Objective:To evaluate the clinical value of a capillary electrophoresis-based method for gene diagnosis of hyperphenylalaninemia.Methods:In this single-center prospective study, 40 newborns with suspected hyperphenylalaninemia detected by neonatal liquid chromatography-tandem mass spectrometry screening at Nanjing Maternity and Child Health Care Hospital from February 2021 to February 2023 were included, with 22 males, 18 females and a mean age at diagnosis of 21.93 days.Capillary electrophoresis was used to detect 85 variants of the phenylalanine hydroxylase ( PAH) gene in 40 newborns with suspected hyperphenylalaninemia.The PAH gene of undiagnosed patients was further analyzed by Sanger sequencing.The detection rate, sensitivity and specificity of capillary electrophoresis were calculated. Results:Among these 40 newborns with suspected hyperphenylalaninemia, 71 PAH variants were detected by capillary electrophoresis, 32 patients were clearly diagnosed, only 1 pathogenic variant was found in 5 patients, and no pathogenic variant was found in the last 3 patients.Therefore, the detection rate, sensitivity and specificity of capillary electrophoresis for analysis of the PAH gene were 80.00%, 88.75% and 100%, respectively. Conclusions:The capillary electrophoresis-based method can rapidly, efficiently and accurately detect PAH gene variants at lower cost and is a promising gene detection method for hyperphenylalaninemia in clinical practice.

Objective:To explore the effect of NOD-like receptor thermal protein 3 ( NLRP3) knockout in γ-aminobutyric acid (GABA)-ergic neurons in the hippocampal CA1 area on improving cognitive dysfunction in mice after traumatic brain injury (TBI). Methods:Forty-eight healthy male NLRP3 flox/flox mice weighing 25-28 g were randomly divided into 4 groups ( n=12): sham-operated+control virus group (SV group), sham-operated+ NLRP3 specific knockout group (SG group), TBI+control virus group (TV group), TBI+ NLRP3 specific knockout group (TG group). TBI in the TV and TG groups was established by free-fall method, while surgical procedures such as scalp incision and cranial window opening without impact were given to the SV and SG groups. Adenovirus was injected into the hippocampal CA1 area of SG and TG groups 21 d before TBI to induce NLRP3 specific knockout in GABA-ergic neurons in the hippocampal CA1 area; empty virus was injected into the CA1 area of SV and TV groups. Cognitive function was evaluated using novel object recognition test 30 and 31 d after TBI, and learning and memory functions were assessed using Morris water maze test 32-36 d after TBI. Field potentials in the hippocampal CA1 area were recorded during novel object recognition 31 d after TBI. After behavioral tests, these mice were sacrificed. Immunofluorescent staining was used to detect the fluorescent intensity of microtubule-associated protein2 (MAP2), glutamic acid decarboxylase 67 (GAD67), and postsynaptic density protein 95 (PSD95) in the hippocampal CA1 area, as well as percentage of pyroptosis-associated inflammatory factor interleukin-18 (IL-18)/GAD67 double-positive neurons in total GAD67 positive neurons. Results:Compared with the SV and SG groups, the TV and TG groups had decreased novel object recognition index, decreased number of platform crossings during the experimental period, increased escape latency on day 3 and day 4 of the training period in Morris water maze test, decreased θ and γ oscillation power in the hippocampal CA1 area during novel object recognition, decreased fluorescent intensity of MAP2, GAD67, and PSD95 in the hippocampal CA1 area, increased percentage of IL-18/GAD67 double-positive neurons, with significant differences ( P<0.05). Compared with the TV group, the TG group had increased novel object recognition index, increased number of platform crossings in Morris water maze test, decreased escape latency during the training period, increased θ and γ oscillation power in the hippocampal CA1 area during novel object recognition, increased fluorescence intensity of MAP2, GAD67, and PSD95 in the hippocampal CA1 area, decreased percentage of IL-18/GAD67 double-positive neurons, with significant differences ( P<0.05). Conclusion:Specific inhibition of NLRP3 expression in GABA-ergic neurons in the hippocampal CA1 area can improve cognitive dysfunction in mice after TBI, whose mechanism may be related to inhibited GABA-ergic neuronal pyroptosis in the hippocampal CA1 area.

Purpose To investigate the value of 99Tcm-(methoxyisobutvlisonitrile,MIBI)bone uptake on hungry bone syndrome(HBS)in renal secondary hyperparathyroidism(SHPT)after parathyroidectomy.Materials and Methods From June 2014 to December 2021,106 renal SHPT patients who underwent successful parathyroidectomy in Jiangyin Hospital Affiliated Nantong University were retrospectively enrolled.Visual analysis was used to evaluate the abnormal bone uptake of 99Tcm-MIBI.The patients were divided into HBS group and non-HBS group based on whether occurred HBS.The clinical features,laboratory indicators and 99Tcm-MIBI bone uptake were compared between the two groups.Results Of 106 renal SHPT patients,42(39.62%)patients with bone uptake on visual assessment,showed diffusely increased tracer accumulation,particularly in sternum,clavicle and ribs.The age in HBS group was younger than that in non-HBS group(t=-3.058),the alkaline phosphatase and parathyroid hormone level in HBS group were higher than that in non-HBS group(Z=-5.148,-2.218),the serum corrected calcium in HBS group was lower than that in non-HBS group(Z=-2.102),the positive rate and number of 99Tcm-MIBI bone uptake in HBS group was 50%and 2(1,3),which was higher than that of 28%and 1(1,1)in non-HBS group(χ2=5.344,Z=-2.970),respectively,all showed statistically significant difference(all P<0.05).Conclusion Renal SHPT patient with HBS after parathyroidectomy is commonly related to a high level of alkaline phosphatase and parathyroid hormone,and more likely to develop abnormal 99Tcm-MIBI bone uptake.

Objective:To explore the current status and correlation of occupational stress and burnout among pediatric nurses.Methods:From April 2023 to January 2024, convenience sampling was used to select 150 pediatric nurses from Taizhou Hospital of Zhejiang Province, East Campus of Taizhou Hospital of Zhejiang Province, and Taizhou Enze Medical Center as research subjects. The survey was conducted using the General Information Questionnaire, Chinese version of the Maslach Burnout Inventory General Survey, and Nurse Job Stressors Scale.Results:A total of 150 nurses were surveyed, and 149 valid questionnaires were collected, with a valid response rate of 99.33%. Pediatric nurses had high scores in emotional exhaustion and depersonalization dimensions in occupational burnout. The job stressors for pediatric nurses were ranked in order of their scores in each dimension, including workload and time management, work environment and resources, nursing profession and work, management and interpersonal relationships, and patient nursing. There were correlations between occupational burnout and occupational stress among pediatric nurses in multiple dimensions (all P<0.05) . Conclusions:Pediatric nurses have a relatively high level of occupational burnout and often face significant occupational stress. In the development of the healthcare system, attention should be paid to the occupational burnout and stress of pediatric nurses. The physical and mental health of pediatric nursing staff can be promoted and work efficiency can be improved by reducing the overloaded work of pediatric nurses, optimizing personnel allocation, and improving social support.

Objective:By investigating the awareness situation of scientific research integrity among scientific researchers,their behavior and attitudes towards scientific research integrity,and the reasons for the occurrence of scientific research integrity,to identify the key influencing factors and risk points,and propose targeted management ideas for scientific research integrity construction.Methods:A web-based questionnaire was used to survey the awareness of scientific research integrity policies,behavior and attitude,and reasons for dishonest behavior in scientific research among scientific researchers at a tertiary A hospital.Results:A total of 1,385 questionnaires were recovered,with 1,253 valid questionnaires.There were 246 males and 1,007 females,aged between 20 and 60 years old,with an average age of(37.98±8.97)years old.The average awareness rate of policies related to scientific research integrity was 39.44%.Scientific researchers believe that the top three risk points for dishonest behavior in scientific research were paper submission,entrusting third-party services,and the use of research funds.According to the different scientific research work involved by different survey subjects,the respondent population was set up with the attributes of the personnel in the basic information of the survey content on attitudes toward violating scientific research behavior norms,and was divided into four groups.The awareness situation of scientific research integrity policies was negatively correlated with the attitudes towards dishonest behavior in scientific research in the first and third groups.Publication of SCI papers and the awareness situation of scientific research integrity policies were negatively correlated with attitudes towards dishonest behavior in scientific research in the second group.Age and publication of SCI papers were related to attitudes towards dishonest behavior in scientific research in the fourth group,which were negatively correlated with the publication of SCI papers and positively correlated with age.Conclusion:The learning and training,and the rate of policy awareness of scientific research integrity were not high,and the awareness of scientific research integrity policies should be further improved.There are still risks and problems in the key links of scientific research activities,and it is necessary to strengthen the integrity management of the whole process of scientific research activities,establish the correct scientific values of scientific researchers,improve the scientific research evaluation system,and create a good scientific research environment.

Helicobacter pylori (H. pylori) neutrophil-activating protein (HP-NAP) is one of the newly discovered main virulence factors of H. pylori, and is expressed in almost all strains of H. pylori. After being activated, HP-NAP can participate in the development of inflammation and tissue damage by stimulating neutrophils, monocytes, dendritic cells, mast cells and lymphocytes. This paper reviewed the latest research progress of HP-NAP in the prevention and treatment of related diseases.

The NLRP3 inflammasome's core and most specific protein, NLRP3, has a variety of functions in inflammation-driven diseases. Costunolide (COS) is the major active ingredient of the traditional Chinese medicinal herb Saussurea lappa and has anti-inflammatory activity, but the principal mechanism and molecular target of COS remain unclear. Here, we show that COS covalently binds to cysteine 598 in NACHT domain of NLRP3, altering the ATPase activity and assembly of NLRP3 inflammasome. We declare COS's great anti-inflammasome efficacy in macrophages and disease models of gouty arthritis and ulcerative colitis via inhibiting NLRP3 inflammasome activation. We also reveal that the α-methylene-γ-butyrolactone motif in sesquiterpene lactone is the certain active group in inhibiting NLRP3 activation. Taken together, NLRP3 is identified as a direct target of COS for its anti-inflammasome activity. COS, especially the α-methylene-γ-butyrolactone motif in COS structure, might be used to design and produce novel NLRP3 inhibitors as a lead compound.

Objective:To explore the views of general practitioners (GPs) on developing special interests in the context of the county medical community.Methods:A survey was conducted using self-designed questionnaire from November and December 2019, among 49 general practice residents trained in the First Affiliated Hospital of Zhejiang University School of Medicine. Meanwhile, structured interviews were undertaken with 14 general practice residents.Results:Forty-nine valid questionnaires were collected with a response rate of 100.0%. All participants were from the county medical community units. The survey showed that 91.8% (45/49) of respondents were willing to develop special interests and 79.6% (39/45) chose one subject, and the top three subjects were endocrinology, gastroenterology and cardiology. The structured interviews demonstrated that most participants did not understand meaning of general practitioners with special interests (GPwSIs) clearly and were unable to distinguish GPwSIs from specialists; they were confused about the status, training mode, and assessment standards of GPwSIs. The interviews also showed that the demand for developing special interests for them was derived from the needs of patients for diagnosis and treatment, the target population of health care services, peer advice and personal interests.Conclusions:Most general practice residents are willing to develop special interests, and internal medicine is the first choice; however, their understanding of the GPwSIs is insufficient. The survey suggests that the position, training model, assessment and certification of GPwSI need to be further clarified.

Chylothorax is one of the complications of surgery and lymphatic diseases. The incidence rate of chylothorax has been increasing in recent years. X-ray-guided interventional therapy is a new method for treating chylothorax, and its curative effect is no less than the traditional surgical treatment. Based on discussing the aetiology and diagnosis of chylothorax, this paper briefly summarizes the application progress of X-ray-guided interventional therapy for chylothorax to provide further reference and a basis for clinical practice.

Objective:To analyze the patent development status of 13 general hospitals in Beijing, for reference in promoting the management and translation of intellectual property rights of public hospitals.Methods:13 tertiary general hospitals in Beijing as one of the top 100 hospitals in China in terms of science and technology evaluaton metrics in 2020 were selected as the research subjects. The patent application and authorization information of 13 hospitals was retrieved from the IncoPat technology innovation intelligence platform, during the period from January 1, 2012 to December 31, 2021. The patent application and authorization status, international patent classification distribution, inventor information, patent validity, and registration of patent transfer were analyzed.Results:The numbers of patent applications and authorizations from 13 hospitals increased from 123 and 13 in 2012 to 2 053 and 2 368 in 2021, respectively. From 2012 to 2021, 13 hospital applied for 10 050 patents and authorized 6 896 patents, including 906 authorized invention patents, 5 863 utility model patents, and 127 appearance design patents. Valid patents amounted to 5 925, accounting for 85.92%, and valid authorized invention patents amounted to 796, accounting for 13.43%. 110 of these patents were transferred or licensed, accounting for 1.60% of the authorized patents.Conclusions:Aapplied and authorizated patents were rising year by year, but the ratios of invention patents and patient translation remained at a low level. The author suggestted that hospitals should highlight the value orientation of patents, strengthen-industry-academia-research cooperation, establish a professional management team and standardized management system for intellectual property, cultivate high-value patents, conduct pre patent application evaluation and screening, and improve patent conversion and technological innovation capabilities.