Objective:A retrospective analysis was performed on clinical characteristics and deoxyguanosine kinase DGUOK gene mutations in a family with hepatocerebral mitochondrial DNA depletion syndrome (MTDPS). Methods:The clinical data, treatment process and gene detection results of a child with MTDPS in the second hospital of Hebei Medical University in April 2019 were analyzed and summarized.Results:Proband was a girl.From the first week of infantile, she suffered from recurrent hypoglycemia, hyperlactic acid, progressive cholestatic liver dysfunction, coagulopathy, difficult feeding, slow growth of body mass, microcephaly, hypotonia, and gradul intermittent binocular tremors, and eventually failed to thrive.Gene testing identified two compound heterozygous mutations c. 42-c.43insTTCA(p.F15fs129X)/c.808-1(IVS6)G>A in DGUOK gene.The former was a frame-shift mutation resulted in truncated protein and the later was a splicing mutation resulted in abnormal splicing.Each parent was a heterozygous carrier, and there were no mutations in the two sites with her elder sister. Conclusions:Both mutations were first reported worldwide. DGUOK gene mutations with MTDPS are important causes of infant liver failure.When hypoglycemia, hyperlactic acidemia and liver dysfunction occur in newborn and infant, MTDPS related gene DGUOK gene sequencing screening should be considered for early definitive diagnosis, or, when acute liver failure happen in infant and childhood, neuromuscular involvement is insufficient.

This article described the background,concept,characteristic and objective of the research-based nursing,systematically introducing the main measures including management mechanism, nursing service,nursing staff training,and nursing scientific development.Other areas covered include innovation management mechanism,updating service philosophy,improving nursing staff training,and constructing scientific research platform.

Objective To optimize the best extraction technology of total flavonoids in Wudang pine needle tea.Methods The contents of total flavonoids were taken as index, and orthogonal design of L9(34) was applied to process ultrasonic extraction.Results The order of factors of affecting extraction technology was solid to liquid ratio > ultrasonic power > ultrasonic time. The optimized extraction technology was as follows: adding 500 times volume of purified water, ultrasonic extracting for 15 minutes with ultrasound power 500 W at 40℃. The average content of total flavonoids was 39.701 mg/g.Conclusions The optimal extraction technology is simple, efficient and feasible, and can be used for extracting total flavonoids from Wudang pine needle tea, providing basis for the formulation of quality standards.

Objective:To explore the current situation of tacit knowledge sharing among nurses and analyze its influencing factors.Methods:From March to June 2023, convenience sampling was used to select 437 nurses from three ClassⅢ Grade A hospitals in Changsha as the research subject. The survey was conducted using the General Information Questionnaire, Tacit Knowledge Sharing Scale, Person-Organization Fit Assessment Scale for Nurses, and Maslach Burnout Inventory. The R software randomForest package was used to construct a random forest model and rank the importance of factors influencing tacit knowledge sharing; LASSO analysis was used for variable selection, and the selected variables were analyzed for influencing factors using multiple linear regression.Results:A total of 437 questionnaires were distributed, and 434 valid questionnaires were collected, with an effective response rate of 99.31% (434/437). The Tacit Knowledge Sharing Scale score of 434 nurses was (13.09±3.03). The random forest algorithm showed that the top four independent variables in importance ranking were occupational burnout, professional title, working years, and person-organization fit. Multiple linear regression analysis indicated that occupational burnout, professional title, and person-organization fit were the main influencing factors of tacit knowledge sharing among nurses ( P<0.01) . Conclusions:The tacit knowledge sharing among nurses needs to be improved. Nursing managers can enhance the tacit knowledge sharing among nurses by reducing their professional burnout, improving the system of professional title promotion, and enhancing the fit between persons and organizations.