To summarize the clinical characteristics and genetic mutation spectrum of patients with SELENON-related myopathy(SELENON-RM), in order to improve awareness among physicians.

Objective:To verify the feasibility of using Elekta accelerated go live (AGL) standard process for the acceptance of multiple accelerators.Methods:The beams of three accelerators were adjusted by PTW Beamscan three-dimensional water tank to reach the AGL standard. Dose verification was performed for three accelerators that met AGL standards. A simple field test example from Cancer Hospital Chinese Academy of Medical Sciences was used to compare the MapCheck 3 surface dose measurement results with the surface dose calculated by the same accelerator model. Images of 10 patients including head and neck, esophagus, breast, lung and rectum were randomly selected. volumetric-modulated arc therapy (VMAT) and intensity modulated radiation therapy (IMRT) treatment techniques were used for planning design, and the measured dose of ArcCheck was compared with the planned dose calculated by the same accelerator model. One-way ANOVA was used to statistically analyze the passing rates of two-dimensional and three-dimensional dose verification.Results:The 6 MV X-ray percentage depth dose at 10 cm underwater (PDD 10) of three accelerators was 67.45%, 67.36%, 67.47%, and the maximum deviation between the three accelerators was 0.11%. The 6 MV flattenting filter free (FFF) mode X-ray PDD 10 was 67.33%, 67.20%, 67.20%, and the maximum deviation between the three accelerators was 0.13%. All required discrete point doses on each energy 30 cm×30 cm Profile spindle of the three accelerator X-rays deviated less than ±1% from the standard data. Absolute γ analysis was performed on the results of MapCheck 3 two-dimensional dose matrix validation. Under the 10% threshold of 2 mm/3% standard, the average passing rate of the test cases in Cancer Hospital Chinese Academy of Medical Sciences was above 99%, and the difference was not statistically significant ( P>0.05). Absolute γ analysis was performed on the ArcCheck verification results. Under the 10% threshold, the pass rate of 2 mm/3% was all above 95%, the maximum average passing rate of the three accelerators with different energy and different treatment techniques was 0.28% (6 MV, VMAT), 0.19%(6 MV FFF, VMAT), 0.56% (6 MV, IMRT) and 0.05% (6 MV FFF, IMRT), and the difference was not statistically significant ( P>0.05). Conclusion:Compared with traditional accelerator acceptance process, the acceptance time of each accelerator is shortened by 4-6 weeks by using the AGL standard process, and the radiotherapy plan of patients can be interchangeably executed among different accelerators.

Objective:To investigate the association between the Doppler variables of the ophthalmic artery with the severity of preeclampsia(PE).Methods:Systematic literature was searched between January 1995 and March 2023 in PubMed,Web of Science,Embase,and the Cochrane Library.Studies comparing ophthalmic artery Doppler variables,including peak systolic velocity(PSV),end-diastolic velocity(EDV),resistive index(Rl),pulsa-tility index(PI),and peak ratio(PR,the ratio of the flow velocity of the second peak to that of the initial peak)in patients with PE,severe preeclampsia(sPE),and healthy pregnant women were included.The random-effects model was adopted as the method of pooled analysis,and the I2value was used to assess heterogeneity.The pooled standardized mean difference(SMD)with 95％confidence interval(CI)was used to estimate the associa-tion between ophthalmic artery Doppler variables and PE patient's characteristics.Results:Eight retrospective studies were eventually included in this Meta-analysis.Our pooled results suggested that compared with PE ca-ses,sPE patients had lower PI levels(SMD-0.56,95％CI-0.92～-0.20,P=0.000),higher EDV levels(SMD 0.47,95％CI 0.12～0.83,P=0.028)and higher PR levels(SMD0.96,95％CI 0.13～1.78,P=0.023).Howev-er,there was no significant difference between PE and sPE patients about the PSV and RI(P=0.361,P=0.626).Conclusions:This review demonstrates that ophthalmic artery Doppler variables(PI,EDV and PR)could be useful for predicting PE and PE development(especially in identifying sPE),which in turn may help the practitioner in the management of these complicated cases and in taking early necessary precautions.

BACKGROUND:Resistance exercise increases muscle mass and improves muscle strength,but there is a lack of conclusive evidence on its effects on body composition.Controversial results from randomized controlled trials and multiple Meta-analyses pose a problem for clinical decisions on exercise interventions. OBJECTIVE:Based on the methodological paradigm of umbrella review,to explore the efficacy and safety of resistance exercise in improving the body composition of overweight and obese people. METHODS:We searched PubMed,Cochrane Library,Web of Science,and CNKI for systematic reviews on the effect of resistance exercise in overweight and obese people published from database inception to August 31,2022.Chinese search terms included"anti-resistance,resistance,self-weight,Meta,Meta-analysis,body composition,body fat percentage,fat mass,lean body mass,body mass."English search terms included"resistance training,strength training,weight-lifting strengthening program,body composition,body fat distribution,Meta-analysis,data pooling,overviews,clinical trial,clinical trial overviews."PRISMA was used to evaluate the methodological quality of the included studies,and a GRADE evaluation system was used to grade the quality of evidence. RESULTS AND CONCLUSION:A total of 14 Meta-analyses were included,and GRADE evidence quality evaluation showed that 2 outcome indicators were intermediate,12 were low,and 14 were extremely low.Compared with the control group,23 of the 32 outcome measures showed significant improvement.The intervention effect of resistance exercise on body fat percentage has reached a consensus,but the intervention effects on lean body mass,body mass index and visceral fat still need further confirmation.Resistance exercise is an effective and safe method to improve the body composition of overweight and obese people.The overall quality of the research and the evidence quality of the outcome indicators included in the systematic evaluation are generally low.In the future,the initial state,exercise goal and willingness of the subjects should be fully combined on the basis of clarifying the sample characteristics and refining the scheme design.Through multiple,scientific and personalized exercise prescription designs of water,air and elastic resistance,the safety and compliance of resistance exercise can be improved,providing clear evidence-based support and decision-making basis for resistance exercise intervention.

OBJECTIVE:Masks are one of the most important defenses against the virus.However,the impact of wearing masks during daily activities or sports on respiratory and circulatory function remains controversial.A comprehensive quantitative evaluation of the effects of mask-wearing on human heart rate,oxygen saturation and end-expiratory carbon dioxide by Meta-analysis was conducted.The effects of wearing different types of masks at different exercise intensities and time of exercise on the human respiratory and circulatory system were explored. METHODS:By February 2023,with"mask,face mask,N95,training,sports,running,walking,cycling"as the Chinese search terms and"masks,respiratory protective devices,N95 respirators,surgical face masks,exercise,resistance training,explosive training,muscle exercises"as English search terms,the experimental studies addressing the influence of exercise with a mask on hemodynamic indexes were retrieved from CNKI,Web of Science,PubMed,Cochrane Library and WanFang databases.The outcome indicators included three continuous variables-exercise center rate,blood oxygen saturation and end-expiratory carbon dioxide.Stata16.0 software was used to analyze the outcome indicators of the included literature.The PEDro scale was used as a quality assessment tool,and the funnel plot was used to analyze the impact of publication bias. RESULTS:Totally 25 articles involving 857 healthy children and adults were included in this Meta-analysis.The overall methodological quality was high,with 22 studies scoring 6 points on the PEDro scale,2 studies scoring 7 points and 1 study scoring 8 points.The meta-analysis results showed that compared with the control group,exercise with masks had no significant effect on heart rate(SMD=0.02,95%CI:-0.11 to 0.15,P=0.81),but increased end-expiratory carbon dioxide(SMD=0.60,95%CI:0.37 to 0.83,P=0.00),decreased oxygen saturation(SMD=-0.28,95%CI:-0.47 to-0.09,P=0.03).Intensity and duration were the factors that affected the heterogeneity between studies.Wearing a mask during high-intensity exercise significantly increased heart rate(SMD=-0.20,95%CI:-0.36 to-0.04,P=0.02).The effect of high-intensity and short-time exercise on blood oxygen saturation was significantly higher than that of other exercises(SMD=-0.40,95%CI:-0.70 to-0.10;SMD=-0.25,95%CI:-0.45 to-0.04).For end-expiratory carbon dioxide,maintaining a certain intensity and increasing the exercise time or increasing the intensity further increased the index significantly,reaching a moderate effect size(SMD=0.61,95%CI:0.06 to 1.15;SMD=0.58,95%CI:0.04 to 1.13). CONCLUSION:Existing evidence suggests that exercise with masks may have the adverse effect of increasing end-expiratory carbon dioxide and decreasing blood oxygen saturation.The influence of different exercise test time and intensities on the three outcome indexes was different.Wearing a mask during high-intensity exercise can significantly increase heart rate and decrease blood oxygen saturation.Maintaining moderate intensity for a long period or further increasing the intensity of exercise will lead to increased end-expiratory carbon dioxide levels.

  Objective  The International Medical Services Department of Peking Union Medical College Hospital (PUMCH) has gradually promoted the concept and practice of palliative care since 2016. This study aims to analyze the death status of end-of-life patients before and after the promotion endeavor, in order to evaluate the impact of palliative care on the death quality.  Methods  All clinical data from end-of-life patients who died at the International Medical Services Department of PUMCH in 2013 and 2019 were retrospectively collected, and their diagnosis and treatment details before death were compared.  Results  A total of 36 end-of-life patients who died in 2013 and 37 end-of-life patients who died in 2019 met the inclusion and exclusion criteria. In 2013, there were 19 males and 17 females, with an average age of (72.1±14.0) years, and 19 cases had advanced cancer. In 2019, there were 19 males and 18 females, with an average age of (70.8±15.3) years, and 27 cases had advanced cancer. Compared to patients who died in 2013, a lower proportion of the patients in 2019 who were transferred to the ICU before death(0 vs. 22.2%, P=0.008), received cardiopulmonary resuscitation (0 vs. 16.7%, P=0.011), had tracheal intubation(5.4% vs. 36.1%, P=0.001), invasive mechanical ventilation(2.7% vs. 33.3%, P=0.001), and total parenteral nutrition(32.4% vs. 61.1%, P=0.014), while a higher proportion received palliative care consultations(29.7% vs. 0, P<0.001), and humanistic care(40.5% vs. 16.7%, P=0.024). In 2019, compared to the patients who did not receive palliative care consultations, those who received palliative care consultations had a higher proportion of humanistic care(90.9% vs. 19.2%, P<0.001).  Conclusions  After concept promotion and practice of palliative care, the proportion of end-of-life patients receiving invasive treatments before death in the International Medical Services Department of PUMCH has significantly decreased, the proportion receiving humanistic care has significantly increased, and the quality of death has been improved to a certain extent.

Objective:To investigate the changes of the liver volume in patients with cirrhotic portal hypertension after splenectomy combined with devascularization, and to analyze the related causes.Methods:Clinical and imaging data of 94 patients with cirrhotic portal hypertension who underwent surgical treatment at the People's Hospital of Ningxia Hui Autonomous Region between January 2014 and May 2024 were included and analyzed before and after surgery. The cohort comprised 61 males and 33 females, aged (47±12) years. The preoperative and postoperative liver volumes were compared, and the patients were divided into two groups based on the changes in postoperative liver volume: the volume increase group ( n=51) and the volume decrease group ( n=43). Clinical data were collected including liver volume, hepatic artery diameter, spontaneous portosystemic shunt (SPSS), portal vein thrombosis (PVT), and hepatic artery dilation. Results:Compared with the volume decrease group, the postoperative liver volume [(1 157±237) cm 3 vs. (977±271) cm 3] and incidence of hepatic artery dilation [92.2%(47/51) vs. 67.4%(29/43)] in the volume increase group both increased, while the incidence of newly formed SPSS [3.9%(2/51) vs. 18.6%(8/43)] and PVT [5.9%(3/51) vs. 34.9%(15/43)] after surgery both decreased, and the differences were statistically significant (all P<0.05). After splenectomy combined with devascularization, patients with decreased liver volume exhibited aggravated conditions of serrated or wavy changes at the edge of the liver and widening of liver fissures, while patients with increased liver volume had significant reduction or disappearance of serrated or wavy changes at the edge of the liver and narrowing of liver fission. Conclusion:Splenectomy combined with devascularization can increase the postoperative liver volume in patients with cirrhotic portal hypertension, and decrease in postoperative liver volume may be related to the occurrence of SPSS and PVT.

Background::With an increasing proportion of multiparas, proper interpregnancy intervals (IPIs) are urgently needed. However, the association between IPIs and adverse perinatal outcomes has always been debated. This study aimed to explore the association between IPIs and adverse outcomes in different fertility policy periods and for different previous gestational ages.Methods::We used individual data from China’s National Maternal Near Miss Surveillance System between 2014 and 2019. Multivariable Poisson models with restricted cubic splines were used. Each adverse outcome was analyzed separately in the overall model and stratified models. The stratified models included different categories of fertility policy periods (2014–2015, 2016–2017, and 2018–2019) and infant gestational age in previous pregnancy (<28 weeks, 28–36 weeks, and ≥37 weeks).Results::There were 781,731 pregnancies enrolled in this study. A short IPI (≤6 months) was associated with an increased risk of preterm birth (OR [95% CI]: 1.63 [1.55, 1.71] for vaginal delivery [VD] and 1.10 [1.03, 1.19] for cesarean section [CS]), low Apgar scores and small for gestational age (SGA), and a decreased risk of diabetes mellitus in pregnancy, preeclampsia or eclampsia, and gestational hypertension. A long IPI (≥60 months) was associated with an increased risk of preterm birth (OR [95% CI]: 1.18 [1.11, 1.26] for VD and 1.39 [1.32, 1.47] for CS), placenta previa, postpartum hemorrhage, diabetes mellitus in pregnancy, preeclampsia or eclampsia, and gestational hypertension. Fertility policy changes had little effect on the association of IPIs and adverse maternal and neonatal outcomes. The estimated risk of preterm birth, low Apgar scores, SGA, diabetes mellitus in pregnancy, and gestational hypertension was more profound among women with previous term births than among those with preterm births or pregnancy loss.Conclusion::For pregnant women with shorter or longer IPIs, more targeted health care measures during pregnancy should be formulated according to infant gestational age in previous pregnancy.

Objective To investigate clinicians'practice and opinions on sedation therapy in end-stage patients at Peking Union Medical College Hospital.Methods From August,2022 to April,2023,an online questionnaire survey was conducted among clinicians involved in end-stage patient management.Results A total of 205 questionnaires were distributed,with an effective response rate of 56.1% .Among the clinicians,55.7% of them had experience of applying sedation therapy in end-stage patients;85.2% of clinicians believed that se-dation could relieve the suffering of terminal patients from physical refractory symptoms;75.7% of clinicians considered that sedation therapy could be used to relieve agony from psycho-existential distress.Most clinicians had concerns about sedation therapy due to the lack of legal support(86.1% )and the lack of understanding of patients or families(59.1% ).The majority(90.4% )of clinicians were willing to receive training on palliative sedation.Conclusions A majority of clinicians agree that sedation therapy could relieve the physical distress and psycho-existential distress in end-stage patients.However,most clinicians have concerns about the application of sedation therapy due to the lack of legal support.It is necessary to enhance the training on palliative sedation.

Objective To summarize the experience of integrative palliative care at Peking Union Medi-cal College Hospital and provide a reference for promoting the integrative palliative care model.Methods Twenty cases receiving integrative palliative care at Peking Union Medical College Hospital were collected.The clinical characteristics,reasons for initiating integrative palliative care,the process of integrative palliative care,and feedback from these cases were summarized.Results Insomnia(11 cases,55％)and pain(9 cases,45％)were the most common symptoms requiring control in the 20 cases.The integrative palliative care team assisted in medical decision-making for 17 cases(85％),prepared end-of-life for 9 cases(45％),assisted in the transfer for 3 cases(15％),and provided comfort care for all the 20 cases(100％).Conclusions The integrative palliative care model can help alleviate suffering in end-of-life patients and provide support to patients'families and the original medical teams.This model is worth further promotion within class A tertiary hospitals.