The construction of a training system for visiting physicians in the department of rare diseases in China is an important measure to improve the overall diagnosis and treatment capacity for rare diseases and address the critical challenge of insufficient knowledge and skills among clinicians in practice. This article systematically describes the visiting physician training system established by the Department of Rare Diseases at Peking Union Medical College Hospital. It summarizes the training objectives and positioning, design logic, and learning modules of the system, aiming to provide a reference for the construction of the specialized talent team for rare diseases in China.

OBJECTIVE To investigate the neuroprotective effect and mechanism of eleutheroside B （ELB） on Parkinson’s disease （PD） model mice by regulating the IκB kinase β （IKKβ）/nuclear factor-κB （NF-κB） signaling pathway. METHODS Fifty mice were randomly divided into normal control group， model group， positive control group （selegiline hydrochloride， 10 mg/kg）， and ELB low-dose and high-dose groups （80， 160 mg/kg）， with 10 mice in each group. Each group was given relevant medicine or normal saline intragastrically for 14 consecutive days. Starting from the 10th day of administration， the model group and all administration groups were intraperitoneally injected with 1-methyl-4-phenyl-1，2，3，6-tetrahydropyridine （MPTP） 30 mg/kg， for five consecutive days to establish the chronic PD model. After the last administration for 24 h， six mice were randomly selected from each group to test their behavioral abilities； detect the levels of interleukin-1β （IL-1β）， IL-10， tumor necrosis factor-α （TNF-α） in brain tissue and their mRNA expressions were measured， and positive expression of tyrosine hydroxylase （TH）， protein expressions of TH， α -synuclein （ α -syn）， ionized calcium-binding adaptor molecule 1 （Iba-1）， as well as phosphorylation levels of IKKβ and NF-κB p65 proteins in the brain tissue were detected. The ultrastructure of neurons in substantia nigra was observed. RESULTS Compared with the model group， rotarod endurance time and climbing score of each administration group （except for the ELB low-dose group） were increased significantly （ P ＜0.05）， while the levels and mRNA expressions of IL-1β， TNF-α， α -syn， and Iba-1， as well as phosphorylation levels of IKKβ and NF-κB p65 proteins in brain tissue were decreased significantly （except for TNF-α in the ELB low-dose group）. Conversely， the level and mRNA expression of IL-10 （except for the ELB low-dose group）， TH positive expression and protein expressions were significantly increased （ P ＜0.05）. Typical neurodegenerative pathological changes， such as neuronal karyopyknosis， mitochondrial swelling and vacuolization， and endoplasmic reticulum dilation， all showed varying degrees of improvement. CONCLUSIONS ELB may exert neuroprotective effects by inhibiting the activation of the IKKβ/NF-κB signaling pathway， alleviating inflammatory responses， reducing abnormal α -syn aggregation and neuronal loss， and further improving motor dysfunction in PD mice.

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 explore the influencing factors of cognitive impairment in non-dialysis patients with chronic kidney disease(CKD).Methods A total of 60 hospitalized non-dialysis patients with CKD in the Department of Nephrology of Northern Jiangsu People's Hospital Affiliated to Yangzhou University from September 2022 to September 2023 were enrolled as research objects.According to the estimated glomerular filtration rate(eGFR),they were divided into stage 1 to 2 of CKD group[eGFR ≥60 mL/(min·1.73 m2)]with 23 cases,the stage 3 of CKD group[eGFR 30～＜60 mL/(min·1.73 m2)]with 20 cases,and stage 4 to 5 of CKD group[eGFR＜30 mL/(min·1.73 m2)]with 17 cases.The Montreal Cognitive Assessment Scale(MoCA)was used to evaluate the cognitive function of the patients.Basic data and common clinical laboratory in-dicators on hospital admission were collected to analyze the differences in cognitive function levels under different renal function statuses and to explore the influencing factors of cognitive impairment.Results The incidence rates of cognitive impairment in the stage 1 to 2 of CKD group,stage 3 of CKD group,and stage 4 to 5 of CKD group were 47.8％,85.0％,and 94.1％respectively,the median MoCA scored 26,24 and 20 respectively,with statistically significant between-group differ-ences(P＜0.05).Cognitive function was significantly negatively correlated with age(r=-0.634,P＜0.001),blood urea nitrogen(BUN)(r=-0.574,P＜0.001),serum creatinine(Cr)(r=-0.417,P＜0.001),cystatin C(Cys-C)(r=-0.327,P=0.011),serum β2-microglobulin(β2-MG)(r=-0.259,P=0.046),and N-terminal pro-brain natriuretic peptide(NT-proBNP)(r=-0.474,P＜0.001),and was significantly positively correlated with hemoglobin(HB)(r=0.401,P=0.001)and eGFR(r=0.485,P＜0.001).Multivariate Logistic regression analysis showed that age(P=0.006)and NT-proBNP(P=0.041)were influencing factors of cognitive im-pairment in non-dialysis patients with CKD.Receiver operating characteristic(ROC)curve analysis showed that the area under the curve(AUC),sensitivity,and specificity of age for prediction were 0.860,0.864 and 0.812 respectively,the AUC,sensitivity,and specificity of NT-proBNP for pre-diction were 0.808,0.795 and 0.875 respectively,and the combined prediction of age and NT-proBNP had an AUC,sensitivity,and specificity of 0.893,0.955,and 0.750,respectively.Conclusion As renal function deteriorates,the incidence rate and severity of cognitive impairment in non-dialysis patients with CKD tend to increase.Advanced age,renal function deterioration,high NT-proBNP level,and anemia are associated with the occurrence of cognitive impairment in non-di-alysis patients with CKD,among which age and NT-proBNP are influencing factors for cognitive im-pairment.

Objective To investigate the relationships of the expression of interferon-induced protein 27(IFI27)and dynamin 3(DNM3)with angiogenesis and platinum-resistant recurrence in epithelial ovarian cancer(EOC).Methods A prospective cohort study design was adopted,and 129 EOC patients scheduled for surgery combined with platinum-based chemotherapy were enrolled as the study subjects.Cancerous tissues and adjacent non-cancerous tissues were collected from the pa-tients.The expression levels of IFI27 mRNA and DNM3 mRNA in the tissue samples were detected using real-time fluorescence quantitative polymerase chain reaction(qRT-PCR).The expressions of angiogenesis markers,microvascular density(MVD)and vascular endothelial growth factor(VEGF),were detected by immunohistochemistry(IHC).After surgery,the patients received standard platinum-based chemotherapy regimen.They were regularly followed up for recurrence and divided into platinum-resistant group and platinum-sensitive group.The associations of the expressions of IFI27 and DNM3 with platinum-resistant recurrence were analyzed.Results All 129 patients completed surgery combined with chemotherapy.Forty-nine patients with chemotherapy-resistant recurrence were included in platinum-resistant group,and the remaining 80 patients were included in platinum-sensitive group.There was a significant difference in the degree of differentiation between the two groups(P=0.001).The expression level of IFI27 mRNA in cancerous tissues was higher than that in adjacent non-cancerous tissues,while the expression level of DNM3 mRNA was lower than that in adjacent non-cancerous tissues.The expression levels of MVD and VEGF were higher than those in adjacent non-cancerous tissues,with statistically significant differences(P＜0.001).In the plati-num-resistant group,the expression level of IFI27 mRNA was higher than that in the platinum-sensi-tive group,the expression level of DNM3 mRNA was lower than that in the platinum-sensitive group,and the expression levels of MVD and VEGF were higher than those in the platinum-sensitive group,with statistically significant differences(P＜0.05 or P＜0.001).Pearson correlation analy-sis showed that the expressions of VEGF and MVD in EOC patients were positively correlated with the expression of IFI27(r=0.643,0.765,P＜0.05),respectively,and negatively correlated with the expression of DNM3(r=-0.573,-0.632,P＜0.05),respectively.Low degree of differen-tiation and high expression of IFI27 were independent risk factors for platinum-resistant recurrence in EOC patients(P＜0.05),while high expression of DNM3 was an independent protective factor(P＜0.05).The receiver operating characteristic curve showed that the area under the curve(AUC)for the combined prediction of platinum-resistant recurrence in EOC patients by IFI27 and DNM3 was 0.863,which was greater than the AUCs for their individual indicators(0.815,0.704).Conclusion High expression of IFI27 and low expression of DNM3 are significantly asso-ciated with angiogenesis and platinum-resistant recurrence in EOC.Their combined detection has a high predictive efficacy for platinum-resistant recurrence in EOC patients within one year.

Objective To investigate the effect of green laser anatomical vaporization on patients with benign prostatic hyperplasia(BPH)and type 2 diabetes mellitus(T2DM),as well as its impact on inflammatory factors,quality of life,and urethral stricture.Methods A total of 120 patients with BPH and T2DM who were treated at the First Affiliated Hospital of Xinxiang Medical University from March 2021 to August 2022 were selected as the research subjects.The patients were divided into a control group and an observation group according to the surgical approach,with 60 cases in each group.Patients in the control group underwent green laser selective photovaporization,while patients in the observation group were treated with green laser anatomical vaporization.The intraoperative and postoperative recovery indicators such as operation time,intraoperative blood loss,prostate resection quality,catheter indwelling time,bladder irrigation time,and hospitalization time of patients between the two groups were compared.The serum levels of interleukin-6(IL-6)and tumor necrosis factor-α(TNF-α)were detected by using enzyme-linked immunosorbent assay,and the serum levels of malondialdehyde(MDA)and lipid peroxide(LPO)were detected by using immunofluorescence assay before surgery,3 days after surgery,and 7 days after surgery.The international prostate symptom score(IPSS)was used to assess prostate symptoms,the urinary symptom distress score(USDS)was used to assess urinary symptoms,the dynamic urodynamic monitor was used to measure urodynamics[maximum flow rate(Qmax),residual urine volume(RUV)],the enzyme-linked immunosorbent assay was used to detect the serum level of total prostate specific antigen(tPSA),the international index of erectile function-5(IIEF-5)was used to assess erectile function,and the BPH quality of life(BPH-QOL)scale was used to assess quality of life before surgery,3 months after surgery,and 6 months after surgery.The incidence of complications such as urethral stricture,urinary tract irritation,bladder neck contracture,urinary retention,and secondary bleeding was compared between the two groups.Results The operation time of patients in the observation group was significantly longer than that in the control group,the intraoperative blood loss was significantly less than that in the control group,and the bladder irrigation time,catheter indwelling time,and hospitalization time were significantly shorter than those in the control group(P＜0.05).There was no statistically significant difference in the quality of prostatectomy between the two groups of patients(P＞0.05).Before surgery,there was no statistically significant difference in the levels of serum TNF-α,IL-6,MDA,and LPO between the two groups of patients(P＞0.05).On postoperative days 3 and 7,the levels of serum TNF-α,IL-6,MDA,and LPO in both groups were significantly higher than those before surgery,and the levels of serum TNF-α,IL-6,MDA,and LPO in the observation group were significantly lower than those in the control group(P＜0.05).Before surgery,there was no statistically significant difference in the IPSS and USDS scores between the two groups of patients(P＞0.05).At 3 and 6 months after surgery,the IPSS and USDS scores of patients in both groups were significantly lower than those before surgery,and the IPSS and USDS scores of patients in the observation group were significantly lower than those in the control group(P＜0.05).Before surgery,there was no statistically significant difference in RUV,Qmax,and serum tPSA levels between the two groups of patients(P＞0.05).At 3 and 6 months after surgery,the RUV and serum tPSA levels of patients in both groups were significantly lower than those before surgery,while the Qmax was signifi-cantly higher than that before surgery(P＜0.05).The RUV and serum tPSA levels of patients in the observation group were significantly lower than those in the control group,while the Qmax was significantly higher than that in the control group(P＜0.05).Before surgery,there was no statistically significant difference in the IIEF-5 and BPH-QOL scores of patients between the two groups(P＞0.05).At 3 and 6 months after surgery,the IIEF-5 scores of patients in both groups were significantly lower than those before surgery,while the BPH-QOL scores were significantly higher than those before surgery(P＜0.05).The IIEF-5 and BPH-QOL scores of patients in the observation group were significantly higher than those in the control group(P＜0.05).The total incidence of complications in the control group and the observation group was 16.67％(10/60)and 5.00％(3/60),respectively,and the total incidence of complications in the observation group was significantly lower than that in the control group(x2=4.227,P＜0.05).Conclusion Green laser anatomical vaporization in the treatment of BPH patients with T2DM can optimize the surgical process,reduce inflammatory stress and the risk of complications,promote early postoperative recovery,improve urodynamics and sexual function,relieve symptoms,and enhance quality of life.

Objective: To explore the differences in the gut microbiota of primary school students with different levels of sugar sweetened beverage intake, so as to provide scientific evidence for better identification of health risks in children and the development of targeted health policies. Methods: In June 2022, a total of 192 healthy primary school students from Chengdu were selected using a stratified cluster random sampling method. The sugar sweetened beverage intake was assessed through a dietary frequency questionnaire. Based on the median daily sugar sweetened beverage intake, primary school students were categorized into a low intake group ( n =96) and a high intake group ( n =96). The gut microbiota in fresh fecal samples from the two groups of primary school students was analyzed using 16S rRNA high throughput sequencing, and the diversity and community structure differences in the gut microbiota were compared. Results: Children in the low intake group had a sugar sweetened beverage intake of (21.3±1.6) mL/d, while the high intake group had an intake of (269.6±37.3) mL/d. Diversity analysis results showed that there were no statistically significant differences between the low intake and the high intake group in terms of α diversity metrics: Observed_otus index [298.50 (259.75, 342.25), 305.50 (244.25, 367.75)], Goods_coverage index [1.00 (1.00, 1.00), 1.00 (1.00, 1.00)], Chao index [304.18 (260.75, 348.78), 305.88 (245.68, 370.88)], Shannon index [5.88 (5.29, 6.45), 5.71 (4.89, 6.28)] and Simpson index [0.95 (0.91, 0.97), 0.94 (0.88, 0.97)] ( Z =-0.64, -0.76, -0.54, -1.76, -1.67, P >0.05). Furthermore, no statistically significant difference was observed in β diversity between the two groups ( R 2=0.006, P >0.05). At the genus level, the abundance of Blautia [0.033 (0.018, 0.055)] and Fusicatenibacter [0.009 (0.005, 0.015)] were higher in the low intake group compared to the high intake group [0.024 (0.013, 0.041),0.006 (0.003, 0.011)]and differences were statistically significant ( Z =-2.52, -2.81, P <0.05). LEfSe analysis highlighted intergroup differences primarily in Blautia, Fusicatenibacter and Sarcina( LDA= 3.56,3.12,3.53, P <0.05). Conclusions There is no significant difference in the diversity and overall structure of the gut microbiota in primary school students with different levels of sugar sweetened beverage intake. However, there are species variations at the genus level. The information can serve as a scientific basis for identifying health risks in primary school students and formulating targeted health strategies.

Objective: To explore the mediating effect of rumination between life events and learning value doubt among junior high school students, so as to provide reference for the psychological health education of junior high school students. Methods: From March to July 2023, a total of 930 junior high school students from Guangdong, Jiangsu and Hebei were selected by a combination of convenient sampling methods. Participants completed the Scales of Value of Learning (SVL), Ruminative Response scale (RRS) and Adolescent Self rating Life Events Checklist (ASLEC). The t test and ANOVA were used for inter group comparisons and Pearson correlation analysis was used for correlation analysis and bootstrap method was used for mediating effect test. Results: The life events of junior middle school students were positively correlated with learning value doubt ( r =0.18), rumination ( r = 0.65 ) and its three dimensions (brooding, reflective pondering, and symptom focused rumination) ( r =0.60, 0.59, 0.66). Learning value doubt was positively correlated with rumination ( r =0.15) and its three dimensions ( r =0.07, 0.13, 0.19) ( P <0.05).The symptom focused rumination (effect size=0.09, 95% CI =0.06-0.11) and brooding (effect size=-0.07, 95% CI =-0.09--0.04) dimensions partially mediated the relationship between life events and learning value doubt. In the two influential pathways, symptom focused rumination exerts a stronger impact, and the difference was statistically significant (effect size=0.15, 95% CI =0.11- 0.20 , excluding 0) ( P <0.05). Conclusions Life events experienced by junior high school students can positively predict learning value doubt through symptom focused rumination, and negatively predict learning value doubt through brooding. Active attention should be paid to the mental health status of junior high school students, intervene promptly when they experience life events, reduce symptom based rumination, and improve their learning enthusiasm.

Objective: To investigate the prospective effects of intake of each food group on the development of lung function of pupils,so as to provide theoretical basis for promoting the healthy development of lung function and preventing chronic respiratory diseases in Chinese children. Methods: A cluster stratified sampling method was used to select a total of 893 pupils in grades 2-5 from Chengdu in November 2021. Dietary data of respondents were collected using a food frequency questionnaire within the past year,then the food group intake was categorized into T1, T2 and T3 from low to high by the trichotomous method, and anthropometric measurements including lung capacity were obtained in 2022. Logistic regression models and test for trend were used to analyze the prospective effects of intake of each food group on lung function development of pupils. Results: Among male students, consumption of vegetables [118.6(50.5, 188.2)g/d] and milk and dairy products [200.0(73.3, 250.0)g/d] were higher in the excellent lung capacity group than in the non excellent lung capacity group [90.0(37.1, 192.9), and 178.6(35.7, 250.0)g/d],with statistically significant differences ( Z =-1.98, -2.24); among girls, the group with excellent lung capacity consumed less staple food [391.1(273.6, 511.4)g/d] than the group with non excellent lung capacity [407.4(309.5, 594.3)g/d], and the group with excellent lung capacity consumed more aquatic products [31.2(14.6, 69.8)g/d] and milk and dairy products [215.0(107.1, 250.1) g/d ] than that of the non excellent lung capacity [19.4(10.7, 58.3), 114.3(35.7, 250.0)g/d] ( Z =-2.01, -3.33, -5.10)( P < 0.05 ). After adjusting for energy, body mass index Z score(BMI Z ), mother s education level, averge family income monthly, whether presence of smokers in the living environment, and whether participation in physical activities during the past week, among male students, T3 group of vegetable intake ( OR =0.48, 95% CI = 0.27-0.86), T2 group of bean and soy product intake ( OR = 0.52 , 95% CI =0.27-0.96)，T2 and T3 groups of milk and dairy products intake (T2： OR =0.54, 95% CI =0.31-0.93; T3： OR = 0.52 , 95% CI =0.30-0.90) were negatively associated with non excellent lung capacity ( P <0.05). Among girls, T3 group of aquatic product intake( OR =0.52, 95% CI =0.28-0.97), T2 and T3 groups of milk and dairy product (T2: OR =0.44, 95% CI =0.25- 0.76 ;T3: OR =0.33, 95% CI =0.19-0.59) were negatively associated with nonexcellent lung capacity, whereas the T2 group of red meat intake ( OR =2.51, 95% CI =1.37-4.67) was positively associated with non excellent lung capacity. Non excellent lung capacity was found to be negatively associated with vegetable and milk and dairy product intake in boys by test for trend; in girls, milk and dairy products intake was negatively associated with non excellent lung capacity, whereas red meat intake was positively associated with non excellent lung capacity ( t =-1.13，-0.44；-3.03，1.95, P trend <0.05). Conclusions Milk and dariy products intakes reduce the risk of non excellent lung capacity in pupils, vegetables intakes reduce the risk of non excellent lung capacity in boys, and the intake of red meat increases the risk of non excellent lung capacity in girls. Promoting rational food choices is necessary for children to improve healthy lung development.