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

Recent data suggest that vascular endothelial growth factor receptor inhibitor (VEGFRi) can enhance the anti-tumor activity of the anti-programmed cell death-1 (anti-PD-1) antibody in colorectal cancer (CRC) with microsatellite stability (MSS). However, the comparison between this combination and standard third-line VEGFRi treatment is not performed, and reliable biomarkers are still lacking. We retrospectively enrolled MSS CRC patients receiving anti-PD-1 antibody plus VEGFRi (combination group, n=54) or VEGFRi alone (VEGFRi group, n=32), and their efficacy and safety were evaluated. We additionally examined the immune characteristics of the MSS CRC tumor microenvironment (TME) through single-cell and spatial transcriptomic data, and an MSS CRC immune cell-related signature (MCICRS) that can be used to predict the clinical outcomes of MSS CRC patients receiving immunotherapy was developed and validated in our in-house cohort. Compared with VEGFRi alone, the combination of anti-PD-1 antibody and VEGFRi exhibited a prolonged survival benefit (median progression-free survival: 4.4 vs. 2.0 months, P=0.0024; median overall survival: 10.2 vs. 5.2 months, P=0.0038) and a similar adverse event incidence. Through single-cell and spatial transcriptomic analysis, we determined ten MSS CRC-enriched immune cell types and their spatial distribution, including naive CD4⁺ T, regulatory CD4⁺ T, CD4⁺ Th17, exhausted CD8⁺ T, cytotoxic CD8⁺ T, proliferated CD8⁺ T, natural killer (NK) cells, plasma, and classical and intermediate monocytes. Based on a systemic meta-analysis and ten machine learning algorithms, we obtained MCICRS, an independent risk factor for the prognosis of MSS CRC patients. Further analyses demonstrated that the low-MCICRS group presented a higher immune cell infiltration and immune-related pathway activation, and hence a significant relation with the superior efficacy of pan-cancer immunotherapy. More importantly, the predictive value of MCICRS in MSS CRC patients receiving immunotherapy was also validated with an in-house cohort. Anti-PD-1 antibody combined with VEGFRi presented an improved clinical benefit in MSS CRC with manageable toxicity. MCICRS could serve as a robust and promising tool to predict clinical outcomes for individual MSS CRC patients receiving immunotherapy.
Humans ; Colorectal Neoplasms/drug therapy* ; Male ; Female ; Immunotherapy ; Middle Aged ; Aged ; Tumor Microenvironment/immunology* ; Retrospective Studies ; Microsatellite Instability ; Transcriptome ; Single-Cell Analysis ; Programmed Cell Death 1 Receptor/immunology* ; Gene Expression Profiling ; Immune Checkpoint Inhibitors/therapeutic use* ; Adult ; Receptors, Vascular Endothelial Growth Factor/antagonists & inhibitors*

Objective:To develop an integrated health management system based on wearable devices for conducting health management to discharged patients,so as to improve the lifestyle and medication compliance of patients with cardiovascular and cerebrovascular diseases,and control risk factors of disease,and maintain patients'safety.Methods:The wearable devices,mobile terminals,and hospital's medical information platform were systematically integrated to develop an integrated health management system.A total of 75 patients with cardiovascular and cerebrovascular diseases(coronary heart disease and hypertension)who admitted to the Department of Cardiovascular Medicine of The 940th Hospital of People's Liberation Army Joint Service Support Force during March 1 and April 1,2024 were selected,and they were randomly divided into an intervention group and a control group using the random number table method,with 38 cases in the intervention group and 37 cases in the control group.During the 6 months of intervention observation period after discharge,patients in the intervention group used the integrated health management system for self-health management,while the patients of control group were managed with the conventional mode.The rate of medication compliance,changes of health behaviors,and changes of measurement data of body between the two groups were compared after the intervention.Results:The smoking rate of patients in the intervention group was 18.42%(7/38),which was lower than 43.24%(16/37)of the control group,and the difference was statistically significant(x2=3.94,P<0.05).The average rate of medication compliance of patients in the intervention group was(89.00±2.39)%,which was higher than(84.8±2.37)%of the control group,and the difference was statistically significant(t=2.15,P<0.05).The increase in diastolic blood pressure of patients in the intervention group was(1.76±2.06)mmHg,which was lower than(3.05±1.94)mmHg of the control group,and the difference was statistically significant(t=2.49,P<0.05).Conclusion:The integrated health management system based on wearable devices is effective for the self-management of patients with cardiovascular and cerebrovascular diseases,and it has a good effect in controlling the level of blood pressure,improving behavioral habits,and enhancing medication compliance of patients.

Objective:To investigate the relationship between work fit, work stress, and occupational fatigue among medical staff, and to analyze the independent and interactive effects of work fit and work stress on occupational fatigue.Methods:A stratified random sampling method was employed to select 1162 employees from two top-tier hospitals as the research subjects. A questionnaire survey was conducted to collect information on basic demographics, work stress, work fit, and occupational fatigue. Chi-square test, analysis of variance, and logistic regression equations were used to evaluate the interactive effects of work fit and work stress on occupational fatigue.Results:This study included 1162 medical Homo sapiens personnel, among whom 884 were job-adapted and 362 were in a stressful state. Univariate logistic regression analysis showed that the risk of occupational fatigue significantly increased in the 40-49 age group ( OR=1.89) and the bachelor's degree group ( OR=2.52, P<0.01). Smoking and alcohol consumption were also associated with a significantly higher risk of occupational fatigue ( P<0.01). In terms of occupational characteristics, compared to clinical doctors, nurses and pharmaceutical/technical Homo sapiens personnel had lower risks ( OR=0.57, 0.43, P<0.05). Compared to high-ranking Homo sapiens personnel, those with no title or junior titles exhibited lower risks ( OR=0.51, 0.43, P<0.05). Working hours exceeding 55 hours per week and night shifts 3-4 times per week significantly increased fatigue risk ( OR=1.94, 1.90, P<0.05). Occupational stress and job discomfort were identified as risk factors for occupational fatigue ( OR=11.94, 3.26, P<0.05). Job adaptation and occupational stress exhibited a multiplicative interaction on physical fatigue after adjusting for confounders[ OR (95% CI) =0.43 (0.22, 0.84) ]. Additionally, job adaptation and occupational stress demonstrated an additive interaction on mental fatigue, with RERI (95% CI) =2.64 (0.11, 5.59), API (95% CI) =0.38 (0.08, 0.67), and SI (95% CI) =1.78 (1.01, 3.14). Medical Homo sapiens personnel in a stressful state had a 7.25-fold higher risk of fatigue compared to those not in a stressful state, while those with job discomfort had a 2.01-fold higher risk compared to those in an adapted state. Conclusion:Occupational stress and job fit have interactive effects on physical fatigue and mental fatigue in medical Homo sapiens personnel. A stressful state combined with job discomfort increases the risk of fatigue occurrence, providing a basis for developing intervention strategies for occupational fatigue among medical Homo sapiens personnel.

[Purpose]To analyze the results of upper gastrointestinal cancer screening in rural areas of Lhasa from 2015 to 2019.[Methods]Upper gastrointestinal cancer screening program was conducted among residents aged 40～69 years old from 3 rural project sites in Lhasa selected by cluster sampling method from 2015 to 2019.The detected malignant tumors and precancerous le-sions were treated and confirmed by pathological examination.The detection rate of esophagus and stomach cancer were calculated by region,sex and age and compared by x2 test.[Results]A total of 1 399 people underwent gastroscopy,among whom 1 288 completed pathological biopsy(92.07％).Three cases of esophageal cancer were detected with a detection rate of 0.21％(3/1 399),all of which were advanced squamous cell carcinoma.Eight cases of gastric cancer were detected with a detection rate of 0.57％(8/1 399),and 2 cases were early gastric cancer.The detection rate of low-grade and high-grade epithelial neoplasia of gastric mucosa was 0.64％(9/1 399)and 0.21％(3/1 399),respectively.There was no significant difference in the detection rate among different regions,sexes and age groups(all P＞0.05).[Conclusion]The detection rate of upper gastrointesti-nal cancer in screening population in Lhasa is lower than the national average level.Men and the elderly are important target populations for upper gastrointestinal cancer screening,and increasing participation rates is necessary for more effective screening outcomes.

Objective:To investigate the impact of immunosuppressive therapy on the severity of SARS-CoV-2 infection and cytokine levels in pediatric patients with kidney diseases.Methods:A retrospective analysis was conducted on the clinical data of 40 hospitalized pediatric patients who were diagnosed with SARS-CoV-2 infection at the 900th Hospital of PLA Joint Logistic Support Force from December 2022 to February 2023. Based on their immunosuppressive status prior to SARS-CoV-2 infection, these patients were categorized into immunosuppressive group and non-immunosuppressive group. Independent sample t-tests, Mann-Whitney U tests, and χ2 test were employed to compare the clinical baseline characteristics and laboratory data, the severity of SARS-CoV-2 infection, and the levels of cytokines between the 2 groups. Results:Among the 40 patients, 11 were in the immunosuppressive group (aged 13 (8, 14) years, 9 males and 2 females) and 29 in the non-immunosuppressive group (aged 2 (1, 4) years, 15 males and 14 females). In the immunosuppressive group, 2 were asymptomatic cases, 8 were mild cases, and 1 was moderate case, and there was no severe or critical cases. In the non-immunosuppressive group, 8 were mild cases, 5 were moderate, 15 were severe cases, 1 was critical case, and no asymptomatic cases. The underlying diseases in the immunosuppressive group included nephrotic syndrome (6 cases), IgA vasculitis nephritis (2 cases), lupus nephritis (1 case), post-renal transplantation (1 case), and renal failure (1 case), with a mean total immunosuppression score (TIS) of (3.6±1.4) points. In the non-immunosuppressive group, 2 patients had a history of epilepsy, and the remaining 27 cases had no underlying conditions, all with TIS scores of 0. Compared to the children in the non-immunosuppressive group, those in the immunosuppressive group were more likely to exhibit asymptomatic or mild infection, with lower risks of severe disease, cytokine storm, fever, and cough, but a higher risk of fatigue ( OR=1.22, 2.66, 0.48, 0.12, 0.12, 0.13, 1.22; 95% CI 0.93-1.62, 0.99-7.15, 0.33-0.70, 0.03-0.57, 0.03-0.57, 0.03-0.65, 0.93-1.62; all P<0.05). The levels of cytokine IL-6, interferon-α and interferon-γ in the immunosuppressive group were all lower than those in the non-immunosuppressive group ( Z=2.23, 2.51, 2.92, respectively; all P<0.05). Conclusion:Pediatric patients with kidney diseases receiving appropriate immunosuppressive therapy may mitigate the severity of SARS-CoV-2 infection by suppressing the expression of cytokines.

Objective:To investigate the drug resistance gene characteristics, plasmid characteristics and genome tracing of Shigella sonnei causing a bacillary dysentery outbreak in Shandong Province. Methods:Sixty-five Shigella sonnei strains isolated from a 2021 outbreak in a county of Shandong Province were analyzed using antimicrobial susceptibility testing, whole genome sequencing (WGS), characterization of resistance and virulence genes, plasmid profiling, core genome multilocus sequence typing (cgMLST), and single nucleotide polymorphism (SNP) analysis. Results:All isolates had the same resistance phenotype and genotypes and were multidrug-resistant ESBL-producing Shigella sonnei, carrying important virulence genes. Plasmid analysis revealed a conserved genetic arrangement, pil( M/ N/ O2/ P)-tra( F/ H/ J/ K/ N/ O/ P/ Q)-IS Ecp1- blaCTX-M-14-Tn 903- yub( J/ I/ F/ G/ E/ D), and shared across strains from diverse regions and bacterial species. The cgMLST and SNP analyses demonstrated concordant clustering, with all 65 outbreak-related strains forming a single cluster alongside human-derived strains from Guangxi. Conclusion:The ESBL-producing Shigella sonnei responsible for the outbreak shares a homologous relationship with Guangxi human-derived strains, and the detected resistance plasmids and virulence genes underscore the need to strengthen drug resistance surveillance and genome tracing.

von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary tumor predisposition syndrome. Central nervous system hemangioblastoma is one of its major clinical manifestations, commonly occurring in the infratentorial region and spinal cord, while supratentorial involvement is rare. A 23-year-old female patient with sellar and spinal hemangioblastomas, along with polycystic pancreas, who presented with back pain and decreased vision in the left eye, was reported. The diagnosis of VHL disease was confirmed based on family history, pathological findings, and genetic testing. This case was reported to enhance clinicians′ awareness of this disease, facilitating early diagnosis and intervention for patients and their families to improve clinical outcomes.

Objective To analyse impact of low-dose CT on radiation dose and image quality of ankle fractures in children.Methods 600 suspected ankle fracture children admitted to Hangzhou Children's Hospital were selected as the research subjects from January to December 2023.All children needed to undergo conventional dose CT examination and low-dose CT examination.Image quality,radiation dose,diagnostic accuracy,and diagnostic efficacy of conventional dose CT and low-dose CT examination were compared.Results No significant difference was found between conventional-dose CT examination and low-dose CT examination in objectivity image quality index(P＞0.05).Effective dose,dose length product,and volume CT dose index of low-dose CT examination were all lower than those of conventional dose CT examination,and the difference was statistically significant(P＜0.05).The diagnostic accuracy of low-dose CT examination and conventional dose CT examination showed no significant difference(P＞0.05).Low-dose CT examination compared with conventional dose CT examination,there was no significant difference in diagnostic specificity(P＞0.05),while there was significant difference in diagnostic sensitivity and accuracy(P＜0.05).Area under the curve of effective dose,dose length product,volume CT dose index alone and combined diagnosis of ankle fractures in children were 0.899(95％CI:0.880-0.918),0.934(95％CI:0.918-0.950),0.928(95％CI:0.911-0.945),and 0.934(95％CI:0.918-0.950),respectively.Conclusion Low-dose CT examination technology has high diagnostic value for ankle fractures in children,can reduce radiation dose,and has little impact on image quality and diagnostic accuracy.It is worth popularizing and applying.

To investigate the clinical utilization of Rituximab biosimilar (Rituximab injection) in order to provide references for evaluating its rationality and economic value in clinical application.