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

Objectives:To explore the association between the r'wave amplitude in lead V1 and impedance changes with left bundle branch pacing electrode implantation depth. Methods:A total of 78 patients with normal heart structure and underwent left bundle branch area pacing(LBBAP)in the Second Affiliated Hospital of Nanchang University from January 1,2019 to December 31,2021 were included in this retrospective analysis.Baseline data,intraoperative and imaging data,and 3,6,9 and 12 months of follow-up results were collected.Correlation and regression analysis were performed to define the feasibility using the r'wave in lead V1 during pacing and impedance changes to estimate the electrode depth. Results:r'waves at the end of the QRS complex in lead V1 during pacing were found in 70 cases(89.7%),and 8 cases(10.3%)showed rS,RS type QRS waves,or no r'wave at the end.Correlation analysis showed that r'wave amplitude was positively correlated with electrode depth(r=0.424,P<0.01),negatively correlated with impedance(r=-0.256,P=0.03).There was no significant statistical correlation between electrode implantation depth and impedance(r=-0.132,P=0.27).Regression analysis found that electrode depth was an important factor affecting r'wave amplitude(regression coefficient=0.056,P=0.000).Combined with the established regression model and impedance,it was found that the amplitude of the r'wave in lead V1 is at the range of 0.24-0.69 mV,and the impedance ranges from 648.30 to 828.90 Ω,and the electrode implantation depth is 6-11 mm,which is most suitable.The risk of perforation is low,and the left bundle branch can be successfully captured with a high probability.The pacing parameters are satisfactory,and the pacing QRS wave duration is narrow.During the intraoperative,postoperative 48 hours,and 12-month follow-up period,the patient did not experience complications such as electrode perforation,thromboembolism,cardiac tamponade,infection,or wire dislocation. Conclusions:Left bundle branch region pacing is a safe and feasible pacing method.During LBBAP,the amplitude of the r'wave in lead V1 at the range of 0.24-0.69 mV,and the impedance ranges from 648.30 to 828.90 Ω can be used to guide the pacing in the left bundle branch region and reduce the risk of electrode perforation.

ObjectiveBioinformatics methods were used to systematically identify the Salvia miltiorrhiza terpenoid synthase （SmTPS） gene family members and predict their functions from the perspective of the genome. MethodThe genome and transcriptome data of S. miltiorrhiza， Arabidopsis thaliana， and tomato were obtained from the national genomics data center （NGDC）， national center for biotechnology information （NCBI）， the Arabidopsis information resource （TAIR）， and tomato functional genomics database （TFGD）， and the whole genome identification and bioinformatics analysis of the SmTPS gene family member were carried out with the help of Perl language programming， Tbtools， and other bioinformatics tools. ResultA total of 52 TPS gene family members were identified， and they were distributed on eight chromosomes of S. miltiorrhiza. Their coding amino acid number was 207-822 aa. The isoelectric points were 4.76-9.16. The molecular mass was 24.11-94.81 kDa， and all members are hydrophilic proteins. Gene structure analysis showed that there were significant differences in the number of introns among different subfamilies. The number of introns in 72.6% of TPS-a， b， and g subfamilies was 6， and that in 88.9% of TPS-c and e/f subfamilies was more than 10. Protein motifs were conserved among TPS subfamilies. The analysis of promoter cis-acting elements showed that all promoters of the SmTPSs contained a large number of light-responsive elements， and most of them had hormone-responsive elements. Gene expression analysis showed that SmTPS gene family members exhibited tissue-specific expression， and 24 of them responded to exogenous methyl jasmonate. ConclusionBased on the published S. miltiorrhiza genome， 52 SmTPS gene family members were identified， and their functions were predicted based on the phylogenetic analysis and expression patterns. This paper provides reference information for the further biosynthesis pathway and regulatory mechanism analysis of terpenoids in S. miltiorrhiza.

Objective:To document the impact of information-based, stepwise, intensive rehabilitation therapy on patients with acute exacerbation of chronic obstructive pulmonary disease (AECOPD).Methods:Eighty such patients in an intensive care unit (ICU) were randomly divided into a control group and an observation group, each of 40. The control group received routine ICU rehabilitation, while the observation group underwent information-based, step-wise ICU rehabilitation. Upon admission to and discharge from the ICU, the forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), FEV1/FVC, and diaphragm functioning were compared between the two groups. The duration of mechanical ventilation, the incidence of ventilator-associated pneumonia (VAP), length of stay in the ICU, incidence of delirium, and the incidence of deep vein thrombosis (DVT) were also recorded. The number of patients readmitted to the ICU after discharge, and the 28-day hospital mortality rate were recorded as well.Results:Significant improvement was observed in both groups in terms of their lung and diaphragm functioning, as well as in the rehabilitation- and hospital-related indicators. At discharge, significantly greater improvements were observed in the observation group compared with the control group in terms of their average FEV1, FVC, FEV1/FVC, inspiratory and expiratory diaphragm thickness, and diaphragm thickening rate. The average duration of mechanical ventilation and of rehabilitation interruptions was significantly less in the observation group. And incidents of accidental extubation, VAP, delirium and DVT were significantly fewer in the observation group as well. Their ICU stays tended to be significantly shorter without any significant difference between the two groups in the 28-day hospital mortality rate. The control group spent significantly less time in their daily rehabilitation sessions, with the result that significantly fewer of them achieved a grading of 2 or better on the mMRC respiratory questionnaire.Conclusion:Information-based stepwise intensive rehabilitation treatment can effectively improve the pulmonary and diaphragmatic function of AECOPD patients admitted to an ICU, shorten their mechanical ventilation time and the length of their ICU stay, and lower their incidence of VAP and DVT during hospitalization.

ObjectiveTo investigate the preventive effect and mechanism of Aurantii Fructus Immaturus and naringin on postoperative intestinal adhesion in rats. MethodThe preventive effect of Aurantii Fructus Immaturus and naringin on intestinal adhesion was studied by cecal scraping model of rats， the model rats were randomly divided into model group， dexamethasone sodium phosphate group and Aurantii Fructus Immaturus low， medium and high dose groups （1.58， 3.15， 6.30 g·kg^-1·d^-1）， tsham-operated group was treated with an incision in the abdomen. Adhesion was assessed by Nair method after 7 d of administration. Hematoxylin-eosin （HE） staining was used to observe the pathological morphology and inflammatory cell infiltration of cecum， the expression of matrix metalloproteinase-9 （MMP-9） in cecal adhesion was detected by immunohistochemistry. Meanwhile， intestinal adhesion model rats were randomly divided into model group， dexamethasone sodium phosphate group， naringin low， medium， high dose groups （50， 100， 200 mg·kg^-1·d^-1）， and the sham-operated group was treated with an incision in the abdomen. Adhesion was assessed after 7 d of administration， HE staining was used to observe the pathological morphology and inflammatory cell infiltration in the cecum， and the expression of MMP-9 in the cecal adhesion tissue was detected by immunohistochemistry. Expressions of transforming growth factor-β₁ （TGF-β₁） and α-smooth muscle actin （α-SMA） in cecum were analyzed by western blot. ResultAurantii Fructus Immaturus could inhibit the formation of postoperative intestinal adhesions in rats， reduce the inflammatory response of damaged cecum tissue， and up-regulate the expression of MMP-9 in the adherent tissue in a dose-dependent manner. All dose groups of naringin could significantly inhibit the formation of postoperative intestinal adhesions in rats， reduce inflammatory cell infiltration and fibrous proliferation in tissue， up-regulate the expression of MMP-9 in the adherent tissue in a dose-dependent manner， and inhibit the expression of TGF-β₁ and α-SMA in cecal tissue. ConclusionAurantii Fructus Immaturus and naringin can reduce postoperative intestinal adhesion formation in rat model， and their effects may be related to reducing tissue fibrosis and accelerating extracellular matrix degradation.

Objective To discuss the anticoagulation effects and safety of sodium citrate in continuous renal replacement therapy (CRRT).Methods A total of 140 cases who underwent CRRT in the intensive care unit (ICU) of Zhejiang Provincial People′s Hospital from January 2014 to December 2016 were selected as research objects and divided into the heparin sodium group (n=56),the low molecular weight heparin calcium group (n=48) and the sodium citrate group (n=36) according to the anticoagulation methods. The differences of anticoagulation effects,the average service life of the pipelines,the overall pipeline quantity,the total cost of blood purification,the blood transfusion amount during CRRT,total bilirubin (Tbil),serum creatinine (Cer), Na+,K+,prothrombin time (PT),activated partial prothrombin time (APTT),platelet count (PLT) before and after CRRT and the complications related to CRRT among the three groups were compared.Results The anticoagulation effect of the sodium citrate group was obviously better than that of the other two groups (χ2=18.809,P<0.05). The average service life of the pipelines of the sodium citrate group was (29.3±5.4) h, which was obviously higher than that of the heparin sodium group (16.5±3.5) h and the low molecular weight heparin calcium group (24.8±4.1) h,and the intergroup difference was statistically significant (F=6.596, P<0.05). The transfusion amount of red blood cells,plasma and platelets of the sodium citrate group was (12.3±2.8) U,(1875.5±198.6) ml and (23.5±8.6) U,which was obviously lower than that of the heparin sodium group [(16.5±5.6) U,(2463.4±265.4) ml and (58.3±18.5) U] and the low molecular weight heparin calcium group [(13.4±3.2) U,(1954.4±214.5) ml and (32.6±11.5) U],and the intergroup differences were all statistically significant (F=4.563,3.896,7.456;P<0.05). The Tbil,Cer,Na+ and K+ of all groups decreased after CRRT,and the differences were all statistically significant (P<0.05). There were statistical significance in PT,APTT and PLT among the three groups (F=4.562,4.153,5.468;P<0.05). The PLT of the sodium citrate group increased significantly after CRRT,with statistical difference (P<0.05). The incidence rate of hemorrhages in skin,digestive tract,puncture point and trachea of the sodium citrate group was respectively 2.8％,5.6％,2.8％ and 5.6％,which was obviously lower than that of the heparin sodium group (23.2％,12.5％, 16.1％,12.5％) and the low molecular weight heparin calcium group (6.3％,10.4％,8.3％,8.3％),and the differences were all statistically significant (χ2=6.678,7.553,6.849,7.534;P<0.05). The incidence rate of heparin induced thrombocytopenia (HIT) of the heparin sodium group was 8.9％ (5/56).Conclusions The anticoagulation effect of sodium citrate in CRRT is remarkable with less complications. It can significantly reduce the amount of blood transfusion and the cost of blood purification and is worth of promotion in clinical application.

Objective To investigate the risk factors of thrombopenia(TP)in septic patients complicated with acute kidney injury (AKI).Methods Two hundred and sixty five septic patients complicated with AKI admitted in Intensive Care Unit ICU of Zhejiang Provincial People''s Hospital during January 2012 and December 2016 were enrolled in the study.The clinical data, results of laboratory tests, Acute Physiology and Chronic Health Evaluation (APACHEII) scores, Sequential Organ Failure Assessment (SOFA) scores, therapeutic intervention, and 28-day mortality were documented.Among 265 patients, TP occurred within 7 days in 112 cases (TP group) and did not occur in 153 cases (non-TP group).Multivariable Logistic regression analysis was performed to analyze the risk factors of TP.Results The 28-day mortality rate in TP group was higher in TP group than that in non-TP group (47.3% vs.33.3%, χ2=5.307,P<0.05).Univariate analysis showed that age, C-reactive protein (CRP), procalcitonin (PCT) and APACHEII score, SOFA score, continuous renal replacement therapy (CRRT), heparin anticoagulation, shock, usage of linezolid and bloodstream infections were associated with TP in septic patients with AKI(all P<0.05).Multivariable Logistic regression analysis showed that age≥65 (OR=4.53, 95%CI 1.23-9.24,P<0.05), CRRT(OR=5.24,95%CI 2.14-14.56,P<0.01), heparin anticoagulation(OR=4.56,95%CI 2.13-8.46,P<0.01), usage of linezolid(OR=2.35,95%CI 1.25-5.24,P<0.01), shock(OR=2.15,95%CI 1.03-4.96,P<0.01)and bloodstream infections(OR=4.26,95%CI 1.36-12.48,P<0.01)were independent risk factors for septic patients with TP.Conclusion For septic patients with AKI having these risk factors, the platelet counts should be closely monitored, and intervention measures should be given to reduce the occurrence of TP.

Respiratory dysfunction is the primary cause of death early after cervical spinal cord injury, and it can be restored by electrical stimulation, magnetic stimulation, nerve transfer surgery, and cell transplantation. This paper reviewed the anatomy, clinical manifestations,and associated restoration techniques of respiratory muscle dysfunction after cervical spinal cord injury.

Objective To evaluate the efficacy of intravertebral augmentation which including transpedicular bone graft, transpediclebody augmenter and vertebroplasty in preventing the correction loss and implant failure of short-segment pedicle instrumentation for thoracolumbarfractures through meta-analysis. Methods Experimental studies (randomized controlled trails, non-randomized controlled trails)and observational studies (cohort studies, case control studies) related with application of posterior short-segment pedicle instrumentationwith intravertebral augmentation for thoracolumbar fractures were searched from Pubmed, EMBASE and CNKI according to the inclusionand exclusion criteria, and hand-searched in Chinese and English journals. RevMan 5.0.18 provided by Cochrane was used to analyse the data.Results 1 randomized controlled trail and 7 observational studies were included. There were 442 patients, in which 216 patients werewith and 226 patients without intravertebral augmentation. There was no significant difference in correction loss and risk of implant failurebetween these two groups. Conclusion Intravertebral augmentation does little about the risk of correction loss and implant failure associatedwith posterior short-segment pedicle instrumention for patients with thoraculumbar fractures.

Objective To investigate the effect of basic fibroblast growth factor (bFGF) on the synthesis of extracellular matrixc (ECM)and expression of chondromodulin in human intervertebral disc cells. Methods 4 intervertebral discs (IVDs) obtained from patients in thetreatment of disc degenerative disease were used for cell culture. The secondary generation of intervertebral disc cells were cultured for 7days, then different concentration of bFGF (0, 0.1 ng/ml, 1 ng/ml, 10 ng/ml)were added to the medium and treated for 72 hours. Real-timeRT-PCR was used to detect the change of Aggrecan and type Ⅱ collagen mRNA expression. The effect of FGF on the expression of ChM-1,a cartilage derived anti-angiogenic factor, was also used by means of Real-time RT-PCR and Western blot. Results Real-time RT-PCRshowed that bFGF can significantly inhibit the expression of Aggrecan and type Ⅱ collagen mRNA. Both Real-time RT-PCR and Westernblot showed that the expression of ChM-1 was down-regulated by administration of bFGF with dose-dependent way. Conclusion bFGFserves primarily as a catabolic factor and induce the angiogenesis in the process of intervertebral disc degeneration.