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

Aim Ischemic stroke(IS)is caused by acute ischemia of cerebral blood vessels,leading to brain tissue damage and neuronal apoptosis.The pathogenesis is complex,involving multiple cell death modes such as pyropto-sis,ferroptosis and disulfide death.Disulfide death is a newly discovered form of death that helps to explore the patholog-ical mechanisms of various diseases from a new perspective.The aim of this study is to discover and validate the differen-tial expression of disulfide death-related genes in blood samples of ischemic patients and their association with immune regu-lation.Methods The relevant datasets of clinical patients(GSE16561 and GSE37587)were obtained through online big data.Differentially expressed genes related to disulfide death were identified,and gene enrichment analysis was con-ducted to further explore the potential mechanisms.Subsequently,immune cell infiltration was analyzed to investigate the dysregulation of immune cells in the context of IS.Finally,the accuracy of key genes was verified through ROC curves,column charts,calibration curves,and decision curves,and a disease prediction model was constructed to predict the risk of stroke.Results Based on this dataset,significant differential expression of 9 genes related to disulfide death was identified.Independent external validation was conducted using the microarray dataset GSE58294.Single item compari-sons were performed on these differentially expressed genes in blood samples from 69 IS patients and 23 normal individuals.The results showed that the trends of LRPPRC,MYH9,NDUFA11,PRDX1 and RPN1,the 5 differentially expressed genes,were consistent.Immune infiltration analysis found that differentially expressed genes such as TLN1,MYH9,PRDX1,LRPPRC,NDUFA11 were also strongly correlated with CD8+T cells,activated NK cells,macrophages,and neu-trophils in IS patients.Functional enrichment analysis emphasized the important role of pathways such as focal adhesion,platelet aggregation,and activation in the occurrence and development of diseases.By using a column chart model for risk prediction,it was shown that the accuracy of these differentially expressed genes was good,and the ROC curve AUC value of the optimized combination of disulfide death-related genes could reach 0.844.Further validation through an external dataset(GSE58294)revealed that the ROC curve AUC value optimized for disulfide death-related genes reached 0.989,which had good clinical guidance significance for the risk of IS.Conclusions This study confirmed the existence of 5 disulfide death-related genes in IS patients through a dataset,including upregulation of MYH9 and downregulation of LRP-PRC,NDUFA11,PRDX1 and RPN1.These gene alterations are suggested to influence IS disease progression and prog-nosis through immune inflammation and bleeding risk.

Aim Ischemic stroke(IS)is caused by acute ischemia of cerebral blood vessels,leading to brain tissue damage and neuronal apoptosis.The pathogenesis is complex,involving multiple cell death modes such as pyropto-sis,ferroptosis and disulfide death.Disulfide death is a newly discovered form of death that helps to explore the patholog-ical mechanisms of various diseases from a new perspective.The aim of this study is to discover and validate the differen-tial expression of disulfide death-related genes in blood samples of ischemic patients and their association with immune regu-lation.Methods The relevant datasets of clinical patients(GSE16561 and GSE37587)were obtained through online big data.Differentially expressed genes related to disulfide death were identified,and gene enrichment analysis was con-ducted to further explore the potential mechanisms.Subsequently,immune cell infiltration was analyzed to investigate the dysregulation of immune cells in the context of IS.Finally,the accuracy of key genes was verified through ROC curves,column charts,calibration curves,and decision curves,and a disease prediction model was constructed to predict the risk of stroke.Results Based on this dataset,significant differential expression of 9 genes related to disulfide death was identified.Independent external validation was conducted using the microarray dataset GSE58294.Single item compari-sons were performed on these differentially expressed genes in blood samples from 69 IS patients and 23 normal individuals.The results showed that the trends of LRPPRC,MYH9,NDUFA11,PRDX1 and RPN1,the 5 differentially expressed genes,were consistent.Immune infiltration analysis found that differentially expressed genes such as TLN1,MYH9,PRDX1,LRPPRC,NDUFA11 were also strongly correlated with CD8+T cells,activated NK cells,macrophages,and neu-trophils in IS patients.Functional enrichment analysis emphasized the important role of pathways such as focal adhesion,platelet aggregation,and activation in the occurrence and development of diseases.By using a column chart model for risk prediction,it was shown that the accuracy of these differentially expressed genes was good,and the ROC curve AUC value of the optimized combination of disulfide death-related genes could reach 0.844.Further validation through an external dataset(GSE58294)revealed that the ROC curve AUC value optimized for disulfide death-related genes reached 0.989,which had good clinical guidance significance for the risk of IS.Conclusions This study confirmed the existence of 5 disulfide death-related genes in IS patients through a dataset,including upregulation of MYH9 and downregulation of LRP-PRC,NDUFA11,PRDX1 and RPN1.These gene alterations are suggested to influence IS disease progression and prog-nosis through immune inflammation and bleeding risk.

Changes in structure of oral solid dosage forms (OSDF) elementally determine the drug release and its therapeutic effects. In this research, synchrotron radiation X-ray micro-computed tomography was utilized to visualize the 3D structure of enteric coated pellets recovered from the gastrointestinal tract of rats. The structures of pellets in solid state and in vitro compendium media were measured. Pellets in vivo underwent morphological and structural changes which differed significantly from those in vitro compendium media. Thus, optimizations of the dissolution media were performed to mimic the appropriate in vivo conditions by introducing pepsin and glass microspheres in media. The sphericity, pellet volume, pore volume and porosity of the in vivo esomeprazole magnesium pellets in stomach for 2 h were recorded 0.47, 1.55 × 10⁸ μm³, 0.44 × 10⁸ μm³ and 27.6%, respectively. After adding pepsin and glass microspheres, the above parameters in vitro reached to 0.44, 1.64 × 10⁸ μm³, 0.38 × 10⁸ μm³ and 23.0%, respectively. Omeprazole magnesium pellets behaved similarly. The structural features of pellets between in vitro media and in vivo condition were bridged successfully in terms of 3D structures to ensure better design, characterization and quality control of advanced OSDF.