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

Humanistic caring for patients in the operating room refers to providing the whole process of caring medical services for patients in the operating room. In order to standardize humanistic caring services for patients in the operating room of medical institutions, improve the comprehensive service level of the operating room, and enhance the surgical experience of patients, the Chinese Association for Life Care released the group standard " Humanistic Caring Management Standards for Patients in the Operating Room" in December 2023. This article interpreted the basic requirements for humanistic caring of patients in the operating room, the environment and facilities for humanistic caring, the procedures and measures for humanistic caring, and the quality management framework, aiming to assist administrators and clinical practitioners across various levels of medical institutions in accurately understanding and effectively implementing the standard, and to provide essential textual reference and practical guidance for promoting the application of the standard.

Objective:To detect pathogenic gene mutations in a patient with CARD14-associated papulosquamous eruption (CAPE) and his family, and to evaluate the short-term and long-term efficacy and safety of ustekinumab treatment.Methods:Clinical data were collected from a 6-year-old child presenting with erythroderma secondary to pityriasis rubra pilaris and from his family members. Peripheral blood samples were collected from the patient, his parents and younger brother, and DNA was extracted from these peripheral blood samples. A next-generation skin-targeted sequencing panel was used to detect gene mutations, and Sanger sequencing was performed for verification. Genomic DNA was also extracted from 100 unrelated healthy controls using the same method.Results:A heterozygous mutation c.349+1G>A in intron 6 of the CARD14 gene was identified in the proband and his mother, but was not identified in his father, younger brother or 100 healthy controls, leading to a diagnosis of CAPE in the proband. The patient received subcutaneous injections of ustekinumab at a dose of 45 mg at weeks 0 and 4, and every 12 weeks thereafter. After 1-week treatment, the skin lesions and symptoms improved markedly. No adverse events were reported during the 18-month treatment and follow-up.Conclusion:The heterozygous mutation c.349+1G>A in the CARD14 gene may be the cause of the disease in this family, and ustekinumab was effective and safe for the treatment of CAPE.

Objective To investigate the potential categories of nutrition literacy in young and middle-aged patients with type 2 diabetes mellitus combined with overweight or obesity,and analyze the relationship between various latent categories and dietary motivation,so as to provide references for clinical working staff to develop relevant dietary intervention strategies.Methods A convenient sampling method was used to enroll 270 young and middle-aged patients with type 2 diabetes mellitus combined with overweight or obesity from the endocrine department of a tertiary general hospital in Zhenjiang City,Jiangsu province,using basic information questionnaire,Nutrition Literacy Measurement Scale for Chinese adults,and the Regulation of Eating Behaviors Scale to collect data,using latent profile analysis to extract nutrition literacy potential categories,with general data and potential categories as independent variables,dietary motivation as the dependent variable for univariate analysis and multiple linear regression analysis.Results A total of 262 young and middle-aged patients with type 2 diabetes mellitus combined with overweight or obesity completed the survey,and their nutrition literacy could be divided into 3 potential categories:low nutrition literacy group(16.41％),transformation dilemma group(54.96％),and middle and high nutrition literacy group(28.63％).The results of multiple linear regression analysis showed that residency style,potential categories of nutrition literacy and presence of diabetic complications were the influencing factors of dietary motivation in young and middle-aged patients with type 2 diabetes mellitus combined with overweight or obesity(P＜0.05).Conclusion There was a group heterogeneity in nutrition literacy in young and middle-aged patients with type 2 diabetes mellitus combined with overweight or obesity.Clinical working staff should provide targeted interventions based on the patient's potential categories of nutrition literacy to stimulate ongoing motivation to maintain a healthy diet.

Objective To investigate the expression and regulatory role of adenosine triphosphate binding transporter C4(ABCC4)in patients with type 2 diabetes mellitus(T2DM)and macrovascular disease(MD).Methods A total of 137 patients with T2DM were enrolled in this study from Zhongnan Hospital of Wuhan University during March 2015 and March 2017.All the participants were divided into T2DM group(n=64)and T2DM with MD group(n=73)according to the presence or absence of MD.Another 75 healthy subjects were selected as normal control(NC)group.The general data and biochemical indicators were collected and compared among the three groups.The mRNA expression level of ABCC4 was detected by real-time fluorescence quantitative PCR(qPCR),and the correlation between each indicator and ABCC4 mRNA was analyzed.ABCC4 knockout stable HepG2 cell line was constructed and transcriptome sequencing analysis(RNA-Seq)was performed.Results BMI,FPG,TC,TG and LDL-C were higher,while HDL-C was lower in T2DM and MD groups than in NC group(P<0.05).The expression levels of SBP and ABCC4 mRNA were higher in MD group than in T2DM and NC groups(P<0.05).Spearman correlation analysis showed that the level of ABCC4 mRNA was negatively correlated with cholinesterase(r=-0.359,P<0.05)and positively correlated with lipoprotein(a)(r=0.241,P<0.05).A total of 280 differentially expressed genes were screened out by RNA-Seq in ABCC4 knockout cell lines.GO and KEGG analysis showed that these differentially expressed genes were mainly enriched in biological functions such as vesicle transport and growth factor activity.It is involved in cytokine receptor interaction,glycation products-receptor for advanced glycation end products signaling pathway of DM complications,leukocyte transendothelial migration and other pathways.Conclusions ABCC4 is elevated in patients with T2DM and macrovascular disease.ABCC4 might play a role in occurrence and development of macrovascular disease through affecting the biological function of membrane,inflammation and glycation metabolism pathways.

Objective To conduct a scoping review of research on the relationship between 24-hour movement behaviours and cognitive function in the elderly,so as to provide references for future research.Methods Totally 8 Chinese and English databases were systematically searched.The search period spanned from the establishment of databases until November 30,2024.According to the scoping review framework,2 researchers independently screened the studies,extracted the data of the included studies,and collaborated on the final analysis.Results A total of 12 papers were included,and the distribution of 24-hour movement behaviors in the elderly was as follows:3.0-5.4 h/d for light physical activity,0.6-1.5 h/d for moderate vigorous physical activity,8.9-12.3 h/d for sedentary behaviour and 6.7-8.4 h/d for sleep.Measures included objective,subjective and subject-objective measures.There are complex interactions among physical activity,sedentary behavior,sleep and cognitive function,which jointly affect cognitive function in the elderly.Conclusion Despite current differences in the measurement of 24-hour movement behaviours,the decreased physical activity,the increased sedentary time,and insufficient or excessive sleep are common problems among the elderly.By increasing physical activity,reducing sedentary behavior and ensuring adequate sleep,the overall cognitive function,executive function of the elderly can be improved.In the future,we should pay attention to the time allocation of 24-hour movement behaviours of the elderly,develop accurate and standardized measurement methods,and promote research in this field.

Objective:To evaluate the clinical status of long-term oral anticoagulant management in patients with atrial fibrillation, and to analyze the obstacles and promoting factors in the implementation of evidence, so as to provide evidence for clinical transformation.Methods:This study was a cross-sectional study. Through the evidence-based nursing method, guided by the Ottawa Model, the review indicators and review methods were developed based on the best evidence. The clinical status review of 100 patients who met the inclusion criteria in the Department of Cardiology, Affiliated Hospital of Jiangsu University was conducted from August to November 2022 using a convenience sampling method, and based on the results of the review, an analysis of obstacles and promoting factors was carried out to develop a clinical response.Results:This study included 100 patients with atrial fibrillation, 60 males and 40 females, aged (70.14 ± 10.39) years. Totally 26 best items were included, and 24 review indicators were developed, among which only 4 indicators had a compliance rate of 100% , 2 indicators had a compliance rate of >60% , 5 indicators had a compliance rate of <60% , and 13 indicators had a compliance rate of 0. According to the results of the review, a comprehensive and systematic analysis was conducted from the three dimensions of system, practitioners, patients and family members. For each dimension, obstacles and promoting factors were analyzed in depth from evidence-based change, potential adopters and practical environment.Conclusions:There is a gap between the best evidence and clinical status of long-term oral anticoagulant management in patients with atrial fibrillation. In-depth analysis of obstacle factors, formulate countermeasures, and promote the efficient implementation of evidence in clinical practice.

Objective:To detect pathogenic gene mutations in a patient with CARD14-associated papulosquamous eruption (CAPE) and his family, and to evaluate the short-term and long-term efficacy and safety of ustekinumab treatment.Methods:Clinical data were collected from a 6-year-old child presenting with erythroderma secondary to pityriasis rubra pilaris and from his family members. Peripheral blood samples were collected from the patient, his parents and younger brother, and DNA was extracted from these peripheral blood samples. A next-generation skin-targeted sequencing panel was used to detect gene mutations, and Sanger sequencing was performed for verification. Genomic DNA was also extracted from 100 unrelated healthy controls using the same method.Results:A heterozygous mutation c.349+1G>A in intron 6 of the CARD14 gene was identified in the proband and his mother, but was not identified in his father, younger brother or 100 healthy controls, leading to a diagnosis of CAPE in the proband. The patient received subcutaneous injections of ustekinumab at a dose of 45 mg at weeks 0 and 4, and every 12 weeks thereafter. After 1-week treatment, the skin lesions and symptoms improved markedly. No adverse events were reported during the 18-month treatment and follow-up.Conclusion:The heterozygous mutation c.349+1G>A in the CARD14 gene may be the cause of the disease in this family, and ustekinumab was effective and safe for the treatment of CAPE.

Objective To investigate the expression and regulatory role of adenosine triphosphate binding transporter C4(ABCC4)in patients with type 2 diabetes mellitus(T2DM)and macrovascular disease(MD).Methods A total of 137 patients with T2DM were enrolled in this study from Zhongnan Hospital of Wuhan University during March 2015 and March 2017.All the participants were divided into T2DM group(n=64)and T2DM with MD group(n=73)according to the presence or absence of MD.Another 75 healthy subjects were selected as normal control(NC)group.The general data and biochemical indicators were collected and compared among the three groups.The mRNA expression level of ABCC4 was detected by real-time fluorescence quantitative PCR(qPCR),and the correlation between each indicator and ABCC4 mRNA was analyzed.ABCC4 knockout stable HepG2 cell line was constructed and transcriptome sequencing analysis(RNA-Seq)was performed.Results BMI,FPG,TC,TG and LDL-C were higher,while HDL-C was lower in T2DM and MD groups than in NC group(P<0.05).The expression levels of SBP and ABCC4 mRNA were higher in MD group than in T2DM and NC groups(P<0.05).Spearman correlation analysis showed that the level of ABCC4 mRNA was negatively correlated with cholinesterase(r=-0.359,P<0.05)and positively correlated with lipoprotein(a)(r=0.241,P<0.05).A total of 280 differentially expressed genes were screened out by RNA-Seq in ABCC4 knockout cell lines.GO and KEGG analysis showed that these differentially expressed genes were mainly enriched in biological functions such as vesicle transport and growth factor activity.It is involved in cytokine receptor interaction,glycation products-receptor for advanced glycation end products signaling pathway of DM complications,leukocyte transendothelial migration and other pathways.Conclusions ABCC4 is elevated in patients with T2DM and macrovascular disease.ABCC4 might play a role in occurrence and development of macrovascular disease through affecting the biological function of membrane,inflammation and glycation metabolism pathways.

Objective:To evaluate the clinical status of long-term oral anticoagulant management in patients with atrial fibrillation, and to analyze the obstacles and promoting factors in the implementation of evidence, so as to provide evidence for clinical transformation.Methods:This study was a cross-sectional study. Through the evidence-based nursing method, guided by the Ottawa Model, the review indicators and review methods were developed based on the best evidence. The clinical status review of 100 patients who met the inclusion criteria in the Department of Cardiology, Affiliated Hospital of Jiangsu University was conducted from August to November 2022 using a convenience sampling method, and based on the results of the review, an analysis of obstacles and promoting factors was carried out to develop a clinical response.Results:This study included 100 patients with atrial fibrillation, 60 males and 40 females, aged (70.14 ± 10.39) years. Totally 26 best items were included, and 24 review indicators were developed, among which only 4 indicators had a compliance rate of 100% , 2 indicators had a compliance rate of >60% , 5 indicators had a compliance rate of <60% , and 13 indicators had a compliance rate of 0. According to the results of the review, a comprehensive and systematic analysis was conducted from the three dimensions of system, practitioners, patients and family members. For each dimension, obstacles and promoting factors were analyzed in depth from evidence-based change, potential adopters and practical environment.Conclusions:There is a gap between the best evidence and clinical status of long-term oral anticoagulant management in patients with atrial fibrillation. In-depth analysis of obstacle factors, formulate countermeasures, and promote the efficient implementation of evidence in clinical practice.