in the treatment of pediatric rare diseases.

Objective:To analyze the distribution and drug resistance of wound pathogenic microorganisms in outpatients of wound healing center so as to provide a basis for the standardized construction of wound healing centers.Methods:A retrospective case series study was used to analyzed the data of 365 outpatients treated at Ruijin Hospital, Shanghai Jiaotong University School of Medicine from December 2017 to October 2019. There were 220 males and 145 females, aged (58.8±18.9)years (range, 18-98 years). The patients included 92 first-visit patients and 273 re-visit patients. The culture results (positive rate of pathogenic microorganisms, bacterial species, bacterial distribution) and drug sensitivity results of the wound secretions were compared and analyzed.Results:(1) Among 365 samples of wound secretions, 198 patients were positive for pathogenic microorganisms with a positive rate of 54.3%. A total of 107 strains (51.0%) of Gram-positive bacteria were detected, mainly Staphylococcus aureus (70 strains, 33.3%); 95 strains (45.2%) of Gram-negative bacteria were detected, mainly Escherichia coli (20 strains, 9.5%), followed by Pseudomonas aeruginosa (17 strains, 8.1%); 8 strains (3.8%) of fungi were detected. (2) A total of 26 (28.3%) first-visit patients were positive for pathogenic microorganisms, and 172 (63.0%) re-visit patients were positive for pathogenic microorganisms. The rate of positive microorganism detection had significant differences between first-visit and re-visit patients ( P<0.05). (3) A total of 29 strains were detected in first-visit patients, including 16 strains (55.2%) of Gram-positive bacteria, 11 strains (37.9%) of Gram-negative bacteria and 2 strains (6.9%) of fungi. A total of 181 strains were detected in re-visit patients, including 91 strains (50.3%) of Gram-positive bacteria, 84 strains (46.4%) of Gram-negative bacteria and 6 strains (3.3%) of fungi. The microbial distribution was significantly different between first-visit and re-visit patients ( P<0.05). (4) Compared with first-visit patients, the resistance of Staphylococcus aureus isolated from the re-visit patients to spenicillin, oxacillin, ciprofloxacin, tetracycline, clindamycin, moxifloxacin, erythromycin, and levofloxacin were increased variably. No vancomycin-resistant Staphylococcus aureus was detected, indicating that the staphylococcus aureus presented in the wound was highly sensitive to vancomycin. Conclusions:Staphylococcus aureus is the most common microorganism in wound secretions in outpatients of wound healing center. The rate of positive pathogenic microorganisms in wound secretions of re-visit patients is significantly higher than that of first-visit patients, and the distribution of pathogenic microorganisms of first-visited and revisited patients differs significantly. The Staphylococcus aureus detected in re-visit patients has a higher resistance to common antibiotics compared with first-visit patients. It is suggested that timely detection of pathogenic microorganisms in outpatients and effective control and supervision of outpatient infections are important contents that cannot be ignored in the construction of wound healing center.

According to a document issued by the General Office of National Health Commission, "one person, one diagnosis, and one room" is required in the process of outpatient consultation. However, the patient will need to go to another room for dressing change after the doctor checks the wound if sticking to the conventional layout of current wound repair specialist outpatient clinic in hospitals and following the regulation of "separation of diagnosis and treatment". To allow a patient walking back and forth with the exposed wounds to different clinics or going to another clinic for dressing change with the original dressing reapplied to the wound is against the regulation of nosocomial infection control and the principle of sterility. To ensure that the layout of the outpatient clinic in the wound repair outpatient department not only conforms to the principle of "one person, one diagnosis, and one room", but also meets the characteristics of the diagnosis and treatment process of chronic wounds, this paper proposes the layout of "large space and small partition" in the wound repair clinic.

Objective:To explore the application value of flexible endoscopy and rigid endoscopy in the clinical examination of chronic sinus tract wounds with different shapes.Methods:A retrospective observational study was conducted. From January 1 to December 23, 2019, a total of 46 patients with chronic sinus tract wounds, who met the inclusion criteria were admitted to the Wound Healing Center of Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, including 23 males and 23 females, aged 18-81 (48±21) years. On admission, computer tomography (CT) imaging and three-dimensional reconstruction were performed to examine the shapes of wound sinus tract and classify the wounds, with the lengths of wound sinus tract by CT imaging examination (hereinafter referred to as reference lengths) recorded. The lengths of wound sinus tract were examined and measured by rigid endoscopy and flexible endoscopy. The wounds with and without obviously curved sinus tract were classified into curve group and linear group respectively, and the deviation rates between the lengths of wound sinus tract measured by flexible endoscopy or rigid endoscopy and the reference lengths (hereinafter referred to as deviation rates of lengths) in each group were calculated. The difference between the deviation rates of lengths examined by flexible endoscopy and rigid endoscopy and the differences between the above two and the deviation rate of reference lengths (0) in each group were compared. Data were statistically analyzed with paired sample t test and Wilcoxon signed rank sum test. Results:CT imaging and three-dimensional reconstruction showed that there were 4 types of wound sinus tract, including tubular (36/46), lamellar (4/46), club-mallet (4/46), and irregular (2/46) shape. Tubular wounds were further divided into type I (23/36), type L (4/36), and type Y (9/36). Wounds with type I tubular, lamellar, and club-mallet sinus tract were classified into linear group (31/46), while those with type Y tubular, type L tubular, and irregular sinus tract were classified into curve group (15/46). In linear group, the deviation rates of lengths examined and measured by rigid endoscopy and flexible endoscopy were 0. In curve group, the deviation rate of lengths examined and measured by flexible endoscopy was 0 (0, 0.58%), which was significantly lower than 41.18% (31.68%, 48.41%) examined and measured by rigid endoscopy, Z=-3.408, P<0.01; the deviation rate of lengths examined and measured by rigid endoscopy (40±19)% was significantly higher than the deviation rate of reference lengths ( t=8.343, P<0.01), while the deviation rate of the lengths examined and measured by flexible endoscopy was similar to the deviation rate of reference lengths ( Z=-1.342, P>0.05). Conclusions:Compared with rigid endoscopy, flexible endoscopy can observe the internal characteristics of chronic sinus tract wounds in a wider range in the clinical examination of this kind of wound, especially for the exploration of curved chronic sinus tract wounds. The promotion of this method will be conducive to the diagnosis and treatment of chronic sinus tract wounds.

Statistics show that 76.74% (4 688) of 6 109 patients with chronic wounds are those over 50 years of age; the proportion of patients with underlying diseases in all age groups above 50 years ranges from 78.25% to 100.00%; among the underlying diseases of chronic wound patients, the top four diseases are diabetes mellitus , cardiovascular and cerebrovascular diseases, hypertension, and respiratory diseases. The above underlying diseases and ages of patients are the susceptibility factors of corona virus disease 2019 released by National Health Commission of China. It is an unavoidable fact that patients with chronic wounds have to go to the hospital for treatment prescribed by the physician. At the same time, we found that there were not a few patients who go far afield because of various reasons when go to the hospital for treatment. During the period of epidemic prevention and control, this kind of "go far afield" style of seeking medical treatment may increase the exposure risk during transportation. Accordingly, we convened 36 wound care clinics in different regions in Shanghai to implement the "Five Measures" to encourage patients with chronic wounds to seek medical treatment proximately. The principle of this operation is that when seeking medical treatment, trying our best to reduce as much as possible the transportation distance for patients with chronic wounds to minimize the exposure risk during the epidemic period and eventually support the epidemic prevention and control campaign.

Objective:To conduct a bibliometric analysis of foreign researches on nursing informatics and to provide references for the future research in China.Methods:Through the bibliometrics method, the Web of Science core collection was used for retrieval. The retrieval was based on SCI-E and SSCI, and the time span was from 2009 to 2019. All analytical data were searched and downloaded on August 9 2019. CiteSpace visualization tool was used for analysis.Results:A total of 6 205 literatures related to nursing informatics published from 2009 to 2019 were obtained, and the number of published literatures was increasing. The UK had an innate advantage in nursing informatics research, with more than half of the top 10 academic journals published in the UK. The research results of nursing informatics were mainly published in clinical medicine, psychology, education and other disciplines. Among the top 10 research institutions with the most publications, most of them were from the United States, which had a great influence in this field. The current hotspots of nursing informatics were mainly in clinical decision support, medical big data, hospital information system, nursing education and so on.Conclusions:In recent years, the research on nursing informatics has attracted much attention and is currently in a rapid development stage. The current hotspots of nursing informatics are mainly in clinical decision support, medical big data, hospital information system, nursing education and other aspects. However, ICU, barrier, cancer, satisfaction and other key words will still have research value in the future.

Immunoglobulin G against myelin oligodendrocyte glycoprotein (MOG-IgG) is detectable in neuromyelitis optica spectrum disorder (NMOSD) without aquaporin-4 IgG (AQP4-IgG), but its pathogenicity remains unclear. In this study, we explored the pathogenic mechanisms of MOG-IgG in vitro and in vivo and compared them with those of AQP4-IgG. MOG-IgG-positive serum induced complement activation and cell death in human embryonic kidney (HEK)-293T cells transfected with human MOG. In C57BL/6 mice and Sprague-Dawley rats, MOG-IgG only caused lesions in the presence of complement. Interestingly, AQP4-IgG induced astroglial damage, while MOG-IgG mainly caused myelin loss. MOG-IgG also induced astrocyte damage in mouse brains in the presence of complement. Importantly, we also observed ultrastructural changes induced by MOG-IgG and AQP4-IgG. These findings suggest that MOG-IgG directly mediates cell death by activating complement in vitro and producing NMOSD-like lesions in vivo. AQP4-IgG directly targets astrocytes, while MOG-IgG mainly damages oligodendrocytes.

The " exploration of treatment technology of chronic wound with sinus tract supported by endoscope and related auxiliary methods" study had been conducted by Wound Healing Center of Ruijin Hospital, Shanghai Jiao Tong University School of Medicine. The feasibility and effectiveness of this technique have been confirmed, and good clinic results have been achieved. In order to further promote the new technology and its related research, the theoretical knowledge and technical experience accumulated in the early stage are summarized as diagnosis and treatment standard for treatment with endoscopy technique in chronic wounds with sinus tract, including four parts: the applicable subjects, the diagnosis and treatment procedures and classification criteria, the healing criteria, and the risk assessment and prevention measures. The purpose of this standard is to facilitate the application of standardized endoscopy technique, to make the most of its technological advantages, prevent risks, and provide a reference for the official version of the diagnosis and treatment standard.

Whether habit stimulation is effective in DOC patient arousal has not been reported. In this paper, we analyzed the responses of DOC patients to habit stimulation. Nineteen DOC patients with alcohol consumption or smoking habits were recruited and 64-channel EEG signals were acquired both at the resting state and at three stimulation states. Wavelet transformation and nonlinear dynamics were used to extract the features of EEG signals and four brain lobes were selected to investigate the degree of EEG response to habit stimulation. Results showed that the highest degree of EEG response was from the call-name stimulation, followed by habit and music stimulations. Significant differences in EEG wavelet energy and response coefficient were found both between habit and music stimulation, and between habit and call-name stimulation. These findings prove that habit stimulation induces relatively more intense EEG responses in DOC patients than music stimulation, suggesting that it may be a relevant additional method for eliciting patient arousal.
Adult ; Alcohol Drinking ; physiopathology ; Brain ; physiopathology ; Consciousness Disorders ; physiopathology ; therapy ; Electroencephalography ; Female ; Habits ; Humans ; Male ; Middle Aged ; Music ; Names ; Nonlinear Dynamics ; Physical Stimulation ; Rest ; Smoking ; physiopathology ; Speech ; Treatment Outcome ; Wavelet Analysis

Objective: To investigate the clinicopathologic features, immunophenotype, pathological diagnosis and treatment of malignant mixed tumor (MMT). Methods: Clinical and pathological features including immunohistochemical phenotypes were analyzed in a case of MMT accompanied with eccrine porocarcinoma (EP) involving both hands, diagnosed definitely in January 2018 along with review of relevant literature. Results: A 64-year-old man presented with multiple rash on both hands for 4 years. Three lesions of 0.5 to 2.2 cm were removed for pathological evaluation. The pathological changes on little finger of left and right hands were MMT with EP, whereas that removed from the right ring finger was EP. MMT showed infiltrative growth with vascular wall invasion and consisted of epithelial (glandular or tube differentiation) and mesenchymal components (mucinous and/or cartilage stroma). The endothelial cells showed moderate to severe cytological atypia, nuclear pleomorphism and increased mitotic activity. The glandular component had histological characteristics of syringocarcinoma with moderately atypical chondrocytes but without myoepithelium. EP was composed of basal cells with visible vacuoles in cytoplasm and the presence of tubular and squamous differentiation, along with obvious atypia. Immunohistochemically cavosurface epithelium of glandular differentiation of MMT showed positivity for CK7, EMA and CD117. Myoepithelium showed S-100, CK5/6 and p63 positivity and stromal cells were positive for S-100. Differential diagnoses included metaplastic carcinoma, malignant myoepithelioma and atypical mixed tumor of skin. Conclusions MMT with EP is extremely rare.The diagnosis of MMT depends on the morphologic features. Immunohistochemical staining is helpful for differential diagnosis. Surgical excision with safety margins is the treatment of choice. Complementary radiotherapy and/or chemotherapy is still controversial. The clinical course of MMT is deemed unpredictable and long-term follow-up is necessary.