VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset, X-linked clonal autoinflammatory disease caused by somatic mutations in the UBA1 gene, characterized by systemic inflammation accompanied by hematologic clonal abnormalities. Somatic UBA1 mutations result in impaired ubiquitination, disruption of protein homeostasis, and sustained activation of inflammatory signaling pathways, including nuclear factor-κB and Janus kinase-signal transducer and activator of transcription (JAK-STAT), which together constitute the core pathogenic mechanism. The disease involves multiple systems and tissues including the hematologic system, skin, cartilage and respiratory system, showing remarkable clinical heterogeneity. Diagnosis depends primarily on the combination of characteristic clinical manifestations and molecular confirmation of UBA1 mutations. Current therapeutic strategies lack a unified standard: glucocorticoids can rapidly control inflammation but are associated with high relapse rates, whereas targeted therapies such as JAK inhibitors, interleukin inhibitors, and hypomethylating agents show variable efficacy, and allogeneic hematopoietic stem cell transplantation offers curative potential in patients with concomitant myelodysplastic syndrome. This review systematically summarizes the genetic background, molecular mechanisms, clinical spectrum, diagnostic criteria, and therapeutic advances of VEXAS syndrome.

Objective To compare the effect of different intensity of neuromuscular training(NMT)on muscle strength and knee joint function of patients after anterior cruciate ligament reconstruction(ACLR).Methods From January,2023 to January,2024,60 ACLR patients in Changhai Hospital were selected,and they received the same intensity of NMT from one to eight weeks after surgery.Eight weeks after surgery,they were randomly divided into low intensity group(n=30)and high intensity group(n=30),and then they received different inten-sities of NMT from nine to 16 weeks after surgery,each training session lasted one hour,with three sessions per week,totaly 48 sessions.The Lysholm score,knee flexor and extensor muscle strength and muscle endurance-were compared at eight weeks and 16 weeks after surgery.Results After group training,the Lysholm score significantly increased in both groups(|t|>13.739,P<0.001),and was higher in the high intensity group than in the low intensity group(t=-2.574,P<0.05);in the high intensity group,the relative peak torque and endurance of the extensor and flexor muscles improved at angular velocities of 60°/s,120°/s and 180 °/s(|t|>2.320,P<0.05);in the low intensity group,the flexor peak torque improved at all the three angular velocities(t>2.177,P<0.05),the extensor peak torque improved at angular velocities of 60°/s and 180°/s(|t|>1.715,P<0.05),and the extensor endurance improved at angular velocity of 60°/s(t=-2.293,P<0.05).However,there was no significant difference in the relative peak torque and endurance of the extensor and flexor muscles at all the three angular velocities(P>0.05).Conclusion Both high and low intensity NMT could improve the muscle strength,muscle endurance and knee joint func-tion.Maybe,high intensity is superior to low intensity.Further verification is still needed.

This study summarized the nursing experience of midline catheter insertion in 12 patients with a body mass index≥50,providing clinical references.Key nursing interventions included:pre-insertion patient positioning was adjusted to semi-Fowler's or sitting position based on respiratory status.The puncture sites were chosen at the middle or distal third of the upper arm with elbow flexion.The widened tourniquets and extended-length needles/catheter sheaths were used according to vascular depth and puncture angle.The modified pre-insertion length measurement and real-time ultrasound-guided vascular puncture were applied.The catheter with crisscross elastic bandage was secured post-insertion.All 12 patients successfully underwent catheterization,with devices removed after completing intravenous therapy and blood sampling.

Objective Using event-related potentials(ERPs)technology to study the effect of long term emotional distress on selective attention distraction inhibition function in college students and its neuroelectrophysiological mechanism.Methods The Eysenck personality questionnaire(EPQ)adult version was used to screen the high and low neuroticism groups among college students,and 35 subjects in each group were included in the long term emotional distress group and the emotional smoothness control group,respectively,and the response time,correct rate,N2 and P3 amplitude and latency results of the participants to complete the negative priming paradigm task were collected and analyzed.Results Compared with the control group,① the long term emotional distress group showed a prolonged response trend(P=0.072).② the long term emotional distress group had a prolonged N2 and P3 latency(P<0.05).Conclusion Selective attention distraction inhibition in college students with long term emotional distress decreased,and the decline mechanism may be related to the decline of inhibition processing and attention resource allocation ability.

Objective To compare the effect of different intensity of neuromuscular training(NMT)on muscle strength and knee joint function of patients after anterior cruciate ligament reconstruction(ACLR).Methods From January,2023 to January,2024,60 ACLR patients in Changhai Hospital were selected,and they received the same intensity of NMT from one to eight weeks after surgery.Eight weeks after surgery,they were randomly divided into low intensity group(n=30)and high intensity group(n=30),and then they received different inten-sities of NMT from nine to 16 weeks after surgery,each training session lasted one hour,with three sessions per week,totaly 48 sessions.The Lysholm score,knee flexor and extensor muscle strength and muscle endurance-were compared at eight weeks and 16 weeks after surgery.Results After group training,the Lysholm score significantly increased in both groups(|t|>13.739,P<0.001),and was higher in the high intensity group than in the low intensity group(t=-2.574,P<0.05);in the high intensity group,the relative peak torque and endurance of the extensor and flexor muscles improved at angular velocities of 60°/s,120°/s and 180 °/s(|t|>2.320,P<0.05);in the low intensity group,the flexor peak torque improved at all the three angular velocities(t>2.177,P<0.05),the extensor peak torque improved at angular velocities of 60°/s and 180°/s(|t|>1.715,P<0.05),and the extensor endurance improved at angular velocity of 60°/s(t=-2.293,P<0.05).However,there was no significant difference in the relative peak torque and endurance of the extensor and flexor muscles at all the three angular velocities(P>0.05).Conclusion Both high and low intensity NMT could improve the muscle strength,muscle endurance and knee joint func-tion.Maybe,high intensity is superior to low intensity.Further verification is still needed.

This case report presents a 16-year-old male patient with deficiency of adenosine deaminase 2(DADA2). The patient had a history of Raynaud′s phenomenon with digital ulcers since childhood. As the disease progressed, the patient developed retinal vasculitis, intracranial hemorrhage, skin necrosis, severe malnutrition, refractory hypertension, and gastrointestinal bleeding. Genetic testing revealed compound heterozygous mutations in the ADA2 gene (paternal c.1072G > A pathogenic mutation + maternal c.1065C > A variant of unknown clinical significance). ADA2 enzyme activity was significantly reduced (0.1 U/L). A multidisciplinary treatment team developed an individualized plan to address key issues, including nutritional support, blood pressure control, rehabilitation, pain management, and balancing anticoagulation/bleeding risks. After systematic intervention, the patient′s condition showed partial improvement. This case reveals clinical challenges such as anticoagulation decisions and nutritional support of DADA2. It also emphasizes the importance of early molecular diagnosis, tumor necrosis factor-α inhibitor targeted therapy, and multidisciplinary collaboration in management, underlining the core value of precision medicine in rare disease management.

OBJECTIVE: To reveal the gap between the evidence of systematic reviews (SRs) and clinical concerns by systematically summarizing the evidence on acupuncture and moxibustion for frozen shoulder and investigating the concerns and needs of clinicians in treatment with acupuncture and moxibustion for this disease. METHODS: The articles of SR and Meta-analysis on acupuncture and moxibustion for frozen shoulder were searched from CNKI, Wanfang, VIP, SinoMed, PubMed, EMbase and Cochrane Library, starting from the inception of each database up to December 31st, 2022. Two researchers screened the articles and extracted data independently. Using AMSTAR-2, the methodological quality of the included studies was evaluated. Based on systematic reviews and expert discussion, a questionnaire on clinical concerns of acupuncture and moxibustion for frozen shoulder was developed and distributed to clinicians. The discrepancies between the evidence and clinical concerns were compared from 5 dimensions, including population, interventions, control measures, outcome indicators and review time points. RESULTS: The evidence gaps existed between SRs and clinical concerns. In the existing studies, the needs of personalized treatment were not fully considered in terms of different syndromes/patterns of frozen shoulder and stages of illness, the outcome indicators were not employed properly, the time for outcome measurement was vague, the control groups were set up outside of standardization, and the methodological quality was lower. CONCLUSION It is suggested that future studies should improve the quality of methodology, lay more consideration to different patient groups, optimize outcome indicators and standardize the setting of control groups, so as to better meet the needs of patients and achieve the best match between evidence and clinicians' needs.
Humans ; Acupuncture Therapy ; Bursitis/therapy* ; Evidence Gaps ; Moxibustion ; Systematic Reviews as Topic ; Meta-Analysis as Topic

Objective Using event-related potentials(ERPs)technology to study the effect of long term emotional distress on selective attention distraction inhibition function in college students and its neuroelectrophysiological mechanism.Methods The Eysenck personality questionnaire(EPQ)adult version was used to screen the high and low neuroticism groups among college students,and 35 subjects in each group were included in the long term emotional distress group and the emotional smoothness control group,respectively,and the response time,correct rate,N2 and P3 amplitude and latency results of the participants to complete the negative priming paradigm task were collected and analyzed.Results Compared with the control group,① the long term emotional distress group showed a prolonged response trend(P=0.072).② the long term emotional distress group had a prolonged N2 and P3 latency(P<0.05).Conclusion Selective attention distraction inhibition in college students with long term emotional distress decreased,and the decline mechanism may be related to the decline of inhibition processing and attention resource allocation ability.

This study summarized the nursing experience of midline catheter insertion in 12 patients with a body mass index≥50,providing clinical references.Key nursing interventions included:pre-insertion patient positioning was adjusted to semi-Fowler's or sitting position based on respiratory status.The puncture sites were chosen at the middle or distal third of the upper arm with elbow flexion.The widened tourniquets and extended-length needles/catheter sheaths were used according to vascular depth and puncture angle.The modified pre-insertion length measurement and real-time ultrasound-guided vascular puncture were applied.The catheter with crisscross elastic bandage was secured post-insertion.All 12 patients successfully underwent catheterization,with devices removed after completing intravenous therapy and blood sampling.

Objective To investigate the appropriate venous access for obese patients undergoing metabolic and bariatric surgery by comparing the clinical outcomes of different lengths of peripheral intravenous catheters.Methods Inpatients who underwent bariatric surgery in a tertiary hospital in Zhejiang from August 2022 to December 2022 were selected as the study population using a fixed-point continuous convenience sampling method.A stratified block randomisation method was used to divide the group into an experimental group 1(mini-midline catheters),an experimental group 2(midline catheters)and a control group(short peripheral intravenous catheters,Short PIVCs).The incidence of catheter-related complications,the rate of extubation due to complications,the duration of catheter retention,the time to first catheter-related complication were compared in the 3 groups.Results A total of 186 patients were included,with 62 patients in each group.The overall incidence of catheter-related complications in experimental group 1,experimental group 2,and control group were 25.81％,8.06％,and 58.06％.The extubation rates due to complications were 19.35％,4.84％,and 41.94％,and the duration of catheter retention was 7.00(6.00,7.00)d,7.00(6.00,7.00)d,6.00(3.00,6.25)d.The differences were statistically different(P＜0.05)when comparing the 3 groups.Among them,the differences in the overall incidence of catheter-related complications and the rate of extubation due to complications were statistically significant when comparing experimental group 1 with the control group,experimental group 2 with the control group,and experimental group 1 with experimental group 2(P＜0.017);the duration of catheter retention in both experimental group 1 and experimental group 2 were higher than it in the control group,and the differences were statistically different(P＜0.017).Conclusion The complication rate of mini-midline catheters and midline catheters is lower than that of short ones,and the indwelling time is consistent with the perioperative period of metabolic and bariatric surgery,which is suitable for use in patients undergoing metabolic and bariatric surgery.