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

The chemical composition of Paeoniae Radix Alba(PRA) is complex, with primary secondary metabolites including monoterpenoids, tannins, triterpenoids, and flavonoids. In previous studies on the material basis of PRA, it was found that, in addition to the widely studied characteristic monoterpene glycosides, tannic acid components also play an important role in the efficacy of PRA. However, their pharmacological effects have not been thoroughly investigated. This paper reviews the tannic acid components in PRA, including pentagaloyl glucose(PGG), tetragaloyl glucose(TGG), trigaloyl glucose(TriGG), and gallic acid, along with their structures, properties, and characteristics to provide a detailed discussion of their pharmacological activities and related mechanisms, aiming to offer a theoretical basis for the material basis research and clinical application of PRA.
Paeonia/chemistry* ; Tannins/chemistry* ; Humans ; Drugs, Chinese Herbal/chemistry* ; Animals ; Plant Extracts

OBJECTIVES: To evaluate the clinical value of next-generation sequencing (NGS) in neonatal disease screening, particularly its advantages when combined with tandem mass spectrometry (MS/MS). METHODS: A prospective study was conducted involving blood samples from 1 999 neonates born at the Shenzhen Guangming District People's Hospital, between May and August 2021. All samples were initially screened using MS/MS and fluorescence immunoassay, followed by NGS to detect high-frequency variation sites in 135 related pathogenic genes. Suspected positive variants were validated using Sanger sequencing or multiplex ligation-dependent probe amplification in family studies. RESULTS: No confirmed positive cases were found in the MS/MS analysis of the 1 999 neonates. Genetic screening identified 58 positive cases (2.90%), 732 carriers of pathogenic genes (36.62%), and 1 209 negative cases (60.48%). One case of neonatal intrahepatic cholestasis was diagnosed (0.05%, 1/1 999). Fluorescence immunoassay identified 39 cases of glucose-6-phosphate dehydrogenase (G6PD) deficiency (1.95%, 39/1 999), while genetic screening identified 43 cases of G6PD deficiency (2.15%, 43/1 999). The fluorescence immunoassay also detected 6 cases of hyperthyrotropinemia (0.30%, 6/1 999), all of whom carried DUOX2 gene variants. The top ten pathogenic gene carrier rates were G6PD (12.8%), DUOX2 (8.7%), HBB (8.2%), ATP7B (6.6%), GJB2 (5.7%), SLC26A4 (5.6%), PAH (5.6%), ACADSB (4.6%), SLC25A13 (4.2%), and SLC22A5 (4.1%). CONCLUSIONS NGS can serve as an effective complement to MS/MS, significantly improving the detection rate of inherited metabolic disorders in neonates. When combined with family validation, it enables precise diagnosis, particularly demonstrating complementary advantages in screening for monogenic diseases such as G6PD deficiency.
Humans ; Infant, Newborn ; High-Throughput Nucleotide Sequencing/methods* ; Neonatal Screening/methods* ; Tandem Mass Spectrometry ; Prospective Studies ; Female ; Male ; Infant, Newborn, Diseases/diagnosis* ; Genetic Testing

OBJECTIVE: To evaluate the efficacy and safety of Chinese medicine (CM) Zihua Wenfei Zhisou Granule (ZWZG) in postinfectious cough (PIC) patients with CM syndrome of wind-cold invading Fei (Lung, WCIF). METHODS: This is a multicenter, randomized, double-blind, parallel-group, placebo-controlled phase II clinical trial. PIC patients with WCIF syndrome were recruited from the Respiratory Departments in 6 hospitals across China between March 2019 and December 2020. Eligible patients were randomly assigned to group A (ZWZG-matched placebo 15 g), group B (active ZWZG 15 g), and group C (active ZWZG 10 g plus ZWZG-matched placebo 5 g) in a 1:1:1 ratio. All medications were taken orally 3 times daily for 14 consecutive days. The primary outcomes were cough relief rate and cough disappearance rate. The secondary outcomes included time to cough relief, time to cough disappearance, and changes in cough symptom score (CSS), cough Visual Analog Scale (VAS) value, Cough-Specific Quality of Life Questionnaire (CQLQ) score, and CM syndrome score from baseline (day 0) to post-treatment (day 14). Adverse events (AEs) in each group were recorded. RESULTS: A total of 198 patients were included in the full analysis set (FAS) and safety analysis set (SS), while 183 were enrolled in the per-protocol analysis set (PPS). In the FAS population, the cough relief rate was 47.76%, 90.77% and 84.85% in groups A, B, and C, respectively; while the cough disappearance rate was 31.34%, 72.31% and 68.18%, respectively. The cough relief rates and cough disappearance rates in groups B and C were significantly higher than group A (P<0.0001). Both the median time to cough relief and cough disappearance in groups B and C were shorter than group A (P<0.0001). Compared with group A, groups B and C showed significantly greater improvements from baseline to post-treatment in CSS during daytime and nighttime as well as VAS (P<0.05). There were no significant differences in changes from baseline to post-treatment in CQLQ and CM syndrome scores among 3 groups (P>0.05). Results in the PPS population were consistent with those in the FAS population. Groups B and C showed lower incidence in AEs than group A (P<0.05), while there was no significant difference between groups B and C (P>0.05). No drug-related severe AEs were reported. CONCLUSIONS ZWZG can increase cough disappearance rate and cough relief rate; and it is beneficial in shortening cough duration and reducing cough severity and frequency in patients suffering from PIC. It is safe and generally well tolerated. (Registration No. ChiCTR1900022078).
Humans ; Cough/drug therapy* ; Double-Blind Method ; Male ; Female ; Drugs, Chinese Herbal/adverse effects* ; Middle Aged ; Treatment Outcome ; Adult ; Aged ; Quality of Life

OBJECTIVE: To evaluate the dynamic changes of glucocorticoid (GC) dose and the feasibility of GC discontinuation in rheumatoid arthritis (RA) patients under the background of Chinese medicine (CM). METHODS: This multicenter retrospective cohort study included 1,196 RA patients enrolled in the China Rheumatoid Arthritis Registry of Patients with Chinese Medicine (CERTAIN) from September 1, 2019 to December 4, 2023, who initiated GC therapy. Participants were divided into the Western medicine (WM) and integrative medicine (IM, combination of CM and WM) groups based on medication regimen. Follow-up was performed at least every 3 months to assess dynamic changes in GC dose. Changes in GC dose were analyzed by generalized estimator equation, the probability of GC discontinuation was assessed using Kaplan-Meier curve, and predictors of GC discontinuation were analyzed by Cox regression. Patients with <12 months of follow-up were excluded for the sensitivity analysis. RESULTS: Among 1,196 patients (85.4% female; median age 56.4 years), 880 (73.6%) received IM. Over a median 12-month follow-up, 34.3% (410 cases) discontinued GC, with significantly higher rates in the IM group (40.8% vs. 16.1% in WM; P<0.05). GC dose declined progressively, with IM patients demonstrating faster reductions (median 3.75 mg vs. 5.00 mg in WM at 12 months; P<0.05). Multivariate Cox analysis identified age <60 years [P<0.001, hazard ratios (HR)=2.142, 95% confidence interval (CI): 1.523-3.012], IM therapy (P=0.001, HR=2.175, 95% CI: 1.369-3.456), baseline GC dose ⩽7.5 mg (P=0.003, HR=1.637, 95% CI: 1.177-2.275), and absence of non-steroidal anti-inflammatory drugs use (P=0.001, HR=2.546, 95% CI: 1.432-4.527) as significant predictors of GC discontinuation. Sensitivity analysis (545 cases) confirmed these findings. CONCLUSIONS RA patients receiving CM face difficulties in following guideline-recommended GC discontinuation protocols. IM can promote GC discontinuation and is a promising strategy to reduce GC dependency in RA management. (Trial registration: ClinicalTrials.gov, No. NCT05219214).
Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Arthritis, Rheumatoid/drug therapy* ; Glucocorticoids/therapeutic use* ; Medicine, Chinese Traditional ; Retrospective Studies

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Background Per- and polyfluoroalkyl substances (PFAS), a group of persistent organic pollutants associated with adverse health effects including hepatotoxicity, immunosuppression, and carcinogenicity, have undergone risk assessments by multiple international organizations, with dietary exposure being the primary pathway. Objective To characterize the exposure to PFAS from food and drinking water sources of elderly residents in Songjiang District of Shanghai and to evaluate associated health risk and health effects. Methods A cross-sectional study was conducted from May to July 2024 in Songjiang District based on the Shanghai Suburban Adult Cohort and Biobank (SSACB) cohort. Dietary surveys were administered via face-to-face interviews among older adults aged 65 years and above, yielding 4 583 valid questionnaires. The estimated daily intake (EDI) of PFAS was calculated by integrating data from the Sixth National Dietary Survey and recent literature on PFAS concentrations in food and drinking water in Shanghai. Health risk assessment was performed using health-based guideline values (HBGV) proposed by various institutions and studies. Additionally, correlation analysis and linear regression modeling of EDI and biochemical indicators in the elderly were conducted to evaluate potential adverse health effects. Results The elderly population in Songjiang District exhibited dietary characteristics consistent with the Eastern Healthy Diet Pattern. Among PFAS compounds, PFOA showed the highest level of oral exposure [mean: 1.495 ng·(kg·d⁻¹)], followed by PFOS [mean: 0.637 ng·(kg·d⁻¹)], PFHxS [mean: 0.636 ng·(kg·d⁻¹)], and PFBS [mean: 0.273 ng·(kg·d⁻¹)]. Specifically, drinking water was the primary source of PFOA [1.415 ng·(kg·d⁻¹), accounting for 94.60%], while aquatic products were the major source of PFOS [0.278 ng·(kg·d⁻¹), accounting for 43.66%]. Using the HBGV derived by China's epidemiological studies, the mean hazard index (HI) for PFAS exposure was 1.39, indicating 54.35% of the population had potential health risks (HI>1). Following the 2024 standard established by the Food Safety Commission of Japan (FSCJ), the HI value dropped to 0.11, suggesting negligible risk. PFAS exposure was negatively associated with triglyceride levels and the indicators of liver and kidney function, but positively associated with low-density lipoprotein cholesterol (LDL-C) and lung cancer markers in the elderly residents. Conclusion PFAS exposure among the elderly residents in Songjiang District is predominantly attributed to PFOA, PFOS, PFHxS, and PFBS, with drinking water and aquatic products identified as primary exposure sources. Current exposure levels demonstrate significant associations with biomarkers of lipid metabolism and lung cancer markers, suggesting potential population health risks. These findings underscore the urgent need to establish HBGV for PFAS compounds based on Chinese population-specific metabolic characteristics.

Objective To investigate the association between the serum creatinine/cystatin C ratio(SCr/Cys C)as a Sarcopenia index(SI)and the incidence of in-hospital adverse events in patients with acute myocardial infarction(AMI)undergoing emergency percutaneous coronary intervention(PCI).Additionally,we evaluate the predictive efficacy of the SI in predicting major adverse cardiovascular events(MACEs)during hospitalization.Methods A total of 306 patients with AMI who underwent emergency PCI in the 904th Hospital of PLA Joint Logistics Support Force from January 2020 to March 2023 were consecutively included in this retrospective analysis.Patients were divided into two groups based on the occurrence of MACEs during hospitalization:MACEs group(n=43)and non-MACEs group(n=263).Clinical characteristics and pre-PCI laboratory test results were collected.Univariate and multivariate logistic regression analyses were performed to identify independent risk factors for MACEs.The predictive performance of SI was assessed using receiver operating characteristic(ROC)curve analysis.Results The incidence of in-hospital MACEs in AMI patients was 14.1%.The results of the independent samples t-test showed that the SI level in MACEs group was significantly lower than that in non-MACEs group,with a statistically significant difference(P<0.001).The results of the multivariate logistic regression analysis suggested that new-onset atrial fibrillation,Killip class 2-4,SI,and TG were independent risk factors for in-hospital adverse events after emergency PCI.The ROC curve results showed that the predictive value of SI(AUC=0.741,95%CI 0.666-0.816)using the SCr/Cys C ratio was superior to that of single Cys C(AUC=0.658,95%CI 0.570-0.746)for predicting post-PCI MACEs,with a statistically significant difference(P<0.05),and the optimal cutoff value for SI was 78.14.After stratifying SI based on the cutoff value,the results of the independent samples t-test showed that compared to the higher SI group,the lower SI group had a higher occurrence of specific adverse events such as heart failure(P<0.001),malignant arrhythmias(P=0.009),and strokes(P=0.003),with statistically significant differences.Conclusions The results highlight SI as an independent risk factor for MACEs during hospitalization after emergency PCI in AMI patients.Furthermore,SI has proven to be an effective prognostic index for patient outcomes.

Objective To provide anatomical,radiological,and clinical diagnostic and therapeutic references for ankylosing spondylitis and spinal surgical operations.Methods Non-measurement spinal observations,X-ray examinations,and measurements were performed on two spinal specimens,along with digital image acquisition and processing.Results The first specimen included thoracic vertebra 7(T7)to lumbar vertebra 3(L3),with an average total length of 29.7 cm;the second specimen ranged from cervical vertebra 7(C7)to lumbar vertebra 2(L2),with an average total length of 38.3 cm.The specimens showed partial or complete calcification of ligaments,ossification of the small joints and intervertebral discs,and osteoporosis;The anterior-posterior diameter(width)of the vertebral foramen was narrower than that of a normal adult,while most of the superior-inferior diameter(height)was wider.Radiographically,the anterior longitudinal ligament calcification appeared as dot-like or striated,but it was actually flaky in the actual specimens.The specimens provided views of the facet joints,costovertebral joints,and intervertebral foramina that was difficult to demonstrate on two-dimensional X-ray images.Conclusion As ankylosing spondylitis progresses,the range of motion in spinal bending and rotation decreases,as does the extent of thoracic expansion,thereby affecting respiration and complicating procedures such as intraspinal anesthesia and sacral canal injections.In terms of diagnosis,bone specimens and X-ray films allow us to understand the development process and severity of ankylosing spondylitis more directly and accurately.

Objective To observe the effect of hyolroxylsafflor(HSYA)on cyclooxygenase-2(COX-2)/prostaglandin E2(PGE2)signaling pathway,and to investigate the protective effect and mechanism of HSYA on cerebral ischemia-reperfusion injury(CIRI).Methods Totally 90 SD male rats were randomly divided into sham-operated group(S group),operation group(CIRI group),HSYA group and celecoxib group(C group),HSYA group subdivided into HSYA low dose group(HSYA-L group),HSYA medium dose group(HSYA-M group)and HSYA high dose group(HSYA-H group),15 rats in each group.CIRI model was prepared by thread embolism method.The rats in each group were given intraperitoneal injection 30 minutes before operation.HSYA groups were given HSYA 10 mg/kg,15 mg/kg,25 mg/kg respectirely;C group was given celecoxib 40 mg/kg;S group and CIRI group were given the same amount of normal saline.Neurofunctional scores of each group of rats were performed immediately after recovery from modeling,cerebral infarction volume was measured 24 hours after reperfusion;At the same time,neuronal injury was observed by Nissl staining,the changes of COX-2 mRNA and protein were detected by Real-time PCR and Western blotting,and the changes of PGE2,tumor necrosis factor α(TNF-α)and interleukin(IL)-1β were detected by ELISA.Results Compared with the S group,in the CIRI group,neurofunctional scores increased dramatically(P＜0.05),the volume of cerebral infarction increased dramatically(P＜0.05),the damage of neurons increased and the number of neurons decreased dramatically(P＜0.05),the expressions of COX-2 mRNA and protein increased dramatically(P＜0.05),meanwhile the expressions of PGE2,TNF-α and IL-1β were also found dramatically increased(P＜0.05);Compared with the CIRI group,in the HSYA group and C group,neurofunctional scores decreased dramatically(P＜0.05),the volume of cerebral infarction was reduced dramatically(P＜0.05),the damage of neurons decreased and the number of neurons increased dramatically(P＜0.05),the expressions of COX-2 mRNA and protein,PGE2,TNF-α and IL-1β decreased dramatically(P＜0.05).The differences between HSYA groups and both HSYA-L group and HSYA-M group compared with the C group were obvious(P＜0.05),while no obvious differences were found in HSYA-H group compared with the C group(P＞0.05).Conclusion HSYA alleviates reperfusion injury in ischemic stroke may be related to the inhibition of COX-2/PGE2 signaling pathway.