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 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.

Objective On the premise of meeting the image quality requirements of simulated location for pediatric radiotherapy,the simulated localizable CT parameters are optimized through phantom scanning to reduce the radiation dose.Methods CatPhan700 phantom was used to simulate the child's body,Philip 24-row large-aperture spiral simulated localizable CT was performed,and the CT images were obtained by scanning the phantom at different mAs and tube voltages.The mAs range was set at 60-400 mAs,the scanning was performed every 20 mAs interval,and the kV was set at 80,100,and 120 kV.Image evaluation was carried out using parameters such as image noise(N10 and mean SD),uniformity,low contrast resolution,high contrast resolution,and the stabilities of HU values of Air,Acrylic,50%bone,LDPE,20%bone,Teflon,Polystyrene,DelrinTM,Lung,PMP and Water.The CTDIVol and DLP automatically calculated by the simulated localizable CT system were read to evaluate the radiation dose.Results At 100 kV,as mAs increased,both CTDI and DLP showed upward trends,and the fitting results were linear correlated,with slopes of 0.034 5 and 0.932 4.Image noise was decreased nonlinearly with the increasing mAs.When mAs increased from 60 to 140 mAs,N10 decreased from 0.25%to 0.14%,and SD reduced from 3.74 HU to 2.54 HU.When mAs reached 180 mAs or higher,N10 fluctuated between 0.1%and 0.12%,the mean SD fluctuated between 2.0 and 2.5 HU,and the downward trends obviously slowed down.When mAs increased from 60 to 200 mAs,the low contrast resolution of the image dropped from 0.53 to 0.29.The image uniformity,high contrast resolution and HU values of different substances were less affected by mAs.The image quality of 100 kV and 200 mAs scanning was close to that of 120 kV scanning,but the image quality of 80 kV scanning failed to meet the clinical requirements.Conclusion In order to reduce the radiation dose as much as possible,the mAs should be set at 200 mAs when the tube voltage is set at 100 kV for a simulated cylinder with a diameter of 20 cm.In the actual simulation scanning for pediatric radiotherapy,the scanning parameters should be fine-tuned according to the phantom results and the actual physical characteristics of children to satisfy the optimization principle for radiation protection.

Patients with severe trauma require an extremely timely treatment and transfusion plays an irreplaceable role in the emergency treatment of such patients. An increasing number of evidence-based medicinal evidences and clinical practices suggest that patients with severe traumatic bleeding benefit from early transfusion of low-titer group O whole blood or hemostatic resuscitation with red blood cells, plasma and platelet of a balanced ratio. However, the current domestic mode of blood supply cannot fully meet the requirements of timely and effective blood transfusion for emergency treatment of patients with severe trauma in clinical practice. In order to solve the key problems in blood supply and blood transfusion strategies for emergency treatment of severe trauma, Branch of Clinical Transfusion Medicine of Chinese Medical Association, Group for Trauma Emergency Care and Multiple Injuries of Trauma Branch of Chinese Medical Association, Young Scholar Group of Disaster Medicine Branch of Chinese Medical Association organized domestic experts of blood transfusion medicine and trauma treatment to jointly formulate Chinese expert consensus on blood support mode and blood transfusion strategies for emergency treatment of severe trauma patients ( version 2024). Based on the evidence-based medical evidence and Delphi method of expert consultation and voting, 10 recommendations were put forward from two aspects of blood support mode and transfusion strategies, aiming to provide a reference for transfusion resuscitation in the emergency treatment of severe trauma and further improve the success rate of treatment of patients with severe trauma.

Objective:This study focuses on the use of narrative medical records in palliative care teams and analyzes text from cases.The goal is to establish a foundation for standardizing and structuring narrative content, providing guidance for incorporating future narratives into medical record writing.Methods:Three cases of narrative medical records from the palliative care team at Peking Union Medical College Hospital were chosen for text analysis to demonstrate the clinical significance of narrative medical records within palliative care teams.These records included medical consultation records, social work service records, and disease progress records, authored by nurses, social workers, and doctors, respectively.Results:Our study discovered that narrative medical records demonstrate traits such as structured writing, cooperative writing, and comprehensive documentation.Furthermore, the narrative information contained in these records can be efficiently organized and condensed.Conclusions:By examining the cases and utilization of narrative medical records in the healthcare community, we emphasize the potential benefits of incorporating narrative elements into existing medical documentation.These results offer valuable insights for enhancing medical education in the future.

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 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.

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.

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.