Objective To compare the mid- and long-term clinical results of mitral valve plasty (MVP) and mitral valve replacement (MVR) in the treatment of functional mitral regurgitation (FMR). Methods Patients with FMR who underwent surgical treatment in the Department of Cardiovascular Surgery of the General Hospital of Northern Theater Command from 2012 to 2021 were collected. The patients who underwent MVP were divided into a MVP group, and those who underwent MVR into a MVR group. The clinical data and mid-term follow-up efficacy of two groups were compared. Results Finally 236 patients were included. There were 100 patients in the MVP group, including 53 males and 47 females, with an average age of (61.80±8.03) years. There were 136 patients in the MVR group, including 72 males and 64 females, with an average age of (61.29±8.97) years. There was no statistical difference in baseline data between the two groups (P＞0.05). There was no statistical difference between the two groups in the extracorporeal circulation time, aortic occlusion time, postoperative hospital and ICU stay, intraoperative blood loss, or hospitalization death (P＞0.05), but the time of mechanical ventilation in the MVP group was significantly shorter than that in the MVR group (P=0.022). The total follow-up rate was 100.0%, the longest follow-up was 10 years, and the average follow-up time was (3.60±2.55) years. There were statistical differences in the left atrial diameter, left ventricular end-diastolic diameter, left ventricular end-systolic diameter and cardiac function between the two groups compared with those before surgery (P<0.05). The postoperative left ventricular ejection fraction in the MVP group was statistically higher than that before surgery (P=0.002), but there was no statistical difference in the MVR group before and after surgery (P=0.658). The left atrial diameter in the MVP group was reduced compared with the MVR group (P=0.026). The recurrence rate of mitral regurgitation in the MVP group was higher than that in the MVR group, and the difference was statistically significant (10.0% vs. 1.5%, P=0.003). There were 14 deaths in the MVP group and 19 in the MVR group. The cumulative survival rate (P=0.605) and cardiovascular events-free survival rate (P=0.875) were not statistically significant between the two groups by Kaplan-Meier survival analysis. Conclusion The safety, and mid- and long-term clinical efficacy of MVP in the treatment of FMR patients are better than MVR, and the left atrial and left ventricular diameters are statistically reduced, and cardiac function is statistically improved. However, the surgeon needs to be well aware of the indications for the MVP procedure to reduce the rate of mitral regurgitation recurrence.

Objective To investigate the impact of various health education intervention strategies on the proper use of personal respiratory protective equipment (RPE) among workers exposed to dust. Methods Dust-exposed workers were recruited from 60 selected enterprises in Guangdong Province using cluster random sampling method. They were randomly allocated to the control, low-intensity intervention, and high-intensity intervention groups, with 358, 346, and 371 workers in each group, respectively. Workers in the control group received no designed intervention. Workers in the low-intensity intervention group received traditional plus mobile health education on the proper use of RPE. Workers in the high-intensity intervention group received all components of the low-intensity intervention, supplemented with peer education. The intervention lasted for six months. RPE usage was compared among the three groups of workers before and after the intervention. Results Workers in the control, low-intensity intervention, and high-intensity intervention groups showed higher rates of both RPE wearing and correct RPE wearing after the intervention than before it within their respective groups (RPE wearing rate: 94.1% vs 99.2%, 95.7% vs 100.0%, 94.6% vs 100.0%, all P<0.01; correct RPE wearing rate: 66.8% vs 91.1%, 67.3% vs 95.7%, 66.6% vs 96.5%, all P<0.01). Post-intervention correct RPE wearing rates were highest in the high-intensity intervention group, followed by the low-intensity intervention group, and the control group, with the percentage of 96.50%, 95.66% and 91.06%, respectively (P<0.01). Binary logistic regression analysis result showed that different intervention strategies affected the correct use of personal RPE among dust-exposed workers after adjusting for gender, age, and other confounding factors (P<0.05). Compared with the control group, the rates of correct RPE use increased in the low-intensity intervention group and the high-intensity intervention group (odd ratio was 2.14 and 3.01; 95% confidence interval was 1.12 - 4.10 and 1.53 - 5.91, respectively). Conclusion The implementation of traditional plus mobile health education interventions on the proper use of RPE can promote correct RPE utilization among dust-exposed workers, and integrating peer education further enhances the intervention effectiveness.

Thyroid hormone（TH）plays an important role in human development and is involved in gene and protein expression in almost all tissues，especially in the development of the central nervous system.TH requires a TH transporter to enter the cell，and three families of TH transporter proteins are known，namely monocarboxylate transporters（MCTs），organic anion transporting polypeptides（OATPs）and L-type amino acid transporter（LAT）.MCT8 has been found to be a specific TH transporter，and OATP1C1 also plays an important role.Deficiency of TH transporters may lead to different degrees of dysfunction in the nervous system and endocrine system.Currently，more studies have been conducted on MCT8 deficiency，which presents with characteristic psychomotor retardation and TH abnormalities，and there are no specific treatment options.In this paper，we summarize the research progress on clinical phenotype，pathogenic mechanism，and treatment of thyroid hormone transporter defects related diseases to provide reference for clinical research.

Background:Acute pancreatitis(AP)is a common disease of the digestive system,among which severe acute pancreatitis(SAP)has a high mortality rate.Finding more accurate and convenient methods for early recognition of SAP is one of the major challenges in clinical treatment.Aims:To explore the application value of the controlled attenuation parameter(CAP)of ultrasound elastography in predicting SAP.Methods:A retrospective cohort study was conducted involving 135 AP patients admitted to Jiading Branch of Shanghai General Hospital from February to October 2024.Patients were categorized into non-SAP and SAP groups according to the severity of the disease.Clinical data,local complications,laboratory indicators,and CAP were compared between the two groups.Univariate and multivariate Logistic regression analyses were used to identify independent risk factors for SAP.A SAP prediction model based on CAP was constructed according to the identified risk factors and the minimum Akaike information criterion(AIC).ROC curve and Bootstrap method were used to evaluate the efficacy of the prediction model and conduct internal validation,respectively.Results:There were statistically significant differences between the non-SAP group and SAP group in body mass index(BMI),incidence of hyperlipidemia,etiological composition,incidence of pleural and ascitic fluid,length of hospital stay,incidence of peripancreatic effusion,incidence of pancreatic necrosis,white blood cell count(WBC),D-dimer(D-D)level,blood glucose,triglyceride(TG),C-reactive protein(CRP),neutrophil count,procalcitonin(PCT),interleukin-6(IL-6),free triiodothyronine(FT3),and CAP(all P<0.05).Multivariate Logistic regression analysis showed that pancreatic necrosis(OR=13.39,95%CI:3.10-57.94,P<0.001)and CAP(OR=1.01,95%CI:1.01-1.02,P=0.038)were independent risk factors for SAP.The SAP prediction model based on CAP was formulated as:Logit(P)=-5.884+0.010×CAP+2.839×pancreatic necrosis+0.169×D-D+0.132×blood glucose+0.006×CRP.The model showed an area under the curve(AUC)of 0.834 for predicting SAP,which was superior to CAP alone(P<0.05).Internal validation indicated that the prediction model had high stability and accuracy(C-index=0.808).Conclusions:The prediction model constructed based on CAP has good clinical value for predicting SAP,providing a new perspective and tool for early identification and prognostic assessment of AP.

Background:Acute pancreatitis(AP)is a common disease of the digestive system,among which severe acute pancreatitis(SAP)has a high mortality rate.Finding more accurate and convenient methods for early recognition of SAP is one of the major challenges in clinical treatment.Aims:To explore the application value of the controlled attenuation parameter(CAP)of ultrasound elastography in predicting SAP.Methods:A retrospective cohort study was conducted involving 135 AP patients admitted to Jiading Branch of Shanghai General Hospital from February to October 2024.Patients were categorized into non-SAP and SAP groups according to the severity of the disease.Clinical data,local complications,laboratory indicators,and CAP were compared between the two groups.Univariate and multivariate Logistic regression analyses were used to identify independent risk factors for SAP.A SAP prediction model based on CAP was constructed according to the identified risk factors and the minimum Akaike information criterion(AIC).ROC curve and Bootstrap method were used to evaluate the efficacy of the prediction model and conduct internal validation,respectively.Results:There were statistically significant differences between the non-SAP group and SAP group in body mass index(BMI),incidence of hyperlipidemia,etiological composition,incidence of pleural and ascitic fluid,length of hospital stay,incidence of peripancreatic effusion,incidence of pancreatic necrosis,white blood cell count(WBC),D-dimer(D-D)level,blood glucose,triglyceride(TG),C-reactive protein(CRP),neutrophil count,procalcitonin(PCT),interleukin-6(IL-6),free triiodothyronine(FT3),and CAP(all P<0.05).Multivariate Logistic regression analysis showed that pancreatic necrosis(OR=13.39,95%CI:3.10-57.94,P<0.001)and CAP(OR=1.01,95%CI:1.01-1.02,P=0.038)were independent risk factors for SAP.The SAP prediction model based on CAP was formulated as:Logit(P)=-5.884+0.010×CAP+2.839×pancreatic necrosis+0.169×D-D+0.132×blood glucose+0.006×CRP.The model showed an area under the curve(AUC)of 0.834 for predicting SAP,which was superior to CAP alone(P<0.05).Internal validation indicated that the prediction model had high stability and accuracy(C-index=0.808).Conclusions:The prediction model constructed based on CAP has good clinical value for predicting SAP,providing a new perspective and tool for early identification and prognostic assessment of AP.

Objective To investigate the safety and efficacy of mitral valve replacement combined with cryoablation Maze surgery in patients with atrial functional mitral regurgitation (AFMR). Methods From January 2014 to June 2020, patients with AFMR who underwent mitral valve replacement in our department were enrolled. They were divided into two groups, a cryoablation Maze group who received cryoablation Maze surgery during mitral valve replacement, and a non-cryoablation Maze group who did not receive cryoablation Maze surgery. The baseline data, surgical data, efficacy, and prognosis between the two groups were compared. Results Finally 85 patients were enrolled. There were 16 males and 24 females with an average age of 58.65±6.86 years in the cryoablation Maze group, and 24 males and 21 females with an average age of 61.29±8.30 years in the non-cryoablation Maze group. There was no statistical difference in baseline data between the two groups (P＞0.05). The aortic occlusion time and extracorporeal circulation time of the cryoablation Maze group were longer than those of the non-cryoablation Maze group with statistical differences (P＜0.01). There was no statistical difference in postoperative ICU retention time, ventilator assistance time, length of hospital stay, intraoperative blood loss, drainage volume on the first day or occurrence rate of complications (temporary pacemaker application, electrical cardioversion, thoracic puncture drainage, hospitalization death) between the two groups (P＞0.05). At the time of discharge, postoperative 3-month, 6-month, 12-month, and 24-month, the maintenance rates of sinus rhythm in the non-cryoablation Maze group were statistically different from those of the cryoablation Maze group (P＜0.001). Compared with the non-cryoablation Maze group, the decrease values of left atrial diameter, left ventricular end-diastolic diameter, left ventricular end-systolic diameter and pulmonary artery systolic pressure were statistically different (P＜0.05). Postoperative cardiac function grading of both groups was grade Ⅰ or Ⅱ, which was significantly improved compared with preoperative level, but there was no statistical significance between the two groups (P＞0.05). There was no statistical difference in the incidence of adverse events during follow-up (P＞0.05). Conclusion Cryoablation Maze surgery combined with mitral valve replacement is safe and effective in the treatment of AFMR patients, which is conducive to the recovery and maintenance of sinus rhythm, and is beneficial to the remodeling of the left atrium and left ventricle, the reduction of pulmonary systolic blood pressure, and the improvement of life quality of the patients.

Objective:To observe and evaluate the clinical efficacy and safety of albumin-bound paclitaxel as first-line treatment for patients with advanced pancreatic cancer in China, and to explore the prognosis-related molecules in pancreatic cancer based on next-generation sequencing (NGS) of tumor tissues.Methods:From December 2018 to December 2020, patients with locally advanced or metastatic pancreatic cancer were recruited to accept albumin-bound paclitaxel as first-line treatment in the oncology departments of 24 hospitals in East China. The primary endpoints were overall survival (OS) and treatment related adverse events, and the secondary endpoint was progression-free survival (PFS). Adverse effects were graded using Common Terminology Criteria for Adverse Events 5.0 (CTCAE 5.0). NGS sequencing on the primary or metastatic tissue samples of pancreatic cancer obtained through surgical resection or biopsy was performed.Results:This study recruited 229 patients, including 70 patients with locally advanced pancreatic cancer (LAPC) and 159 patients with metastatic pancreatic cancer (mPC). The disease control rate was 79.9% and the objective response rate is 36.3%.The common adverse effects during treatment were anaemia (159 cases), leucopenia (170 cases), neutropenia (169 cases), increased aminotransferases (110 cases), and thrombocytopenia (95 cases), and the incidence of grade 3-4 neutropenia is 12.2% (28/229). The median follow-up time was 21.2 months (95% CI: 18.5-23.1 months). The median PFS (mPFS) was 5.3 months (95% CI: 4.37-4.07 months) and the median OS (mOS) was 11.2 months (95% CI: 9.5-12.9 months). The mPFS of patients with LAPC was 7.4 months (95% CI: 6.6-11.2 months), and their mOS was 15.5 months (95% CI: 12.6-NA months). The mPFS of patients with mPC was 3.9 months (95% CI: 3.4-5.1 months), and their mOS was 9.3 months (95% CI: 8.0-10.8 months). Multivariate Cox regression analysis showed that clinical stage ( HR=1.47, 95% CI: 1.06-2.04), primary tumor site ( HR=0.64, 95% CI: 0.48-0.86), Eastern Cooperative Oncology Group Performance Status (ECOG PS) score ( HR=2.66, 95% CI: 1.53-4.65), and whether to combine radiotherapy ( HR=0.65, 95% CI: 0.42-1.00) were independent influencing factors for the PFS of these patients. The primary tumor site ( HR=0.68, 95% CI: 0.48-0.95), ECOG score ( HR=5.82, 95% CI: 3.14-10.82), and whether to combine radiotherapy ( HR=0.58, 95% CI: 0.35-0.96) were independent influencing factors of the OS of these patients. The most frequent gene mutations in these advanced stage pancreatic patients were KRAS (89.66%), TP53 (77.01%), CDKN2A (32.18%), and SMAD4 (21.84%) by NGS of tumor tissues from 87 pancreatic cancer patients with sufficient specimens. Further analysis revealed that mutations in CDKN2B, PTEN, FGF6, and RBBP8 genes were significantly associated with an increased risk of death ( P＜0.05). Conclusion:Albumin-bound paclitaxel as first-line treatment demonstrated feasible anti-tumor efficacy and manageable safety for patients with advanced pancreatic cancer in China.

Objective:To observe and evaluate the clinical efficacy and safety of albumin-bound paclitaxel as first-line treatment for patients with advanced pancreatic cancer in China, and to explore the prognosis-related molecules in pancreatic cancer based on next-generation sequencing (NGS) of tumor tissues.Methods:From December 2018 to December 2020, patients with locally advanced or metastatic pancreatic cancer were recruited to accept albumin-bound paclitaxel as first-line treatment in the oncology departments of 24 hospitals in East China. The primary endpoints were overall survival (OS) and treatment related adverse events, and the secondary endpoint was progression-free survival (PFS). Adverse effects were graded using Common Terminology Criteria for Adverse Events 5.0 (CTCAE 5.0). NGS sequencing on the primary or metastatic tissue samples of pancreatic cancer obtained through surgical resection or biopsy was performed.Results:This study recruited 229 patients, including 70 patients with locally advanced pancreatic cancer (LAPC) and 159 patients with metastatic pancreatic cancer (mPC). The disease control rate was 79.9% and the objective response rate is 36.3%.The common adverse effects during treatment were anaemia (159 cases), leucopenia (170 cases), neutropenia (169 cases), increased aminotransferases (110 cases), and thrombocytopenia (95 cases), and the incidence of grade 3-4 neutropenia is 12.2% (28/229). The median follow-up time was 21.2 months (95% CI: 18.5-23.1 months). The median PFS (mPFS) was 5.3 months (95% CI: 4.37-4.07 months) and the median OS (mOS) was 11.2 months (95% CI: 9.5-12.9 months). The mPFS of patients with LAPC was 7.4 months (95% CI: 6.6-11.2 months), and their mOS was 15.5 months (95% CI: 12.6-NA months). The mPFS of patients with mPC was 3.9 months (95% CI: 3.4-5.1 months), and their mOS was 9.3 months (95% CI: 8.0-10.8 months). Multivariate Cox regression analysis showed that clinical stage ( HR=1.47, 95% CI: 1.06-2.04), primary tumor site ( HR=0.64, 95% CI: 0.48-0.86), Eastern Cooperative Oncology Group Performance Status (ECOG PS) score ( HR=2.66, 95% CI: 1.53-4.65), and whether to combine radiotherapy ( HR=0.65, 95% CI: 0.42-1.00) were independent influencing factors for the PFS of these patients. The primary tumor site ( HR=0.68, 95% CI: 0.48-0.95), ECOG score ( HR=5.82, 95% CI: 3.14-10.82), and whether to combine radiotherapy ( HR=0.58, 95% CI: 0.35-0.96) were independent influencing factors of the OS of these patients. The most frequent gene mutations in these advanced stage pancreatic patients were KRAS (89.66%), TP53 (77.01%), CDKN2A (32.18%), and SMAD4 (21.84%) by NGS of tumor tissues from 87 pancreatic cancer patients with sufficient specimens. Further analysis revealed that mutations in CDKN2B, PTEN, FGF6, and RBBP8 genes were significantly associated with an increased risk of death ( P＜0.05). Conclusion:Albumin-bound paclitaxel as first-line treatment demonstrated feasible anti-tumor efficacy and manageable safety for patients with advanced pancreatic cancer in China.

The clinical data, diagnosis and treatment of a child with Robinow syndrome (RS) caused by the DVL1 gene mutation, who was treated in the Department of Endocrinology, Genetics and Metabolism, Children′s Hospital of Nanjing Medical University in May 2023, were retrospectively analyzed.The male child, 2 years old, presented with 2 years of external genital abnormality.The main clinical features included intrauterine growth retardation, external genital abnormalities, craniofacial anomalies, skeletal malformations and congenital heart diseases.Whole exome sequencing revealed that the patient carried a heterozygous mutation c. 1529delG(p.G510Vfs*139) in exon 14 of the DVL1 gene.Cases of the DVL1 gene mutation have not been documented in Chinese.A review of literature identified 25 (including the case in this report) cases of RS in children attributed to DVL1 gene mutations, revealing common clinical features such as craniofacial anomalies, skeletal malformations, external genital abnormalities, heart diseases, short stature, and hearing impairments.Cognitive abilities are typically unaffected, and reproductive function remains normal.Notably, 19 identified DVL1 gene mutations are clustered within a specific genomic region (c.1496-c.1631), with no discernible genotype-phenotype correlation observed.

Objective:To screen the key exosomal long non-coding RNAs (lncRNAs) molecules that cause nasopharyngeal carcinoma cells to develop chemoradiotherapy resistance.Methods:In vitro, a model of concurrent chemoradiotherapy for human nasopharyngeal carcinoma cells was constructed, and the continuous shock method of high-dose concurrent chemoradiotherapy was used to induce the establishment of chemoradiotherapy-resistant nasopharyngeal carcinoma cell lines, and its resistance formation was verified. Exosomes produced by chemoradiotherapy-resistant cell lines and respective mother cell lines for nasopharyngeal carcinoma were extracted and identified. Finally, biochip technology was used to detect the differential expression levels of exosomal lncRNAs. Results:After 10 repeated treatments of concurrent chemoradiotherapy, CNE-1 CRR and CNE-2 CRR were successfully obtained. Compared with the mother cell lines, CNE-1 CRR and CNE-2 CRR had a tendency to transform from epithelial to interstitial morphology, and the number of cell clones was higher, and the values of average lethal dose (D 0), quasi-threshould dose (D q), survival fraction after 2 Gy irradiation (SF 2) and cell survival rate were higher. Nasopharyngeal carcinoma cells were detected by PCR chip of exosomal lncRNAs. Compared with their respective mother cell lines, 18 lncRNAs in CNE-1 CRR exosomes were significantly up-regulated and 31 lncRNAs were significantly down-regulated, and 15 lncRNAs were significantly up-regulated and 38 lncRNAs were significantly down-regulated in CNE-2 CRR exosomes. CNE-1 CRR also had similar expression profiles to CNE-2 CRR. Conclusion:There are significantly up-regulated and down-regulated lncRNAs in the exosomes of CNE-1 CRR and CNE-2 CRR.