OBJECTIVE To analyze the current status and challenges in importing rare disease drugs in China， providing references for optimizing the import process and improving relevant policies. METHODS Questionnaires and interviews were conducted with stakeholders involved in rare disease drug importation， including government departments， multinational pharmaceutical enterprises， healthcare institutions， and patient organizations. This explored the current situation and challenges encountered by each party. Expert opinions were synthesized to propose improvement suggestions. RESULTS A questionnaire survey of representatives from 25 multinational pharmaceutical companies in the rare disease field revealed that these companies had a strong willingness to import rare disease drugs， with 58.33% of them practicing diverse import models. However， significant challenges hindered this process， including unclear regulations （54.17%）， complex approval procedures （45.83%）， and excessively long approval cycles （41.67%）， negatively impacting their motivation. Meanwhile， interviews with 13 experts from government departments， healthcare institutions， pharmaceutical enterprises， and patient organizations identified deficiencies in policy design， approval processes， sampling inspection costs， and communication efficiency with regulators. Additionally， the drug import model in special medical zones also required improvement. CONCLUSIONS The importation of rare disease drugs in China faces challenges such as incomplete policies， inflexible regulatory mechanisms， and insufficient communication channels. It is recommended to enhance the rare disease definition criteria， optimize import incentive policies， and refine regulatory models， so as to further optimize the import process of rare disease drugs and improve relevant policies.

ObjectiveTo adapt the Stanford Expectations of Treatment Scale（SETS） into Chinese（C-SETS） and test the feasibility， validity and reliability of its application in patients with diarrhea-predominant irritable bowel syndrome（IBS-D） with liver-constraint and spleen-deficiency syndrome treated with traditional Chinese medicine（TCM）. MethodsWe obtained authorisation from the developer of the SETS， and followed the principle of "two-way translation" to translate the SETS by literal translation and back translation to form the C-SETS. Ninety-six IBS-D patients with liver-constraint and spleen-deficiency syndrome were enrolled as respondents and filled out C-SETS before receiving treatment； the feasibility was assessed by the recall rate， completion rate and the duration of filling out the scale； the reliability was assessed by Cronbach's α； the structural validity was assessed by exploratory and confirmatory factor analysis， and the content validity was assessed by correlation analysis. ResultsThe C-SETS consists of 10 items， with the 1st， 3rd， and 5th rating items constituting the Positive Expectations subscale， and the 2nd， 4th， and 6th rating items constituting the Negative Expectations subscale， each of which is rated on a 7-point Likert Scale. The recall of C-SETS was 100%（96/96）， the completion rate was 89.58%（86/96）； Cronbach's α for the Positive and Negative Treatment Expectations subscales were 0.845 and 0.854， respectively； exploratory factor analysis showed that the coefficient of commonality for all six entries was larger than 0.4， and that the six entries could be used by both factors to explain 77.092% of the total variance； validation factor analysis showed that the goodness-of-fit index， comparative fit index， root mean square of approximation error， canonical fit coefficient， and chi-square degrees of freedom ratio took the values of 0.943， 1.003， 0， 0.943， and 0.626， respectively； and the results of Spearman's analysis suggested that the C-SETS had good content validity. ConclusionThe C-SETS has well feasibility， reliability， and validity， which initially proves that it can be used as a tool to assess the treatment expectation of patients with IBS-D with liver-constraint and spleen-deficiency syndrome before receiving TCM treatment.

Objective To explore the influence of continuous thermal exposure on human cognitive performance.It provides solutions for the reduction of cognitive performance caused by high temperature emergency,and reduces the misoperation problems or even safety accidents of astronauts in high temperature emergency.Methods 14 right-handed male subjects were recruited and continuously exposed to four thermal environments of 32℃,35℃,38℃,and 41℃for 150 minutes,during which 5 different cognitive tasks(Posner Cueing Task,Deary-liewald Task,N-back Task,Wisconsin Card Task,Visual Search Task)were continuously performed to obtain relative cognitive performance.At the same time,a portable 2-lead holter was used to continuously capture ECG R signals during the cognitive test.Thereafter,the heart rate variability(HRV)analysis was conducted based on the ECG data,to explore the correlations between nonlinear indices(SD1,Shannon entropy,α2,D2)and the cognitive performance.Results Tresults showed that all nonlinear indexes changed with the exposure time and exposure temperature with certain patterns.In addition,through the correlation analysis between relative cognitive performance and nonlinear indicators,the most relevant indicator is α2(correlation coefficient 0.7).The correlation analysis results showed that the nonlinear index α2 had a U-shaped relationship with relative cognitive performance(correlation coefficient was 0.7).Based on that,a quantitative relationship between α2 and relative cognitive performance was obtained.It is found that when α2 was equal to 0.98,the worst relative cognitive performance was observed.Conclusion This study showed that the HRV nonlinear index α2 could serve as a reliable indicator of the cognitive performance of human during hot exposure,which provided a potential solution for the productivity and health evaluation of astronauts.

Objective:To investigate the effect of liver cirrhosis complicated with thrombocytopenia on perioperative period.Methods:The retrospective and descriptive study was conducted. The clinical data of 75 cirrhosis patients complicated with thrombocytopenia who were admitted to the Fuyang City Second People's Hospital from July 2020 to February 2022 were collec-ted. There were 56 males and 19 females, aged (58±11)years. Observation indicators: (1) preoperative conditions; (2) intraoperative and postoperative conditions. Measurement data with normal distribution were represented as Mean± SD, and comparison between groups was conducted using the one way ANOVA. Pairwise comparison was conducted using the LSD- t test. Count data were descri-bed as absolute numbers or percentages, and comparison between groups was conducted using the chi-square test or Fisher exact probability. Comparison of ordinal data was analyzed using the rank sum test. Results:(1) Preoperative conditions. Of 75 cirrhosis patients complicated with thrombocytopenia, there were significant differences in the preoperative Child-Pugh grading and platelet count between patients with mild, moderate, and severe thrombocytopenia ( P<0.05). Results of further analysis showed that compared with patients with mild thrombocytopenia, patients with moderate and severe thrombocytopenia had significantly lower preoperative platelet count ( P<0.05). Compared with patients with moderate thrombocytopenia, patients with severe thrombocytopenia had significantly lower preoperative platelet count ( P<0.05). (2) Intraoperative and postoperative conditions. Of 75 cirrhosis patients complicated with thrombocytopenia, there were significant differences in the surgical anesthesia methods, operation time, postoperative bleeding, duration of hospital stay between patients with mild, moderate, and severe thrombocytopenia ( P<0.05). Results of further analysis showed that compared with patients with mild or moderate thrombocytopenia, patients with severe thrombocytopenia had a higher proportion of general anesthesia during surgery ( P<0.05) and compared with patients with mild thrombocytopenia, patients with severe thrombocytopenia significantly increased operation time and duration of hospital stay ( P<0.05). Of the 6 patients with severe thrombocytopenia, 3 of 5 cases with intraoperative platelet transfusion and 1 case without intraoperative platelet transfusion experienced postoperative bleeding, respectively, showing no significant difference between them ( P>0.05). Four patients with postoperative bleeding were successfully stopped bleeding after treatment and there was no death in the 75 patients. Conclusions:Patients with severe thrombocytopenia have longer operation time and duration of hospital stay, and higher proportion of general anesthesia than those with mild or moderate thrombocytopenia. Perioperative platelet transfusion cannot reduce the risk of postoperative bleeding.

In 2020, the European Society of Paediatric and Neonatal Intensive Care first issued the international point-of-care ultrasound(POCUS) guideline for the critically ill newborns and children, which provided an evidence-based foundation to standardize the use of POCUS in the critically ill neonates and children.It has been widely recognized by neonatology and pediatric critical physicians worldwide.In order to keep our domestic colleagues updated and promote the POCUS application based on this international standard, the guideline was summarized and interpreted in this review.

Objective: To develop a model incorporating radiomic features and clinical factors to accurately predict acute ischemic stroke (AIS) outcomes. Materials and Methods: Data from 522 AIS patients (382 male [73.2%]; mean age ± standard deviation, 58.9 ± 11.5 years) were randomly divided into the training (n = 311) and validation cohorts (n = 211). According to the modified Rankin Scale (mRS) at 6 months after hospital discharge, prognosis was dichotomized into good (mRS ≤ 2) and poor (mRS > 2); 1310 radiomics features were extracted from diffusion-weighted imaging and apparent diffusion coefficient maps. The minimum redundancy maximum relevance algorithm and the least absolute shrinkage and selection operator logistic regression method were implemented to select the features and establish a radiomics model. Univariable and multivariable logistic regression analyses were performed to identify the clinical factors and construct a clinical model. Ultimately, a multivariable logistic regression analysis incorporating independent clinical factors and radiomics score was implemented to establish the final combined prediction model using a backward step-down selection procedure, and a clinical-radiomics nomogram was developed. The models were evaluated using calibration, receiver operating characteristic (ROC), and decision curve analyses. Results: Age, sex, stroke history, diabetes, baseline mRS, baseline National Institutes of Health Stroke Scale score, and radiomics score were independent predictors of AIS outcomes. The area under the ROC curve of the clinical-radiomics model was 0.868 (95% confidence interval, 0.825–0.910) in the training cohort and 0.890 (0.844–0.936) in the validation cohort, which was significantly larger than that of the clinical or radiomics models. The clinical radiomics nomogram was well calibrated (p > 0.05). The decision curve analysis indicated its clinical usefulness. Conclusion The clinical-radiomics model outperformed individual clinical or radiomics models and achieved satisfactory performance in predicting AIS outcomes.

OBJECTIVETo evaluate the effect of supply of fresh poultry products on reducing environment contamination of avian influenza virus (AIV) in markets in Guangzhou.

METHODSA total of 40 markets, including 20 selling alive poultry and 20 selling fresh poultry products, were selected randomly in Guangzhou to conduct environment surveillance in 80 poultry stalls every 4 months from July 2014 to April 2015. Four smear samples were collected from different sites of each poultry stall to detect nucleic acid of AIV. The positive samples were further detected for AIV subtype H5, H7 and H9 nucleic acids.

RESULTSAmong 40 alive poultry stalls, 95.0% (38/40) kept alive poultry overnight, 25.0% (10/40) were disinfected daily, 95.0% (38/40) were cleaned up weekly, 95.0% (38/40) were closed for one day every month. Among 40 fresh poultry product stalls, 20.0% (8/40) were disinfected daily, 90.0% (36/40) were cleaned up weekly, and 96.0% (38/40) ever sold dressed poultry from alive poultry markets. The positive rate of AIV in alive poultry markets was 40.4% (252/623), higher than that in fresh poultry product markets (32.3%, 197/610), the difference was significant (χ(2)=8.85, P=0.003), and the positive rate of subtype H9 virus in alive poultry markets was 28.6% (178/623), higher than that in fresh poultry product markets (16.2%, 99/610), the difference was significant (χ(2)=26.95, P<0.001). In fresh poultry product markets, the positive rate of AIV in stalls selling dressed poultry was 37.3% (180/482), higher than that in stalls selling no dressed poultry (13.3%, 17/128), the difference was significant (χ(2)=26.78, P<0.001), and the positive rate of subtype H9 virus in stalls selling dressed poultry was 19.1% (92/482), higher than that in stalls selling no dressed poultry (5.5%, 7/128), the difference was significant (χ(2)=13.80, P<0.001). Both the positive rate of AIV and the positive rate of subtype H9 virus were highest in the second round surveillance (October 2014). The differences in AIV and its subtype H5, H7 and H9 virus positive rates of environmental samples from four different sites were not significant, respectively. In the same sample site, the positive rate of subtype H9 virus in alive poultry markets was higher than that in fresh poultry product markets the difference was significant (P<0.05).

CONCLUSIONSThe supply of fresh poultry products could effectively reduce the level of environment contamination of AIV in markets. Dressed poultry supplement caused the risk of AIV spread in fresh poultry product markets.

Animals ; China ; Commerce ; statistics & numerical data ; Disinfection ; statistics & numerical data ; Environmental Microbiology ; Influenza A virus ; isolation & purification ; Poultry ; Poultry Products ; supply & distribution

Pancreatic ductal carcinoma accounts for 85％-90％ in pancreatic cancer, followed by the pancreatic cellendocrine tumors and pancreatic acinar cell carcinoma.In addition, mesenchymal cell carcinoma of pancreas is rare, and a mixed pancreatic cancer from 3 cells carcinoma is extremely rare.And pancreatic cancer always assume hypovascular tumor,spontaneous rupture of pancreatic cancer is rarely reported.A patient with a mixed duct-acinar-islet cell tumor of pancreas was admitted to the Second Affiliated Hospital of Harbin Medical University in June 2014, and underwent emergent operation of intraabdominal bleeding due to spontaneous rupture of tumor.The follow-up was done up to January 20, 2015.The patient died of intraabdominal widespread implantation metastasis of pancreatic cancer.A mixed duct-acinar-islet cell tumor of pancreas is extremely rare and easy to metastasis and diffusion of tumor with a rapid disease progression and a poor prognosis of patient,while operation is the key to terminate deterioration of the condition, and is the last line of defense to save lives.

OBJECTIVESTo investigate a survey about acceptance of central slaughtering of live poultry in residents of Guangzhou.

METHODSWe conducted a telephone survey by sampling residents with fixed-line telephone and with normal hearing, whose age is more than 15 years, by Mitofsky-Waksberg two-stage method during Jan 6(th) to 8(th), 2014. 358 residents finished the telephone questionnaire by 12 320 health hot line. We investigated the acceptance rate of city-wide central slaughtering permanently. We compared the difference between the respondents and the 2010 Guangzhou census data by Cohen's effect sizes (w) and weighted by population age and sex. We used χ(2) test to compare the acceptance rate of central slaughtering in residents with different characteristic. We used multiple logistic regression analysis to analyze the factors.

RESULTSThe difference in gender and age was small between respondents and the 2010 Guangzhou census data (w value was 0.13, 0.28, respectively), but that in education and marital status was large (w value was 0.52, 0.31, respectively). 49.0% (95% CI: 43.7%-54.3%) accept city-wide central slaughtering permanently. The acceptance rate of city-wide central slaughtering permanently in those who bought fresh, chilled and frozen poultry in their family in previous year was 54.3% (133/245), 60.0% (57/95) and 59.8% (49/82), respectively. It was more than those who didn't buy fresh, chilled and frozen poultry (38.1% (43/113), 44.9% (118/263) and 45.7% (126/276); χ(2) values were 8.15, 6.40 and 5.03; P values were 0.004, 0.011 and 0.025, respectively). The acceptance rate of city-wide central slaughtering permanently in those who deem fresh poultry taste better than live poultry was 64.9% (24/38). It more than those who deem not (47.0%, 151/320) (χ(2) = 4.22, 6.02, P = 0.040, 0.014, respectively). The acceptance rate of city-wide central slaughtering permanently in the male (OR = 2.68, 95% CI: 1.64-4.37) and those who deem getting sick due to buying live birds from LPM (OR = 1.72, 95% CI: 1.05-2.82), who can accept only fresh poultry carcass supply (OR = 2.39, 95% CI: 1.33-4.30), Who bought live poultry in their family in previous year (OR = 0.29, 95% CI: 0.11-0.74), who will decrease the consumption after ban on live poultry sale (OR = 0.50, 95% CI: 0.30-0.83) was 58.6% (109/186), 59.0% (92/156), 60.7% (139/230), 44.9% (132/295), 36.6% (68/186), respectively.

CONCLUSIONIn the early stage of avian influenza A(H7N9) epidemic in Guangzhou, the rate of acceptance of central slaughtering permanently in residents was not so high. Who deem getting sick due to buying live birds from LPM, who could accept only fresh poultry carcass supply and the male more accept city-wide central slaughtering permanently.

Animals ; Attitude to Health ; Birds ; Epidemics ; Humans ; Influenza A Virus, H7N9 Subtype ; Influenza in Birds ; Influenza, Human ; Male ; Meat-Packing Industry ; Poultry ; Surveys and Questionnaires

[ ABSTRACT] AIM:To investigate the changes of migration of lung adenocarcinoma cells promoted by IL-8 and the inner and outer mitochondrial membrane dynamic changes during this process.METHODS:Human lung adenocarci-noma cell line A549 was divided into control group and IL-8 group.Cell migration was analyzed by scratch detection and Transwell assay.The secretion of endogenous IL-8 was detected by ELISA.The protein levels of mitochondrial cytochrome C ( Cyt C) and mitochondrial outer membrane protein Tom20 was detected by Western blot.The mRNA expression of mito-chondrial fusion genes Mfn1, Mfn2 and OPA1 and fission genes Fis1, Drp1 and MTP18 was detected by RT-PCR.The morphological changes of mitochondria were observed by MitoTracker Red CMXRos dye staining and confocal microscopy. RESULTS:The migratory rate of A549 cells and endogenous secretion of IL-8 in A549 cells were higher than those in SPC-A-1 cells.The migratory rate of A549 cells was improved by IL-8 in a time-dependent manner.Compared with control group, the Tom20 protein expression was increased ( P<0.05 ) , and the Cyt C protein expression was decreased ( P<0.05).The expression of mitochondrial outer membrane fusion genes Mfn1 and Mfn2 was increased (P<0.05), and the expression of mitochondrial inner membrane fusion gene OPA1 was decreased (P<0.05).The expression of fission genes Drp1 and MTP18 were decreased (P<0.05), while the expression of Fis1 was no change (P>0.05).Under confocal microscope, the punctate aggregates in the mitochondria of the A549 cells treated with IL-8 were observed.CONCLU-SION:The migratory rate of A549 cells is increased by IL-8, which is related to the changes of mitochondrial fusion genes and the fission genes.