ObjectiveTo investigate the effect of video-based educational intervention combined with maternal presence on perioperative adverse outcomes in preschool children undergoing general anesthesia， including cooperation in anesthesia induction， perioperative anxiety， pain and agitation during recovery. MethodsA total of 300 preschool children scheduled for general anesthesia in our hospital from June to December 2023 were randomly assigned to control group （n=150） and intervention group （n=150）. The control group received routine recovery care. For the intervention group， in addition to routine recovery care， a preoperative visit was scheduled one day before surgery. During this visit， mothers were guided to watch anesthesia videos with their children. During the waiting period in the operating room and 30 minutes after awakening， the mothers were guided to accompany the children for more than 30 minutes. Recovery conditions were recorded using the surgical anesthesia information system， and the children’s anesthetic induction compliance， perioperative anxiety， pain， and agitation were evaluated and recorded using the modified Yale Preoperative Anxiety Scale （m-YPAS）， the Induction Compliance Scale （ICC）， the Children’s Pain Behavior Scale （FLACC）， and the Pediatric Agitation and Emergence Delirium Scale （PAED）. ResultsOn the preoperative visit day， there were no statistically significant differences in baseline data between the two groups （P > 0.05）. For perioperative anxiety， the m-YPAS scores of the intervention group were significantly lower than those of the control group， both when entering the operating room waiting area （35.27±6.48 vs. 41.79±6.68， P < 0.05） and 30 minutes after postoperative recovery （20.13±7.05 vs. 35.75±9.51， P < 0.05）. In terms of anesthesia induction cooperation， the ICC scores of the intervention group were significantly lower than those of the control group （1.84±0.95 vs. 3.17±0.62， P < 0.05）， and the proportion of good induction cooperation was significantly higher than that of the control group （24.00% vs. 12.67%， P < 0.05）. There was no significant difference in awakening duration between the two groups， but the intervention group had a significantly shorter length of stay in the post-anesthesia care unit than the control group （0.90±0.29 hours vs. 1.29±0.42 hours， P < 0.001）. For perioperative agitation， the PAED scores of the intervention group were significantly lower than those of the control group （entering in the operating room waiting area： 8.5 vs. 9.2， P < 0.05； 30 minutes after postoperative recovery： 4.2 vs. 7.8， P < 0.05）. In terms of pain scores， the FLACC scores of the intervention group were also significantly lower than those of the control group， both when entering the operating room waiting area （ 5.3 vs. 6.7， P < 0.05； 30 minutes after postoperative recovery： 2.1 vs. 4.9， P < 0.05）. ConclusionsVideo-based educational intervention combined with maternal presence reduces the perioperative anxiety， pain and agitation of preschool children undergoing general anesthesia， and improved the compliance of anesthesia induction. It is recommended to promote this intervention measure in clinical practice.

Objective:To report the clinical, electrophysiological, and genetic mutation characteristics of a family with paramyotonia congenita, to enhance clinical understanding of ion channelopathies, provide better guidance for clinical diagnosis, and optimize treatment plans.Methods:The clinical data, nerve conduction studies, needle electromyography, long exercise test, short exercise test, and whole-exome sequencing results of the proband with paramyotonia congenita and his family members admitted to Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, in August 2023 were collected and analyzed.Results:The proband was a 15-year-old male who presented with episodic muscle weakness for 1 year, accompanied by elevated creatine kinase during episodes. Between the ages of 3 and 4, he exhibited facial and bilateral hand myotonia triggered by cold exposure. His mother, sister, maternal grandmother, maternal aunt and uncle also displayed similar cold-induced facial and hand myotonia during childhood. Additionally, his cousin exhibited walking difficulties, frequent falls, mild difficulty in opening eyes after closure, and mild chewing and swallowing difficulties at the age of 1. Electromyography of the proband showed widespread myotonic discharges and myopathic impairment, while the electromyography of his mother and sister revealed only widespread myotonic discharges. The proband′s long exercise test results were consistent with periodic paralysis, while the short exercise test results aligned with paramyotonia congenita. His sister′s long exercise test results were within normal range, while the short exercise test results of his mother and sister were consistent with paramyotonia congenita. Genetic testing of the family revealed that all tested patients carried a heterozygous SCN4A mutation, c.4343G>A (R1448H). Conclusions:The R1448H mutation in the SCN4A gene is the genetic cause of this family. Patients carrying the SCN4A R1448H mutation may exhibit clinical features of cold-induced myotonia with or without episodic muscle weakness. In addition to widespread myotonic discharges on electromyography, certain patients may also show myopathic impairment. The long and short exercise tests can further assist in differentiating various types of myotonia and periodic paralysis for clinical diagnosis.

In recent years, as societal perceptions have evolved and women's social standing has improved, the average age of childbearing for women in China has generally increased. This shift has contributed to a notable decline in fertility rates among Chinese women. The introduction of the "three-child" policy and the inclusion of assisted reproductive technology (ART) in medical insurance coverage across many regions have further fueled the rapid rise in demand for ART among older women. However, numerous high-level evidence-based medical studies have confirmed that age remains a critical factor influencing the success rates of ART treatments. Many older women seeking pregnancy assistance, particularly than aged 43 years and above, continue to experience unsatisfactory outcomes despite multiple attempts at ART. Therefore, considering the principles of beneficence, protection of offspring, and social welfare, and in light of relevant domestic and international guidelines, consensus, policies, regulations, and research data, should there be an age limit for high-aged patients undergoing ART treatments? This issue warrants a thorough ethical examination.

Objective:To report the clinical, electrophysiological, and genetic mutation characteristics of a family with paramyotonia congenita, to enhance clinical understanding of ion channelopathies, provide better guidance for clinical diagnosis, and optimize treatment plans.Methods:The clinical data, nerve conduction studies, needle electromyography, long exercise test, short exercise test, and whole-exome sequencing results of the proband with paramyotonia congenita and his family members admitted to Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, in August 2023 were collected and analyzed.Results:The proband was a 15-year-old male who presented with episodic muscle weakness for 1 year, accompanied by elevated creatine kinase during episodes. Between the ages of 3 and 4, he exhibited facial and bilateral hand myotonia triggered by cold exposure. His mother, sister, maternal grandmother, maternal aunt and uncle also displayed similar cold-induced facial and hand myotonia during childhood. Additionally, his cousin exhibited walking difficulties, frequent falls, mild difficulty in opening eyes after closure, and mild chewing and swallowing difficulties at the age of 1. Electromyography of the proband showed widespread myotonic discharges and myopathic impairment, while the electromyography of his mother and sister revealed only widespread myotonic discharges. The proband′s long exercise test results were consistent with periodic paralysis, while the short exercise test results aligned with paramyotonia congenita. His sister′s long exercise test results were within normal range, while the short exercise test results of his mother and sister were consistent with paramyotonia congenita. Genetic testing of the family revealed that all tested patients carried a heterozygous SCN4A mutation, c.4343G>A (R1448H). Conclusions:The R1448H mutation in the SCN4A gene is the genetic cause of this family. Patients carrying the SCN4A R1448H mutation may exhibit clinical features of cold-induced myotonia with or without episodic muscle weakness. In addition to widespread myotonic discharges on electromyography, certain patients may also show myopathic impairment. The long and short exercise tests can further assist in differentiating various types of myotonia and periodic paralysis for clinical diagnosis.

In recent years, as societal perceptions have evolved and women's social standing has improved, the average age of childbearing for women in China has generally increased. This shift has contributed to a notable decline in fertility rates among Chinese women. The introduction of the "three-child" policy and the inclusion of assisted reproductive technology (ART) in medical insurance coverage across many regions have further fueled the rapid rise in demand for ART among older women. However, numerous high-level evidence-based medical studies have confirmed that age remains a critical factor influencing the success rates of ART treatments. Many older women seeking pregnancy assistance, particularly than aged 43 years and above, continue to experience unsatisfactory outcomes despite multiple attempts at ART. Therefore, considering the principles of beneficence, protection of offspring, and social welfare, and in light of relevant domestic and international guidelines, consensus, policies, regulations, and research data, should there be an age limit for high-aged patients undergoing ART treatments? This issue warrants a thorough ethical examination.

Objective To study the characteristics of work disability and its influencing factors in patients with ankylosing spondylitis (AS). Methods The demographic data, work conditons and disease related characteristics of 277 patients with AS were recorded, and randomly selected from the Department of Rheumatology, Chinese PLA General Hospital from November 2014 to January 2016. Work and productivity activity impair-ment questionnaire (WPAI) was used to survey the work disability and productivity loss, then explore its in-fluencing factors and the relationships between patient-reported outcomes and WPAI scores. Logistic regression was used to analyze the associated factors of work disability. Multivariate linear regression was used to analyze the predictive factors of lose of work productivity. Results The prevalence of work disability was 30.3%. Twenty patients were unemployed because of working disability. Two hudreds patients were employed, with average 36.5 (24.0, 50.0) hours workingtime in the past week. Average AS related absenteeism was 4.4 (0, 10) hour. Average workproductivity loss was 26.4%(2.5%, 40.0%). Logistic multiple regression analysis showed that Bath AS disease activity index (BASDAI), SF-36 physical component summary (PCS) scores might be the important influencing factors among those clinical measures ( OR=1.270, 0.959). Presenteeism and overall work impairment were moderately correlated with patients' global assessment of disease activity (VAS), BASDAI, bath AS functional index (BASFI), SF-36 physical Functioning (PF), SF-36 body pain (BP) and SF-36 Physical Component Summary (PCS) (|r|=0.539-0.648). Linear multivariate analyses indicated that work presente-eismand absenteeismwere significantly associated with BASDAI (P<0.01). Conclusion High prevalence of work disability in patients with AS is noted, which is closely related with disease activity and body function;High attention should be paid to AS patients with work disability.

Objective To assess the dignosis value of CT three-dimensional reconstruction with Fisher discriminant model in small solitary pulmonary nodules before operation.Methods CT data of 40 cases with SPN were retrospectively analyzed and divided into into malignant pulmonary nodules (25 cases),squamous cell carcinoma (4 cases),adenocarcinoma (13 cases),lung cancer (4 ca-ses),small cell lung cancer (2 cases),large cell carcinoma (1 case),metastases tumor (1 case),benign nodules (1 5 cases,6 cases of tuberculosis,2 cases of hamartoma,and 7 cases of non-specific inflammatory nodules)by pathology and follow-up results.The CT features of pulmonary nodules were evaluated through multi-planar reformation (MPR),curved-planar reformation (CPR),volume rendering (VR),maximum intensity proj ection (MIP)and other three-dimensional reconstruction.The three-dimensional data were divided into benign and malignant groups.In each of the two groups,the significant signs of morphological signs of discrimination indicators were adminstrated Fisher discriminant,and the probalitiy of false positives were estimated using cross-validation method. Results The positive features of pulmonary nodules in there-dismensional images were much more than in two-dimensional images. Fisher discriminant formula of solitary pulmonary nodules in three-dimensional images was Z=1.143X1 + 0.454X2+1.606X3-0.262X4+0.04X5+0.483X6+1.611X7-2.164.Discriminant boundary value Zc was-0.516.When Zcgreater than -0.516,nodules were proneed to considere as malignant nodules.In 25 cases of malignant nodules,4 cases mistook for benign.When Zc less than -0.516,nodules were proneed to considere as benign nod-ules.In 1 5 benign nodules,2 cases mistook for malignant.The total misdiagnosis and accuracy rate were 15 % and 85% respec-tively.Conclusion CT three-dimensional reconstruction combined with Fisher discriminant model have a high clinical value in dif-fereiating diagonsis of pulmonary nodules were proneed to considere as malignant nodules.In 25 cases of malignant nodules,4 cases mistook for benign.When Zc less than -0.516,nodules were proneed to considere as benign nodules. In 15 benign nodules,2 cases mistook for malignant.The total misdiagnosis and accuracy rate were 15 % and 85% respec-tively.Conclusion CT three-dimensional reconstruction combined with Fisher discriminant model have a high clinical value in differeiating diagonsis of pulmonary nodules.

Objective To explore the value of CT diagnosis in broadening of right upper mediastinum.Methods X-ray plain film and CT manifestations of broadening of right upper mediastinum in 56 cases proved by surgery and pathology or CT enhancement or heart ultrasound were analyzed. All cases were undergone radiography and CT scan. The contrast-enhanced CT scans were carried out in 51 cases.Results The study showed:un-invasive thymoma in 6 cases,invasive thymoma resulted in superior vena cava syndrome in 2 cases,intrathoracic thyroid tumors in 2 cases,ascending aorta or innominate artery dilatation in 14 cases,thoracic aortic aneurysm in 3 cases,pulmonary artery stenosis resulted in aortopulmonary dilatation in 1 cases,right sided aorta in 2 cases,lymphoma in 4 cases,the right mediastinal lung carcinoma in 16 cases,larvate focus of lung carcinoma in 2 cases,low esophageal carcinoma resulted in upper esophagectasia in 2 cases,foreign body of esophageal secondary upper mediastinal abscess in 2 cases. The cases proved by plain and contrast-enhanced CT scan,operation,pathology and heart ultrasound were 33,10,12 and 1 cases respectively. Conclusion CT scan is a effective method in the diagnosis of broadening of right upper mediastinum.

Objective To assess the feasibility and curative effects of laser surgery in treatment of early-stage glottic carcinoma under self-retaining laryngoscope with nasal endoscopic assistance.Methods Thirteen cases of glottic carcinoma(TisN0M0:2 cases;T1aN0M0:7 cases;T1bN0M0:3 cases;T2N0M0:1 case) extirpated with Nd:YAG laser or Diomed laser from June 1999 to March 2003 were retrospectively analyzed.Because of the poor glottic exposure with self-retaining laryngoscope under general anesthesia,various angular nasal endoscopes were used.The tumor was distinctly displayed on monitor screen with nasal endoscopic assistance and were completely removed through vapourization or removed with Nd:YAG laser or Diomed laser.Results The follow-up period was three to five years.Two patients were found to have local recurrence.One patient received treatment with Nd:YAG laser surgery under electronic laryngoscope with local and superfacial anesthesia,and the patient was followed up for three years without showing recurrence.The other patient underwent vertical hemilaryngectomy and was followed up for three and half years without showing recurrence.All 13 cases were followed up for three years,and all of them were alive.Three-year survival rate was 100%(13/13).When they were followed up for five years,one patient lost track(counted as death),therefore the five-year survival rate was 92.3%(12/13).Conclusions With the assistance of various angular nasal endoscope,the glottic tumors can be exposed distinctly under self-retaining laryngoscope and removed completely.Nd:YAG laser or Diomed laser is an effective and safe procedure with minimal invasion for the treatment of early-stage glottic carcinoma.The advantages of this technique include lower complication rate and high rate of preservation of laryngeal functions,avoidance of tracheotomy and the sequela of laryngeal fissure.