Standardizing the emergency assessment and management process for patients with implantable continuous-flow left ventricular assist device (LVAD) in emergency and intensive care settings is of great significance for reducing delays in diagnosis and treatment, lowering the mortality associated with LVAD-related emergencies, and improving overall prognosis and long-term survival. To this end, a multidisciplinary expert committee was convened to develop this consensus, integrating international evidence-based findings with clinical practice experience in China, with an emphasis on highlighting the characteristics of domestically manufactured devices. The aim is to establish an actionable standardized emergency management protocol to enhance clinical identification and response efficiency, reduce the risk of LVAD-related emergencies, and improve patient outcomes. Using a modified Delphi method, this consensus proposes a structured decision-making pathway that integrates an "ABC" rapid assessment with parallel device troubleshooting, prioritizing the use of point-of-care echocardiography for hemodynamic evaluation and complication assessment. Key emergency scenarios covered include low-flow alarms, pump thrombosis, right heart dysfunction, bleeding and anticoagulation imbalance, arrhythmias, and cardiac arrest. This consensus applies to implantable continuous-flow LVADs commonly used in China, including domestically manufactured devices such as Corheart 6, CH-VAD, EVAHEART, and HeartCon, as well as the imported device HeartMate 3.

Objective To explore the valve regurgitation status of left heart after the implantation of left ventricular assist device (LVAD) and its effect on prognosis of patients with LVAD implantation. Methods A total of 35 patients with cardiomyopathy who underwent magnetic levitation LVAD implantation at Zhongshan Hospital, Fudan University from February 2021 to July 2024 were retrospectively selected. Clinical data during hospitalization were collected, including preoperative basic data and postoperative valve regurgitation status. Telephone follow-ups were conducted to monitor patients’ survival status and transthoracic echocardiography was used to assess left valve function. Kaplan-Meier survival curves and log-rank test were employed to compare the survival rate of patients with different levels of valve regurgitation. Results The 35 patients had a mean age of (53.9±11.1) years, with 85.7% male, and 3 patients (8.6%) died during hospitalization. Preoperatively, 17 patients (48.6%) had moderate or greater mitral regurgitation, while all 35 patients had less than moderate aortic regurgitation. One month postoperatively, thirty patients were followed up, among which 24 patients (80%) had less than moderate mitral regurgitation, including 11 cases with alleviated regurgitation compared to pre-surgery; 6 patients (20%) had moderate or greater mitral regurgitation, including 4 cases with stable regurgitation and 2 cases with progression of regurgitation compared to pre-surgery; 2 patients (6.7%) had progression of aortic regurgitation to moderate or greater. The follow-up time was 1.2 (1.0, 2.1) years, with 1-year survival rate of 91.4% and 3-year survival rate of 71.1%. Survival analysis showed that the 3-year survival rate of patients with moderate or greater mitral regurgitation one month postoperatively was significantly lower than that of patients with less than moderate regurgitation (66.7% vs 83.3%, P＝0.046). Conclusions After the implantation of magnetic levitation LVAD, most patients showed improvement in mitral regurgitation, while aortic regurgitation remained unchanged. The degree of mitral regurgitation one month postoperatively is associated with prognosis.

OBJECTIVE: To explore the clinical presentation and genetic etiology of a case with Xeroderma pigmentosum in conjunct with basal cell carcinoma and melanoma. METHODS: A male patient with Xeroderma pigmentosum treated at Xinxiang Central Hospital in October 2022 was selected as study subject. Whole exome sequencing (WES) was carried out. Candidate variants were verified by Sanger sequencing of his family members. This study was approved by the Ethics Committee of the hospital (Ethics No.: 2021-167). RESULTS: Magnetic resonance imaging showed that the patient has a solid soft tissue mass in the anterior and lower part of his right eyeball and a small nodule on the left nasal wing. Histopathological biopsy showed that the periocular tumor was basal cell carcinoma in conjunct with malignant melanoma, and the nasal wing tumor was basal cell carcinoma. WES and Sanger sequencing revealed that he has harbored compound heterozygous variants of the XPC gene, namely c.2391delT (p.F797Lfs*11) and IVS1+1G>A, which were inherited from his father and mother, respectively. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variants were rated as likely pathogenic (PVS1+PM2_Supporting+PM3) and pathogenic (PVS1+PM2_Supporting+PM3+PP5), respectively. The c.2391delT variant was unreported previously. Bioinformatic analysis suggests that it could significantly affect the tertiary structure of XPC protein. CONCLUSION The c.2391delT(p.F797Lfs*11) and IVS1+1G>A compound heterozygous variants probably underlay the pathogenesis in this patient. The detection of the novel variant has enriched the mutational spectrum of the XPC gene.
Humans ; Male ; Xeroderma Pigmentosum/genetics* ; Basal Cell Carcinoma/genetics* ; DNA-Binding Proteins/genetics* ; Melanoma/genetics* ; Mutation ; Skin Neoplasms/genetics* ; Middle Aged ; Exome Sequencing ; Pedigree

Objective To observe the value of traditional pelvic floor ultrasound parameters combined with middle urethral sphincter elasticity parameters for diagnosing female stress urinary incontinence(SUI).Methods Fifty two female SUI patients(SUI group)and 45 healthy women(control group)were prospectively enrolled.Traditional pelvic floor ultrasound parameters and middle urethral sphincter elasticity parameters were compared between groups,and logistic regression analysis was performed,the efficacy of each parameter alone and their combination for diagnosing SUI was analyzed.Results Significant differences of bladder neck descent(BND),urethral rotation angle(URA),posterior urethrovesical angle(PUA),shear modulus of the middle urethral anterior wall sphincter at rest state(Q1),shear modulus of the middle urethral anterior wall sphincter under maximum Valsalva maneuver(Q2),and shear modulus of the middle urethral posterior wall sphincter at resting-state(H1)were found between groups(all P＜0.05).BND,PUA,Q1 and Q2 were all influencing factors of female SUI(all P＜0.05),with the area under the curve(AUC)for diagnosing SUI of 0.721,0.718,0.659 and 0.288,respectively.Then traditional ultrasound model,elasticity ultrasound model and combined model were constructed based on traditional pelvic floor ultrasound parameters(BND,PUA),middle urethral sphincter elasticity parameters(Q1,Q2)and their combination,respectively,with AUC for diagnosing SUI of 0.837,0.754 and 0.908,respectively.The AUC of combined model was higher than that of traditional ultrasound model,elasticity ultrasound model and each ultrasound parameter alone(all P＜0.05).Conclusion Traditional pelvic floor ultrasound parameters combined with middle urethral sphincter elasticity parameters had high value for diagnosing female SUI.

Objective:To analyze the clinical effect of Jingling oral liquid combined with methylphenidate hydrochloride and thiopride hydrochloride in the treatment of children with attention deficit hyperactivity disorder (ADHD) and its influence on serum dopamine transporter (DAT), interleukin-6 (IL-6) and 8 isoprostaglandin F2α[8-iso-PGF-(2α)] levels.Methods:A total of 120 children with ADHD co-induced by epilepsy diagnosed and treated in Handan Second Hospital from May 2022 to June 2023 were retrospectively selected and divided into observation group and control group according to treatment method, with 60 cases in each group. The control group was treated with methylphenidate hydrochloride tablets and tiapride hydrochloride, and the observation group was treated with Jingling oral liquid on the basis of the control group. Both groups were treated continuously for 16 weeks. The clinical efficacy and number of seizures of the two groups were compared, the clinical symptoms of the two groups were assessed by the Conner Parent Symptom Questionnaire (PSQ) and the Swanson Nolan and Pelham Version Ⅳ(SNAP-Ⅳ), and the intelligence level of the two groups was assessed by the Wechsler Intelligence Scale. The serum levels of DAT, IL-6, 8-iso-PGF-(2α), neurotrophic factors before and after treatment and the occurrence of adverse reactions were compared between the two groups.Results:After treatment, the effective rate in the observation group was higher than that in the control group: 93.33% (56/60) vs. 80.00% (48/60), there was statistical difference ( χ2 = 4.62, P<0.05). After treatment, the number of seizures in the observation group was less than that in the control group at 3, 4 to 6, 7 to 12 months: (10.78 ± 1.45)times vs.(14.18 ± 1.56) times, (4.86 ± 0.53) times vs.(8.63 ± 0.89) times, (2.64 ± 0.32) times vs. (4.11 ± 0.45) times, there were statistical differences ( P<0.05). After treatment, the scores of PSQ and SNAP-Ⅳ in the observation group were lower than those in the control group: (13.67 ± 1.48)scores vs. (15.18 ± 1.59) scores, (22.12 ± 2.35) scores vs. (25.37 ± 2.68)scores; while the scores of Wechsler intelligence scale was higher than that in the control group: (93.65 ± 9.77)scores vs.(89.42 ± 8.89) scores, there were statistical differences ( P<0.05). After treatment, the serum levels of DAT, IL-6 and 8-iso-PGF-(2α) in the observation group were lower than those in the control group: (0.46 ± 0.05) μg/L vs. (0.52 ± 0.06) μg/L, (8.26 ± 0.89) ng/L vs. (8.74 ± 0.92) ng/L, (60.38 ± 6.46) ng/L vs.(79.25 ± 8.14) ng/L, there were statistical differences ( P<0.05). After treatment, the serum levels of S100βprotein, brain-derived nerve growth factor and prolactin in the observation group were higher than those in the control group: (41.55 ± 4.28) ng/L vs. (37.26 ± 3.87) ng/L, (498.33 ± 54.26) ng/L vs.(442.15 ± 45.78) ng/L, (10.18 ± 1.14) μg/L vs. (9.69 ± 0.97) μg/L, there were statistical differences ( P<0.05).There was no significant difference in gelatinous-derived nerve growth factor level between the two groups after treatment ( P>0.05). There was no significant difference in the incidence of adverse reactions between the two groups ( P>0.05). Conclusions:Jingling oral liquid combined with methylphenidate hydrochloride and thiopride hydrochloride in the treatment of ADHD co-induced by epilepsy has good efficacy, and can significantly improve their clinical symptoms, regulate the levels of serum DAT, IL-6, 8-iso-PGF-(2α). It is safe and reliable.

Objective:To investigate the therapeutic efficacy and safety of conditioning regimen with oral melphalan in tandem autologous hematopoieticstem cell transplantation (ASCT) for patients with malignant plasma cell diseases.Methods:A retrospective case series study was conducted. The clinical data of 13 patients with malignant plasma cell diseases who underwent tandem ASCT between October 2019 and March 2024 in the First Affiliated Hospital of Xi'an Jiaotong University were collected. Compared with the use of intravenous melphalan as conditioning regimen for the first ASCT, hematopoietic reconstruction after transplantation, the therapeutic effects, adverse reactions after drug usage and survival of conditioning regimen with oral melphalan after tandem ASCT were analyzed.Results:Among the 13 patients, there were 10 males and 3 females, with a median age [ M ( Q1, Q3)] of 53 (48, 61) years; 11 cases were multiple myeloma and 2 cases were plasma cell leukemia. Before the first ASCT, tandem ASCT was performed 2-6 months later. The median reconstruction time of neutrophils after the first and second ASCT were both 9 (9, 10) d, and the median reconstruction time of platelets after the first and second ASCT were both 10 (9, 11) d, and there were no statistically significant differences in reconstruction rate of granulocytes on day 9 [69.2% (9/13) vs. 61.5% (8/13)] and platelets on day 10 [46.2% (6/13) vs. 53.8% (7/13)] between the first and second transplantation (all P > 0.05). There were 4 cases of strict complete remission (sCR), 3 cases of complete remission (CR), 4 cases of very good partial remission (VGPR), and 2 cases of partial remission (PR) before the first ASCT. After the first ASCT 1 month later, 1 case achieved VGPR, 1 case achieved PR, 11 cases achieved sCR; all 13 patients achieved sCR at 6 months after second ASCT. Compared with conditioning regimen of intravenous melphalan for the first ASCT, the non-hematological adverse reactions such as nausea (7 cases vs. 9 cases), vomiting (4 cases vs. 13 cases), diarrhea (4 cases vs. 13 cases) and oral mucositis (2 cases vs. 9 cases) in the conditioning regimen of oral melphalan after the second ASCT was reduced, and the differences were statistically significant (all P < 0.01). After the 2 transplantation conditioning regimen with melphalan, Ⅳ degree myelosuppression occurred in 13 cases. After the second ASCT, the median follow-up time was 14 (10, 22) months, 7 patients received maintenance therapy containing lenalidomide, 3 patients received maintenance therapy containing bortezomib, 2 patients received pomalidomide maintenance therapy, and 1 patient received maintenance therapy containing CD38 monoclonal antibody. At the last follow-up, all patients survived, among which 6 multiple myeloma patients relapsed; and the median recurrence time was 13 (10, 22) months after the second ASCT. The estimated 5-year progression-free survival rate was 28.6%. Conclusions:Conditioning regimen with oral melphalan for the second ASCT is safe and well tolerated, and it may further improve the efficacy of the first transplantation.

Objective To observe the value of middle urethral motion and sphincter elasticity for diagnosing female stress urinary incontinence(SUI).Methods Totally 97 female patients,including 52 with SUI(SUI group)and 45 without SUI(control group)were prospectively enrolled.Pelvic floor ultrasound was performed under resting state and the maximum Valsalva maneuver,respectively,and bladder neck mobility(BND),upper-lower mobility of middle urethra(UMupper-lower),anterior-posterior mobility of middle urethra(UM anterior-posterior),elasticity parameter of the anterior wall of middle urethral sphincter(ΔEanterior wall),as well as elasticity parameter of the posterior wall of middle urethral sphincter(ΔEposterior wall)were measured.Patients'general data and the above ultrasound parameters were compared between groups,and the efficacy of them for diagnosing SUI was analyzed.Results Significant differences of BND,UM upper-lower,ΔEanterior wall and ΔEposterior wall,of also the proportion of lateral episiotomy history were found between groups(all P＜0.05).Among them,UMupper-lower,ΔEanterior wall and ΔEposterior wall were all correlated with female SUI(rs=0.231,-0.533,-0.428,all P＜0.05).The area under the curve(AUC)of UMupper-lower,ΔEanterior wall,ΔEposterior wall and their combination for diagnosing SUI was 0.634,0.820,0.748 and 0.867,respectively.The AUC of the combination was significantly higher than that of each parameter alone(all P＜0.001).Conclusion The combination of middle urethral motion and sphincter elasticity was helpful for diagnosing female SUI.

Objective:To compare the impact of preoperative pulmonary vascular resistance(PVR) levels on the prognosis of patients undergoing left ventricular assist device(LVAD)implantation.Methods:A retrospective analysis was conducted on the clinical data of 33 patients among September 2021 and June 2024, who underwent LVAD implantation at Zhongshan Hospital, Fudan University. 29 males and 4 females, with a mean age of(53.61±9.65)years old. The cohort included 30 cases of dilated cardiomyopathy, 2 cases of ischemic cardiomyopathy, and 1 case of noncompaction cardiomyopathy. Patients were grouped based on a preoperative PVR threshold of 3 WU, Preoperative data from Swan-Ganz catheterization, liver and renal function indicators, as well as other relevant clinical parameters were recorded. Patients were followed up to assess differences in survival outcomes.Results:Preoperative PVR was elevated in 18 cases(60%)of the patients, with a mean preoperative PVR of(3.16±1.71)WU. Immediate postoperative right heart catheterization was performed in 20 patients, showing a significant reduction in PVR from(3.85±1.72)WU preoperatively to(1.80±1.38)WU postoperatively( P<0.05). The cardiac index improved significantly from(1.95±0.63)L·min -1·m -2 preoperatively to(4.25±1.26)L·min -1·m -2 postoperatively( P<0.001). The mean follow-up duration was(14.78±12.06)months, no significant difference in postoperative survival was observed between the two groups(100% vs. 80%, P=0.667). Conclusion:Increased pulmonary resistance is often secondary to left heart dysfunction. After the implantation of a LVAD, pulmonary resistance can be improved. For patients with relatively normal right heart function before surgery, elevated pulmonary resistance does not affect the short-term outcomes of the implantation.

Paraplegia caused by spinal cord injury is a serious neurological complication, for which surgery is currently the main treatment method. Due to different surgical approaches, patients are usually expected to maintain a passive prone position for a long time or switch between the supine and prone positions. Affected by multiple factors such as neurogenic sensory disorders, pathological changes in muscle tone and operative duration, the risk of intraoperative acquired pressure injury (IAPI) is significantly increased. Current clinical prevention strategies for IAPI in these patients predominantly focus on localized pressure relief during positioning, lacking systematic, standardized comprehensive prevention protocols or evidence-based guidelines. To address it, Department of Nursing, Orthopedics Branch, China International Exchange and Promotive Association for Medical and Health Care, Spinal Trauma Professional Committee, Orthopedics Branch, Chinese Medical Doctor Association, Nursing Group of Spine and Spinal Cord Professional Committee of Chinese Association of Rehabilitation Medicine organized experts in relevant fields to formulate Guideline for the prevention of intraoperative acquired pressure injury in paraplegic patients with spinal cord injury ( version 2025), based on evidence-based medical evidence and latest research results and clinical practice at home and abroad. Eleven recommendations were put forward from the aspects of preoperative risk assessment, intraoperative prevention strategies, postoperative handover and monitoring, and supportive mechanisms for IAPI prevention, aiming to standardize the prevention measures and management strategies of IAPI in paraplegic patients with spinal cord injury and accelerate the recovery of patients and improve the therapeutic effect.

Objective To investigate the role and mechanism of metastasis-associated protein in colorectal cancer 1(MACC1)in the proliferation and migration of colorectal cancer.Methods The expression of MACC1 in colorectal cancer samples and para-cancerous samples from TCGA database was analyzed.The survival difference between the groups with high and low expression of MACC1 was studied.HCT116 cells were divided into Vector group(no treatment group)and MACC1 OE group(transfected with pcDNA3.1-MACC1 plasmid),si-NC group(negative control group),and si-MACC1 group(transfected with MACC1 siRNA).MTT assay was used to detect cell viability;EDU and cell clonal formation assay were used to detect cell proliferation.The migration and invasion of cells were detected by scratch and invasion assays,respectively.The mRNA expression level of cellular-mesenchymal epithelial transition factor(c-MET)was detected by RT-qPCR,and the protein expression of MACC1 and c-MET was detected by Western blotting.Colon cancer cell HCT116 transfected with MACC1 OE was inoculated subcutaneously into nude mice to establish tumor model,and the volume and weight of tumor tissue were measured.Results The expression level of MACC1 was upregulated in colorectal cancer tissue and cells(P＜0.05).Patients with high MACC1 expression had shorter overall survival than those with low MACC1 expression(P=0.003).Overexpression of MACC1 significantly increased cell viability(F=86.070,P＜0.001).Compared with those in si-NC group,the proliferation rate,migration,invasion and number of clone formation of HCT116 in si-MACC1 group were significantly decreased(P＜0.01).The expression of MACC1 protein was positively correlated with the expression of c-MET protein in colorectal cancer(r=0.802,P=0.002).Overexpression of MACC1 promoted the c-MET expression(t=13.532,P＜0.001),while knockdown of MACC1 inhibited the c-MET expression(t=14.626,P＜0.001).Luciferase reports assay demonstrated that c-MET was a transcriptional target of MACC1.MACC1 overexpression increased the tumor volume and weight of nude mice(P＜0.01).Conclusion MACC1 can promote the invasion and migration of colorectal cancer through hepatocyte growth factor/c-MET pathway.