Myasthenia gravis is an autoimmune disorder characterized by impaired neuromuscular transmission. Thymectomy is one of the therapeutic options for acetylcholine receptor antibody-positive myasthenia gravis patients. The quality of perioperative care is directly associated with surgical safety and patient outcomes. However, there is currently a lack of specialized nursing consensus or guidelines specifically addressing the care of these patients domestically or internationally. To promote the standardization and normalization of perioperative nursing care for myasthenia gravis patients undergoing thymectomy and to ensure treatment efficacy, a panel of 57 experts from relevant fields was convened. Based on evidence-based medicine and clinical practice experience, discussions were held on various aspects including condition assessment, nutritional support, medication management, and airway care, resulting in a consensus with 18 final recommendations by using the Delphi method through two rounds of expert consultation. This consensus aims to provide a scientific reference for the perioperative nursing care of myasthenia gravis patients undergoing thymectomy.

ObjectiveTo adapt the Stanford Expectations of Treatment Scale（SETS） into Chinese（C-SETS） and test the feasibility， validity and reliability of its application in patients with diarrhea-predominant irritable bowel syndrome（IBS-D） with liver-constraint and spleen-deficiency syndrome treated with traditional Chinese medicine（TCM）. MethodsWe obtained authorisation from the developer of the SETS， and followed the principle of "two-way translation" to translate the SETS by literal translation and back translation to form the C-SETS. Ninety-six IBS-D patients with liver-constraint and spleen-deficiency syndrome were enrolled as respondents and filled out C-SETS before receiving treatment； the feasibility was assessed by the recall rate， completion rate and the duration of filling out the scale； the reliability was assessed by Cronbach's α； the structural validity was assessed by exploratory and confirmatory factor analysis， and the content validity was assessed by correlation analysis. ResultsThe C-SETS consists of 10 items， with the 1st， 3rd， and 5th rating items constituting the Positive Expectations subscale， and the 2nd， 4th， and 6th rating items constituting the Negative Expectations subscale， each of which is rated on a 7-point Likert Scale. The recall of C-SETS was 100%（96/96）， the completion rate was 89.58%（86/96）； Cronbach's α for the Positive and Negative Treatment Expectations subscales were 0.845 and 0.854， respectively； exploratory factor analysis showed that the coefficient of commonality for all six entries was larger than 0.4， and that the six entries could be used by both factors to explain 77.092% of the total variance； validation factor analysis showed that the goodness-of-fit index， comparative fit index， root mean square of approximation error， canonical fit coefficient， and chi-square degrees of freedom ratio took the values of 0.943， 1.003， 0， 0.943， and 0.626， respectively； and the results of Spearman's analysis suggested that the C-SETS had good content validity. ConclusionThe C-SETS has well feasibility， reliability， and validity， which initially proves that it can be used as a tool to assess the treatment expectation of patients with IBS-D with liver-constraint and spleen-deficiency syndrome before receiving TCM treatment.

Objective To systematically evaluate the efficacy of β-blocker in pediatric patients with congestive heart failure and congenital heart disease,and to provide evidence for clinician.Methods Before-and-after self-control study and randomized controlled trials were retrieved from PubMed,EMbase,the Cochrane Library,CNKI,WanFang,VIP databases,and the search time was from the establishment of the database to October 31,2023.All outcomes included left ventricular ejection fraction(LVEF),left ventricular fraction shortening,LVFS),left ventricular end-diastolic dimension(LVDD),Left ventricular end-systolic dimension(LVSD),N-terminal proB brain natriuretic peptide(NT-proBNP),heart rate,blood pressure and cardiac function improvement.Results A total of 20 trials involving 1 068 children with heart failure(dilated cardiomyopathy and endocardial fibroelastosis were included.Meta-analysis results showed that the addition of β-blockers(metoprolol succinate,bisoprolol and carvedilol)on the basis of conventional drug therapy for heart failure had significant effects on LVEF[MD=13.06,95%CI(11.67,14.45),P<0.001],LVFS[MD=6.96,95%CI(6.54,7.37),P<0.001],LVDD[MD=-6.43,95%CI(-7.58,-5.28),P<0.001]and LVSD[MD=-8.30,95%CI(-8.83,-7.76),P<0.001]were significantly improved.In addition,blood pressure,heart rate,NT-proBNP and cardiac function could also be improved.Conclusion The combination regimen of β-blockers on the basis of conventional drug therapy for heart failure can improve cardiac function and symptoms of heart failure in children with congestive heart failure.Therefore,it is recommended that β-blockers should be actively used in the conventional treatment regimen for children with congestive heart failure.

Objective To investigate the prognostic value of serum bispecific phosphatase 1(DUSP1)ex-pression level in patients with acute pulmonary thromboembolism(APTE).Methods A total of 112 patients with APTE admitted to the hospital from March 2020 to July 2022 were enrolled as the observation group,and 50 healthy individuals who underwent physical examinations in the hospital during the same period were en-rolled as the control group.The APTE patients were followed up for 6 months after treatment,and were grouped into a good prognosis group(90 cases)and a poor prognosis group(22 cases)based on their progno-sis.The serum DUSP1 relative expression level and pulmonary embolism severity index(PESI)score were compared among the groups before admission.Spearman correlation was applied to analyze the relationship between serum DUSP1 relative expression level and PESI score.Receiver operating characteristic(ROC)curve was applied to analyze the predictive value of serum DUSP1 relative expression level and PESI score on the prognosis of APTE patients.Results Compared with the control group,the serum DUSP1 relative expres-sion level in the observation group was increased(P<0.05).There were statistically significant differences in serum DUSP1 relative expression level and PESI score among patients with different risk levels(P<0.05),the serum DUSP1 relative expression level and PESI score in high-risk APTE patients were higher than those in medium-risk patients(P<0.05),and those in medium-risk patients were higher than those in low-risk pa-tients(P<0.05).Spearman correlation analysis showed that serum DUSP1 relative expression level was posi-tively correlated with PESI score(r=0.561,P<0.05).ROC curve results showed that the area under the curve(AUC)of DUSP1 and PESI score alone for predicting the poor prognosis in APTE patients was 0.789 and 0.867,with sensitivity of 65.8%and 86.8%,specificity of 44.2%and 67.2%,respectively.The AUC of the combination of the two for predicting the poor prognosis in APTE patients was 0.952,with sensitivity and specificity of 92.1%and 75.6%,respectively.Conclusion The serum DUSP1 relative expression level in APTE patients is elevated,and with the aggravation of the disease,the serum DUSP1 relative expression level gradually increases.DUSP1 is an effective indicator for predicting poor prognosis in APTE patients.

Objective:To analyze the gene mutation type and clinical phenotype of patients with PRRT2 mutation, and explore their relations with prognosis. Methods:A total of 18 patients with PRRT2 gene mutation (1 patient with novel mutation in PRRT2 gene, and 17 probands in 17 families with PRRT2 gene mutation) were enrolled in Department of Neurology, First Affiliated Hospital of Air Force Medical University from January 2018 to July 2023. Serum of the patients was collected for whole exon sequencing, and mutation sites and types of PRRT2 gene were analyzed. SWISS-MODEL website was used to predict the changes in protein structure caused by PRRT2 gene mutation. The relations of gene mutation type and clinical phenotype with prognosis of these patients were analyzed. Results:(1) All 18 patients with PRRT2 gene mutation were heterozygous mutation, including 12 frameshift mutations, 5 missense mutations, and 1 integer mutation. The clinical phenotype included benign familial infantile epilepsy (BFIE) in 5 patients, epilepsy in 6 patients, exercise-induced paroxysmal kinesigenic dyskinesia (PKD) in 5 patients, and infantile convulsion and choreoathetosis (ICCA) in 2 patients. A total of 8 mutation sites were found in 18 patients with PRRT2 gene mutation, of which 3 mutation sites have been reported, and 5 mutation sites have not been reported, including c.647(exon2)C>A, c.647(exon2)C>G, c.170(exon2)delC, c.981(exon3)C>G, and lossl(EXON: 2)(all). (2) Eighteen patients mainly accepted oxcarbazepine, levetiracetam, and sodium valproate in combination or monotherapy. Among them, 5 BFIE patients, 2 ICCA patients and 3 epilepsy patients were seizure-free after treatment. PKD patients did not respond well to oxcarbazepine. (3) Three frameshift mutations (mutation sites: c.649 [exon2]_c.650 [exon2] insC, c.640 [exon2]_c.641 [exon2] insC, and c.170 [exon2] delC) led to premature termination of protein translation, resulting in significant changes in protein structure. Four missense mutations (mutation sites: c.640[exo2]G>C, c.647[exon2]C>A, c.647[exon2]C>G, and c.981[exon3]C>G) had little effect on protein structure changes. No relation was found between changes of protein structure caused by different mutation types and prognosis. Conclusion:PRRT2 gene mutation patients with clinical phenotypes of BFIE and ICCA have good prognosis, but the mutation type is not related with the prognosis of patients.

Objective:To understand the current status of financial toxicity in patients with heart failure and the factors affecting it, and to provide ideas for making personalized and informed decisions.Methods:Using a scoping review methodological framework, PubMed, Embase, Cochrane Library, Web of Science, China National Knowledge Infrastructure, VIP, Wanfang, and SinoMed Databases were searched and screened for relevant literature on financial toxicity in patients with heart failure, with a timeframe of January 1, 2014-October 31, 2023, respectively. Relevant literature was identified based on inclusion and exclusion criteria, and data were extracted, collected, summarized, and the findings were reported.Results:Ten literatures that met the inclusion and exclusion criteria were identified. The results showed 5 cross-sectional surveys, 2 qualitative studies, and 1 each of reviews, mixed studies and commentaries. Heart failure patients generally faced high levels of financial toxicity, the incidence and severity of patient financial toxicity varied somewhat between study outcomes. Factors influencing financial toxicity in heart failure patients included age, education level, family income level, discussion of medical costs with physicians, type of insurance the patient had, and occupational status.Conclusions:In the future, we can develop and apply a specialized assessment tool for financial toxicity in heart failure patients in China, further explore the factors affecting financial toxicity in heart failure patients, and formulate personalized treatment plans and financial support strategies for patients according to the influencing factors, so as to reduce the impact of financial toxicity on heart failure patients.

Gray matter heterotopia is a neurodevelopmental disorder characterized by abnormal distribution of gray matter in the brain cortex,and epilepsy is the most common clinical symptom of this disease.This article summarizes the etiology,pathogenesis,clinical manifestations,examination,and treatment of gray matter heterotopia and epilepsy,so as to improve the understanding of the disease.

Objective To explore the efficacy and safety of medical thoracoscopic bulla volume reduction for the treatment of chronic obstructive pulmonary disease(COPD)combined with giant emphysematous bullae(GEB).Methods A total of 66 patients with COPD combined with GEB were enrolled in the study.All the subjects received treatment at Zhengzhou Central Hospital affiliated with Zhengzhou University between March 2021 and December 2022.The subjects were divided into two groups,a medical thoracoscope group consisting of 30 cases treated with medical thoracoscopic bulla volume reduction and a surgical thoracoscope group consisting of 36 cases treated by video-assisted thoracoscopic surgery.All patients were followed up before discharge and 3 months and 6 months after discharge.The preoperative and postoperative levels of the pulmonary function,6-minute walk distance(6MWD),and St.George's Respiratory Questionnaire(SGRQ)scores and differences in postoperative complications were compared between the two groups.The operative duration,postoperative length-of-stay,and surgical costs and hospitalization bills,and the maximum visual analog scale(VAS)pain scores at 24 h after the procedure were assessed.Results The baseline data of the two groups were comparable,showing no statistically significant difference.The forced expiratory volume in 1 second(FEV1)6 months after the procedures improved in both the medical thoracoscopy group([0.78±0.29]L vs.[1.02±0.31]L,P＜0.001)and the surgical thoracoscopy group([0.80±0.21]L vs.[1.03±0.23]L,P＜0.001)compared to that before the procedures.Improvements to a certain degree in 6MWT and SGRQ scores were also observed in the two groups at 3 months and 6 months after the procedures(P＜0.05).In addition,no statistically significant difference in these indexes was observed during the follow-up period of the patients in the two groups.There was no significant difference in operating time between the two groups.The medical thoracoscopy group had shorter postoperative length-of-stay([7.3±2.6]d)and 24-hour postoperative VAS pain scores(3.0[2.0,3.3])than the surgical thoracoscopic group did([10.4±4.3]d and 4.5[3.0,5.0],respectively),with the differences being statistically significant(P＜0.05).Surgical cost and total hospitalization bills were lower in the medical thoracoscopy group than those in the surgical thoracoscopy group(P＜0.05).The complication rate in the medical thoracoscopy group was lower than that in the surgical thoracoscopy group(46.7%vs.52.8%),but the difference was not statistically significant.Conclusion Medical thoracoscopic reduction of bulla volume can significantly improve the pulmonary function,quality of life,and exercise tolerance of patients with COPD combined with GEB,and it can reduce postoperative short-term pain and shorten postoperative length-of-stay.The procedure has the advantages of minimal invasiveness,quick recovery,and low costs.Hence extensive clinical application is warranted.

【Objective】 To investigate the incidence of clinical massive blood transfusion in hospitals, the proportion of departments conducted massive blood transfusion and the current situation of component transfusion, so as to provide a theoretical basis for medical decision-making and further research on massive blood transfusion. 【Methods】 The basic clinical data and transfusion of blood components were retrospectively collected from 489 patients (514 occasions) who received massive blood transfusion at Sun Yat-sen Memorial Hospital of Sun Yat-sen University from Jan. 1 2014 to Dec. 31 2018. 【Results】 The incidence of massive blood transfusion during the 5-year period was 1.2/1 000 inpatients (95%CI: 1.1-1.3), and the 30-day all-cause mortality was 21.88%; in the departments where massive blood transfusion occurred, the mortality rate was the highest in the trauma emergency department (60%), followed by intensive care unit (56.25%) and other surgery department (46.67%), while there was no death in the obstetric department. All patients received red blood cells [median 14 U (11.5-19.13)] and plasma [median 1 600 mL (1 200-2 200)], of which 47% received platelet [median 0 U (0-10)] and 32.68% received cryoprecipitate [0 U (0-10)]. The results of logistics regression analysis of all-cause mortality risk showed that compared with the youth group, the risk of all-cause death at 30 days of elderly patients over 65 years old (65 < age ≤79 years old: OR=2.471, 95%CI=[1.044, 5.847], P<0.05; patients with age > 80 years old: OR=7.563, 95%CI=[1.587, 36.049], P<0.05) and 24-hour RBC infusion volume greater than 18 U (18≤RBC<27: OR=2.948 95%CI=[1.592, 5.462], P<0.05; RBC≥28: OR=3.992, 95%CI=[1.178, 13.536], P<0.05) was higher. 【Conclusion】 A dynamic definition should be included in massive transfusion studies. If only a 24-hour RBC infusion volume ≥18 U was used as the mass transfusion definition, about 68% of cases would be lost. The mortality rate of patients with massive blood transfusion was higher, and the incidence of massive blood transfusion was higher in the departments of cardiac surgery, general surgery and orthopedics surgery. More attention should be paid to the increasing number of female patients with massive blood transfusion. In addition, the risk of 30-day all-cause death was highest in elderly patients over 65 years of age and those with a 24-hour erythrocyte transfusion level of ≥18 U.

Objective To observe the value of quantitative CT(QCT)for predicting the efficacy of medical thoracoscopic giant emphysematous bullae(GEB)volume reduction.Methods Data of chest QCT and pulmonary function tests before and 6 months after medical thoracoscopic volume reduction in 26 chronic obstructive pulmonary disease(COPD)patients complicated with GEB who underwent medical thoracoscopic GEB volume reduction were retrospectively analyzed.According to the improvement rate of lung function(forced expiratory volume in one second[FEV1])6 months after treatment compared with that before treatment,the patients were divided into significant improvement group(FEV1 improvement rate≥15％,n=20)and non-significant improvement group(FEV1 improvement rate＜15％,n=6).QCT parameters before treatment were compared between groups,and the correlations of QCT parameters being significantly different between groups with FEV1 improvement 6 months after treatment were analyzed.Then receiver operating characteristic(ROC)curves were drawn to evaluate the efficacy of the above parameters for predicting the curative effect of medical thoracoscopic volume reduction of GEB.Results Significant differences of preoperative target lung lobe volume,total lung volume and bullae volume were found between groups(all P＜0.05).Preoperative target lung lobe volume,total lung volume,target lobar bullae volume and total lung bullae volume were positively correlated with FEV1 improvement(r/rs=0.600,0.470,0.699,0.523,all P＜0.05),with the area under the curve(AUC)for predicting FEV1 improvement of 0.817,0.817,0.892 and 0.833(all P＜0.05),respectively.Conclusion QCT could effectively predict the efficacy of medical thoracoscopic GEB volume reduction.