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 observe value of real-time functional MRI neurofeedback(rtfMRI-NF)for modulating brain activity changes in obese adults.Methods Twenty-two obese adults were prospectively recruited,3-week rtfMRI-NF training intervention was conducted.Barratt impulsivityness scale version 11(BIS-11),three-factor eating questionnaire(TFEQ)and food rating scales were scored before and after intervention,and whole-brain resting state fMRI(rs-fMRI)data were obtained.Clinical scale scores,rs-fMRI regional homogeneity(ReHo)values and amplitude of low frequency fluctuation(ALFF)values were compared before and after intervention.Then brain regions with differences of ReHo and ALFF values before and after intervention were explored,and correlations of values of rs-fMRI data differences and clinical scale score differences were analyzed.Results BIS-11 score,TFEQ emotional eating(TFEQ-EE)score and food rating scales scores decreased,while TFEQ cognitive restraint(TFEQ-CR)scores increased in obese adults after intervention(all P＜0.05).ReHo values in right inferior frontal gyrus,right anterior cingulate and left precuneus increased,while of left middle temporal gyrus decreased(all corrected P＜0.05).ALFF values of left precuneus increased,whereas of left middle occipital gyrus,right superior occipital gyrus,left calcarine fissure and surrounding cortex and left supramarginal and angular gyrus decreased(all corrected P＜0.05).ReHo difference in right anterior cingulate was negatively correlated with BIS-11 scores difference(r=-0.601,P＜0.05),and ALFF difference in left precuneus was negatively correlated with TFEQ-EE difference(r=-0.478,P＜0.05).Conclusion rtfMRI-NF intervention could correct disorder of cerebral functional areas in obese adults and change high-calorie food preference and poor dietary habits.

Objective:To explore the direct effect of safety organization on work engagement of Operating Room nurses, as well as the mediating effect of psychological capital between safety organization and work engagement, so as to provide theoretical basis for developing intervention programs to enhance work engagement of Operating Room nurses.Methods:From October to December 2024, convenience sampling was used to select 260 Operating Room nurses from three ClassⅢ Grade A hospitals as research subjects. Psychological Capital Questionnaire, Safety Organizing Scale, and Work Engagement Scale were used to survey Operating Room nurses. AMOS 26.0 software was used to analyze the mediating effect of Operating Room nurses' psychological capital between safety organization and work engagement.Results:A total of 260 questionnaires were distributed and 241 valid questionnaires were recovered, with a valid recovery rate of 92.69%. Among 241 Operating Room nurses, the total scores of Work Engagement Scale, Psychological Capital Questionnaire, and Safety Organizing Scale were (37.35±7.30), (90.10±11.62), and (41.58±10.82), respectively. The total score and scores of each dimension of the Work Engagement Scale were positively correlated with the total score and scores of each dimension of the Psychological Capital Questionnaire and the total score of the Safe Organizing Scale ( P<0.05). The total score and scores of each dimension of the Psychological Capital Questionnaire were positively correlated with the total score of the Safe Organizing Scale ( P<0.05). Mediating effect analysis showed that the direct effect of safety organization on work engagement of Operating Room nurses was established, with an effect value of 0.266 ( P<0.05). Psychological capital played a partially mediating effect between safety organization and work engagement of, with an effect value of 0.275, and the mediating effect accounted for 50.83% of the total effect. Conclusions:Safety organization has a positive impact on work engagement of Operating Room nurses, and psychological capital partially mediates the effect between safety organization and work engagement of Operating Room nurses. Hospital nursing administrators should be aware of the importance of building a safety organization and pay attention to building the psychological capital of Operating Room nurses so as to improve work engagement.

Objective:To explore the direct effect of safety organization on work engagement of Operating Room nurses, as well as the mediating effect of psychological capital between safety organization and work engagement, so as to provide theoretical basis for developing intervention programs to enhance work engagement of Operating Room nurses.Methods:From October to December 2024, convenience sampling was used to select 260 Operating Room nurses from three ClassⅢ Grade A hospitals as research subjects. Psychological Capital Questionnaire, Safety Organizing Scale, and Work Engagement Scale were used to survey Operating Room nurses. AMOS 26.0 software was used to analyze the mediating effect of Operating Room nurses' psychological capital between safety organization and work engagement.Results:A total of 260 questionnaires were distributed and 241 valid questionnaires were recovered, with a valid recovery rate of 92.69%. Among 241 Operating Room nurses, the total scores of Work Engagement Scale, Psychological Capital Questionnaire, and Safety Organizing Scale were (37.35±7.30), (90.10±11.62), and (41.58±10.82), respectively. The total score and scores of each dimension of the Work Engagement Scale were positively correlated with the total score and scores of each dimension of the Psychological Capital Questionnaire and the total score of the Safe Organizing Scale ( P<0.05). The total score and scores of each dimension of the Psychological Capital Questionnaire were positively correlated with the total score of the Safe Organizing Scale ( P<0.05). Mediating effect analysis showed that the direct effect of safety organization on work engagement of Operating Room nurses was established, with an effect value of 0.266 ( P<0.05). Psychological capital played a partially mediating effect between safety organization and work engagement of, with an effect value of 0.275, and the mediating effect accounted for 50.83% of the total effect. Conclusions:Safety organization has a positive impact on work engagement of Operating Room nurses, and psychological capital partially mediates the effect between safety organization and work engagement of Operating Room nurses. Hospital nursing administrators should be aware of the importance of building a safety organization and pay attention to building the psychological capital of Operating Room nurses so as to improve work engagement.

Objective To observe value of real-time functional MRI neurofeedback(rtfMRI-NF)for modulating brain activity changes in obese adults.Methods Twenty-two obese adults were prospectively recruited,3-week rtfMRI-NF training intervention was conducted.Barratt impulsivityness scale version 11(BIS-11),three-factor eating questionnaire(TFEQ)and food rating scales were scored before and after intervention,and whole-brain resting state fMRI(rs-fMRI)data were obtained.Clinical scale scores,rs-fMRI regional homogeneity(ReHo)values and amplitude of low frequency fluctuation(ALFF)values were compared before and after intervention.Then brain regions with differences of ReHo and ALFF values before and after intervention were explored,and correlations of values of rs-fMRI data differences and clinical scale score differences were analyzed.Results BIS-11 score,TFEQ emotional eating(TFEQ-EE)score and food rating scales scores decreased,while TFEQ cognitive restraint(TFEQ-CR)scores increased in obese adults after intervention(all P＜0.05).ReHo values in right inferior frontal gyrus,right anterior cingulate and left precuneus increased,while of left middle temporal gyrus decreased(all corrected P＜0.05).ALFF values of left precuneus increased,whereas of left middle occipital gyrus,right superior occipital gyrus,left calcarine fissure and surrounding cortex and left supramarginal and angular gyrus decreased(all corrected P＜0.05).ReHo difference in right anterior cingulate was negatively correlated with BIS-11 scores difference(r=-0.601,P＜0.05),and ALFF difference in left precuneus was negatively correlated with TFEQ-EE difference(r=-0.478,P＜0.05).Conclusion rtfMRI-NF intervention could correct disorder of cerebral functional areas in obese adults and change high-calorie food preference and poor dietary habits.

Gaucher Disease (GD) is an autosomal recessive lysosomal storage disorder characterized by high heterogeneity. This study aimed to further understand the correlation between clinical phenotypes and genotypes in GD patients through a retrospective analysis of 20 cases in Shanxi Bethune Hospital, including their clinical manifestations, laboratory tests, enzyme studies, and genetic results. Among the 20 GD patients, 16 were classified as Type Ⅰ GD with a median age of diagnosis of 24 years, and 4 were classified as Type Ⅲ GD with a median age of diagnosis of 19 years. All patients exhibited splenomegaly and thrombocytopenia, with 16 patients showing skeletal imaging changes, and 5 of them presenting with bone pain symptoms. Genetic analysis revealed 15 distinct mutations, predominantly missense mutations, with L483P being the most prevalent (35.7%), followed by V414L, L303I, and F252I. Mutation sites were predominantly located in exon 7. Noteworthy findings included the first report of the S310G mutation by our research group and the first occurrence of the K196R mutation in the Chinese population. Additionally, the N227S mutation was implicated in a potential association with neuropathy. Despite advancements, Uncertainties still exist in the correlation between clinical phenotypes and genotypes in GD patients.

Objective:To evaluate the efficacy and safety of CD30 antibody-drug conjugates (ADC) brentuximab vedotin (BV) combined with chemotherapy in children with refractory or relapsed classic Hodgkin′s lymphoma (R/R cHL).Methods:Clinical data (including age, gender, B symptoms, clinical stage, previous treatment, etc.) of the 10 R/R cHL children diagnosed and treated at Beijing Children′s Hospital Affiliated to Capital Medical University from October 2021 to August 2023 were analyzed retrospectively. According to the different intensity of chemotherapy drugs, the dose of BV applied in the same course of treatment was 1.8 mg/kg for BV applied once every 3 weeks, and 1.2 mg/kg for BV applied once every 2 weeks. All 10 patients received at least 2 cycles of BV combined with chemotherapy and were evaluated every 2 cycles. The patients were followed up until May 31, 2024. The infusion reactions and adverse reactions after treatment were recorded.Results:In all 10 patients, there were 7 males and 3 females, the age ranged from 5.3-16.9 years, and there were 6 cases of refractory and 4 cases of relapsed. There were 6 cases of nodular sclerosis type, 2 cases of mixed cell type, 1 case of lymphocyte-rich type, and 1 case of lymphodepletion type. There were 5 cases of stage Ⅳ and 5 cases of stage Ⅲ. Previous treatment was mainly chemotherapy, 4 cases received radiotherapy and 1 case received programmed cell death protein 1 (PD-1) antibody therapy. The follow-up time ranged from 9 to 27 months. A total of 43 courses with 49 doses of BV alone or combined with chemotherapy were recorded, and the number of courses was 2 to 10 times. All 10 children responded to the treatment, and 9 achieved complete remission. BV infusion was successfully completed in all cases. A total of 28 cases of grade 3 or above adverse events were recorded, mainly myelosuppression, all of which were related to chemotherapy and did not affect sequential treatment.Conclusion:Brentuximab vedotin has demonstrated efficacy and a tolerable safety profile in the treatment of refractory and relapsed CD30-positive Hodgkin′s lymphoma in children.

Threatened abortion is a common disease of obstetrics and gynecology and one of the diseases responding specifically to traditional Chinese medicine （TCM）. The China Association of Chinese Medicine organized experts in TCM obstetrics and gynecology， Western medicine obstetrics and gynecology， and pharmacology to deeply discuss the advantages of TCM and integrated Chinese and Western medicine treatment as well as the medication plans for threatened abortion. After discussion， the experts concluded that chromosome， endocrine， and immune abnormalities were the key factors for the occurrence of threatened abortion， and the Qi and blood disorders in thoroughfare and conception vessels were the core pathogenesis. In the treatment of threatened abortion， TCM has advantages in preventing miscarriages， alleviating clinical symptoms and TCM syndromes， relieving anxiety， regulating reproductive endocrine and immune abnormalities， personalized and diversified treatment， enhancing efficiency and reducing toxicity， and preventing the disease before occurrence. The difficulty in diagnosis and treatment of threatened abortion with traditional Chinese and Western medicine lies in identifying the predictors of abortion caused by maternal factors and the treatment of thrombophilia. Recurrent abortion is the breakthrough point of treatment with integrated traditional Chinese and Western medicine. It is urgent to carry out high-quality evidence-based medicine research in the future to improve the modern diagnosis and treatment of threatened abortion with TCM.

Objective To explore the correlations of lumbar bone mineral density(BMD)and metabolic syndrome(MS)in adult males.Methods Data of low dose chest CT and quantitative CT of 13 490 adult males were retrospectively analyzed,and lumbar BMD were measured to judge whether MS existed and the degree of MS,and the correlations of lumbar BMD with MS or not and the degree of MS,as well as of lumbar BMD value and the related indicators of MS were assessed.Taken lumbar BMD as the dependent variable,the age,low density lipoprotein cholesterol(LDL-C),blood uric acid(BUA),hemoglobin(Hb)and MS or not were included in multiple linear regression analysis to observe the impact of MS and related indicators on lumbar BMD.Results Among 13 490 adult males,3 900 were found with MS(MS group),while 9 590 were found without MS(non-MS group).Significant difference of lumbar BMD was detected between groups(P=0.001).Lumbar BMD values were negatively correlated with MS(rs=-0.025,P=0.004)and the degree of MS(rs=-0.038,P＜0.001),whereas positively correlated with abdominal obesity,high triglyceride and low HDL-C or not(rs=0.024,0.061,0.036,all P＜0.001)but negatively correlated with hypertension and hyperglycemia or not(rs=-0.135,-0.104,both P＜0.05).After adjustment of age,lumbar BMD of adult males was negatively correlated with MS or not as well as LDL-C(both P＜0.05),but positively correlated with BUA and Hb(both P＜0.001).Conclusion Lumbar BMD was associated with MS in adult males.

Patients with severe trauma require an extremely timely treatment and transfusion plays an irreplaceable role in the emergency treatment of such patients. An increasing number of evidence-based medicinal evidences and clinical practices suggest that patients with severe traumatic bleeding benefit from early transfusion of low-titer group O whole blood or hemostatic resuscitation with red blood cells, plasma and platelet of a balanced ratio. However, the current domestic mode of blood supply cannot fully meet the requirements of timely and effective blood transfusion for emergency treatment of patients with severe trauma in clinical practice. In order to solve the key problems in blood supply and blood transfusion strategies for emergency treatment of severe trauma, Branch of Clinical Transfusion Medicine of Chinese Medical Association, Group for Trauma Emergency Care and Multiple Injuries of Trauma Branch of Chinese Medical Association, Young Scholar Group of Disaster Medicine Branch of Chinese Medical Association organized domestic experts of blood transfusion medicine and trauma treatment to jointly formulate Chinese expert consensus on blood support mode and blood transfusion strategies for emergency treatment of severe trauma patients ( version 2024). Based on the evidence-based medical evidence and Delphi method of expert consultation and voting, 10 recommendations were put forward from two aspects of blood support mode and transfusion strategies, aiming to provide a reference for transfusion resuscitation in the emergency treatment of severe trauma and further improve the success rate of treatment of patients with severe trauma.