Objective:To analyze and summarize the clinical, genotypic and pathological characteristics of children with PAX2 gene mutation in China, and to provide information for the monitoring, treatment and prognosis of the disease. Methods:It was a case series analysis study. The clinical data of children with PAX2 gene mutation in Pediatric Nephrology Department, Tongji Hospital Affiliated to Tongji Medical College, Huazhong University of Science and Technology from January 2014 to December 2022 were collected, and peripheral blood gene DNA was extracted and sequenced for whole exome sequencing. The clinical, pathological and genotypic characteristics of PAX2 gene variation of children in China were summarized by searching PubMed, Medline, China National Knowledge Infrastructure and Wanfang database and compared with the cases in this single center. Results:Among the 13 children with PAX2 gene mutation, there were 9 males and 4 females, 12 patients with abnormal urine tests, 7 patients with small kidney volume by imaging examination, and 5 patients with renal cysts. The clinical phenotypes were congenital renal and urinary tract malformations in 8 cases, renal coloboma syndrome in 1 case, and hematuria or proteinuria in 3 cases. Five patients underwent renal biopsies, showing focal segmental glomerulosclerosis and C3 glomerulopathy in 1 case, focal segmental glomerulosclerosis in 1 case, thin basement membrane lesion in 1 case, and IgA nephropathy in 2 cases. The genetic testing in 13 children showed 9 de novo mutations and 4 new mutations of c.321G>A, c.213-8C>G, c.63C>A and c.449C>T. There were 2 cases of 76dupG (p.V26Gfs*28) mutant. A total of 51 Chinese children with PAX2 gene mutation were found in the literature search. There were 32 males and 19 females, 8 cases with small kidney volume and 12 cases with renal cysts. The clinical phenotypes were congenital anomalies of kidney and urinary tract in 28 cases, renal coloboma syndrome in 17 cases, and hematuria or proteinuria in 6 cases. Seven patients underwent renal biopsies, including 2 cases with focal segmental glomerulosclerosis, 1 case with minimal lesion, 1 case with mesangial proliferative glomerulonephritis, 1 case with IgA nephropathy, 1 case with membranous nephropathy and a case with focal proliferative sclerosing purpura nephritis combined with glomerular hypertrophy. Thirty-four cases were de novo mutations, and 12 mutations were from the father or mother. The father or mother of 5 children had no clinical manifestations, with normal renal function. There were 11 cases of 76dupG (p.V26Gfs*28) mutant. Conclusions:The clinical phenotypes and genotypes of PAX2 gene variation in Chinese children are diverse. The most common clinical phenotype of PAX2 gene variation is congenital anomalies of kidney and urinary tract. c.76dupG (p.V26Gfs*28) is the most common of PAX2 gene variant.

Fire safety is crucial for the regular operation of large public hospitals.This paper elaborates on the funda-mental guidelines for fire safety management in large hospitals.Under the framework of collaborative governance theory,it also analyzes and points out that the standardization of peoples behavior in hospitals and the integrity of fire safety facilities are the pri-mary factors influencing the efficacy of fire safety management.Subsequently,based on management practices,it suggests focu-sing on"five synergies"in enforcing primary responsibility for fire safety,namely,the implementation of fire safety main respon-sibilities in synergy,the regular inspection and rectification of fire hazards in synergy,the training and evaluation of basic fire safety skills in synergy,the lifecycle management of fire safety facilities in synergy,and the building of a fire safety management team in synergy.This approach aims to maximize overall fire safety effectiveness and comprehensively enhance the level of fire safety management in hospitals.Finally,the paper discusses and calls for further enhancements in the management of fire safety in large public hospitals.

Objective:To investigate the prognosis and risk factors of IgA vasculitis nephritis (IgAVN) in children.Methods:A retrospective cohort study was conducted. Clinical data were collected from 264 children who were pathologically diagnosed with IgAVN at Department of Pediatric Nephrology, Tongji Hospital, affiliated with Tongji Medical College, Huazhong University of Science and Technology, between January 2011 and December 2017. All patients had a follow-up period of more than 3 years. Clinical characteristics, renal pathology, 3-year and 5-year prognosis were analyzed. The patients were grouped based on gender, age of onset (≤6 years, >6-9 years, and >9 years), pathological classification (≤Ⅲ and>Ⅲ),whether the prognosis was complete remission at 3 and 5 years. Independent sample t-tests, ANOVA or chi-squared test were used for intergroup comparisons. Spearman correlation analysis was applied for ordinal data, and multivariate Logistic regression was used to analyze factors affecting the prognosis. Receiver operating characteristic (ROC) curve was utilized to evaluate the predictive value of these factors. Results:Of the 264 children with IgAVN, 153 were male and 111 were female, the age of onset was 8.3 (6.7, 10.3) years, 118 patients (45%) with onset age >6-9 years accounted for the highest proportion. All patients presented with skin purpura and renal involvement, primarily manifesting as hematuria and/or proteinuria. Microscopic hematuria was observed in 253 patients (95.8%), while 246 patients (93.2%) showed proteinuria. In 256 patients (97.0%), hematuria or proteinuria urinalysis was detected within 6 months of skin purpura onset, and 243 patients (92.0%) underwent renal biopsy within 6 months of renal involvement. The most common clinical subtype in 264 IgAVN children was hematuria and proteinuria (204 cases, 77.3%), with grade Ⅲ being the predominant pathological classification (181 cases, 68.6%). Among children ≤6 years old, the 3-year complete remission rate was higher in males than in females (83.9% (26/31) vs. 7/16, χ2=8.12, P=0.012). Factors independently associated with poor 5-year prognosis included time from hematuria or proteinuria urinalysis to renal biopsy >6 months, elevated serum cholesterol levels, and incomplete remission 3 years post-biopsy ( OR=5.41, 1.39, 6.02, 95% CI 1.40-20.86, 1.04-1.84, 2.61-13.88, all P<0.05). The serum cholesterol has a predictive value for 5-year prognosis ( P=0.020, AUC=0.62, 95% CI 0.52-0.71, Youden index=0.27, cutoff=4.37). Conclusions:For children with IgAVN aged≤6 years, the 3-year prognosis is better in males than in females. Time from hematuria or proteinuria urinalysis to renal biopsy >6 months, elevated serum cholesterol levels, and incomplete remission at 3 years post-biopsy may be independent risk factors for poor 5-year prognosis in children with IgAVN.

Objective:Peritoneal free cancer cells can negatively impact disease progression and patient outcomes in gastric cancer.This study aimed to investigate the feasibility of using golden-angle radial sampling dynamic contrast-enhanced magnetic resonance imaging(GRASP DCE-MRI)to predict the presence of peritoneal free cancer cells in gastric cancer patients.Methods:All enrolled patients were consecutively divided into analysis and validation groups.Preoperative magnetic resonance imaging(MRI)scans and perfusion were performed in patients with gastric cancer undergoing surgery,and peritoneal lavage specimens were collected for examination.Based on the peritoneal lavage cytology(PLC)results,patients were divided into negative and positive lavage fluid groups.The data collected included clinical and MR information.A nomogram prediction model was constructed to predict the positive rate of peritoneal lavage fluid,and the validity of the model was verified based on data from the verification group.Results:There was no statistical difference between the proportion of PLC-positive cases predicted by GRASP DCE-MR and the actual PLC test.MR tumor stage,tumor thickness,and perfusion parameter Tofts-Ketty model volume transfer constant(Ktrans)were independent predictors of positive peritoneal lavage fluid.The nomogram model featured a concordance index(C-index)of 0.785 and 0.742 for the modeling and validation groups,respectively.Conclusions:GRASP DCE-MR could effectively predict peritoneal free cancer cells in gastric cancer patients.The nomogram model constructed using these predictors may help clinicians to better predict the risk of peritoneal free cancer cells being present in gastric cancer patients.

Objective:To investigate the tissue methylation features of adenocarcinoma patients presenting as pulmonary nodules on CT scans.Methods:A retrospective analysis was conducted on 70 adenocarcinoma patients with pulmonary nodules diagnosed at the Shanghai General Hospital from June 1, 2022 to January 20, 2024. Participants were assigned to two groups using the random number table, with 40 in the discovery group and 30 in the validation group. In the discovery group, tissue samples were analyzed using reduced representation bisulfite sequencing (RRBS) technology to compare the average methylation levels between cancer tissues and paired adjacent non-cancerous tissues. Differentially methylated regions (DMRs) were screened for analysis of their distribution across various genomic functional elements, and hierarchical clustering was plotted. GO and KEGG pathway enrichment analyses were further conducted on the DMRs. Subsequently, candidate DMRs associated with lung adenocarcinoma were validated using TCGA lung adenocarcinoma cohort and targeted bisulfite sequencing technology in the validation group. The comparison of methylation levels between groups was conducted using t-tests or non-parametric tests, while rates and composition ratios were analyzed using chi-square tests or Fisher′s exact test.Results:In discovery cohort, the average methylation level in cancer tissues was lower compared to adjacent normal tissues [(42.369±4.627) vs (44.370±4.046), t=?2.059, P=0.043]. A total of 37 995 DMRs were identified, including 16 889 upregulated regions and 21 106 downregulated regions, predominantly locating in promoter regions (48.917%), introns (36.457%), and exons (10.812%). The DMR clustering heatmap revealed two distinct clusters corresponding to cancer tissues and adjacent non-cancerous tissues. GO analysis showed that DMRs associated genes were mainly located in the cell membrane and nuclear chromatin, and were primarily involved in RNA polymerase Ⅱ-related transcription and regulation. KEGG pathway enrichment analysis indicated that DMRs associated genes were mainly involved in neuroactive ligand-receptor interaction, cancer pathways, calcium signaling pathway, cAMP signaling pathway, and MAPK signaling pathway. Validation in the TCGA cohort confirmed 11 potential characteristic DMRs. In the validation group, TBS confirmed that the methylation levels of DMRs associated with MIR10B, DMRTA2, HOPX, TFAP2B and MARCH11 in cancer tissues were significantly higher than those in adjacent non-cancerous tissues [11.200(4.305, 27.088) vs 2.650(1.298, 4.645), Z=?4.539, P<0.05; 18.610(13.600, 33.025) vs 8.675(5.488, 13.085), Z=?4.554, P<0.05; 17.600(2.183, 76.015) vs 1.085(0.898, 1.835), Z=?5.131, P<0.05; 5.250(3.220, 7.693) vs 3.495(2.165, 4.383), Z=?2.861, P<0.05; 11.515(7.525, 21.033) vs 7.830(5.518, 11.488), Z=?2.440, P<0.05 ], and the differences were statistically significant. Conclusions:Lung adenocarcinoma tissue exhibits different methylation patterns compared with adjacent normal lung tissue. The identified DMRs are involved in the regulation of several key pathways. Results from the TCGA cohort and an independent validation group support the potential diagnostic value of DMRs such as MIR10B, DMRTA2, HOPX, TFAP2B, and MARCH11 in lung adenocarcinoma, though their clinical application requires further validation.

Objective:To study the feasibility of a modified pancreatogastric anastomosis in laparoscopic duodenum preserving pancreatic head resection (LDPPHR).Methods:The clinical data of 25 patients with benign or low-grade malignant tumors of pancreatic head undergoing LDPPHR at the Second Hospital of Hebei Medical University from January 2019 to May 2023 were retrospectively analyzed, including 7 males and 18 females, aged (44.9±6.2) years old. According to the methods of pancreatic digestive reconstruction, patients were divided into the observation group ( n=10), who underwent the modified pancreatogastric anastomosis, and the control group ( n=15) who underwent conventional pancreaticojejunal anastomosis and jejuno-jejunal anastomosis. The general data, intraoperative pancreatic digestive reconstruction time, maximum levels of amylase in abdominal drainage within three days postoperatively, postoperative complications, and hospital stay were compared between the groups. Results:All procedures were performed successfully. The intraoperative pancreatic digestive reconstruction time was shorter in the observation group [(27.8±2.4) min vs. (45.8±3.6) min, P=0.010]. The intraoperative blood loss were comparable between the groups [(140.5±14.8) ml vs. (145.2±9.7) ml, P=0.843]. The maximum level of amylase in abdominal drainage within three days postoperatively was lower in the observation group [(809.1±185.5) U/L vs. (1 385.4±481.1) U/L, P=0.031]. No grade C pancreatic fistula or postoperative hemorrhage occurred in either group, and the incidence of grade B pancreatic fistula was lower in the observation group [20.0% (2/10) vs. 60.0% (9/15), P=0.048], with a shorter postoperative hospital stay [(7.9±1.3) d vs. (10.3±2.7) d, P=0.017]. No decrease of life quality or reoperation due to pancreatic fistula, hemorrhage or digestive tract malfunction occurred in either group within a median follow-up of 15.6 months. Conclusion:In LDPPHR, the modified pancreatogastric anastomosis could help shorten the pancreatic digestive reconstruction and lower the risk of postoperative pancreatic fistula.

Previous studies have comprehensively evaluated predictive models related to peripherally inserted central catheter-related venous thrombosis (PICC-RVT) , but have not further elaborated on its application and effect in the tumor patient population. This article reviews the PICC-RVT evaluation tools that are clinically mature and applicable to cancer patients, analyzes the presentation forms, statistical methods, and limitations of different models, in order to provide suggestions and references for model selection by clinical healthcare professionals and the construction of future PICC-RVT prediction models for cancer patients.

Objective:To observe the effects of different kinesio taping methods on hand swelling, shoulder pain, upper limb motor function and ability in the activities of daily living of stroke survivors with shoulder-hand syndrome.Methods:Sixty stroke survivors with shoulder-hand syndrome were randomly divided into groups A, B, C and a control group, each of 15. In addition to routine rehabilitation training and drug treatment, as well as claw-shaped and I-shaped taping of the hand and wrist, group A received I-shaped kinesio taping, B received Y-shape and C received I-shape plus Y-shaped taping of the shoulder. Before and after 4 weeks, the drainage method was employed to calculate the difference in volume between the two hands. Their temperatures were also measured. The subjects reported shoulder pain using a visual analog scale (VAS). Upper limb motor functioning was quantified using Fugl-Meyer scores, and difficulties in the activities of daily living were evaluated using the modified Barthel index (MBI).Results:Before the treatment there were no significant differences among the four groups in terms of any of the measurements. Afterward the treatment, significant improvement was observed in the volume and temperature differences between hands, as well as in the VAS, FMA and MBI scores. After the treatment, group C′s average FMA score was significantly higher than those of the other groups. There was no significant difference in MBI scores among the four groups.Conclusions:Supplementing rehabilitation training with I-shaped plus Y-shaped kinesio taping can effectively reduce the volume and temperature differences between the hands, relieve shoulder pain, and improve effectiveness in the activities of daily living of persons with shoulder-hand syndrome after a stroke. Hand-claw and wrist-I taping also have some effect.

Objective:To compare the specific IgE positive rates of the patients between allergic respiratory diseases and respiratory infectious diseases in Guangzhou, the relationship between the co-sensitization of house dust mite (HDM) allergen and Aspergillus fumigatus (AF) allergen and asthma, allergic rhinitis with asthma, pneumonia, upper respiratory infections, bronchitis, serum total immunoglobulin E (total Immunoglobulin E, tIgE) and age were analyzed, to provide the basis for the prevention and treatment of respiratory allergic diseases and respiratory infectious diseases in this area.Methods:A total of 2 535 patients with confirmed respiratory allergic diseases and respiratory infectious diseases were selected retrospectively from the outpatient or inpatient department of the First Affiliated Hospital of Guangzhou Medical University from April 2017 to June 2021 and detected HDM and AF specific IgE (sIgE) by the ImmunoCAP system. The age range was 1 to 89 years. The median age was 5 years. The average age was 9. ≤3 years old group n=894, 4-6 years old group n=721, 7-18 years old group n=615, 19-49 years old group n=207, >49 years old group n=98. There were 1 596 males (62.96%) and 939 females (37.04%). There were 1 279 cases of allergic diseases and 1 256 cases of respiratory infectious diseases. The different disease groups were divided into asthma group (411 cases), allergic rhinitis group (458 cases), allergic rhinitis combined with asthma group (410 cases), pneumonia group (463 cases), upper respiratory tract infection group (299 cases) and bronchitis group (494 cases). The difference of specific IgE (sIgE) and tIgE between HDM and AF was analyzed. For statistical analysis, continuous variables were tested by Mann-Whitney U. Classification data by chi-square test or Fisher′s exact test. Results:1 313 (51.79%) patients were sIgE positive for HDM allergen, 65 (2.56%) were sIgE positive for AF allergen, and 50 (1.97%) were both positive. In the respiratory allergic disease group, 877 cases (68.57%,877/1 279) were positive for HDM allergen sIgE, 57 cases (4.46%,57/1 279) were positive for AF allergen sIgE, and 44 cases (3.44%,44/1 279) were both positive; 436 cases (34.71%,436/1 256) of respiratory infectious diseases were positive for HDM allergen sIgE, 8 cases (0.64%,8/1 256) were positive for AF allergen sIgE, and 6 cases (0.48%,6/1 256) were both positive. In monosensitization, the HDM allergen sIgE sensitization rate was the highest in the allergic rhinitis & asthma group, at 80.24% (329/410). The positive rate of HDM allergen sIgE in male patients was 53.76%(858/1 596), and the positive rate in female patients was 46.22%(434/939), and the difference between the two was statistically significant (χ 2=13.449, P<0.001). In polysensitization, asthma patients (5.35%,22/411) had the highest positive rate of HDM sensitization with AF, followed by allergic rhinitis patients (3.06%,14/458), allergic rhinitis with asthma (1.95%,8/410). The positive rate of respiratory infectious diseases such as pneumonia (0.43%,2/463), upper respiratory infections (0.33%,1/299), and bronchitis (0.61%,3/494) with AF was extremely low. The positive rate of HDM combined with AF in infants(≤3 years old group,0.34%, 3/894; 4-6 years old group, 0.97%, 7/721)was significantly lower than that in teenagers and adults(7-18 years old group,3.58%, 22/615; 19-49 years old group,6.28%, 13/207;>49 years old group,5.10%, 5/98).In the patients with HDM and AF combined sensitization, HDM sIgE levels were distributed in all grades, and AF sIgE levels were mainly in grades 1, 2, and 3. Conclusion:The positive rate of HDM combined with AF was higher in patients with respiratory allergic diseases such as asthma, allergic rhinitis, and allergic rhinitis combined with asthma, suggesting that clinical attention should be paid to the combination of HDM and AF in patients with asthma and allergic rhinitis, especially adults, more likely to be combined with AF.

Objective:To compare the specific IgE positive rates of the patients between allergic respiratory diseases and respiratory infectious diseases in Guangzhou, the relationship between the co-sensitization of house dust mite (HDM) allergen and Aspergillus fumigatus (AF) allergen and asthma, allergic rhinitis with asthma, pneumonia, upper respiratory infections, bronchitis, serum total immunoglobulin E (total Immunoglobulin E, tIgE) and age were analyzed, to provide the basis for the prevention and treatment of respiratory allergic diseases and respiratory infectious diseases in this area.Methods:A total of 2 535 patients with confirmed respiratory allergic diseases and respiratory infectious diseases were selected retrospectively from the outpatient or inpatient department of the First Affiliated Hospital of Guangzhou Medical University from April 2017 to June 2021 and detected HDM and AF specific IgE (sIgE) by the ImmunoCAP system. The age range was 1 to 89 years. The median age was 5 years. The average age was 9. ≤3 years old group n=894, 4-6 years old group n=721, 7-18 years old group n=615, 19-49 years old group n=207, >49 years old group n=98. There were 1 596 males (62.96%) and 939 females (37.04%). There were 1 279 cases of allergic diseases and 1 256 cases of respiratory infectious diseases. The different disease groups were divided into asthma group (411 cases), allergic rhinitis group (458 cases), allergic rhinitis combined with asthma group (410 cases), pneumonia group (463 cases), upper respiratory tract infection group (299 cases) and bronchitis group (494 cases). The difference of specific IgE (sIgE) and tIgE between HDM and AF was analyzed. For statistical analysis, continuous variables were tested by Mann-Whitney U. Classification data by chi-square test or Fisher′s exact test. Results:1 313 (51.79%) patients were sIgE positive for HDM allergen, 65 (2.56%) were sIgE positive for AF allergen, and 50 (1.97%) were both positive. In the respiratory allergic disease group, 877 cases (68.57%,877/1 279) were positive for HDM allergen sIgE, 57 cases (4.46%,57/1 279) were positive for AF allergen sIgE, and 44 cases (3.44%,44/1 279) were both positive; 436 cases (34.71%,436/1 256) of respiratory infectious diseases were positive for HDM allergen sIgE, 8 cases (0.64%,8/1 256) were positive for AF allergen sIgE, and 6 cases (0.48%,6/1 256) were both positive. In monosensitization, the HDM allergen sIgE sensitization rate was the highest in the allergic rhinitis & asthma group, at 80.24% (329/410). The positive rate of HDM allergen sIgE in male patients was 53.76%(858/1 596), and the positive rate in female patients was 46.22%(434/939), and the difference between the two was statistically significant (χ 2=13.449, P<0.001). In polysensitization, asthma patients (5.35%,22/411) had the highest positive rate of HDM sensitization with AF, followed by allergic rhinitis patients (3.06%,14/458), allergic rhinitis with asthma (1.95%,8/410). The positive rate of respiratory infectious diseases such as pneumonia (0.43%,2/463), upper respiratory infections (0.33%,1/299), and bronchitis (0.61%,3/494) with AF was extremely low. The positive rate of HDM combined with AF in infants(≤3 years old group,0.34%, 3/894; 4-6 years old group, 0.97%, 7/721)was significantly lower than that in teenagers and adults(7-18 years old group,3.58%, 22/615; 19-49 years old group,6.28%, 13/207;>49 years old group,5.10%, 5/98).In the patients with HDM and AF combined sensitization, HDM sIgE levels were distributed in all grades, and AF sIgE levels were mainly in grades 1, 2, and 3. Conclusion:The positive rate of HDM combined with AF was higher in patients with respiratory allergic diseases such as asthma, allergic rhinitis, and allergic rhinitis combined with asthma, suggesting that clinical attention should be paid to the combination of HDM and AF in patients with asthma and allergic rhinitis, especially adults, more likely to be combined with AF.