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

Crigler-Najjar syndrome (CNS) and Gilbert syndrome (GS; OMIM: 143500) are rare autosomal recessive diseases that cause unconjugated hyperbilirubinemia due to decreased UGT1A1 enzyme activity. Crigler-Najjar syndrome type 2 (CNS2; OMIM: 606785) increases the risk of gallbladder stone formation and cholecystitis, while GS seldom causes health issues. We found a 28-year-old male patient with recurring right upper abdomen pain who experienced persistent jaundice from birth. CNS2 with gallbladder stones and cholecystitis was diagnosed after genetic testing revealed rare double homozygous mutations A(TA)₇TAA (rs3064744) and P229Q (rs35350960) in the UGT1A1 gene. After pedigree investigation, we found that the patient's parents with modestly increased bilirubin had compound heterozygous mutations A(TA)₇TAA and P229Q, which were GS. Bioinformatics analysis showed that A(TA)₇TAA is in the TATA-box region of the gene UGT1A1 promoter, affecting gene transcriptional initiation, whereas P229Q modifies protein three-dimensional structure and may be harmful. In this pedigree, double homozygous mutations have a more severe phenotype than compound heterozygous mutations. Inherited causes of hyperbilirubinemia should be suspected after ruling out biliary obstruction, and early bilirubin reduction (< 103 µmol/L (6 mg/dL)) may reduce the risk of complications like cholecystitis in CNS2 patients, though further studies with longer follow-up are needed to confirm this observation.
Humans ; Male ; Glucuronosyltransferase/genetics* ; Adult ; Crigler-Najjar Syndrome/complications* ; Cholecystitis/etiology* ; Homozygote ; Mutation ; Pedigree

In order to help medical institutions use and manage lead protective equipment in a scientific and standardized manner and promote the occupational safety and health of medical personnel,this《Expert consensus on the application and management of ionizing radiation lead protective equipment》(hereinafter referred to as"the consensus")is formulated.The consensus is formulated through conducting systematic literature search as well as through integrating the opinions of domestic experts in various fields after two rounds of letter inquiry.The consensus provides professional guidance in four aspects,including the selection,configuration,cleaning and maintenance,and inspection of appliances,aiming to provide clinical reference for the majority of medical workers engaged in interventional radiology.

Platelets contribute to maintaining brain homeostasis in a physiological environment,and platelet dysfunction is closely associated with the occurrence of many diseases,including neurodegenerative diseases.This article provides a comprehensive summary of the various functions of platelets and their role in the occurrence and development of one specific neurodegenerative disease,Parkinson's disease.It discusses the correlation between platelet functional status and Parkinson's disease pathology,the potential and possible clinical significance of platelet heterogeneity as a peripheral biomarker for Parkinson's disease diagnosis and prognosis,as well as the therapeutic potential of healthy platelets in Parkinson's disease.

Purpose To observation the relationship be-tween the β-catenin/Slug signal specific inhibitor FH535 and EMT,and to explore the role of LPCAT1 in regulating the inva-sion,metastasis,and growth of cervical cancer cells.Methods Hela cells were transfected with sh-NC and sh-LPCAT1,and SiHa cells were transfected with Vector group and LPCAT1 over-expression plasmid.SiHa cells were divided into control group(Con),LPCAT1 group,LPCAT1+FH535 group and FH535 group.The proliferation of cervical cancer cells was detected by CCK-8 analysis and colony formation test.The metastasis and invasion ability of cervical cancer cells were detected by wound healing test and Transwell test.Western blot was used to analyze the expression of LPCAT1,β-catenin/Slug signaling pathway and EMT-related proteins in cells.Results Compared with Vector group,the cell viability,colony number,migration and invasion number of SiHa cells in LPCAT1 group increased signif-icantly(P＜0.05).Compared with sh-NC group,the cell via-bility,colony number,migration and invasion number of Hela cells in sh-LPCAT1 group decreased significantly(P＜0.05).Compared with LPCAT1 group,the levels of Wnt4(1.18±0.05 vs 0.80±0.06),β-catenin(1.05±0.08 vs 0.77±0.05),Slug(1.13±0.06 vs 0.28±0.02),Cyclin D1(0.99±0.06 vs 0.44±0.02),N-cadherin(0.91±0.07 vs 0.46±0.03)and vimentin(0.95±0.06 vs 0.49±0.03)in SiHa cells in LPCAT1+FH535 group decreased significantly(P＜0.05),and the level of E-cadherin(0.44±0.03 vs 0.58±0.03)in-creased significantly(P＜0.05).In addition,compared with LPCAT1 group,the number of colonies(224±15 vs 146±11),migration(85±3vs51±4)and invasive(166±10 vs 90±5)cells of SiHa cells in LPCAT1+FH535 group decreased signifi-cantly(P＜0.05).Conclusion The increase of LPCAT1 ex-pression may promote the metastasis and progress of CC by acti-vating β-catenin/Slug signaling pathway,and LPCAT1 may be a potential marker for predicting CC metastasis.

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 investigate the effects of Aikeqing(AKQ),a compound of Chinese medicine,on drug-metabolizing activity and on the pharmacokinetic parameters of HIV-1 protease inhibitors lopinavir(LPV)and ritonavir(RTV)in Kaletra tablets.Methods Human liver microsomes were co-incubated with mixed probes and AKQ.HPLC-MS was employed to measure the content of probe.Half-inhibitory concentration(IC50)of AKQ on different subtypes of cytochrome P450 enzymes involved in drug metabolism of liver microsomes was calculated.The P450 subtype,whose activity was significantly inhibited by AKQ was then identified.HPLC-MS analytical method for simultaneous determination of LPV and RTV in rat plasma was established.SD rats were orally given AKQ or vehicle once a day for 7 consecutive days.After half an hour of the last gavage of AKQ,the rats were given Kaletra by intragastric administration.Then,the blood concentration of LPV and RTV were measured and the effect of AKQ on pharmacokinetic parameters of LPV and RTV were analyzed.Results The methanol extract of AKQ at the concentrations of 0～500 μg·mL-1 showed inhibitory effects on the metabolic activity of CYP2D6,CYP2C8、CYP2E1,CYP2C19,CYP1A2,CYP2B6,CYP2C9 and CYP3A4,with IC50 values of 7.7,75.3,144.0,99.5,43.5,104.5,49.3 and 204.9 μg·mL-1,respectively.An HPLC-MS/MS method was established for simultaneous quantification of LPV and RTV in blood samples.LPV and RTV showed linear relationships in the ranges of 30～10 000 ng·mL-1 and 3～1 000 ng·mL-1,respectively.The lowest limits of quantification were 30 ng·mL-1 and 3 ng·mL-1.Intra-day and inter-day precision were all less than 5%,and the accuracy of LPV and RTV was in the range of 96.3%～109%.The extraction recovery rates were not less than 88.7%,the matrix effects were 93.8%～105.0%,and the plasma samples were stable.Compared with Kaletra group,there was no significant changes in non-compartmental model parameters including AUC0-t,AUC0-∞,MRT0-t,t1/2z,tmax,Vz/F,Clz/F and Cmax of LPV in AKQ+Kaletra group(P＞0.05).But MRT0-∞ was found to be obviously affected by AKQ(P＜0.05).All pharmacokinetic parameters of RTV showed no significant change in AKQ+Kaletra group(P＞0.05).Conclusion Aikeqing exhibited varying degrees of inhibitory effects on 8 human drug-metabolizing cytochromes P450,especially CYP2D6.A human-equivalent dose of Aikeqing does not affect the pharmacokinetic parameters of lopinavir and ritonavir in rats.

BACKGROUND:Research has shown that fatty acid metabolism genes are closely related to the development of rheumatoid arthritis.Therefore,exploring the progression of rheumatoid arthritis based on fatty acid metabolism genes is of clinical significance. OBJECTIVE:To investigate whether fatty acid metabolism genes can serve as reliable biomarkers for predicting the progression of rheumatoid arthritis. METHODS:Gene data related to synovial tissue were downloaded from the Gene Expression Comprehensive Database(GEO).STRING was used to construct the protein-protein interaction network analysis.Cytoscape was utilized for biological annotation(gene ontology)and signaling pathway enrichment analysis(Kyoto Encyclopedia of Genes and Genomes).Fatty acid metabolism related genes were screened from the molecular feature database(MSigDB).Least absolute shrinkage and selection operator and support vector machine recursive feature elimination feature were used to screen for potential biomarkers.Immune cell infiltration levels in normal individuals and rheumatoid arthritis patients were assessed using the CIBERSORT algorithm.Finally,the expression levels of fatty acid metabolism related genes were verified using the receiver operating characteristic curve in GSE77298. RESULTS AND CONCLUSION:361 differentially expressed genes in rheumatoid arthritis were identified,of which 13 overlapped with the reported fatty acid metabolism related genes.Based on machine learning algorithms,five genes were selected,and the receiver operating characteristic curve showed that five genes(PCK1,PDK1,PTGS2,PLA2G2D,and DPEP2)could predict the development of rheumatoid arthritis.The CIBERSORT algorithm results showed that five genes were associated with activated mast cells,neutrophils,resting mast cells,and memory resting CD4+ T cells.The receiver operating characteristic curve showed that PLA2G2D and PCK1 have high diagnostic value.To conclude,the expression characteristics of fatty acid metabolism related genes can serve as potential biomarkers for predicting clinical outcomes,which can further improve the accuracy of prediction in RA patients.

Objective To analyze the metabolic changes of myocardial tissue in rats under acute exposure to macleaya cordata by gas chromatography mass spectrometry(GC-MS),explore forensic identifications of its characteristic metabolites,and verify its toxicological mechanism in poisoning cases.Methods The rats in the exposure group were given 382 mg/kg macleaya extract solution by gavage,and the rats in the control group were given the same dose of solvent.The myocardial samples were analyzed by GC-MS,and pattern recognition was conducted through partial least squares discriminant analysis(PLSDA).The differential metabolites with characteristic changes were identified by variable importance projection(VIP value＞1)and Student's t test(P＜0.01).Results Compared with the control group,21 potential characteristic metabolites were identified.Through KEGG pathway enrichment analysis,it was found that these metabolites were mainly involved in the pathways of glycine,serine and threonine metabolism;pyruvate metabolism and glycerolipid metabolism.Conclusion Through the study of myocardial metabolism in rats exposed to macleaya cordata,we found the information on metabolites closely related to poisoning,which provides new insight and reference for studies on the mechanisms of macleaya cordata poisoning in the field of forensic medicine.

BackgroundSibling relationships play a critical role in shaping anxiety symptoms in firstborn children. Anxiety symptoms often originate in early childhood and can persist into adolescence and adulthood. However， there is insufficient research on anxiety symptoms in preschool children， especially firstborn preschool children. ObjectiveTo explore the influencing factors of anxiety symptoms among firstborn preschool children， so as to provide references for the intervention of anxiety symptom for children in families with multiple children. MethodsFrom October to December 2021， a total of 8 449 children from 234 kindergartens in Longhua District of Shenzhen were included using a cluster sampling method. Sibling Inventory of Behavior （SIB） and Spence Preschool Anxiety Scale （SPAS） were used to investigate. Logistic regression analysis was used to identify influencing factors of anxiety symptoms in firstborn preschool children. ResultsA total of 8 419 （99.64%） valid questionnaires were collected. Anxiety symptoms were detected in 344（4.09%） firstborn preschool children. Statistically significant differences were observed between anxiety group and non-anxiety group in terms of household registration， monthly family income， maternal age， maternal education level， paternal education level， family living conditions and whether they are left-behind children （χ²/t=9.906， 33.490， 5.136， 13.485， 9.690， 17.332， 21.975， P<0.05 or 0.01）. Compared with non-anxiety group， children in the anxiety group scored higher on the SIB dimensions of rivalry， aggression and avoidance （t=165.322， 74.471， 286.419， P<0.01）， and lower on companionship， empathy and teaching （t=59.133， 42.417， 39.112， P<0.01）. Risk factors for anxiety symptoms in firstborn preschool children included left-behind children， as well as negative sibling relationships characterized by rivalry and avoidance （OR=1.195， 1.143， 1.260， P<0.05 or 0.01）. ConclusionFirstborn preschool children who are left-behind are more susceptible to anxiety symptoms. Negative sibling relationships， characterized by competition and avoidance， may also contribute to the emergence of anxiety symptoms in firstborn preschool children.