Alacrima-achalasia-mental retardation syndrome(AAMR) is an extremely rare autosomal recessive genetic disorder caused by mutation in the GMPPA gene. Its pathogenesis is associated with congenital disorders of glycosylation. The clinical manifestations involve multiple system impairments, with alacrima, achalasia, and mental retardation as the core features. This article reports the diagnosis, treatment process and long-term follow-up outcomes after deep brain stimulation in one patient with AAMR complicated with dystonia, aiming to improve clinicians' understanding of this disease.

Objective To examine the expression pattern of apoptosis signal-regulating kinase 1(ASK1)in the intestinal tissues of patients with Crohn's disease(CD),and analyze its mechanistic impact on intestinal epithelial barrier function and inflammatory responses.Methods Ileal tissue samples from Crohn's disease patients and healthy controls were collected.ASK1 protein level was assessed by immunohistochemistry,and its relationship with the Crohn's disease activity index(CDAI)was analyzed.A mouse model of acute colitis was constructed using TNBS,and subjected to qRT-PCR,Western blotting and immunohistochemistry for ASK1 expression,and the association between ASK1 expression and the disease activity index was examined.Lentivirus transfection was employed to create stable Caco-2 cell lines with altered ASK1 expression,and the intestinal barrier integrity and inflammation were assessed by measuring transepithelial electrical resistance(TEER),FITC-dextran leakage,and IL-6,IL-1β levels.Furthermore,the effects of ASK1 expression on Krüppel-like factor 4(KLF4)levels was examined using qRT-PCR and Western blotting.Results ASK1 was highly expressed in the ileum of CD patients and positively correlated with CDAI.In a TNBS-induced mouse model of acute colitis,ASK1 expression was up-regulated and positively correlated with DAI.Inflammation-induced ASK1 overexpression weakened the Caco-2 cell intestinal barrier,whereas ASK1 knockdown strengthened it.Moreover,ASK1 had the capability to enhance the expression of inflammatory factors.Additionally,ASK1 knockdown increased KLF4 expression,while overexpression decreased it,indicating a negative correlation between ASK1 and KLF4.Conclusion ASK1 expression is notably higher in CD and positively correlates with disease activity.ASK1 can influence intestinal barrier integrity and inflammatory factor expression,possibly through its impact on KLF4 expression.

In the context of high-quality development,enhancing hospital petition management is essential for fostering a harmonious doctor-patient relationship.This study uses a tertiary general hospital in Shanghai as a case to retrospectively analyze the handling of hospital complaints from 2022 to 2024.It examines the current petition landscape,identifies significant issues re-flected in these petitions,and explores the underlying causes of challenges in resolving petition-related conflicts.Furthermore,it discusses the hospital's strategies in innovating management mechanisms,engaging diverse stakeholders,developing professional teams,and comprehensively addressing patient needs.These approaches aim to offer valuable insights for public hospitals to im-prove petition management efficiency as they advance in high-quality development.

Objective To develop a method of pulsed direct current electrospray ionization mass spectrometry(pulsed-dc-ESI-MS)for rapid detection of 4 kinds of amphetamines in saliva.Methods Saliva samples were adjusted to optimal pH using NaOH,followed by protein precipitation with anhydrous magnesium sulfate and liquid-liquid extraction with ethyl acetate.The supernatant was then analyzed via pulsed-dc-ESI-MS.Results The limits of detection were 0.01～0.5 ng/mL and the limits of quantitative were 1.0～5.0 ng/mL.In addition,in the concentration range,4 amphetamines have good linear relationships,the correlation coefficients(R2)were all greater than 0.999,the recoveries of 97.21%～104.70%and the relative standard deviations(RSDs)were less than 10%.Conclusion The method has the advantages of simple operation,fast detection,high sensitivity,selectivity and good stability.It can be used for on-site rapid detection of amphetamines in saliva.

Objective:To construct a quality assessment tool for teaching plans designed for clinical scenario-based simulation, test its reliability and validity, and provide an effective tool for accurately and objectively evaluating the simulation instructors' capabilities in clinical scenario-based simulation teaching designs and the quality of teaching plans.Methods:Through literature review and two rounds of expert consultation using the Delphi method, an initial draft of the assessment tool was formed. Subsequently, an empirical study was conducted with 85 clinical scenario-based simulation teaching plans collected nationwide to test the reliability and validity of the assessment tool.Results:The quality assessment tool for teaching plans designed for clinical scenario-based simulation consists of 5 primary items and 17 secondary items. The Cronbach's α coefficient of the scale was 0.872. The inter-rater reliability, measured by the intraclass correlation coefficient, was 0.838. The scale level content validity index was 0.949. The results of confirmatory factor analysis showed χ2/d f of 2.385, a root mean square error of approximation of 0.090, and a comparative fit index of 0.861, indicating an acceptable model fit. Conclusions:The quality assessment tool for teaching plans designed for clinical scenario-based simulation demonstrates high reliability and validity and can be used as a tool for evaluating the simulation instructors' capabilities in clinical scenario-based simulation teaching designs and the quality of teaching plans.

Objective To investigate the features of resting-state electroencephalography(EEG)in stroke patients with limited upper limb movement,and assess its potential utility in evaluating upper limb motor function.Methods From March to August,2024,a total of 71 stroke patients with limited upper limb movement were enrolled as stroke group at the Rehabilitation Hospital Affiliated to Fujian University of Traditional Chinese Medicine;while 63 healthy participants matched for age and sex were recruited as control group.They were tested with 19-chan-nel resting-state EEG(rsEEG),calculating of the pairwise derived brain symmetry index(pdBSI)and Delta/Al-pha ratio(DAR).The motor function was assessed with Fugl-Meyer Assessment-Upper Extremities(FMA-UE),the upper limb recovery was evaluated with Brunnstrom Stages and the activities of daily living was assessed with modified Barthel index(MBI)in stroke patients,Results Compared with the control group,the pdBSI of Global lead,Frontal region,Central region and Posterior region across Delta,Alpha and Beta1 frequency bands were significantly higher in the stroke group than in the control group(|Z|>2.289,P<0.05);as well as the pdBSI of Global lead,Central region and Posterior region across The-ta bands(|Z|>3.016,P<0.01),the pdBSI of Global lead,Frontal region,Central region and Posterior region across Beta2 bands(|Z|>3.222,P<0.01),DAR of Global lead,Frontal region,Central region and Posterior re-gion(|Z|>6.565,P<0.001).In the stroke group,the pdBSI of Global lead(r=-0.280,P=0.018)and Central region(r=-0.304,P=0.010)across the Delta band were significantly negatively correlated with FMA-UE,as well as the pdBSI of Global leads(r=-0.289,P=0.014),Central region(r=-0.244,P=0.040)and Posterior region(r=-0.356,P=0.002)across the Beta1 band,and the DAR of Global lead(r=-0.431,P<0.001),Fron-tal region(r=-0.429,P<0.001),Central region(r=-0.491,P<0.001)and Posterior region(r=-0.482,P<0.001).Conclusion Asymmetry in spectral power between hemispheres in the Delta(0.5 to 4 Hz)and Beta(13 to 20 Hz)bands is found in stroke patients,especially in the central region,which correlates with upper limb function limitations.pdBSI Delta,pdBSI Beta1 and DAR are potential neuroelectrophysiological markers for assessing upper limb motor function in stroke patients.

Objective:To explore the construction of an assessment tool for the implementation of scenario-based simulation courses, and provide a reference for the standardized implementation of scenario-based simulation courses and the key direction of teacher training.Methods:Based on the elements of the implementation of scenario-based simulation courses, the Scenario-Based Simulation Course Implementation Quality Scale was developed through expert interviews and expert panel meetings. This scale was used to rate the implementation of scenario-based simulation courses by 12 teaching teams in Shanghai, China. The evaluation scores were collected and analyzed using R language.Results:The reliability of the evaluation scale was assessed using Cronbach's α (0.893) and inter-rater consistency was assessed using the intraclass correlation coefficient (0.226). Among the 12 teaching teams, the lowest score rate was 67.07% for "debriefing". A correlation analysis between scenario-based simulation teaching design and course implementation showed a Pearson correlation coefficient r of -0.08 ( P=0.809), which indicated no significant correlation between them. Conclusions:The assessment of the implementation of scenario-based simulation courses should include all aspects of course implementation. The rater's understanding of scenario-based simulation courses can affect the results. Debriefing remains a challenge in implementing scenario-based simulation courses. A comprehensive assessment of the teaching ability of simulation teaching teams should include both teaching design and course implementation.

In the context of high-quality development,enhancing hospital petition management is essential for fostering a harmonious doctor-patient relationship.This study uses a tertiary general hospital in Shanghai as a case to retrospectively analyze the handling of hospital complaints from 2022 to 2024.It examines the current petition landscape,identifies significant issues re-flected in these petitions,and explores the underlying causes of challenges in resolving petition-related conflicts.Furthermore,it discusses the hospital's strategies in innovating management mechanisms,engaging diverse stakeholders,developing professional teams,and comprehensively addressing patient needs.These approaches aim to offer valuable insights for public hospitals to im-prove petition management efficiency as they advance in high-quality development.

Objective To investigate the role of the prefrontal cortex in the generation and adaptation of chro-nic subjective tinnitus using resting-state functional magnetic resonance imaging(rs-fMRI).Methods Resting-state functional magnetic resonance imaging scan were acquired from 20 patients with chronic subjective tinnitus and 20 healthy controls.Fractional amplitude of low-frequency fluctuations(fALFF)and seed-based whole-brain functional connectivity(FC)methods were used to detect abnormal prefrontal cortex activity in tinnitus patients and to investi-gate interactions between prefrontal cortex activity and brain regions associated tinnitus perception.The analysis aimed to assess the relationship between prefrontal cortex spontaneous neural activity,atypical functional connectivi-ty across various brain regions,and clinical characteristics of tinnitus.Results Compared with healthy controls,pa-tients with chronic tinnitus showed a significant reduction in fALFF values in some specific brain areas of prefrontal cortex,including the left/right medial superior frontal gyrus and the left/right middle frontal gyrus.Functional con-nectivity values were notably enhanced between the left medial superior frontal gyrus and the left anterior insula,as well as between the right medial superior frontal gyrus and the left superior temporal gyrus.Furthermore,increased functional connectivity was observed between the left middle frontal gyrus and the right middle temporal gyrus,as well as between the right middle frontal gyrus and the left parahippocampal gyrus,left superior parietal lobule,and left supplementary motor area.Importantly,the functional connectivity between the left middle frontal gyrus and the right superior temporal gyrus exhibited a negative correlation with tinnitus handicap inventory scores(r=-0.627,P=0.003)and visual analogue scale scores(r=-0.596,P=0.005).Conclusion There are abnormal brain function changes in medial prefrontal cortex and dorsolateral prefrontal cortex in patients with chronic subjec-tive tinnitus,accompanied by changes in the intensity of functional connections with the salience and auditory net-works.These abnormalities are highly related to the severity of tinnitus.The prefrontal cortex may play an impor-tant role in the sensory prediction and auditory regulation of tinnitus.

Objective To examine the expression pattern of apoptosis signal-regulating kinase 1(ASK1)in the intestinal tissues of patients with Crohn's disease(CD),and analyze its mechanistic impact on intestinal epithelial barrier function and inflammatory responses.Methods Ileal tissue samples from Crohn's disease patients and healthy controls were collected.ASK1 protein level was assessed by immunohistochemistry,and its relationship with the Crohn's disease activity index(CDAI)was analyzed.A mouse model of acute colitis was constructed using TNBS,and subjected to qRT-PCR,Western blotting and immunohistochemistry for ASK1 expression,and the association between ASK1 expression and the disease activity index was examined.Lentivirus transfection was employed to create stable Caco-2 cell lines with altered ASK1 expression,and the intestinal barrier integrity and inflammation were assessed by measuring transepithelial electrical resistance(TEER),FITC-dextran leakage,and IL-6,IL-1β levels.Furthermore,the effects of ASK1 expression on Krüppel-like factor 4(KLF4)levels was examined using qRT-PCR and Western blotting.Results ASK1 was highly expressed in the ileum of CD patients and positively correlated with CDAI.In a TNBS-induced mouse model of acute colitis,ASK1 expression was up-regulated and positively correlated with DAI.Inflammation-induced ASK1 overexpression weakened the Caco-2 cell intestinal barrier,whereas ASK1 knockdown strengthened it.Moreover,ASK1 had the capability to enhance the expression of inflammatory factors.Additionally,ASK1 knockdown increased KLF4 expression,while overexpression decreased it,indicating a negative correlation between ASK1 and KLF4.Conclusion ASK1 expression is notably higher in CD and positively correlates with disease activity.ASK1 can influence intestinal barrier integrity and inflammatory factor expression,possibly through its impact on KLF4 expression.