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

Rapid and ultrasensitive detection of pathogen-associated biomarkers is vital for the early diagnosis and therapy of bacterial infections. Herein, we developed a close-packed and ordered Au@AgPt array coupled with a cascade triggering strategy for surface-enhanced Raman scattering (SERS) and colorimetric identification of the Staphylococcus aureus biomarker micrococcal nuclease (MNase) in serum samples. The trimetallic Au@AgPt nanozymes can catalyze the oxidation of 3,3',5,5'-tetramethylbenzidine (TMB) molecules to SERS-enhanced oxidized TMB (oxTMB), accompanied by the color change from colorless to blue. In the presence of S. aureus, the secreted MNase preferentially cut the nucleobase AT-rich regions of DNA sequences on magnetic beads (MBs) to release alkaline phosphatase (ALP), which subsequently mediated the oxTMB reduction for inducing the colorimetric/SERS signal fade away. Using this "on-to-off" triggering strategy, the target S. aureus can be recorded in a wide linear range with a limit of detection of 38 CFU/mL in the colorimetric mode and 6 CFU/mL in the SERS mode. Meanwhile, the MNase-mediated strategy characterized by high specificity and sensitivity successfully discriminated between patients with sepsis (n = 7) and healthy participants (n = 3), as well as monitored the prognostic progression of the disease (n = 2). Overall, benefiting from highly active and dense "hot spot" substrate, MNase-mediated cascade response strategy, and colorimetric/SERS dual-signal output, this methodology will offer a promising avenue for the early diagnosis of S. aureus infection.

L-citrulline is a nonprotein amino acid that plays an important role in human health and has great market demand. Although microbial cell factories have been widely used for biosynthesis, there are still challenges such as genetic instability and low efficiency in the biosynthesis of L-citrulline. In this study, an efficient, plasmid-free, non-inducible L-citrulline-producing strain of Escherichia coli BL21(DE3) was engineered by combined strategies. Firstly, a chassis strain capable of synthesizing L-citrulline was constructed by block of L-citrulline degradation and removal of feedback inhibition, with the L-citrulline titer of 0.43 g/L. Secondly, a push-pull-restrain strategy was employed to enhance the L-citrulline biosynthesis, which realized the L-citrulline titer of 6.0 g/L. Thirdly, the NADPH synthesis and L-citrulline transport were strengthened to promote the synthesis efficiency, which achieved the L-citrulline titer of 11.6 g/L. Finally, fed-batch fermentation was performed with the engineered strain in a 3 L fermenter, in which the L-citrulline titer reached 44.9 g/L. This study lays the foundation for the industrial production of L-citrulline and provides insights for the modification of other amino acid metabolic networks.
Citrulline/biosynthesis* ; Escherichia coli/genetics* ; Metabolic Engineering/methods* ; Fermentation ; NADP/biosynthesis*

Objective To observe the value of 18F-FDG PET/CT semi-quantitative parameters for predicting spread through air spaces(STAS)of clinical stage Ⅰa—Ⅲa lung adenocarcinoma.Methods Data of 85 patients with clinical stage Ⅰa—Ⅲ a lung adenocarcinoma who underwent preoperative 18F-FDG PET/CT were retrospectively analyzed.The patients were divided into positive group(n=23)or negative group(n=62)according to whether pathology showed STAS or not.Clinical and PET/CT data were compared between groups,and logistic analysis was performed to explore the efficacy of each parameter for predicting STAS.Results Significant differences of gender,carcinoma embryonic antigen,clinical stage,pathological grade,micropapillary growth and proportion were found between groups(all P＜0.05).The maximum,the mean,the peak standard uptake value(SUVmax,SUVmean,SUVpeak),as well as the maximum,the mean and the peak standard uptake value normalized by lean body mass(SULmax,SULmean,SULpeak),also the total lesion glycolysis(TLG)in positive group were all significantly higher than those in negative group(all P＜0.05).Patients'gender,proportion of micropapillary growth,SUVmax and SULmax were all independent risk factors of STAS of clinical stage Ⅰa—Ⅲa lung adenocarcinoma.The area under the curve(AUC)of the above parameters for predicting STAS was 0.666,0.912,0.839 and 0.842,respectively,and of the combination was 0.957.Conclusion 18 F-FDG PET/CT semi-quantitative parameters SUVmax and SULmax were helpful for predicting STAS of clinical stage Ⅰa—Ⅲ a lung adenocarcinoma,and further combination of gender and proportion of micropapillary growth could improve diagnostic efficacy.

Objective To observe the value of the scoring model of MRI features for predicting proliferative hepatocellular carcinoma(HCC).Methods Data of 241 patients with pathologically confirmed HCC,including 90 cases of proliferative HCC and 151 cases of non-proliferative HCC were analyzed retrospectively.Univariate and multivariate logistic regression were used to compare the clinical and MRI findings evaluated according to liver imaging reporting and data system version 2018 between groups.The independent predictive factors of proliferative HCC were screened,and scores were assigned according to the weight,then a scoring model was constructed.Receiver operating characteristic(ROC)curve was drawn,and the area under the curves(AUC)were calculated to assess the predictive efficacy of this model.The patients were divided into high and low proliferation risk subgroups based on the optimal score thresholds.The recurrence free survival(RFS)rates and early RFS rates were compared between groups and subgroups.Results MRI showed tumor corona enhancement,arterial phase annular hyper-enhancement,intratumoral vessels,much focus parenchymal low enhancement and irregular tumor margins were all independent predictive factors for proliferative HCC(OR=3.287,2.362,4.542,2.997,2.379,all P＜0.05),which were then were scored with 7,5,9,7 and 5,respectively,with a total score of 0-33.AUC of the obtained scoring model for predicting proliferative HCC was 0.818.Taken 9 points as the optimal score thresholds,97 cases were assigned into high proliferation subgroup and 144 into low proliferation risk subgroups).Significant differences of RFS rates and early RFS rates were found between groups and subgroups(all P＜0.05).Conclusion MRI features scoring model could effectively predict proliferative HCC.

This study was designed to investigate the effect of hypoxia on preeclampsia(PE)by modulating the Src/Siglec-6/SHP2 signaling pathway in the cytoplasm of trophoblast cells.Mouse model of PE was established in normal control and Siglec-6 knockdown mice by L-NAME administration,with aims of studying the changes in vascular diameter of spiral arteries in vivo and examining the expression levels of Siglec-6,p-Src,p-Shp2 and p-ERK1/2 proteins in mouse uterine vascular tissues.While,the effect of Src/Siglec-6/SHP2 on the invasive proliferation of trophoblast cells was explored by culturing human chorionic trophoblast cells HTR-8/SVneo with hypoxia in vitro.In vivo experimental assays showed that the diameter of spiral arteries was reduced in the Siglec-6 knockdown group of mice,and the expression levels of Siglec-6,p-Src,p-SHP2 and p-ERK1/2 proteins were significantly reduced.In vitro hypoxic HTR-8/SVneo cell model results revealed that Siglec-6 overexpression could promote trophoblast cell invasion and proliferation by regulating p-Src,p-SHP2,p-ERK1/2,MMP2,P53 and P21.While,suppression of Src and SHP2 eliminated Siglec-6 overexpression-mediated Siglec-6,p-Src,p-SHP2 and p-ERK1/2 expression,and inhibited the ability of Siglec-6 overexpression to mediate trophoblast invasion and proliferation.Taken together,Siglec-6 plays an important role in preeclampsia,and can alleviate preeclampsia by promoting trophoblast invasion and proliferation through the Src/SHP2 signalling pathway.

Objective To explore the application value of Three-Dimensional rectal cavity ultrasound combined with contrast agent imaging in necrotizing fasciitis of the anal region.Methods Before surgery,standard three-dimensional rectal cavity ultrasound examinations(referred to as the conventional group)and contrast agent imaging examinations(referred to as the imaging group)were conducted for 40 patients clinically diagnosed with anal region necrotizing fasciitis.Separate observations were made for the primary lesion,as well as for the depth and superficial necrosis of the fascia,and injuries to the anal sphincter muscle.Comparative analysis with surgical results was undertaken to assess the diagnostic sensitivity of both the conventional and imaging groups.Results In comparing the conventional group with the imaging group,the rates of primary lesion visibility rose significantly from 70%to 97.5%,deep fascial necrosis visibility increased from 50%to 88.8%,superficial fascia visibility improved from 70%to 100%,and the visibility of anal sphincter muscle injury escalated from 62.5%to 97.2%,all demonstrating statistical significance at P<0.05.Conclusions Three-dimensional rectal cavity ultrasound combined with contrast agent imaging exhibits significantly enhanced accuracy in identifying primary lesions associated with perianal necrotizing fasciitis,as well as the necrosis affecting deep and superficial fascia,in contrast to conventional three-dimensional rectal cavity ultrasound.This advancement offers more precise guidance for clinicians in devising surgical plans,thereby augmenting the success rate of surgical interventions.

Objective To investigate the value of qualitative and quantitative characteristics of gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid(Gd-EOB-DTPA)enhanced MRI in preoperative prediction of vessels encapsulating tumor clusters(VETC)pattern in hepatocellular carcinoma(HCC).Methods A total of 234 patients diagnosed with HCC by pathology were analyzed retrospectively.A total of 101 VETC-positive HCC patients and 133 VETC-negative HCC patients were included.All patients were divided into training group and validation group according to 7︰3.The training group data were used to construct a prediction model for VETC-positive HCC.Receiver operating characteristic(ROC)curve was drawn and the area under the curve(AUC)was calculated to verify the diagnostic efficiency of the model.Calibration curve was drawn to verify the calibration of the model.Results Multivariate logistic regression analysis predicted the independent risk factors for VETC-positive HCC:portal phase peripheral washout[odds ratio(OR)6.493],necrosis or severe ischemia(OR 4.756),targetoid transitional phase or hepatobiliary phase(OR 0.307),and lesion to liver signal intensity ratio(LLR)on arterial phase(OR 0.074).The AUC of the training group in predicting VETC-positive HCC was 0.790[95%confidence interval(CI)0.720-0.859].The AUC of the validation group in predicting VETC-positive HCC was 0.779(95%CI 0.668-0.889).The calibration curve diagram showed that the calibration curve(the slope was 0.91)almost coincides with the ideal curve,indicating that the prediction model had better calibration.Conclusion The qualitative and quantitative characteristics of Gd-EOB-DTPA enhanced MRI can be used to predict VETC-positive HCC preoperatively,the independent risk factors of VETC include portal phase peripheral washout,necrosis or severe ischemia,targetoid transitional phase or hepatobiliary phase,and LLR on arterial phase.

Objective:To investigate the current situation of coping styles in Crohn's disease patients and its related influencing factors.Methods:A total of 80 patients with Crohn's disease admitted to our hospital from Apri 2021 to Dec 2022 were selected to evaluate their coping styles with a simple coping style questionnaire,and relevant data were collected.The factors affecting the coping styles of Crohn's disease were analyzed by multivariable logistic regression.Results:Among the 80 patients,29 cases were negative coping,the incidence was 36.25% .There were 51 patients with positive coping(63.75% ).Educational level,simplified Crohn's disease activity index(CDAI)score,adverse psychology,social support and type D personality were associated with negative coping(P＜0.05).Gender,age,family history,working status,monthly family income,place of residence,and marital status were not associated with negative coping in patients with Crohn's disease(P＞0.05).Multivariable logistic regression analysis showed that education level of high school or below(OR=2.945,95% CI:1.139-7.614),higher CDAI score(OR=11.999,95% CI:4.387-32.815),poor psychology(OR=5.950,95% CI:2.180-16.239),low social support(OR=3.598,95% CI:1.370-9.448)and type D personality(OR=3.208,95% CI:1.118-8.904)were risk factors for negative coping in patients with Crohn's disease(P＜0.05).Conclusions:The incidence of negative coping in patients with Crohn's disease is higher,which is related to high school education or below,high CDAI score,poor psychology,low social support,and type D personality.Therefore,clinical measures can be taken to promote patients to actively cope with the disease.