OBJECTIVES: To study the clinical characteristics of pediatric Behçet syndrome (BS). METHODS: A retrospective review was conducted on the medical records of children hospitalized in the Department of Pediatrics at the Second Hospital of Hebei Medical University between December 2014 and December 2024 who met diagnostic criteria for BS. RESULTS: Among 45 children with BS, 26 (58%) were male. Oral aphthous ulcers were the most common manifestation (43/45, 96%), followed by genital ulcers (23/45, 51%) and gastrointestinal involvement (18/45, 40%). Genital ulcers were more frequent in girls, whereas ocular involvement was more common in boys (P<0.05). The pathergy test was positive in 10 (22%), and HLA-B51 was positive in 13 (29%). Fecal calprotectin (FC) was elevated in 16 (36%); gastrointestinal involvement was more frequent in children with elevated FC than in those with normal FC (P<0.05). According to the respective criteria, 17 (38%) patients met the International Study Group criteria (1990), 33 (73%) met the International Criteria for Behçet Disease (2014), and 13 (29%) met the Pediatric Behçet Disease criteria (2015). CONCLUSIONS Pediatric BS shows marked clinical heterogeneity. HLA-B51 is associated with disease susceptibility.
Humans ; Behcet Syndrome/genetics* ; Male ; Female ; Child ; Retrospective Studies ; Adolescent ; Child, Preschool ; Leukocyte L1 Antigen Complex/analysis* ; HLA-B51 Antigen

The density and composition of lymphocytes infiltrating colon tumors serve as predictive factors for the clinical outcome of colon cancer.Our previous studies highlighted the potent anti-cancer properties of the principal compounds found in Garcinia yunnanensis(YTE-17),attributing these effects to the regu-lation of multiple signaling pathways.However,knowledge regarding the mechanism and effect of YTE-17 in the prevention of colorectal cancer is limited.In this study,we conducted isobaric tags for relative and absolute quantification(iTRAQ)analysis on intestinal epithelial cells(IECs)exposed YTE-17,both in vitro and in vivo,revealing a significant inhibition of the Wnt family member 5a(Wnt5a)/c-Jun N-terminal kinase(JNK)signaling pathway.Subsequently,we elucidated the influence and mechanism of YTE-17 on the tumor microenvironment(TME),specifically focusing on macrophage-mediated T helper 17(Th17)cell induction in a colitis-associated cancer(CAC)model with Wnt5a deletion.Additionally,we performed the single-cell RNA sequencing(scRNA-seq)on the colonic tissue from the Wnt5a-deleted CAC model to characterize the composition,lineage,and functional status of immune mesenchymal cells during different stages of colorectal cancer(CRC)progression.Remarkably,our findings demon-strate a significant reduction in M2 macrophage polarization and Th17 cell phenotype upon treatment with YTE-17,leading to the restoration of regulatory T(Treg)/Th17 cell balance in azoxymethane(AOM)/dextran sodium sulfate(DSS)model.Furthermore,we also confirmed that YTE-17 effectively inhibited the glycolysis of Th17 cells in both direct and indirect co-culture systems with M2 macrophages.Notably,our study shed light on potential mechanisms linking the non-canonical Wnt5a/JNK signaling pathway and well-established canonical β-catenin oncogenic pathway in vivo.Specifically,we proposed that Wnt5a/JNK signaling activity in IECs promotes the development of cancer stem cells with β-catenin activity within the TME,involving macrophages and T cells.In summary,our study undergoes the po-tential of YTE-17 as a preventive strategy against CRC development by addressing the imbalance with the immune microenvironment,thereby mitigating the risk of malignancies.

OBJECTIVES: To improve the understanding of the clinical phenotypes and genetic characteristics of nephronophthisis (NPHP) and related syndromes in children. METHODS: A retrospective analysis was performed on the medical data of eight children with NPHP and related syndromes who were diagnosed and treated in the Department of Pediatrics of the Second Hospital of Hebei Medical University, from January 2018 to November 2022. The clinical characteristics and genetic testing results were analyzed. RESULTS: Among these eight children, there were five boys and three girls, with an age of onset ranging from 15 months to 12 years. All 8 children exhibited different degrees of renal function abnormalities when they attended the hospital. Among the eight children, two had the initial symptom of delayed development, two had the initial symptom of anemia, and two were found to have abnormal renal function during physical examination. The extrarenal manifestations included cardiovascular abnormalities in two children, skeletal dysplasia in two children, liver dysfunction in one child, retinitis pigmentosa in one child, and visceral translocation in one child. All eight children had renal structural changes on ultrasound, and four children had mild to moderate proteinuria based on routine urine test. Of all eight children, five had NPHP1 gene mutations and one each had a gene mutation in the NPHP3, IFT140, and TTC21B genes, and four new mutation sites were discovered. CONCLUSIONS Children with NPHP and related syndromes often have the initial symptom of delayed development or anemia, and some children also have extrarenal manifestations. NPHP and related syndromes should be considered for children with unexplained renal dysfunction, and high-throughput sequencing may help to make a confirmed diagnosis.
Child ; Humans ; Retrospective Studies ; Syndrome ; Kidney Diseases, Cystic/genetics* ; Mutation ; Phenotype

Background Long working hours (LWH) and shift work are harmful to the physical and mental health of occupational groups. Objective To understand the status quo of LWH and shift work of migrant workers in Shanghai, and to analyze the influence of the above two factors on sleep of the population. Methods From July to September 2018, a cross-sectional survey using questionnaires was conducted among 3215 in-service employees at seven workplaces with more than 300 employees in six selected districts of Shanghai using multi-stage random sampling method. A total of 2976 valid questionnaires were collected, with a valid response rate of 92.6%. The questionnaires included general demographic characteristics, personal health behavior, work-related factors, weekly working hours, shift work, and sleep. Univariate analysis and logistic regression analysis were used to investigate the relationships of LWH and shift work with sleep, and an interaction item was included to evaluate potential interaction between LWH and shift work. Result The average age of the subjects was (30.98±9.49) years old, the male to female ratio was 1∶1.20, and 2382 workers were reported without local residency (80.0%). Among the migrant workers, the average weekly working hours was (57.23±13.14) h, the proportions of LWH (weekly working hours>40 h), extra LWH (weekly working hours>55 h), and shift work were 80.6% (1919/2382), 60.3% (1436/2382), and 25.4% (600/2366), respectively, all above were significantly higher than those of local workers. The prevalence rates of insomnia and lack of sleep among the migrant workers were 46.3% (1091/2356) and 25.4% (597/2354), respectively. The results of logistic regression model showed that after adjusting demographic characteristics such as gender, age, education level, and monthly income, as well as occupation and personal health behavior, compared with working hours ≤40 h per week, working 55-60 h per week was a risk factor for insomnia of migrant workers (OR=1.33, 95%CI: 1.02-1.72), while working >60 h per week was a risk factor for both insomnia (OR=1.37, 95%CI: 1.05-1.78) and insufficient sleep (OR=1.73, 95%CI: 1.28-2.35) of migrant workers. Shift work was only associated with insomnia (OR=1.37, 95%CI: 1.11-1.69). Meanwhile, working hours>60 h per week and shift work had an interaction effect on insomnia of migrant workers (OR=2.35, 95%CI: 1.20-4.60). Conclusion LWH and shift work are prominent among migrant workers, and there is an interaction between LWH and shift work with insomnia.

Objective:To explore the clinical effects of transplantation of turbocharged bipedicle deep inferior epigastric perforator (DIEP) flap in breast reconstruction.Methods:A retrospective observational study was used. From December 2008 to December 2016, 24 patients who met the inclusion criteria were treated in the Department of Plastic Surgery of Hunan Cancer Hospital, all patients were female, aged 28-51 (36.5±1.6) years. All cases received turbocharged bipedicle DIEP flap for two-staged breast reconstruction. According to the patterns of turbocharged vessels anastomosis, the turbocharged bipedicle DIEP flaps with length of (27.5±0.3) cm and width of (12.8±1.4) cm, were divided into three types: distal end of pedicle anastomosis type, main branch of pedicle anastomosis type, and muscular branch of pedicle anastomosis type. After complete hemostasis in the donor region, the anterior sheath was repaired with intermittent suture, and umbilical reconstruction was completed. Two negative pressure drainage tubes were indwelled, and subcutaneous tissue and skin were sutured layer by layer. The specific ways of vascular anastomosis of the flap pedicle with the internal thoracic vessels of recipient site included anastomosing the proximal end of one artery and one vein, anastomosing the proximal and distal end of one artery and one vein, and anastomosing the proximal end of one artery and two veins. Postoperatively, the survival and blood supply of flaps were observed. The patients were followed up to observe the reconstructed breast shape satisfaction, donor site complications, abdominal wall function, and scar hyperplasia.Results:All turbocharged bipedicle DIEP flaps for two-staged breast reconstruction survived well, with good blood supply. During follow-up for 14 to 56 (20±6) months, the shape of reconstructed breasts was satisfied. Only linear scar was left in the donor sites of abdomen with no complications, and the function of abdominal wall was not affected.Conclusions:For patients with clear indications, transplantation of free turbocharged bipedicle DIEP flap is a safe, reliable, and satisfactory choice for breast reconstruction with autologous tissue.

Objective To investigate the risk factors of adult urolithiasis in China. Methods 14 areas including 11 communities and 19 villages were randomly selected from 7 provinces of China by multi-stage stratified cluster sampling method during the period of May 2013 to July 2014. Individuals were investigated by a face-to-face questionnaire and a physical examination including urinary tract ultrasonographic examinations, routine blood and urine tests and blood biochemical examination ect. Results In total, 1 447 participants were found with the urolithiasis among 9 310 individuals and the overall prevalence was 15.5% (1 447/9 310). The prevalence of urolithiasis was significantly different among 14 areas ( 2=711.523，P<0.001), the lowest was the village in Shanxi (0.76%) and the highest was the village in Guangdong(35.99%). The intercept-only model further indicated the reginal aggregation for the individuals of urolithiasis (t=2.48, P=0.027) and the ICC was 48.74%. The two-level Logistic regression model showed that the gender (OR=1.235, 95% CI:1.082-1.411, P=0.005), age (OR=1.101, 95% CI:1.047-1.158, P=0.001), diabetes mellitus (OR=1.411，95%CI:1.192-1.670, P=0.001), family history of urinary calculi (OR=1.867, 95% CI:1.500-2.323, P<0.001), LDL (OR=1.150, 95% CI:1.050-1.260, P=0.006), drinking coffee (OR=1.352, 95% CI:1.065-1.716, P=0.017) and drinking sodas (OR=1.547, 95% CI:1.203-1.990, P=0.002) were the risk factors for urolithiasis. By contrast, consumed more fermented vinegar (OR=0.567, 95% CI:0.498-0.645, P<0.001) and had a amount of legume (OR=0.726, 95% CI:0.628-0.839, P<0.001) were protective factors of urolithiasis. Conclusion The prevalence of urolithiasis among adults reveal an aggregation in area-level, influenced by life environment and dietary habits of individual.

Objective: This study was to understand the current situation of long working hours and identify the association of long working hours and job stress and depression among workers from one district company of state grid. Methods: The project was done with the cross-sectional survey. All questionnaires were completed by self-administered with informed consent. Employees’ weekly working hours was reported by themselves; Using the PHQ-9 scale to assess depression, the JDC Model to evaluate job stress, and SPSS software to analyze data, the single factor chi-square test and multivariate Logistic analysis were performed to identify the association between long working hours and job stress, depression. Results: there were 35.3 percent employees with over 44 hours a week. The association between increasing weekly working hours and job stress and depression was statistically significant. Compared to the reference group (≤44 h) , for those who worked more than 44 hours per week, the odds ratio of job stress was 2.02 (95%CI=1.27-3.23) times increased, and the odds ratio of depression was 1.60 (95%CI=1.01-2.55) times increased, especially for those (≥60 h) , the odds ratio was 3.69 (95%CI=1.45-9.41) and 2.76 (95%CI=1.09-7.03) respectively. Conclusion Long working hours can significantly increase the risk of job stress and depression among workers. When their weekly working hours exceeds 60, long working hours will be the independent risk factor for both job stress and depression.

Objective To explore the application of various forms of anterolateral thigh flap in lower extremity and ankle amputation with extensive skin and soft tissue defect reconstruction.Methods From September 2011 to December 2015,26 cases of lower extremity and ankle amputation with extensive skin and soft tissue defect reconstruction (included 20 male patients and 6 female patients) were admitted,the age ranged from 22 to 61 years old (38.6 ± 4.5).Among these cases,10 cases received flow-through anterolateral thigh flap,16 cases used chimeric anterolateral thigh flap with vastus lateralis thigh muscle flap,15 flaps were thinned in one-staged,5 flaps were harvested in double-paddled form.The flap size ranged from 10 cm × 6 cm to 28 cm × 12 cm.The donor site was closed directly in all cases.Results All flaps and replanted extremities survived uneventfully.All patients were followed up for 8 to 30 months with satisfied esthetic and functional results in reconstructed foot and ankle,the texture of flaps was good,no bulky was noted and no second revision was needed.No local ulcer happened and regained protective sensation.Only linear scar left in the donor sites,no hernia occurred.Conclusions Various forms of anterolateral thigh flap is the effective choice in lower extremity and ankle amputation with extensive skin and soft tissue defect reconstruction.

Background and objectiveEpidermal growth factor receptor-tyrosine kinase inhibitors (EGFR-TKIs) are the standard ifrst-line treatment regimen forEGFR mutated non-small cell lung cancer (NSCLC) patients. However, the ef-ifcacy of EGFR-TKIs widely varies. hTe aim of this study is to determine whether the pretreatment serum cytokeratin-19 frag-ments (CYFAR21-1) and carcinoembryonic antigen (CEA) are associated with the effcacy of EGFR-TKIs inEGFR-mutated NSCLC patients.MethodsWe retrospectively enrolled 194 NSCLC patients harboringEGFR mutations who received EGFR-TKIs. Clinical characteristics were collected, and the relation between the effcacy of EGFR-TKIs and pretreatment serum CYFAR21-1 and CEA was analyzed.Results In all cases, progression-free survival (PFS) in patients with high CYFAR21-1 level was signiifcantly shorter than PFS in patients with normal CYFAR21-1 (7.0vs 11.9 months,P<0.001). Overall survival (OS) in patients with high CYFAR21-1 was signiifcantly shorter than in the normal-CYFAR21-1 group (12.6vs28.0 months, P<0.001). In adenocarcinoma patients, PFS in the high-CYFAR21-1 level group was signiifcantly shorter than in patients with normal CYFAR21-1 (7.0vs 12.0 months,P<0.001). OS in patients with high CYFAR21-1 was signiifcantly shorter than that in the normal-CYFAR21-1 group (13.1vs 28.1 months,P<0.001). Among squamous carcinoma patients, CYFAR21-1 level did not affect survival. No signiifcant difference in PFS and OS was observed between patients with high CEA and patients with normal CEA.ConclusionEGFR-mutated patients with high CYFAR21-1 had signiifcantly shorter PFS and OS than patients with normal CYFAR21-1 atfer receiving EGFR-TKIs. Pretreatment serum CYFR21-1 level was a predictive marker of EGFR-TKI treatment inEGFR-mutated NSCLC patients.

PURPOSE: Autophagy is one of the ways to degrade unfolded proteins after endoplasmic reticulum (ER) stress. The purpose of this study is to determine whether a blockade of autophagy leads to aggravated endoplasmic reticulum stress, which then induces cells apoptosis in HeLa cells treated with paclitaxel. MATERIALS AND METHODS: Autophagy activation and the proapoptotic effects were characterized using monodansylcadaverine labeling and Hoechest staining, respectively. A Western blot analysis was used to detect the expression of apoptotic and autophagy-related genes. A flow cytometry was used to assess the cell apoptosis ratio. RESULTS: Paclitaxel exposure induced the aggregation of autophagosomes in the cytoplasms of cervical cancer HeLa cells. The expression of Beclin 1 and LC3 II were upregulated, but p62 was downregulated, which suggests that autophagy was promoted by paclitaxel. On the other hand, the expression of GRP78 obviously increased, suggesting that ER stress was induced after paclitaxel treatment. The cell proliferation assay indicated that a knockdown of Beclin 1 sensitized HeLa cells to paclitaxel. Furthermore, paclitaxel-mediated apoptotic cell death was further potentiated by the pretreatment with autophagy inhibitor chloroquine or small interfering RNA against Beclin 1. These results suggest that an induction of autophagy by paclitaxel may induce cell survival rather than cell death in HeLa cells; moreover, inhibition of autophagy led to an aggravated ER stress and an induction of downstream apoptosis. CONCLUSION: Our results reveal autophagy induced by paclitaxel conferred protection of tumor cells against apoptosis, and blockade of autophagy subsequently aggravated ER stress, enhancing the apoptosis associated with paclitaxel treatment in HeLa cells.
Apoptosis ; Autophagy* ; Blotting, Western ; Cell Death ; Cell Proliferation ; Cell Survival ; Chloroquine ; Cytoplasm ; Endoplasmic Reticulum ; Endoplasmic Reticulum Stress* ; Flow Cytometry ; Hand ; HeLa Cells ; Humans ; Paclitaxel* ; RNA, Small Interfering ; Uterine Cervical Neoplasms*