Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease caused by abnormal expression of glycosylphosphatidylinositol (GPI) on the cell membrane due to mutations in the phosphatidylinositol glycan class A(PIGA) gene. It is commonly characterized by intravascular hemolysis, repeated thrombosis, and bone marrow failure, as well as multiple systemic involvement symptoms such as renal dysfunction, pulmonary hypertension, swallowing difficulties, chest pain, abdominal pain, and erectile dysfunction. Due to the rarity of PNH and its strong heterogeneity in clinical manifestations, multidisciplinary collaboration is often required for diagnosis and treatment. Peking Union Medical College Hospital, relying on the rare disease diagnosis and treatment platform, has invited multidisciplinary clinical experts to form a unified opinion on the diagnosis and treatment of PNH, and formulated the Expert Consensus of Multidisciplinary Diagnosis and Treatment for Paroxysmal Nocturnal Hemoglobinuria (2024), with the hope of promoting the standardization of PNH diagnosis and treatment.

Objective    To explore the safety and feasibility of uni-portal video-assisted thoracic surgery (VATS) for the treatment of bronchopulmonary sequestration (BPS). Methods    The clinical data of BPS patients with surgical resection in Shanghai Pulmonary Hospital from February 2010 to June 2021 were reviewed. The patients were divided into a VATS group and a thoracotomy group according to the operation method. The operation time, intraoperative blood loss, hospital stay and postoperative complication rate were compared between the two groups. The VATS group was subdivided into a uni-portal VATS group and a multi-portal VATS group for subgroup analysis. Results    Finally 131 patients were enrolled, including 62 males and 69 females with an average age of 39.3±13.2 years. There were 103 patients in the VATS group and 28 patients in the thoracotomy group. A total of 104 patients were diagnosed with left lower BPS, 26 with right lower BPS and 1 with bilateral lower BPS. The main symptom was cough (88 patients, 67.2%). There were 119 patients diagnosed by thoracic enhanced CT before operation. Compared with the thoracotomy group, the operation time was not statistically different (P=0.717), but the blood loss was less, the rate of postoperative complication was lower and hospital stay was shorter in the VATS group (P<0.05). The rate of conversion to open surgery in the uni-portal VATS group and multi-portal VATS group was 11.8% and 13.5%, respectively. Meanwhile, patients in the uni-portal VATS group had shorter operation time and postoperative hospital stay, less blood loss and lower postoperative complication rate than those in the multi-portal VATS group (P<0.05). Conclusion     In order to improve the rate of diagnosis, the lung enhanced CT scan should be selected as an optimal noninvasive method in adult suspected patients (especially those with solid cystic and solid lesions in the lower lobe). Uni-portal VATS is a safe and feasible method for BPS which can be widely promoted.

Objective To compare and analyze the results of hysterosalpingography(HSG)in primary infertility patients who received ultra-liquefied ethiodized poppyseed oil-based contrast medium or iodized oil-based contrast medium.Methods A total of 212 patients with primary infertility who received HSG examination were enrolled,of which 96 patients were in the ultra-liquefied ethiodized pop-pyseed oil-based contrast medium group,while 116 patients were in the iodized oil-based contrast medium group.The visual analogue scale(VAS)score of pain during HSG,adverse reactions(allergic reaction of contrast medium,contrast medium reflux,etc.),24 h-peri-toneal distribution and natural pregnancy rate within 1 year after HSG were analyzed between the two groups.Results The VAS score[(1.62±0.92)points]and the incidence of contrast medium reflux(5.2％)in the ultra-liquefied ethiodized poppyseed oil-based con-trast medium group were lower than those in the iodized oil-based contrast medium group[(3.2±1.42)points and 13.8％],respectively and the differences between the two groups were statistically significant(P＜0.05).In the 24 h-peritoneal distribution,the diagnosis rate of mild pelvic adhesion in the ultra-liquefied ethiodized poppyseed oil-based contrast medium group(26％)was lower than that in the iodized oil-based contrast medium group(39.7％),and the difference was statistically significant(P=0.041).The natural pregnancy rate of the two groups within 1 year was significantly improved,but there was no significant difference between the two groups(P＞0.05).Conclusion Ultra-liquefied ethiodized poppyseed oil-based contrast medium has less pain,lower incidence adverse reactions and better peritoneal distribution,and improve the natural pregnancy rate in primary infertility patients undergoing HSG.It is a high priority contrast medium for HSG.

This article summarizes the research progress on situation awareness in nursing through literature review. Currently, research on situation awareness in nursing is mostly focused on foreign countries, and there is relatively little attention and research on it in China. This article reviews the relevant theories of situation awareness in nursing and its application status in medical settings, related factors, and improvement strategies, in order to provide theoretical references for future research on situation awareness in the nursing and even medical industries.

Objective:To analyze and elaborate on the concept and connotation of elder self-neglect, so as to provide reference for nursing practice.Methods:Literature on elder self-neglect was systematically searched on China National Knowledge Infrastructure, WanFang Data, China Biology Medicine disc, PubMed, Web of Science, and CINAHL. The search period was from database establishment to July 5, 2023. The literature was analyzed using Rodgers' evolutionary concept analysis as the theoretical framework.Results:A total of 63 articles were included. Elder self-neglect consisted of four defining attributes of disorder, danger, concealment, and closure. Antecedents contained physiological, psychological, social environment, and other factors. Consequences included the impact on the elderly themselves as well as the impact on others and the society.Conclusions:Elder self-neglect has a profound impact on individuals and society. By analyzing the concept of elder self-neglect and clarifying its conceptual attributes, researchers can conduct in-depth and comprehensive research on it, promoting the development of healthy aging.

Objective:To analyze the research progress of family psychological education in schizophrenia population, in order to understand the elements and current situation of family psychological education application, and provide guidance for future research in this field.Methods:According to the basic idea of scoping review, research on family psychological education for schizophrenia patients was systematically searched in PubMed, Embase, CINAHL, Web of Science, Cochrane Library, China National Knowledge Infrastructure, WanFang Data, and China Biology Medicine. The search period was from database establishment to March 15, 2023.Results:A total of 15 articles were included, including 11 completely randomized controlled trials, 2 semi-randomized controlled trials, and 2 follow-up studies. The content consisted of the evaluation and intervention forms of family psychological education, and the development and implementation of specific intervention plans.Conclusions:There is limited research on family psychological education for patients with schizophrenia, and the implementation efforts around the world are insufficient. In the future, relevant research can be conducted to develop cost-effective education programs that are in line with China's culture and environment, so that schizophrenia patients and their families can receive evidence-based treatment to improve patient and family outcomes.

Objective:To translate the English version of the Beliefs Toward Mental Illness Scale (BTMI) into Chinese and test the reliability and validity of the Chinese version in college students.Methods:According to the Beaton cross-cultural adaptation guideline, the Chinese version of BTMI was formed. From April to May 2023, a questionnaire survey was conducted on 915 university students from 171 universities in 26 provinces, autonomous regions, and municipalities across China using convenience sampling, to analyze the reliability and validity of the scale.Results:A total of 915 questionnaires were distributed, and 814 valid questionnaires were collected, with an effective recovery rate of 88.96% (814/915). The Chinese version of BTMI had a total of 21 items. The Cronbach's α coefficient of the total scale was 0.922, and the Cronbach's α coefficients of each dimension were 0.745 to 0.921. The half reliability coefficient of the total scale was 0.797, and the retest reliability coefficient of the total scale was 0.903 ( P<0.01). Exploratory factor analysis extracted four common factors, namely social function, risk, incurability, and awkwardness, with a cumulative variance contribution rate of 62.29%. Confirmatory factor analysis showed that all important fitting indicators were greater than 0.900, indicating a good overall model fit. Conclusions:The Chinese version of BTMI has good reliability and validity, which can be used to evaluate the beliefs of Chinese university students about mental illness.

Objective     To summarize the progress and trend on clinical drug trials of esophageal squamous cell carcinoma in China. Methods     Based on the clinical drug trial registration and information disclosure platform and the drug data query system of the National Medical Products Administration, the characteristics of clinical trials, investigational drugs and listed drugs of esophageal squamous cell carcinoma in China from 2012 to 2021 were analyzed. Results     From 2012 to 2021, a total of 49 clinical drug trials of esophageal squamous cell carcinoma were registered in China, accounting for 1.6% of all clinical trials of anticancer drugs. Among them, there were 39 (79.6%) trials initiated by domestic pharmaceutical enterprises, 6 (12.2%) for adjuvant and neoadjuvant treatment, and 9 (18.4%) for local treatment. There were differences in the treatment line distribution between global and domestic enterprise-initiated trials (P=0.032). The above trials covered 29 investigational drugs, including 23 (79.3%) targeted drugs, most of which targeted programmed death-1, programmed death-ligand 1 and epidermal growth factor receptor. From 2012 to 2021, there were 2 drugs for esophageal squamous cell carcinoma listed in China, both of which were approved for the first-line and second-line treatment. Conclusion     Great achievements have been made in the clinical development of esophageal squamous cell carcinoma drugs in China. It is suggested that domestic enterprises increase the investment of esophageal squamous cell carcinoma, pay attention to adjuvant and local treatment, explore novel targets and drug categories, and focus on the details of pivotal trials.

Objective:To explore the rules of prescription and medication of Traditional Chinese Medicine (TCM) syndrome differentiation treatment for insomnia.Methods:We screened the medical records related to insomnia from the ancient medical cases, modern medical cases, famous medical cases and shared medical record database, and then we put data into the Ancient and Modern Medical Case Cloud Platform (V 1.5.7). The platform software Data mining was applied and performed by frequency analysis, correlation analysis, and drug pair analysis. Results:This study collected 664 cases related to insomnia, like difficulty in falling sleep, early waking up, easy to wake up, dreaminess. The top 5 syndromes included internal phlegm-heat, imbalance between heart-yang and kidney-yin, liver stagnation transforming into fire, heart and spleen deficiency, restless mind. There were 664 prescriptions, 414 TCM medications, where the top 5 medications included Glycyrrhizae Radix et Rhizoma, Poria, Rehmanniae Radix, Ziziphi Spinosae Semen, Angelicae Sinensis Radix. The effect of high-frequency medications were nourishing the heart and calming the mind, clearing heat and dissipating phlegm, soothing the liver and relieving depression, and nourishing yin and yang. The top five drug-combinations included Ostreae Concha- Longgu, Poria- Glycyrrhizae Radix et Rhizoma, Citri Reticulatae Pericarpium- Poria, Angelicae Sinensis Radix- Rehmanniae Radix, Bupleuri Radix- Glycyrrhizae Radix et Rhizoma. Conclusions:TCM syndrome differentiation for insomnia focus on the heart. The medications are mainly to nourish the heart and calm the mind, supplemented by clearing heat, replenishing heart and spleen, soothing the liver and relieving stagnation, nourishing yin and blood for the symptoms relief, with treatment priciple of seeking both temporary and permanent solutions.

OBJECTIVE：To study the pr otective effect of schisandrin A （SA）on CCl 4-induced liver fibrosis model mice and its mechanism. METHODS ：Mice were randomly divided into blank control group ，model group ，silymarin group （positive control，100 mg/kg），SA low-dose and high-dose groups （20，40 mg/kg），with 10 mice in each group. Except for blank control group，other groups were given CCl 4 subcutaneously to induce liver fibrosis model. After successful modeling ，administration groups were given relevant medicine intragastrically ，once a day ，for consecutive 6 weeks；blank control group and model group were given constant volume of 0.5％sodium carboxymethyl cellulose solution intragastrically by the same way. HE staining was used to observe the pathological changes of liver tissue in mice. UV spectrophotometry and ELISA assay were adopted to detect the serum levels of liver injury indexes （ALT and AST ）and the contents of inflammatory factors （TNF-α，IL-1β，IL-6）. Western blotting assay was used to detect the expression of NOD like receptor protein 3（NLRP3）/NF-κB and TGF-β/Smad signaling pathway protein. RESULTS ：Compared with blank control group ，obvious pathological changes of liver fibrosis were observed in model group. The serum levels of liver injury indexes and contents of inflammatory factors were significantly increased （P＜0.01）. The expression of NLRP 3，apoptosis associated spot-like protein ，Caspase-1 and IL- 1β，TGF-β1 and ratios ofp-NF-κB p65/NF-κB p65，p-IκBα/IκBα，p-Samd3/Smad3 were increased significantly （P＜0.01）. Compared with model group ，SA could significantly relieve hepatic fibrosis in mice ，reduce serum levels of liver injury indexes and contents of inflammatory factors ，as well as the expression of NLRP 3/NF-κB and TGF-β/Smad signaling pathway protein and phosphorylation level（P＜0.01）. CONCLUSIONS ： SA can effectively relieve liver injury and inflammation of CCl 4-induced hepatic fibrosis model mice ，which may be through the regulation of NLRP 3/NF-κB and TGF-β/Smad3 signaling pathways ，thus inhibiting the process of liver fibrosis.