BACKGROUND: Generalized pustular psoriasis (GPP), a rare and recurrent autoinflammatory disease, imposes a substantial burden on patients and society. Awareness of GPP in China remains limited. METHODS: This cross-sectional survey, conducted between September 2021 and May 2023 across 14 hospitals in China, included GPP patients of all ages and disease phases. Data collected encompassed demographics, clinical characteristics, economic impact, disease severity, quality of life, and treatment-related complications. Risk factors for GPP recurrence were analyzed. RESULTS: Among 127 patients (female/male ratio = 1.35:1), the mean age of disease onset was 25 years (1st quartile [Q1]-3rd quartile [Q3]: 11-44 years); 29.2% had experienced GPP for more than 10 years. Recurrence occurred in 75.6% of patients, and nearly half reported no identifiable triggers. Younger age at disease onset ( P  = 0.021) and transitioning to plaque psoriasis ( P  = 0.022) were associated with higher recurrence rates. The median diagnostic delay was 8 months (Q1-Q3: 2-41 months), and 32.3% of patients reported misdiagnoses. Comorbidities were present in 53.5% of patients, whereas 51.1% experienced systemic complications during treatment. Depression and anxiety affected 84.5% and 95.6% of patients, respectively. During GPP flares, the median Dermatology Life Quality Index score was 19.0 (Q1-Q3: 13.0-23.5). This score showed significant differences between patients with and without systemic symptoms; it demonstrated correlations with both depression and anxiety scores. Treatment costs caused financial hardship in 55.9% of patients, underscoring the burden associated with GPP. CONCLUSIONS The substantial disease and economic burdens among Chinese GPP patients warrant increased attention. Patients with early onset disease and those transitioning to plaque psoriasis require targeted interventions to mitigate the high recurrence risk.
Humans ; Male ; Female ; Psoriasis/pathology* ; Adult ; Cross-Sectional Studies ; Adolescent ; Child ; Young Adult ; Quality of Life ; Middle Aged ; China/epidemiology* ; Recurrence ; Risk Factors ; Surveys and Questionnaires ; East Asian People

OBJECTIVES: To evaluate the safety and efficacy of thiotepa (TT)-containing conditioning regimens for allogeneic hematopoietic stem cell transplantation (HSCT) in children with inborn errors of immunity (IEI). METHODS: Clinical data of 22 children with IEI who underwent HSCT were retrospectively reviewed. Survival after HSCT was estimated using the Kaplan-Meier method. RESULTS: Nine patients received a traditional conditioning regimen (fludarabine + busulfan + cyclophosphamide/etoposide) and underwent peripheral blood stem cell transplantation (PBSCT). Thirteen patients received a TT-containing modified conditioning regimen (TT + fludarabine + busulfan + cyclophosphamide), including seven PBSCT and six umbilical cord blood transplantation (UCBT) cases. Successful engraftment with complete donor chimerism was achieved in all patients. Acute graft-versus-host disease occurred in 12 patients (one with grade III and the remaining with grade I-II). Chronic graft-versus-host disease occurred in one patient. The incidence of EB viremia in UCBT patients was lower than that in PBSCT patients (P<0.05). Over a median follow-up of 36.0 months, one death occurred. The 3-year overall survival (OS) rate was 100% for the modified regimen and 88.9% ± 10.5% for the traditional regimen (P=0.229). When comparing transplantation types, the 3-year OS rates were 100% for UCBT and 93.8% ± 6.1% for PBSCT (P>0.05), and the 3-year event-free survival rates were 100% and 87.1% ± 8.6%, respectively (P>0.05). CONCLUSIONS TT-containing conditioning for allogeneic HSCT in children with IEI is safe and effective. Both UCBT and PBSCT may achieve high success rates.
Humans ; Retrospective Studies ; Transplantation Conditioning/methods* ; Thiotepa/therapeutic use* ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Male ; Female ; Child, Preschool ; Infant ; Child ; Transplantation, Homologous ; Graft vs Host Disease ; Adolescent

Ovarian tumor (OT) is the most lethal form of gynecologic malignancy, with minimal improvements in patient outcomes over the past several decades. Metastasis is the leading cause of ovarian cancer-related deaths, yet the underlying mechanisms remain poorly understood. Psychological stress is known to activate the glucocorticoid receptor (NR3C1), a factor associated with poor prognosis in OT patients. However, the precise mechanisms linking NR3C1 signaling and metastasis have yet to be fully elucidated. In this study, we demonstrate that chronic restraint stress accelerates epithelial-mesenchymal transition (EMT) and metastasis in OT through an NR3C1-dependent mechanism involving nuclear protein 1 (NUPR1). Mechanistically, NR3C1 directly regulates the transcription of NUPR1, which in turn increases the expression of snail family transcriptional repressor 2 (SNAI2), a key driver of EMT. Clinically, elevated NR3C1 positively correlates with NUPR1 expression in OT patients, and both are positively associated with poorer prognosis. Overall, our study identified the NR3C1/NUPR1 axis as a critical regulatory pathway in psychological stress-induced OT metastasis, suggesting a potential therapeutic target for intervention in OT metastasis.

Peri-implant keratinized mucosa (PIKM) augmentation refers to surgical procedures aimed at increasing the width of PIKM. Consensus reports emphasize the necessity of maintaining a minimum width of PIKM to ensure long-term peri-implant health. Currently, several surgical techniques have been validated for their effectiveness in increasing PIKM. However, the selection and application of PIKM augmentation methods may present challenges for dental practitioners due to heterogeneity in surgical techniques, variations in clinical scenarios, and anatomical differences. Therefore, clear guidelines and considerations for PIKM augmentation are needed. This expert consensus focuses on the commonly employed surgical techniques for PIKM augmentation and the factors influencing their selection at second-stage surgery. It aims to establish a standardized framework for assessing, planning, and executing PIKM augmentation procedures, with the goal of offering evidence-based guidance to enhance the predictability and success of PIKM augmentation.
Humans ; Consensus ; Dental Implants ; Mouth Mucosa/surgery* ; Keratins

Objective:This study aims to determine the risk perception, evacuation decision-making, and influencing factors related to typhoon disasters among public.Methods:A cross-sectional study will be conducted in residents of Hainan from September 2024 to January 2025. The study will employ chi-square tests, t-tests, and binary logistic regression analysis to examine factors influencing evacuation behavior decisions. Furthermore, structural equation modeling based on ordered choice model will be constructed to validate the mechanisms through which risk perception affects evacuation decision-making processes.Results:Among 517 respondents from 11 administrative districts (counties) in Hainan Province, 62.09% of the residents were willing to evacuate. The decision of public evacuation behavior was significantly related to disaster knowledge, official trust, risk perception, source of warning information and some demographic characteristics. Structural equation modeling demonstrated that warning information sources positively correlated with disaster knowledge and official trust (path coefficients: 0.363, 0.315, both P < 0.001). Both disaster knowledge and official trust showed positive correlations with risk perception (path coefficients: 0.157, 0.165, both P < 0.001), while risk perception positively influenced evacuation decisions (path coefficient: 0.137, P = 0.003). Moreover, source of warning information, disaster knowledge and official trust further positively affect evacuation behavior decision through risk perception, a key mediating variable. Conclusions:Disaster knowledge, official trust, risk perception and other factors can directly or indirectly affect the evacuation behavior of the public following the typhoon disaster. In typhoon-prone areas, it is recommended to enhance public risk perception through strategies such as authoritative training, diversified early warning channels, and improving government credibility, thereby promoting disaster preparedness and evacuation behaviors.

OBJECTIVE To analyze the relationship between microRNA-186(miR-186),forkhead box D1(FOXD1)expression carcinoma tissues and epithelial-mesenchymal transition(EMT)and prognosis in nasopharyngeal carcinoma.METHODS A total of 155 patients with nasopharyngeal cancer(nasopharyngeal cancer group)admitted to Shaanxi University of Traditional Chinese Medicine Affiliated Hospital from March 2018 to May 2021 were selected,and 155 patients with chronic nasopharyngeal mucosal inflammation(control group)were selected.The expression of miR-186 and FOXD1 and the expression of EMT genes[E-cad,N-cad,and Snail]were detected in the diseased mucosal tissue.The relationship between miR-186 and FOXD1 expression and EMT gene,clinicopathological characteristics and prognosis of patients with nasopharyngeal carcinoma was analyzed.RESULTS Compared with the control group,the expression of miR-186 and E-cad mRNA in the nasopharyngeal carcinoma group was decreased,and the expression of FOXD1 mRNA,Snail mRNA and N-cad mRNA was increased(P<0.05).Pearson said,miR-186 was positively correlated with the expression of E-cad mRNA,and negatively correlated with the expression of FOXD1,Snail and N-cad mRNA(r=0.506,-0.336,-0.438,-0.626,P<0.05).FOXD1 mRNA was negatively correlated with the expression of E-cad mRNA,and positively correlated with the expression of Snail and N-cad mRNA(r=-0.469,0.417,0.562,P<0.05).There were significant differences in the relative expression levels of miR-186 and FOXD1 mRNA in nasopharyngeal carcinoma patients with different degrees of differentiation,TNM stage,lymph node metastasis,and distant metastasis(P<0.05).The 3-year overall survival rate of the low miR-186 expression group was lower than that of the high miR-186 expression group(P<0.05).The 3-year overall survival rate of the low FOXD1 expression group was higher than that of the high FOXD1 expression group(P<0.05).CONCLUSION The expression of miR-186 and FOXD1 in patients with nasopharyngeal carcinoma is correlated with EMT gene,tissue differentiation,TNM stage,lymph node metastasis,distant metastasis and prognosis,which can be used as potential markers to evaluate the malignant progression and prognosis of nasopharyngeal carcinoma.

The 2024 National Education Work Conference pointed out that at the current juncture of the critical period for achieving the goals and tasks of the 14th Five-Year Plan,the implementation of the Education Powerhouse Construction Plan Outline should be taken as the main line of work,and building first-class disciplines is an crucial task for a higher education powerhouse.In 2022,forensic medicine was officially listed as a first-level discipline under the medical category,presenting an un-precedented historical opportunity for the development of forensic medicine.The forensic medicine dis-cipline of Southern Medical University comprehensively improves the quality of talent cultivation and facilitates the construction of first-class disciplines as its main direction.It aims to initiate and imple-ment a high-level faculty team building plan featuring"combining recruitment and cultivation,inter-disciplinary integration";make vigorous efforts to establish a first-level doctoral program,refine advan-tageous second-level disciplines and research directions;and establish an innovative research platform from a high starting point with deep integration.The discipline adheres to moral cultivation and the Five Domains of Education simultaneous development,to build a high-quality talent joint training model.Guided by the construction of the national legal system and industry needs,the discipline will enhance social service capabilities.The forensic medicine construction in our university will continue to contribute to the rule of law in China and educational power.

To explore the understanding of the target population regarding the Get Active Questionnaire for Pregnancy(GAQ-P)and the Companion Health Care Provider Consultation Form for Prenatal Physical Activity(cHCP-CF-PPA)in the Chinese context,and to verify the consistency of the Chinese version of the prenatal physical activity dual screening questionnaire with the original version in terms of language expression,27 pregnant women and 12 healthcare providers were selected from Obstetrics and Gynecology Hospital,Fudan University during Aug and Oct 2023,and were interviewed using purposive sampling.Two rounds of cognitive interviews were conducted.The first round revealed that some respondents experienced ambiguities in understanding the meanings of 5 items in the questionnaire.Following modifications,the second round indicated that the revised items were consistent in meaning with the original questionnaire.Cognitive interviews can facilitate the adaptation of the prenatal physical activity dual screening questionnaire to the Chinese cultural context,improve the understanding of the questionnaire items among the target population,and promote the localization of the screening tool.

Objective:To evaluate the therapeutic efficacy of an upper limb rehabilitation robot on the recovery of elbow function recovery following arthroscopic elbow joint release.Methods:Seventy-two patients who underwent arthroscopic elbow joint release at Shanghai Sixth People’s Hospital from December 2022 to December 2023 were recruited. All patients were randomly assigned to either the conventional group (n=36; 16 males, 20 females; age 34.39±9.04 years, range 24-56; fractures: 4 intercondylar humerus, 18 olecranon, 8 radial head, 6 other) which received conventional rehabilitation postoperatively, or the robot-assisted group (n=36; 18 males, 18 females; age 33.78±9.98 years, range 20-59; fractures distribution identical to the conventional group) receiving conventional rehabilitation combined with robot-assisted upper limb rehabilitation therapy. The active range of motion (ROM) of elbow joint, Mayo elbow performance score (MEPS), visual analogue scale (VAS) of elbow joint were recorded preoperatively and at 4 weeks and 3 months postoperatively. The surface electromyography were analyzed at 3 months postoperatively.Results:All patients completed the 3-month rehabilitation program. At 4 weeks postoperatively, the conventional group exhibited a mean active ROM of 106.78°±9.91°, MEPS of 67.78±7.68, VAS of 2.11±0.74; the robot-assisted group showed active ROM of 113.72°±7.06°, MEPS of 73.33±9.28, VAS of 21.89±0.46. By 3 months postoperatively, the conventional group achieved a mean active ROM of 118.11°±6.75°, MEPS of 85.00±8.66, VAS of 0.67±0.67; robot-assisted achieved a mean active ROM of 127.61°±6.61°, MEPS of 91.11±6.57, VAS of 0.39±0.49. Both groups exhibited significant improvements in active ROM and MEPS, and significant reductions in VAS scores at 4 weeks and 3 months postoperatively compared with preoperatively values ( P<0.05). The robot-assisted group demonstrated significantly higher active ROM and MEPS at both 4 weeks and 3 months postoperatively, and a significantly lower VAS score at 3 months postoperatively, compared with the conventional group ( P<0.05). Surface electromyography at 3 months revealed significantly higher biceps brachii root mean square and significantly lower co-contraction index in the robot-assisted group compared to the conventional group ( P<0.05). No adverse symptoms were reported in the treated elbows of either group during the operation and follow-up period. Conclusion:The integration of upper limb rehabilitation robot-assisted therapy and conventional rehabilitation program significantly enhances the recovery of elbow range of motion and functional outcomes at 3 months following arthroscopic elbow joint release.

Objective:To investigate the the differential expression of EIF5A2 in intrahepatic cholangiocarcinoma cell lines RBE,HCCC9810,and HUCCT1,and its effects on HCCC9810 cell migration and invasion,epithelial mesenchymal transition,and PI3K/AKT signaling pathway.Methods:The differential expression of EIF5A2 in RBE,HCCC9810,and HUCCT1 cell lines was detected using WB method.The HCCC9810 cell line,with the highest expression of EIF5A2,was selected for this experiment.The expression of EIF5A2 in HCCC9810 cell line was silenced by transient transfection of small interfering RNA.The best silencing effect of small interfering RNA was screened by WB.Scratch assay and Tran-swell migration invasion assay were used to detect the effect of silencing EIF5A2 on the migration and invasion ability of HCCC9810 cells.WB was used to detect the effect of silencing EIF5A2 on PI3K/AKT signaling pathway and epithelial mesenchymal transition in HCCC9810 cells.Results:The WB results showed that EIF5A2 had the highest expression in the HCCC9810 cell line,and siRNA1 had the best silencing effect on EIF5A2 in the HCCC9810 cell line.Scratch assay and Transwell migration invasion assay results showed that silencing EIF5A2 in the HCCC9810 cell line resulted in a decrease in cell invasion and metastasis ability(P＜0.05).At the same time,the expression of p-PI3K and p-AKT in the PI3K/AKT signaling pathway was significantly decreased(P＜0.05),while the epithelial cell marker E-cadherin expression increased(P＜0.05)and the stromal cell marker N-cadherin expression decreased(P＜0.05).Conclusion:EIF5A2 may promote epi-thelial mesenchymal transition and enhance the migration and invasion ability of intrahepatic cholangiocarcinoma cells through the PI3K/AKT signaling pathway.