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

Objective:To investigate clinical characteristics, treatment approaches, and prognosis of drug-induced hypersensitivity syndrome (DIHS) in children.Methods:A retrospective analysis was performed on clinical data from pediatric inpatients with DIHS in Department of Dermatology, Beijing Children's Hospital from 2009 to 2023. The clinical data included demographic characteristics, clinical manifestations, laboratory findings, treatment regimens, and outcomes.Results:A total of 103 children with DIHS were included, comprising 54 males (52.4%) and 49 females (47.6%), with ages ( M [ Q1, Q3]) of 2.3 (1.2, 4.5) years. Primary causative drugs were antibiotics (52 cases, 45.2%), antiepileptic drugs (41 cases, 35.7%), and nonsteroidal anti-inflammatory drugs (19 cases, 16.5%), with a median latency period of 12 days. All patients presented with rashes, including 72 (69.9%) with maculopapular rashes, 69 (67.0%) with edema (including 46 with facial edema). Lip involvement occurred in 25 cases (24.3%), and mucosal involvement was noted in 11 cases (10.7%). Additionally, 102 (99.0%) patients had fever, and 79 (76.7%) presented with lymphadenectasis. Eosinophilia was present in 64 cases (62.1%). Among 84 patients tested for atypical lymphocytes, 51 (60.7%) showed elevated percentages of atypical lymphocytes. Liver involvement was noted in 94 cases (91.3%), followed by pulmonary involvement in 31 (30.1%), gastrointestinal symptoms in 25 (24.3%), cardiac involvement in 14 (13.6%), renal involvement in 10 (9.7%), and pancreatic involvement in 7 cases (6.8%). Among 82 patients tested for blood immunocytes, 49 (59.8%) showed decreased percentages of B lymphocytes, and 69 (84.1%) showed decreased percentages of natural killer cells. Of 88 patients tested for serum immunoglobulins, 40 (45.5%) showed decreased IgA levels. Among 20 patients tested for serum cytokines, 15 (75.0%), 15 (75.0%), 13 (65.0%), and 12 (60.0%) showed elevated levels of interleukin (IL) -5, IL-6, IL-10, and interferon-γ, respectively. All patients received systemic glucocorticoid therapy, among whom 86 additionally received intravenous immunoglobulin therapy, 4 received Janus kinase inhibitors, and 3 received dupilumab. Five patients died, 9 developed hemophagocytic lymphohistiocytosis, 6 developed bronchiolitis obliterans, and 5 experienced long-term immune-related sequelae. Conclusions:Among these children with DIHS, antibiotics were the most common causative drugs, and the latency period could be shorter than 2 weeks. In addition to the common involvement of the liver and lungs, gastrointestinal and cardiac impairments were relatively frequent, while renal involvement was rare. Immunological features included decreased percentages of B lymphocytes and natural killer cells, reduced IgA levels, and elevated levels of cytokines such as IL-5, IL-6, IL-10, and interferon-γ.

Objective To investigate the longitudinal relationship between sleep duration(SD)and positive youth development(PYD)among primary and secondary school students in Chengdu city using a cross-lagged model,and to provide scientific evidence for enhancing sleep management practices for students.Methods A total of 4061 students of grades 3 through 9 from the Chengdu Child Positive Development Cohort were included in this three-wave longitudinal study.There was a one-year interval between one survey and the following round of survey,and the time points for the baseline,12-month follow-up,and 24-month follow-up surveys were designated T0,T1,and T2.The PYD of the participants was assessed using the Chinese version of the Positive Youth Development Scale.The demographic data and the average daily SD over the past month were collected.Spearman correlation analysis was performed to examine the associations between SD and PYD,and a cross-lagged model was used to investigate the longitudinal relationship between them.Results The average daily SD for the 3 rounds of surveys conducted at T0,T1,and T2 was 9.00(8.04,10.00)hours,10.44(9.67,11.11)hours,and 10.39(9.83,11.00)hours,respectively,while the PYD scores were 5.30(4.73,5.71),5.27(4.73,5.73),and 5.39(4.89,5.77),respectively.Statistical significance was found in the differences of SD and PYD scores across the 3 rounds(P＜0.05).Spearman correlation analysis revealed synchronous correlations between SD and PYD at all three time points(r=0.10 at T0,r=0.18 at T1,and r=0.21 at T2,P＜0.05)and significant lagged correlations(e.g.,r=0.10 for T1-PYD and T2-SD,and likewise,significant correlation was found for the 3 other cross-lagged paths).The cross-lagged model demonstrated that PYD at T0 and T1 positively predicted SD at T1 and T2,respectively(β0-1=0.116[95％CI,0.083-0.150],β1-2=0.097(95％CI,0.067-0.127),P＜0.05),and that SD at T0 and T1 also positively predicted PYD at T1 and T2(β0-1=0.028[95％CI,0-0.056],β1-2=0.042[95％CI,0.010-0.074],P＜0.05).According to these findings,a bidirectional predictive relationship between SD and PYD across different time points was observed in primary and secondary school students.Furthermore,PYD demonstrated better performance for predicting SD than SD did for PYD.Subgroup analysis by sex confirmed the robustness of the predictive power of PYD for SD.Conclusion This study reveals a positive bidirectional predictive relationship between SD and PYD among primary and secondary school students,suggesting that higher levels of PYD may contribute to adequate sleep.These findings provide critical scientific evidence for schools and families to strengthen sleep management and promote the holistic development and well-being of adolescents.

Extracorporeal membrane oxygenation (ECMO) serves as an advanced life support technology and plays a critical role in treating critically ill patients with severe cardiopulmonary failure. However, the unique physiological and pathological changes during pregnancy present numerous challenges and special considerations for the application of ECMO in obstetrics. Given the current lack of unified guidelines specifically for obstetric ECMO, clinical practice often relies on adapting general ECMO guidelines in combination with obstetric-specific factors. This article systematically reviews the core principles of existing general ECMO guidelines, provides an in-depth analysis of the impact of gestational physiological and pathological characteristics on ECMO application, and discusses the particularities and necessary adaptations of obstetric ECMO in terms of indications, anticoagulation strategies, parameter adjustment, multidisciplinary collaboration, and emergency management. Furthermore, it proposes strategies suitable for domestic clinical practice. The article also addresses existing difficulties and limitations not covered by current guidelines and calls for the urgent development of specialized obstetric ECMO guidelines to provide clinicians with more standardized and safer decision-making support, ultimately improving outcomes for critically ill pregnant patients and newborns.

Objective:To investigate clinical characteristics, treatment approaches, and prognosis of drug-induced hypersensitivity syndrome (DIHS) in children.Methods:A retrospective analysis was performed on clinical data from pediatric inpatients with DIHS in Department of Dermatology, Beijing Children's Hospital from 2009 to 2023. The clinical data included demographic characteristics, clinical manifestations, laboratory findings, treatment regimens, and outcomes.Results:A total of 103 children with DIHS were included, comprising 54 males (52.4%) and 49 females (47.6%), with ages ( M [ Q1, Q3]) of 2.3 (1.2, 4.5) years. Primary causative drugs were antibiotics (52 cases, 45.2%), antiepileptic drugs (41 cases, 35.7%), and nonsteroidal anti-inflammatory drugs (19 cases, 16.5%), with a median latency period of 12 days. All patients presented with rashes, including 72 (69.9%) with maculopapular rashes, 69 (67.0%) with edema (including 46 with facial edema). Lip involvement occurred in 25 cases (24.3%), and mucosal involvement was noted in 11 cases (10.7%). Additionally, 102 (99.0%) patients had fever, and 79 (76.7%) presented with lymphadenectasis. Eosinophilia was present in 64 cases (62.1%). Among 84 patients tested for atypical lymphocytes, 51 (60.7%) showed elevated percentages of atypical lymphocytes. Liver involvement was noted in 94 cases (91.3%), followed by pulmonary involvement in 31 (30.1%), gastrointestinal symptoms in 25 (24.3%), cardiac involvement in 14 (13.6%), renal involvement in 10 (9.7%), and pancreatic involvement in 7 cases (6.8%). Among 82 patients tested for blood immunocytes, 49 (59.8%) showed decreased percentages of B lymphocytes, and 69 (84.1%) showed decreased percentages of natural killer cells. Of 88 patients tested for serum immunoglobulins, 40 (45.5%) showed decreased IgA levels. Among 20 patients tested for serum cytokines, 15 (75.0%), 15 (75.0%), 13 (65.0%), and 12 (60.0%) showed elevated levels of interleukin (IL) -5, IL-6, IL-10, and interferon-γ, respectively. All patients received systemic glucocorticoid therapy, among whom 86 additionally received intravenous immunoglobulin therapy, 4 received Janus kinase inhibitors, and 3 received dupilumab. Five patients died, 9 developed hemophagocytic lymphohistiocytosis, 6 developed bronchiolitis obliterans, and 5 experienced long-term immune-related sequelae. Conclusions:Among these children with DIHS, antibiotics were the most common causative drugs, and the latency period could be shorter than 2 weeks. In addition to the common involvement of the liver and lungs, gastrointestinal and cardiac impairments were relatively frequent, while renal involvement was rare. Immunological features included decreased percentages of B lymphocytes and natural killer cells, reduced IgA levels, and elevated levels of cytokines such as IL-5, IL-6, IL-10, and interferon-γ.

Extracorporeal membrane oxygenation (ECMO) serves as an advanced life support technology and plays a critical role in treating critically ill patients with severe cardiopulmonary failure. However, the unique physiological and pathological changes during pregnancy present numerous challenges and special considerations for the application of ECMO in obstetrics. Given the current lack of unified guidelines specifically for obstetric ECMO, clinical practice often relies on adapting general ECMO guidelines in combination with obstetric-specific factors. This article systematically reviews the core principles of existing general ECMO guidelines, provides an in-depth analysis of the impact of gestational physiological and pathological characteristics on ECMO application, and discusses the particularities and necessary adaptations of obstetric ECMO in terms of indications, anticoagulation strategies, parameter adjustment, multidisciplinary collaboration, and emergency management. Furthermore, it proposes strategies suitable for domestic clinical practice. The article also addresses existing difficulties and limitations not covered by current guidelines and calls for the urgent development of specialized obstetric ECMO guidelines to provide clinicians with more standardized and safer decision-making support, ultimately improving outcomes for critically ill pregnant patients and newborns.

The effect and mechanism of chronic stress on psychosomatic diseases are important topics in stress research.The establishment of animal models to simulate human chronic stress is of great significance to investigations of stress responses,the pathogenesis of related diseases,clinical treatment,and drug development.In this paper,animal models of chronic stress commonly used in China and abroad are reviewed;the classification of stressors,animal selection,model construction,pathological manifestations,and evaluation indexes are summarized;and the advantages and disadvantages and application of various models are discussed.The paper provides a reference for the study and selection of models of chronic stress response.

The Nod-like receptor pyrin domain-associated protein 3(NLRP3)-mediated pyroptosis of pulmonary parenchymal and immune cells plays a key role in the pathogenesis of lung injury during sepsis.NF-κB,JAK2/STAT3 and MAPK signaling pathways are involved in NLRP3-mediated pyroptosis.Targeting NLRP3-pyroptosis and its related signaling pathways,pharmacological interventions with Physalin B,schisandrin,erythropoietin,and physical therapies such as acupuncture at Zusanli and Feishu points,as well as NLRP3-specific inhibitors like ergolide,have all shown effective anti-septic effects in treating lung injuries.This article reviewed the roles and mechanisms of NLRP3-pyroptosis in sepsis-induced lung injury,as well as the experimental progresses made in targeting NLRP3 pyroptosis as a therapy.We aim to highlight the importance of NLRP3-pyroptosis as a target,providing insights for the prevention and treatment of sepsis-induced lung injury.

Objective : To explore the method of intraperitoneal injection of allogenic fecal filtrate to establish the rat model of moderate and severe sepsis． Methods: The preparation method of allogeneic fecal filtrate was determined．Allogeneic fecal filtrate of different concentrations (0. 5，1，2 g / kg) was injected intraperitoneally to observe the general situation，survival time and severe degree of sepsis of rats. After determining the optimal concentration，the success rate of the model，serum inflammatory factors，serum concentration of D-lactic acid ( D-LA) and serum intestinal fatty acid binding protein (I-FABP) ，lung function changes，lung，liver and kidney tissue injury were further observed. Results: After intraperitoneal injection of allogenic fecal filtrate for 24 h，the rats of 1 g / kg group presented fever，tachypnea and hypotension，the survival rate was 83. 3% at 24 h and 16. 7% at 48 h， 2 g / kg group rats all died within 24 h，the dose of 1 g / kg was determined for subsequent experiments．Injected fecal filtrate for 24 h，the success rate of the sepsis model was 77. 8% . The levels of interleukin-6 ( IL-6) ，tumor necrosis factor-α ( TNF-α) ，D-LA and I-FABP in serum significantly increased． There were severe edema and bleeding in lung tissue，Pulmonary function appeared respiratory dysfunction，included functional residual capacity (FRC) ，quasi static compliance ( Cdyn) ，forced expiratory volume for the first 100 milliseconds(FEV100) ，peak expiratory flow (PEF) decreased，airway resistance (RI) ，inspiratory capacity (IC) increased．Liver and kidney tissues also showed varying degrees of edema and inflammatory cell infiltration，the levels of alanine aminotransferase (ALT) ，aspartate aminotransferase (AST) ，blood urea nitrogen (BUN) and creatinine ( Cr) in serum significantly increased． Conclusion Intraperitoneal injection of allogenic fecal filtrate ( 1 g / kg) can produce a relative typical septic model in rats.

Esophageal squamous cell carcinoma (ESCC) is one of the leading causes of cancer death worldwide. It is urgent to develop new drugs to improve the prognosis of ESCC patients. Here, we found benzydamine, a locally acting non-steroidal anti-inflammatory drug, had potent cytotoxic effect on ESCC cells. Benzydamine could suppress ESCC proliferation in vivo and in vitro. In terms of mechanism, CDK2 was identified as a target of benzydamine by molecular docking, pull-down assay and in vitro kinase assay. Specifically, benzydamine inhibited the growth of ESCC cells by inhibiting CDK2 activity and affecting downstream phosphorylation of MCM2, c-Myc and Rb, resulting in cell cycle arrest. Our study illustrates that benzydamine inhibits the growth of ESCC cells by downregulating the CDK2 pathway.
Humans ; Benzydamine ; Esophageal Neoplasms/drug therapy* ; Esophageal Squamous Cell Carcinoma/drug therapy* ; Molecular Docking Simulation ; Phosphorylation ; Cell Proliferation ; Cell Line, Tumor ; Apoptosis ; Cyclin-Dependent Kinase 2