This study investigated the role of the nuclear factor of activated T cells c3 (NFATc3) in vascular smooth muscle cells (VSMCs) during aortic aneurysm and dissection (AAD) progression and the underlying molecular mechanisms. Cytoplasmic and nuclear NFATc3 levels were elevated in human and mouse AAD. VSMC-NFATc3 deletion reduced thoracic AAD (TAAD) and abdominal aortic aneurysm (AAA) progression in mice, contrary to VSMC-NFATc3 overexpression. VSMC-NFATc3 deletion reduced extracellular matrix (ECM) degradation and maintained the VSMC contractile phenotype. Nuclear NFATc3 targeted and transcriptionally upregulated matrix metalloproteinase 9 (MMP9) and MMP2, promoting ECM degradation and AAD development. NFATc3 promoted VSMC phenotypic switching by binding to eukaryotic elongation factor 2 (eEF2) and inhibiting its phosphorylation in the VSMC cytoplasm. Restoring eEF2 reversed the beneficial effects in VSMC-specific NFATc3-knockout mice. Cabamiquine-targets eEF2 and inhibits protein synthesis-inhibited AAD development and progression in VSMC-NFATc3-overexpressing mice. VSMC-NFATc3 promoted VSMC switch and ECM degradation while exacerbating AAD development, making it a novel potential therapeutic target for preventing and treating AAD.

Objective:To investigate the current status of fear of falling in postoperative cardiac surgery patients, and to analyze its influencing factors and interrelationships.Methods:A convenience sample of 246 postoperative cardiac surgery patients hospitalized in the Department of Cardiac Surgery, the First Affiliated Hospital of Xiamen University, from December 2023 to December 2024 was surveyed. The Body Image Scale, the Personal Mastery Scale, the Fear of Falling Questionnaire-Revised, and a self-designed demographic and clinical questionnaire were used for data collection. Pearson correlation analysis was conducted to examine the relationships among body image, personal mastery, and fear of falling. PROCESS Model 4 was applied to test the mediating role of personal mastery between body image and fear of falling, with bootstrapping for verification.Results:A total of 246 questionnaires were distributed, and 236 valid responses were obtained (valid response rate: 95.93%) . The body image score was (14.02±6.36) , personal mastery score was (20.44±4.16) , and fear of falling score was (38.28±10.83) . Body image was positively correlated with fear of falling ( r=0.324, P<0.01) , while personal mastery was negatively correlated with fear of falling ( r=-0.552, P<0.01) . After controlling for variables with statistically significant differences in univariate analysis (including sex, age, education level, body mass index, history of syncope, pain level, and self-rated preoperative exercise capacity) , PROCESS analysis showed that body image had a direct positive effect on fear of falling ( β=0.288, P<0.05) , and also indirectly affected fear of falling through personal mastery, with the indirect effect accounting for 49.5% of the total effect (0.237/0.525) . Conclusions:Postoperative cardiac surgery patients require improvement in body image, personal mastery, and fear of falling. Understanding how body image influences fear of falling through personal mastery not only enriches theoretical frameworks but also provides guidance for psychological interventions in clinical practice, thereby meeting patients' psychological support needs during rehabilitation.

Objective:To investigate the current status of fear of falling in postoperative cardiac surgery patients, and to analyze its influencing factors and interrelationships.Methods:A convenience sample of 246 postoperative cardiac surgery patients hospitalized in the Department of Cardiac Surgery, the First Affiliated Hospital of Xiamen University, from December 2023 to December 2024 was surveyed. The Body Image Scale, the Personal Mastery Scale, the Fear of Falling Questionnaire-Revised, and a self-designed demographic and clinical questionnaire were used for data collection. Pearson correlation analysis was conducted to examine the relationships among body image, personal mastery, and fear of falling. PROCESS Model 4 was applied to test the mediating role of personal mastery between body image and fear of falling, with bootstrapping for verification.Results:A total of 246 questionnaires were distributed, and 236 valid responses were obtained (valid response rate: 95.93%) . The body image score was (14.02±6.36) , personal mastery score was (20.44±4.16) , and fear of falling score was (38.28±10.83) . Body image was positively correlated with fear of falling ( r=0.324, P<0.01) , while personal mastery was negatively correlated with fear of falling ( r=-0.552, P<0.01) . After controlling for variables with statistically significant differences in univariate analysis (including sex, age, education level, body mass index, history of syncope, pain level, and self-rated preoperative exercise capacity) , PROCESS analysis showed that body image had a direct positive effect on fear of falling ( β=0.288, P<0.05) , and also indirectly affected fear of falling through personal mastery, with the indirect effect accounting for 49.5% of the total effect (0.237/0.525) . Conclusions:Postoperative cardiac surgery patients require improvement in body image, personal mastery, and fear of falling. Understanding how body image influences fear of falling through personal mastery not only enriches theoretical frameworks but also provides guidance for psychological interventions in clinical practice, thereby meeting patients' psychological support needs during rehabilitation.

Objective:To develop a Knowledge-Attitude-Practice (KAP) Scale for Fall Prevention in Hospitalized Aged Patients with Cardiovascular Disease and test its reliability and validity.Methods:Using the Knowledge-Attitude-Practice (KAP) framework, scale items were initially constructed through literature review, expert consultation, and a pilot survey. Totally 277 hospitalized cardiovascular patients from the cardiovascular surgery and cardiology departments of the First Affiliated Hospital of Xiamen University were recruited by convenience sampling from September to November 2023 to evaluate the reliability and validity of the scale.Results:A total of 277 questionnaires were distributed, with 254 valid responses, with a response rate of 91.70%. Exploratory factor analysis using principal component analysis and varimax rotation extracted four factors in each dimension (knowledge, attitude, and practice) , explaining 66.506%, 67.847%, and 69.241% of the variance, respectively. Confirmatory factor analysis demonstrated a stable and reliable factor structure. The overall Cronbach's α coefficient of the scale was 0.829, with Cronbach's α coefficients for the knowledge, attitude, and practice dimensions of 0.697, 0.756, and 0.726, respectively. The finalized KAP Scale for Fall Prevention in Hospitalized Aged Patients with Cardiovascular Disease comprised 3 dimensions, 12 factors, and 32 items.Conclusions:KAP Scale for Fall Prevention in Hospitalized Aged Patients with Cardiovascular Disease demonstrated good reliability, validity, and feasibility, making it a suitable tool for clinical nursing practice to better prevent fall incidents in hospitalized aged patients with cardiovascular disease.

Objective:To develop a Knowledge-Attitude-Practice (KAP) Scale for Fall Prevention in Hospitalized Aged Patients with Cardiovascular Disease and test its reliability and validity.Methods:Using the Knowledge-Attitude-Practice (KAP) framework, scale items were initially constructed through literature review, expert consultation, and a pilot survey. Totally 277 hospitalized cardiovascular patients from the cardiovascular surgery and cardiology departments of the First Affiliated Hospital of Xiamen University were recruited by convenience sampling from September to November 2023 to evaluate the reliability and validity of the scale.Results:A total of 277 questionnaires were distributed, with 254 valid responses, with a response rate of 91.70%. Exploratory factor analysis using principal component analysis and varimax rotation extracted four factors in each dimension (knowledge, attitude, and practice) , explaining 66.506%, 67.847%, and 69.241% of the variance, respectively. Confirmatory factor analysis demonstrated a stable and reliable factor structure. The overall Cronbach's α coefficient of the scale was 0.829, with Cronbach's α coefficients for the knowledge, attitude, and practice dimensions of 0.697, 0.756, and 0.726, respectively. The finalized KAP Scale for Fall Prevention in Hospitalized Aged Patients with Cardiovascular Disease comprised 3 dimensions, 12 factors, and 32 items.Conclusions:KAP Scale for Fall Prevention in Hospitalized Aged Patients with Cardiovascular Disease demonstrated good reliability, validity, and feasibility, making it a suitable tool for clinical nursing practice to better prevent fall incidents in hospitalized aged patients with cardiovascular disease.

The paper reports two cases of lipoprotein glomerulopathy (LPG) in children. The Sanger sequencing results in 2 cases indicated apolipoprotein E gene mutation[c.127 (exon3) C>T, p.R43C (p.Arg43Cys); c.494 (exon4) G>C, p.R165P (p.Arg165Pro),respectively]. Renal pathological presentation of two children showed that a large number of lipoprotein emboli were formed in the glomerular capillary loop, and the diagnosis of LPG was confirmed. The onset of LPG has no specific clinical manifestation, which is easy to be undiagnosed or misdiagnosed. Renal biopsy is a diagnostic means, glucocorticoid treatment is ineffective, and long-term lipid-lowering treatment may be required for LPG.

Objective:To analyze the clinicopathologic features and prognosis of children with Henoch-Sch?nlein purpura nephritis (HSPN).Methods:The clinicopathological data of children with HSPN who were followed up for more than 5 years and underwent renal biopsy in Jinling Hospital affiliated to Medical School of Nanjing University from January 2001 to June 2015 were retrospectively analyzed. The follow-up endpoint event was defined as estimated glomerular filtration rate (eGFR)<90 ml·min -1·(1.73 m 2) -1. Participants were divided into two groups according to whether the children had reached the primary endpoint event or not. Cox proportional hazards model was used to analyze the influencing factors of renal poor prognosis in children with HSPN. Kaplan-Meier survival curve method was used for survival analysis, and log-rank test was used to compare the difference of renal cumulative survival rate between segmental sclerosis/adhesion (S1) group and non-segmental sclerosis/adhesion (S0) group. Receiver operating characteristic curve (ROC curve) and area under the curve ( AUC) were used to evaluate the diagnostic value. Results:A total of 130 children with HSPN were enrolled in the study. The median onset age was 11.7(8.6, 13.3) years old, of whom 71 cases were males (54.6%). At a median follow-up time of 100.0(75.8, 119.0) months, 12 cases (9.23%) with HSPN reached the primary endpoint event. Compared with the non-endpoint event group, the endpoint event group had higher proportion of hypertension, higher levels of 24-hour urinary protein, serum cholesterol, serum uric acid, and serum creatinine, and lower levels of serum albumin (all P<0.05). There was no statistical difference in treatment between the two groups (all P>0.05). In terms of pathological features, compared with the non-endpoint event group, the endpoint event group had higher proportion of mesangial hyperplasia (M1), S1, tubular atrophy/interstitial fibrosis (T1/T2) and Glomerulus-Bowman's capsule adhesion (all P<0.05). Multivariate Cox regression model showed that S1 was significantly correlated with renal poor prognosis ( HR=7.739, 95% CI 1.422-42.114, P=0.018). As was revealed in a Kaplan-Meier plot, renal cumulative survival rate in the S1 group was significantly lower than that in the S0 group (log-rank χ2=17.069, P<0.001). The ROC curve showed S1 accurately predicted the outcome ( AUC=0.710, 95% CI 0.549-0.872) with specificity of 0.667(95% CI 0.349-0.901) and specificity of 0.754(95% CI 0.667-0.829). Conclusions:S1 is an independent risk factor affecting renal poor prognosis and has a diagnostic value.

Objective:To investigate the efficacy and safety of Rituximab (RTX) in treating children with refractory steroid-resistant nephrotic syndrome (SRNS).Methods:The clinical data of 10 children with refractory SRNS receiving RTX in the Department of Pediatrics, Jinling Hospital from September 2013 to March 2018 were analyzed retrospectively.Results:The age of onset of 10 children (including 5 males and 5 females) was (4.47±2.75) years old.The renal biopsy showed focal segmental glomerular sclerosis in 5 cases (50%), minimal change nephropathy in 3 cases (30%), IgM nephropathy in 1 case (10%), and mesangial proliferative glomerulonephritis in 1 case (10%). Ten children received RTX treatment (1 or 4 doses; 375 mg/m 2 once; maximum: 500 mg) at the age of (6.74±2.62) years old.There were 8 patients (80%) receiving a single dose of RTX, 1 patient (10%) receiving 3 doses, and 1 patient (10%) receiving 8 doses.The follow-up time was 11.93 (5.17, 25.66) months.The remission rates at the 3-month follow-up, 6-month follow-up and last follow-up were 30% (3 patients), 40% (4 patients), and 40% (4 patients), respectively.The 24-hour urinary proteinuria and serum albumin levels were improved in 10 children after RTX treatment, but there were no significant statistical difference(all P>0.05). No significant difference was found in humoral immunity and renal function before and after RTX treatment (all P>0.05). During the treatment and follow-up, 3 patients (30%) developed infusion reaction, 2 patients (20%) showed severe pulmonary infection, and 1 patient (10%) died of severe pulmonary infection. Conclusions:RTX is effective in treating some children with refractory SRNS, and a long-term follow-up should be conducted to prevent infection.

Objective:To discuss the etiology, clinical manifestations and renal pathological features of acute interstitial nephritis (AIN) in children.Methods:The etiology, clinical manifestations, pathological characteristics, clinical effects and outcome of the children with AIN diagnosed by renal biopsy from January 2010 to December 2019 in Nanjing Jinling Hospital were analyzed retrospectively. The Kaplan-Meier method was used to evaluate the kidney survival rate. Cox regression model was built to analyze the risk factors for developing end-stage renal disease (ESRD) at baseline in AIN children.Results:A total of 51 children with AIN were diagnosed by renal biopsy, including 36 males and 15 females. The age was (12.94±2.55) years old (2-17 years old). The clinical manifestations of AIN in children were various and lack of specificity. Only 2 cases (3.92%) had triad. All of the 51 children with AIN showed acute renal injury (AKI), accompanied by increased serum creatinine and decreased estimated glomerular filtration rate. The stage of AKI was mainly stageⅢ(33 cases, 64.71%). Infection was the main cause (38 cases, 74.51%) and drug factor was the second cause (27 cases, 52.94%) in children with AIN. Nonsteroidal antiinflammatory drugs (NSAIDs) were the main inducers of drug-induced AIN (18 cases, 35.29%). The interstitial inflammatory cell infiltration or interstitial edema was found in 51 children. The infiltration of inflammatory cells was mainly mononuclear cells (46 cases, 90.20%). After 4 weeks of treatment, 32 cases (62.75%), 11 cases (21.57%) and 8 cases (15.69%) showed complete, partial and no recovery of renal function, respectively. After 12 weeks of treatment, 49 cases (96.08%), 0 cases (0) and 2 cases (3.92%) showed complete, partial and no recovery of renal function, respectively. After an average follow-up of 4.0(2.0-15.0) months, 2 case (3.92%) patients developed ESRD. The cumulative survival rates of ESRD at 1 year and 2 years after renal biopsy both were 100%, and renal survival rates at 5 years and 10 years were 96.55% and 72.41%, respectively. Multivariate Cox regression analysis results showed that N-acetyl-β-D-glucosidase (NAG) enzyme level>17.6 U/g·cr ( HR=15.729, 95% CI 1.045-15.977, P=0.042) and IgM deposition in renal tissue ( HR=7.523, 95% CI 1.142-9.541, P=0.033) were independent risk factors for developing ESRD in AIN children. Conclusions:AKI is the main clinical manifestation of AIN in children. The characteritic of renal pathology in AIN is tubulointerstitial lesions. After active treatment, most of the patients have a good prognosis. Prevention of infection and rational use of drugs are the key to reduce the incidence rate of AIN in children. The N-acetyl-β-D-glucosidase enzyme level>17.6 U/g·cr and IgM deposition in renal tissue are independent risk factors for developing ESRD in AIN children.

Objective The article was to analyze the features of gene mutation and clinical phenotype in Alport syndrome. Methods Next-generation sequencing was applied to capture the exons of COL4A3, COL4A4, COL4A5 genes in 30 cases of children with suspected or confirmed diagnosis of Alport syndrome and Sanger method was used to identify gene mutations of related family mem-bers.Provean database was applied in protein function prediction.We collected and analyzed clinical data of AS patients on the basis of gene mutation. Results All 30 children were diagnosed with AS by gene sequencing, among whom 4 boys were autosomal reces-sive inheritance, 16 boys and 10 girls were X-linked Alport syndrome.Next-generation sequencing detected 35 different gene mutations of COL4A3, COL4A4, COL4A5, including 19 missense mutations, 2 synonymous mutations, 4 splice-site mutations, 3 truncating mu-tations, 2 insertion mutations, 4 deletion mutations and 1 compound mutations.It was observed by Sanger sequencing that 20 mutations were inherited from the mother, 8 from the father, homozygous mutation in 1 propositus from the parents respectively, 8 novel mutations and 1 with unidentified source.All the 30 children had an onset of hematuria or proteinuria, 17 cases had a positive family history, 1 case had hearing loss, and no pathogenesis or renal insufficiency was found in the children.Renal biopsy was performed on 23 children, 13 minimal change disease ( MCD) and 10 mesangial proliferative glo-merulonephritis ( MsPNG) by light microscope.Extensive lamination and split of glomerular basement membrane dense layers were found in 9 children by electron microscope. Conclusion XLAS ac-counts for most AS patients and missense mutation is the main type in pathogenic mutations.Altogether, 31 mutations without disease notification were found.Most of children showed MCD in renal biopsy, with atypical electron microscope manifestations and rare extra renal manifestations.