Gorham-Stout disease(GSD) is a rare osteolytic disorder characterized by spontaneous and progressive osteolysis, along with abnormal angiogenesis and lymphangiogenesis, with no new bone formation. We present a case of a 15-year-old female admitted due to " recurrent right leg pain for 5 years, 11 months after undergoing right femoral fracture surgery". Through comprehensive integration of the patient's clinical phenotype, laboratory tests, imaging findings, pathological examinations, and molecular biological test results, GSD was considered highly likely. A multidisciplinary treatment approach was conducted, including a combination of zoledronic acid and sirolimus to inhibit osteolysis, along with rehabilitation training and orthopedic intervention, providing a personalized and comprehensive treatment strategy.

Objective: To define the mechanism by which swine TRIM25 restricts vesicular stomatitis virus replication. Methods: Porcine 3D4/21 cells with TRIM25 overexpression or knockdown were infected with vesicular stomatitis virus. Viral replication was quantified by immunoblotting, quantitative reverse transcription polymerase chain reaction, and 50% tissue culture infectious dose assays. Type I interferon signaling was assessed by transcript quantification, interferon-beta and interferon-stimulated response element reporter assays, and co-immunoprecipitation.Viral RNA binding was tested by RNA immunoprecipitation. Results: TRIM25 overexpression reduced viral RNA and infectious titers, whereas TRIM25 knockdown increased replication (p < 0.01). TRIM25 increased interferon-beta and interferon-stimulated gene expression and enhanced interferon-beta and interferonstimulated response element promoter activity (p < 0.01). Mechanistically, TRIM25 promoted Lys63-linked ubiquitination of RIG-I and increased phosphorylation of TANK-binding kinase 1 and interferon regulatory factor 3. TRIM25 also bound vesicular stomatitis virus genomic RNA, and binding required the C-terminal region. Conclusions and Relevance: Porcine TRIM25 restricts vesicular stomatitis virus replication by amplifying type I interferon signaling and directly binding viral RNA.

Objective:To investigate the effects of auditory integration training combined with applied behavior analysis(ABA)rehabilitation training on the rehabilitation effect and social skills of children with autism spectrum disorder(ASD).Methods:This study was a prospective randomized controlled study,60 children with ASD admitted to Tongren Rehabilitation Hospital from June 2023 to June 2024 were selected as the research subjects,and divided into control group(n=30)and treatment group(n=30)according to the random double chromosphere method.The control group received ABA rehabilitation training,The treatment group received auditory integration training on the basis of the control group,Both groups were treated for 6 months.The improvement of clinical symptoms,Sensory Integration Function Scale(SIFS),Repetitive Behavior Scale-Revised(RBS-R)between two groups before and after treatment and Self-Rating Anxiety Scale(SAS)score for parents caring for the sick child were compared between two groups.Results:The sensation,communication,physical movement,language,self-care,and total score of the treatment group were all lower than those of the control group(P＜0.05).The language,social skills,sensory perception,behavior and overall score of the treatment group were lower than those of the control group(P＜0.05).The RBS-R and SAS scores of the child's foster parents in the treatment group were lower than those in the control group,while the SIFS score was higher than that in the control group(P＜0.05).Conclusion:The combination of auditory integration training and ABA rehabilitation training can improve the rehabilitation effect of children with ASD,improve their social skills,sensory integration ability,and symptoms of repetitive stereotyped behavior,Relieve the anxiety of parents caring for sick children.

Objective:To integrate the best evidence of non drug intervention for blood lipid management of breast cancer survivors used the evidence-based method, and provide evidence-based evidence for clinical medical staff to carry out dynamic monitoring and management of blood lipid abnormalities.Methods:According to the 6S model, the evidence summary, clinical decision, recommended practice, guidelines, systematic review, expert consensus, scientific position statement and other non-drug interventions on lipid management of breast cancer survivors were systematically searched from the evidence-based guidelines website and related databases from database establishment until June 10, 2024. The quality of the included literature was evaluated, and the evidence was extracted, summarized and formed.Results:A total of 14 literatures were included, including 7 guidelines, 5 expert consensus and 2 systematic reviews. Totally 21 best evidences were collected from the necessity of lipid management, lipid management objectives, individualized lipid monitoring and lipid management strategies.Conclusions:The quality and level of the best evidence collected in this study are generally high, which has reference significance for clinical medical staff to carry out scientific and effective non drug intervention in lipid management of breast cancer survivors. In the evidence transformation stage, it is necessary to fully consider the individual differences of patients, in order to improve the intervention effect and the quality of life of patients.

Objective To investigate the effects of m6A methylation modification and changes in oxidative stress levels on the progression of colorectal cancer(CRC)in Apemin/+mice with normal and high-fat diets.Methods C57BL/6J mice and Apcmin/+mice were fed with a normal or high-fat diet(60％fat)for 2(S group),6(M group),or 12 weeks(L group),respectively.Food intake,body mass,and the size,number,and volume of small intestinal polyps and colon tumors were then measured.Protein expression levels of the cancer markers Ki-67 and proliferating cell nuclear antigen in colon tissues were detected by immunohistochemical staining and the serum levels or activities of the antioxidant enzymes catalase,reduced glutathione,and lipid peroxide malondialdehyde were detected using appropriate kits.mRNA expression levels of m6A methylation-related enzymes in colon tissues were detected by RT-qPCR and total levels of m6A methylation modification in colon tissues were detected.Results(1)Apcmin/+mice showed rapid tumor growth from the early to middle stages of cancer.Tumor proliferation from the middle to late stages of cancer was slowed in mice fed a normal diet,while a high-fat diet promoted the further development of cancer.(2)Decreased m6A methylation levels and enhanced antioxidant capacity may have delayed advanced tumor development in Apcmin/+mice fed a normal diet.(3)In contrast,a high-fat diet may have promoted the sustainable development of CRC by increasing the total level of m6A methylation,while the enhanced antioxidant capacity may have been insufficient to resist the promoting effect of m6A methylation on CRC.Conclusions A high-fat diet may promote the advancement of CRC compared with a normal diet by affecting m6A methylation modification.Both normal and high-fat diets enhanced the antioxidant capacity,suggesting that antioxidant effects may initiate self-protection mechanisms during cancer progression.

Objective To investigate the role of Sneathia sanguinegens(S.sanguinegens)in the development of unexplained recurrent spontaneous abortion(URSA).Methods A case-control study was conducted to analyze the vaginal flora characteristics of 65 patients with URSA and 18 healthy controls through 16S rRNA gene sequencing.Toxicity profile of S.sanguinegens on human cervical cancer cells(ME-180),human umbilical vein endothelial cells(HUVEC)and human placental choriocarcinoma cells(JEG-3)was analyzed at the cellular level to assess the mechanism of it in adverse pregnancy outcomes.And S.sanguinegens was used to infect C57BL/6J mice to explore the toxic effect on living organisms.Results The relative abundance of Sneathia was increased in patients with URSA compared with healthy controls.It was positively correlated with the number of miscarriages,and was attributed to S.sanguinegens.We also found that S.sanguinegens damaged ME-180,JEG-3 and HUVEC cells.The degree of cellular damage was related to the level of S.sanguinegens added.Intravenous infection with S.sanguinegens caused inflammatory damage in several organs and extramedullary hematopoiesis in the spleen.Conclusion S.sanguinegens is closely related to URSA and should be emphasized in patients with high vaginal bacterial load.

Case report form(CRF)is an important tool to obtain clinical research data.Scientifically designed and properly recorded CRF is of great significance to improve the quality of clinical research.The author summarized the common problems existing in the design and recording of CRF in traditional Chinese medicine(TCM)clinical studies,including:①Delayed design time point.②Unstandardized CRF design,recording and modification.③Imperfect records of combined medication/therapy.④Insufficient quantification of syndrome efficacy indicators and objectification of tongue and pulse.⑤ Insufficient protection of subjects' privacy.⑥ Unstandardized records of adverse events.Accordingly,this paper discusses some solutions to provide reference for other TCM researchers,including:①Advance CRF design time.②Standardize CRF design,recording and modification.③Design drug combinations/therapies according to Clinical Data Acquisition Standards Harmonization(CDASH).④Standardization of syndrome and tongue and pulse.⑤Use of subject identification codes to protect privacy.⑥AE records should vary from study to study.

Objective To investigate the practical effects of pediatric rapid sequence intubation(RSI)nursing coordination training based on the LSPPDM(learn,see,practice,prove,do,maintain)framework in order to provide evidence for optimizing pediatric RSI nursing training programs.Methods Nurses from the intensive care unit(ICU)of Children's Hospital,Fudan University during Feb 2023 and Jan 2024 were divided into the experimental group(n=35)and the control group(n=35)by block randomization.The experimental group received LSPPDM framework-based training,while the control group underwent conventional training with theoretical lectures and procedural demonstrations.Outcomes included training satisfaction,theoretical knowledge and procedural skill assessment scores,team collaboration compliance and RSI procedure time were compared between the two groups.Results The experimental group demonstrated significantly higher training satisfaction(123.80±2.04 vs.117.26±9.82,P<0.05),superior post-training theoretical knowledge and procedural skills(P<0.05),enhanced team collaboration compliance(P<0.05),and shorter RSI completion time(P<0.05)compared with the control group.Conclusion Pediatric RSI nursing coordination training based on the LSPPDM framework can effectively increase training satisfaction,promote theoretical and procedural skills and reduce completion time in nurses.

Purpose To characterize the clinicopathological features of gastrointestinal mesenchymal tumors(GMTs)resected via endoscopic techniques.Methods A retrospective analysis was conducted on 495 cases of endo-scopically resected GMTs.Clinical information,histomorphological findings,immunohistochemical profiles,and mo-lecular characteristics were reviewed.Results The cohort included 495 patients aged 20-78 years(median:53 years).The majority of tumors were located in the stomach(58.8％)and esophagus(36.8％).Histologically,most tumors consisted of spindle cells,with a minority composed of epithelioid cells;fibrocollagenous or myxoid stroma was occasionally observed.Immunohistochemically,leiomyomas showed diffuse positivity for α-SMA(98.8％)and desmin(99.3％),gastrointestinal stromal tumors(GISTs)expressed CD117(99.4％),DOG1(97.6％),and CD34(97.0％),and schwannomas were positive for S-100(93.7％).The predominant tumor types were leiomyomas(54.1％)and GISTs(33.7％),while the remaining 12.2％comprised other rare types.Various endoscopic resection techniques were employed based on the tumors' anatomical depth,including endoscopic submucosal dissection(ESD,40.5％),submucosal tunneling endoscopic resection(STER,17.1％),endoscopic mucosal resection(EMR,16.5％),endoscopic full-thickness resection(EFTR,13.9％),and endoscopic submucosal excavation(ESE,12.0％).EMR and ESD were primarily used for superficial lesions,while deeper tumors with were more often treated with STER,EFTR,and ESE.The rate of negative resection margins was lower in GISTs(72.2％)and other tumors with indistinct margins,compared to leiomyomas(92.6％)and those with well-defined boundaries.Conclusion Leiomyomas and GISTs are the most common gastrointestinal mesenchymal tumors resected via endoscopy.A variety of resection techniques are applicable depending on tumor location and depth.Accurate pathological diagnosis should be based on HE morphology,supplemented by endoscopic findings,margin status,immunohistochemistry,and necessary molecular tests.

Objective:To investigate the effect of extracorporeal membrane oxygenation (ECMO) in assisting neonates with complex congenital heart diseases (CHD) and the survival rate of the patients.Methods:A retrospective case-control study was made on 22 newborns with complex CHD assisted by ECMO during the perioperative period in Fuwai Central China Cardiovascular Hospital from January 2018 to June 2024.There were 19 males and 3 females in the newborns included, with an average age of (10.4±8.7) days (range: 1-26 days) and an average weight of (3.1±0.3) kg.Complex CHD included total anomalous pulmonary venous connection in 9 cases (40.9%), interrupted aortic arch and coarctation in 8 cases (36.4%), transposition of great arteries in 3 cases (13.6%), double outlet right ventricle in 1 case (4.5%) and cardiac tumor in 1 case (4.5%).The patients were divided into the <5 d group, 5-12 d group, and >12 d group according to the duration of ECMO support.Data were compared using the t-test or χ2 test. Results:There were 8 cases (36.4%) successfully weaned from ECMO support and 6 cases (27.3%) survived.ECMO support was used routinely in 15 cases (68.2%), and for extracorporeal cardiopulmonary resuscitation in 7 cases (31.8%).The duration of ECMO support was (5.9±3.7) days.There were no statistically differences in age, weight, gender, disease composition, operation time, cardiopulmonary bypass time, cross-clamp time, assistant time, blood routine, liver and kidney function and other biochemical indicators, preoperative cardiac ejection fraction value, procalcitonin, C-reactive protein and other infection indicators between the death group and the survival group (all P>0.05).The highest lactate values 24 hours before [(8.1±5.4) mmol/L] and after ECMO support [(10.5±7.1) mmol/L] in the survival group were significantly lower than those in the death group [(18.7±9.2) mmol/L, (21.3±8.6) mmol/L] ( t=2.606, P=0.018; t=2.729, P=0.013).It was found that the survival rate was 0/9 (0) in the <5 d group, 6/12 (50.0%) in the 5-12 d group, and 0/1 (0) in the >12 d group.The survival rate of the 5-12 d group was the highest, which was significantly higher than that of the <5 d group ( χ2=6.300, P=0.012). Conclusions:ECMO support is an effective treatment for severe circulatory failure in neonates with complex CHD in the perioperative period.The highest lactate levels 24 hours before and after ECMO support affect the survival rate.Patients receiving 5-12 d ECMO support can achieve the highest survival rate.