The Ehlers-Danlos syndrome(EDS)is a rare inherent connective tissue disorder.The prev-alence of EDS in the population is estimated at one out of ten thousand to one out of a hundred thousand.The vascular EDS(vEDS)are rare among the subtypes but are the worst in prognosis.The article reports a case of vEDS admitted to the hospital.The patient was a young man complaining of a sudden onset of aphasia in right hemiparalysis and severe left abdominal pain for unknown reasons.The diagnosis was made after the genetic testing.The patient suffered from vEDS.Then,the multi-disciplinary team(MDT)made a treatment plan tailored to this young patient.The complexity in classification and delusive presentations of the EDS make the correct diagnosis very challenging.This article hopes to report this case and to share the experiences to the bet-ter understanding of this disease.

To summarize the nursing experience of a patient with gastric cancer who developed cytokine release syndrome after using immune checkpoint inhibitors.Key points of nursing care:development of nursing assessment decisions with a holistic view to guide safe nursing care;taking into account the contradiction between bleeding and thrombosis and providing good care for upper gastrointestinal bleeding;implementing a nursing strategy focusing on cleaning and anti-infection for IV oral mucositis;implementing risk management for severe pulmonary lesions;providing good hormone medication care and discharge follow-up management.The patient was successfully discharged on 52nd day with a 3-month follow-up in good condition.

To summarize the nursing experience of a patient with multiple enterostomies after segmental small bowel resection for acute mesenteric vein embolism.Nursing points:early identification of necrosis of retained intestinal tubes and improvement of early warning care;active improvement of tissue perfusion for retained indeterminate necrotic intestinal tubes;relay enteral nutritional support for 4 stomas based on the collaborative care model;implementation of combined dressing changes for surgical incisions and stomas,control of incisional infections and peristoma dermatitis;attention to the psychological aspects of the patients and their families and provision of psychological support.The patient successfully underwent stoma retraction on the 42nd day after surgery,and no obvious short bowel syndrome occurred in the postoperative period.

To summarize the nursing experience of a patient with median arcuate ligament compression syndrome.Nursing points:to implement effective measures to relieve local compression and improve gastrointestinal symptoms;to establish a monitoring and evaluation plan focusing on abdominal pain to prevent serious adverse vascular events;to implement pre-rehabilitation care with psychological and nutritional support to reduce surgical risks;to actively provide postoperative gastroparesis care and discharge follow-up care.The patient was discharged from the hospital 22 days after surgery and was followed up for 6 months.His weight increased and his quality of life was high.

Objective To construct an intervention programme for breastfeeding among mothers of infants with congenital heart disease and to verify its effect.Methods Taking the capability-opportunity-motivation-behaviour model as the theoretical basis,the first draft of the intervention programme was formed through literature search and semi-structured interviews,and 8 experts in the relevant fields were selected to conduct expert validation and determine the content of the program.By the consecutive sampling method,infants and their mothers who attended the cardiothoracic disease clinic of a tertiary-level children's specialist hospital in Shanghai from 4 May to 24 June 2023,with a day-old age of≤14 d and a confirmed diagnosis of CHD,were selected as the study subjects.They were divided into an experimental group and a control group,with 16 cases in each group,by the method of randomized grouping by district.The experimental group received breastfeeding intervention for mothers of infants with CHD on the basis of routine care;the control group received routine care and basic disease education and breastfeeding counselling.At 1 and 3 months of the infants with CHD,the 2 groups were compared in terms of exclusive breastfeeding rate,daily human milk as a percentage of total feeds,weight for age Z-score and height for age Z-score.Results A total of 1 round of expert validation was conducted,with a valid questionnaire recovery rate of 100％and an expert authority coefficient of 0.94.The breastfeeding intervention programme for mothers of infants with CHD is a comprehensive approach that encompasses 3 key dimensions,including capability,opportunity,and motivation.The programme is designed to enhance mothers'ability to recognize feeding signals,master breastfeeding techniques,and maintain successful breastfeeding practices.It also addresses common breastfeeding challenges and provides effective solutions.Additionally,it includes strategies for breastmilk management and fosters family support for breastfeeding.The programme offers medical information support on breastfeeding and aims to elevate mothers'understanding of its benefits.It involves systematic monitoring and recording of breastfeeding volumes,as well as thorough assessments and guidance on infant growth and development,including but not limited to the 10 specific entries related to the infant's progress.The results of the generalized estimating equations and repeated measures analysis of variance revealed statistically significant between-group differences(P＜0.05)in the rates of exclusive breastfeeding,the proportion of daily human milk as a percentage of total feeds,and the weight-for-age(WAZ)and height-for-age(HAZ)z-scores of infants across the 2 groups at various time points.The results of simple effect analysis showed that the differences in exclusive breastfeeding rate,the proportion of daily human milk as a percentage of total feeds,WAZ and HAZ between the 2 groups before intervention were not statistically significant(P＞0.05).At the age of 3 months of the infants after the intervention,the rate of exclusive breastfeeding in the experimental group was higher than that in the control group,and the difference was statistically significant(P=0.003).The proportion of daily human milk as a percentage of total feeds for infants in the intervention group at 1 and 3 months of age was higher than that in the control group,with a statistically significant difference(P＜0.05).At 3 months of age,WAZ of the experimental group was higher than that of the control group,and the difference was statistically significant(P=0.037);HAZ of the experimental group was higher than that of the control group at 1 and 3 months of age after intervention,and the difference was statistically significant(P＜0.05).Conclusion The breastfeeding intervention programme for mothers of infants with CHD,constructed on the basis of the COM-B model in this study,was comprehensive,feasible and acceptable.Implementation of the programme has potential positive effects on increasing exclusive breastfeeding rates and the daily human milk as a percentage of total feeds,and may also have a positive impact on WAZ and HAZ of infants.

Objective:To investigate the short-term therapeutic effect and long-term survival of multiple myeloma patients with extramedullary disease (EMD) in the new drug era.Methods:The data of 74 patients with multiple myeloma diagnosed and treated in Anhui Wanbei Coal and Electricity Group General Hospital from January 2015 to January 2020 were retrospectively analyzed, including 17 patients with soft tissue infiltration (EM-S), 9 patients with bone infiltration (EM-B), and 48 patients without EMD (No-EMD). The short-term efficacy, the 4-year progression-free survival (PFS) rate and overall survival (OS) rate, and their influencing factors in three groups of patients after receiving bortezomib regimen were analyzed.Results:After 3-4 courses of early induction therapy of bortezomib regimen, the overall response rate of patients in the EM-S group was lower than that in the No-EMD group and the EM-B group [58.8% (10/17) vs. 85.4% (41/48), 100.0% (9/9)], and the differences were statistically significant ( χ2 = 13.7, P = 0.036; χ2 = 26.5, P = 0.003), while the difference between No-EMD group and EM-B group was not statistically significant ( χ2 = 12.7, P = 0.211). Survival analysis showed that the 4-year PFS rate of No-EMD group was higher than that of the EM-S group and EM-B group (41.0% vs. 7.6%, 0), and the differences were statistically significant ( χ2 = 10.835, P < 0.01; χ2 = 8.276, P = 0.004). Meanwhile, the 4-year OS rate of EM-S group was lower than that of the No-EMD group and EM-B group (16.5% vs. 54.3%, 59.3%), and the differences were statistically significant ( χ2 = 9.146, P = 0.002; χ2 = 4.066, P = 0.044). Conclusion:The early treatment effect of bortezomib regimen, PFS and OS in multiple myeloma patients with EM-S are poor, while the EM-B has no effect on OS.

Hospitals need to explore based on their own specifics how to establish a modern hospital governance system and how a lean hospital administration level be achieved. This paper introduced the practice and exploration of modern hospital governance system construction in Beijing Tsinghua Changgung Hospital as follows. The hospital is run by a board of administration featuring co-administration by both Beijing Municipal Hospital Administration Center and Tsinghua University; A hospital charter is developed and agreed upon to regulate decision-making, day-to-day operations and supervision; The hospital president′s responsibility is under the leadership of the hospital′s Communist Party Committee; Benefiting from best practices of Taiwan Changgung′s medical system, the hospital′s specialized medical teams and professional administrative teams operate in cooperative governance, and under professional management model and a lean operation management system. The practice and exploration of Beijing Tsinghua Changgung Hospital can serve as references for building and perfecting a modern hospital governance system.

Objective:To assess the utility of contrast-enhanced ultrasound (CEUS) targeted biopsy (TB) for clinically significant prostate cancer (PCa) detection.Methods:A total of 983 consecutive patients scheduled for prostate biopsy from October 2015 to March 2019 in Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine were enrolled in this retrospective study. All patients had suspicious lesions on CEUS, defined as increased focal contrast enhancement, rapid contrast enhancement and low enhancement lesions with ill-defined borders. Suspicious lesions on CEUS were sampled in addition with standard 12-core systematic biopsy(SB). Clinically significant PCa was defined using Epstein criteria. The clinically significant PCa detection rate by CEUS-TB and combined biopsy was evaluated in comparison with SB.Results:In 502 of the 983 patients, the diagnosis of PCa was histologically confirmed, including 445 patients with clinically significant PCa and 57 patients with clinically insignificant PCa. The clinically significant PCa by CEUS-TB and combined biopsy were 41.9% (412/983) and 45.3% (445/983) respectively, which was significantly higher than SB (36.8%, 362/983)(all P<0.001). CEUS-TB resulted in additional 83 cases of clinically significant PCa, including 61 patients missed by SB and 22 patients under-graded by SB. Conclusions:CEUS is helpful in the detection of PCa lesions. Combined CEUS-TB and SB can improve the clinically significant PCa detection rate.

Objective:To investigate the clinicopathologic features and prognosis of breast mucosa associated lymphoid tissue(MALT) lymphoma.Methods:Clinical data of 8 breast MALT lymphoma patients were retrospectively analyzed at Tianjin Medical University Cancer Institute and Hospital from Jan 1, 2004 to Jan 31, 2017.Results:All were females, median age was 50 years. Tumors were located in the left breast in 3 cases while 5 cases in the right. All tumors were single in the outer-upper 1/4 quadrant of the breast. By Ann Arbor clinical stageⅠ, Ⅱ, Ⅲ and Ⅳ were 3, 2, 1 and 2, respectively. None of patients had B symptoms. 7 cases underwent surgery.All of the 8 cases received chemotherapy, 6 cases had complete response, one partial response, one with stable in efficacy evaluation. The follow-up time was 2-154 months. The 1, 3, 5-year overall survival rate were 100.0%, 50.0%, 50.0%, median survival time was 144.00 months. The 1-year progress free survival rate was 78.0% and median progress free survival time 28.29 months.Conclusion:Breast MALT is extremely rare without specific clinical features. Almost all are single tumors without B symptom. It′s sensitive to chemotherapy and the patients have fair prognosis.

Objective: To investigate the clinical characteristics, therapy modality and prognosis of primary breast diffuse large B-cell lymphoma(PB-DLBCL). Methods: A total of 68 patients with PB-DLBCL treated in Tianjin Medical University Cancer Institute and Hospital were enrolled between January 1, 2004 and January 31, 2017. Clinicopathological data were retrospectively analyzed. 67 patients were female and only one male. The median age was 56 years old. 46 patients had Ann Arbor clinical stageⅠ~Ⅱ disease, and the other 22 were stage Ⅲ~Ⅳ. The patients with and without B symptom were 11 and 57, respectively. Kaplan-Meier method was used for univariate analysis to calculate the 5-year overall survival (OS) rate and 5-year progress-free survival (PFS) rate, compared using the log rank test. Cox regression analysis was used for multivariate analysis. Results: The 1, 3, 5-year OS rate were 84.0%, 78.0% and 73.0%, and 1, 3, 5-year PFS rate were 80.0%, 71.0% and 51.0%, respectively. Univariate analysis indicated that eastern cooperative oncology group (ECOG) score, Ann Arbor clinical stage, international prognostic index (IPI) score, risk stratification, B symptom, β2-microglobulin(β2-MG) level, size of the tumor and cycles of chemotherapy were prognostic factors for OS (all P<0.05), and Ann Arbor clinical stage, IPI score, risk stratification and B symptom were prognostic factors for PFS (all P<0.05). Multivariate analysis indicated that Ann Arbor clinical stage was independent prognostic factor for OS(P=0.029) and B symptom was independent prognostic factor for PFS(P=0.028). Conclusions Prognosis of PB-DLBCL was relatively good. Ann Arbor clinical stage and B symptom were independent prognostic factors for OS and PFS, respectively.