Objective:To investigate the impact of Waardenburg syndrome（WS） -associated Sox10 p.S100Rfs*9 mutation on inner ear function and its mechanism in noise-induced hearing impairment. Methods:A mice model carrying the Sox10 p.S100Rfs*9 mutation was established using CRISPR-Cas9 gene editing technology. Auditory phenotypes were assessed under baseline conditions and after noise exposure（96 dB SPL, 2 hours）. Auditory brainstem response（ABR） tests were performed to evaluate hearing function, combined with immunofluorescence staining of cochlear basilar membrane whole-mounts to observe hair cells and ribbon synapses. Transcriptome sequencing was conducted to analyze molecular mechanisms. Results:Sox10 p.S100Rfs*9 heterozygous mice exhibited normal hearing thresholds with characteristic ventral pigmentation abnormalities under baseline conditions. Following noise exposure, mutant mice showed significantly higher ABR thresholds at 24 000 Hz compared to wild-type controls（[60.00±6.12]vs[48.13±4.28]dB SPL, P<0.000 1）, and a significant reduction in ribbon synapses（CtBP2-positive puncta） in the basal turn（[55.0±2.3]vs[64.8±3.3]per inner hair cell, P=0.006 6）, while hair cell morphology and number remained intact. Transcriptome analysis revealed altered expression of genes involved in immune regulation, membrane structures, ion channels, and neuroactive ligand-receptor interactions. Conclusion:The Sox10 p.S100Rfs*9 mutation does not alter baseline hearing function but significantly increases inner ear susceptibility to noise damage, primarily manifested as enhanced ribbon synapse vulnerability, especially in high-frequency regions. This gene-environment interaction reveals that Sox10 haploinsufficiency may compromise noise tolerance by affecting synaptic stability and inner ear protective mechanisms. These findings provide new perspectives on the phenotypic heterogeneity in WS patients and theoretical basis for individualized noise protection strategies for patients carrying SOX10 mutations.
Animals ; SOXE Transcription Factors/genetics* ; Waardenburg Syndrome/physiopathology* ; Mice ; Hearing Loss, Noise-Induced/genetics* ; Evoked Potentials, Auditory, Brain Stem ; Mutation ; Noise ; Disease Models, Animal ; Ear, Inner/physiopathology*

Objective:To analyze the correlation between nasal resistance and lung function in children with allergic rhinitis（AR）, and explore whether AR children with increased nasal resistance are accompanied by potential lower respiratory tract involvement. Methods:A total of 88 children diagnosed with AR from December 2021 to December 2022 were selected as the study group, while 20 normal children were selected as the control group during the same period. Both the study group and the control group children underwent lung function tests, bronchodilator tests, and nasal resistance measurements. Spearman correlation analysis and multiple linear regression analysis were performed on the results of nasal resistance and lung function tests to explore the relationship and influencing factors between the two groups.According to the results of nasal resistance measurement, children with increased nasal resistance and abnormal lung function were divided into a mild increase in nasal resistance with abnormal lung function group and a moderate to severe increase in nasal resistance with abnormal lung function group. The degree of increased nasal resistance was analyzed to determine whether it would affect lung function. Results:The FEF25, FEF50, and FEF75 levels in the study group were significantly lower than those in the control group（P<0.05）. The FEV1of children with moderate to severe increase in AR nasal resistance was significantly lower than that of children with mild increase in AR nasal resistance（P<0.05）. There was a correlation between nasal resistance and FEV1/FVC, R20 in AR children, and FEV1/FVC, R20 were the influencing factors of nasal resistance in AR children（P<0.05）. There was no correlation between total serum IgE, lung function, and bronchodilation test in AR patients（P>0.05）. Conclusion:The nasal ventilation function of AR patients has changed, and there is a downward trend in small airway function. Children with moderate to severe increase in AR nasal resistance have a more significant decrease in lung ventilation function than those with mild increase. The nasal resistance of AR children is influenced by FEV1/FVC and R20, and FEV1/FVC and R20 decrease as the nasal resistance value increases. The improvement rate of lung function and FEV1 are not influencing factors for the elevation of total serum IgE.
Humans ; Child ; Rhinitis, Allergic/diagnosis* ; Nasal Polyps ; Respiratory Function Tests ; Nose ; Immunoglobulin E

Objective:To evaluate the methodological quality and reporting quality of clinical practice guidelines or consensuses in the field of Chinese pancreatic diseases from 2016 to 2021, and provide reference for formulating clinical practice guidelines and consensuses in this field.Methods:VIP, Wanfang , CNKI and CBM databases were searched for articles published from January 2016 to March 2021. The main index terms were " pancreas" , " guideline" , " consensus" , and the supplementary index terms were " pancreatitis" , " pancreatic cancer" , " pancreatic head cancer" . Two researchers independently selected the literature. The appraisal of guidelines for research and evaluation (AGREE-China) was utilized to assess the methodological quality of the guidelines or consensuses, and the reporting items for practice guidelines in healthcare (RIGHT) tool was used to assess the reporting quality.Results:A total of 14 literature were included, including 7 literature on pancreatic cancer, 3 literature on acute pancreatitis, 1 literature on chronic pancreatitis and 3 literature on others. The results of the assessment by the AGREE-China tool showed that there were no document with a total score greater than or equal to 60.0 points, two with 40.0 to 59.9 points, eleven with 20.0 to 39.9 points, and one with less than 20.0 points. Among the results of RIGHT list, basic information was reported the highest(72.62%) and funding and declaration was the lowest(0).Conclusions:The methodological and reporting quality of the guidelines or consensuses on pancreatic disease in China from 2016 to 2021 are generally not high. In the process of developing domestic guidelines or consensuses on pancreatic diseases, the guideline developer should refer to AGREE-China and RIGHT to improve the quality of clinical practice guidelines or consensuses.

OBJECTIVE：To inv estigate the effects of 4-hydroxy-2（3H）-benzoxazolone on inflammatory and apoptosis signaling pathways in non-alcoholic fatty liver disease （NAFLD）model rats. METHODS ：SD rats were divided into normal control group（10 rats）and modeling group （50 rats）. Normal control group was given basic diet ，and modeling group were given high-fat diet to induce NAFLD model. After modeling ，the rats were divided into normal control group ，model group ，silibinin group （26.25 mg/kg），and 4-hydroxy-2（3H）-benzoxazolone high-dose ，medium-dose and low-dose groups （100，50，25 mg/kg），with 8 rats in each group. Normal control group and modeling group were given 0.6％ CMC-Na intragastrically ，and other groups were given relevant medicine 10 mL/kg intragastrically ，once a day ，for consecutive 4 weeks. After last medication ，the serum levels of albumin（ALB），total protein （TP），globulin（GLB），ALB/GLB and free fatty acid （FFA）were detected ；TUNEL staining was used to observe the apoptosis of rat hepatocytes. Western blot assay was used to detect the protein expression and phosphorylation level of inflammatory signaling pathway related proteins [Toll-like receptor 4（TLR4），myeloid differentiation factor 88（MyD88）， nuclear factor-κB p65（NF-κB p65），NF-κB inhibitor protein（IκBα）] in liver tissue as well as the expression of apoptosis signaling pathway related proteins [B cell lymphoma 2（Bcl-2），Bax，caspase-3]. RESULTS ：Compared with model group ，serum levels of TP （except for low-dose group ），GLB and FFA ，the protein expression of TLR 4（except for low-dose group ），MyD88 （except for medium-dose group ）and caspase- 3，the phosphorylation levels of NF-κB p65 and IκBα protein were decreased significantly（P＜0.05 or P＜0.01）. The ratio of A LB/GLB in serum and the ratio of Bcl- 2/Bax in liver tissue were significantly increased（P＜0.05 or P＜0.01），and the phenomenon of hepatocyte apoptosis was improved. CONCLUSIONS ：4-hydroxy-2 （3H）-benzoxazolone can ameliorate NAFLD in rats ，and the mechanism may be associated with inhibiting the expression TLR 4/ MyD88/NF-κB signaling pathway-related proteins and apoptosis-related proteins in liver tissues.

OBJECTIVE：To s tudy improvement effect and mech anism of ilicifoliosids alkaloid A （HBOA）on non-alcoholic fatty liver disease in rats. METHODS ：SD rats were randomly divided into blank control group ，model group ，Silybin capsule group（positive control ，26.25 mg/kg），HBOA high-dose ，medium-dose and low-dose groups （100，50，25 mg/kg），with 10 rats in each group. Except that blank control group fed normal feed ，the other groups were continuously fed with high-fat diet for 8 weeks to induce non-alcoholic fatty liver disease model. Form the 9th week ，blank control group and model group were given constant volume of 0.6％ CMC-Na solution ，and administration groups were given corresponding drugs by intragastric admini- stration，once a day ，for consecutive 4 weeks. The general information of rats were observed and the body weight increase ，organ （liver，kidney and spleen ）indexes were calculated ；the contents of AST ，ALT，TC，TG and NEFA in liver tissue were detected ， and SOD，GSH-Px activities and MDA content in the serum were also determined. The protein expression of PPARα in liver tissue was detected by immunohistochemistry. RESULTS ：Compared with blank control group ，the body mass increase and liver index of rats in model group were increased significantly （P＜0.01）；fat deposition could be observed in the liver ；the activities of SOD and GSH-Px in serum were reduced significantly ，and the contents of MDA ，the contents of AST ，ALT，TC，TG and NEFA in liver tissue were significantly increased ，and the protein expression of PPARα was decreased significantly（P＜0.01）. Compared with model group ，the body mass increase and liver index of the rats were decreased significantly in administration groups （P＜0.05 or P＜0.01），liver fat deposition was improved ，the activity of SOD and GSH-Px in serum （except for HBOA low-dose group ）were increased significantly while MDA content ，the contents of AST ，ALT，TC（except for HBOA low-dose group ），TG（except for HBOA low-dose group ） and NEFA in liver tissue were decreased significantl y，while protein expression of PPAR α 15177460685@163.com was increased significantly （P＜0.05 or P＜0.01）. Some of the above indexes of HBOA high-dose group were 电话：0771-5302433。E-mail：junlin898@126.com significantly better than HBOA medium- and low-dose group（P＜0.05）. CONCLUSIONS ：HBOA has a certain improvement effect on non-alcoholic fatty liver disease in rats caused by high-fat diet ，and its mechanism may be related to improving lipid metabolism disorders ，anti-oxidative stress and up-regulating the expression of PPARα.

Hepatocellular carcinoma (HCC) accounts for approximately 75%-85% of primary liver cancer cases and is one of the most frequently diagnosed malignancies worldwide. Immunotherapy is currently considered to be the most promising treatment to prevent the progression and postoperative recurrence of HCC. At present, the treatment strategies of immunotherapy for HCC are classified as active immunotherapy and passive immunotherapy, including tumor vaccine therapy, immune checkpoint inhibitors, and adoptive cell therapy. Here we review the current clinical progression and discuss the future perspective on immune therapy for HCC.

Objective To investigate the pressure of male nurses in women and children′s Hospital and analyze it, so as to provide decision support for nursing administrators. Methods Used general information questionnaire, Perceptual Stress Scale, China Nurses'Pressure Source Scale (CPSS) in Sichuan,Chongqing,Yunnan,Guizhou,Tibet and other places of the maternal and child hospital male nurses, and analyzed the results. Results According to the CPSS scoring standard,63 in 101 subjects(62.4%)male nurses were in the state of stress, and the average score of stress was 26.0 ± 7.4. Binary Logistic regression showed that the main factors of male nurses′pressure were the lack of support from parents and family,low income, less promotion opportunities and non-nursing work. Conclusion The pressure situation of male nurses in maternity and child care hospitalis affected by family, work, society and so on. Managers should timely relieve the stress for male nurses and maintain the stability of male nurses′team.

Objective To investigate the therapeutic effect of Pingyangmycin combined with Triamcinolone Acetonide on the huge faciocervical lymphatic malformations (LMs) in infants.Methods Sixty-seven infants with LMs located in head and neck from January 2009 to June 2014 were retrospectively analyzed in Kunming Children's Hospital.Thirty-five males and 32 females were enrolled,aged from 1 month to 4 years,with a median age of 1.3 years.Computed tomography and ultrasonography were used to evaluate the location,size and extent of LMs before treatment in all the patients.The size of lesion varied from 5.2 cm ×7.5 cm to 9.2 cm × 10.5 cm.All patients were given local injection of Pingyangmycin combined with Triamcinolone Acetonide after puncturing fluid with uhrasonography guiding under general anesthesia.The injection was repeated every 30 d when necessary.Results The number of injections varied from 2 to 5 times,with a median number of 3.9 times.All cases were followed up for 5 to 36 months.Thirty-two cases (62.68％) were cured,improvement in 19 cases (28.36％) and no effect in 6 cases (8.96％).The total effective rate was 91.04％.There was no severe allergic reaction or pulmonary fibrosis.Secondary operation was performed after 6 months in 12 cases.Two post-operative complications were found,1 was minor paralyses of mandibular branch of facial nerve,with mouth askew,the other was trachyphonia,who were both improved after rehabilitation treatment.Conclusions In order to avoid serious complications,the huge LMs may be given local injection of Pingyangmycin combined with Triamcinolone Acetonide after puncturing fluid with ultrasonography guiding.Graded sclerotherapy provides for a less invasive and shorter course of treatment.The complications and risk of secondary resection increase slightly if sclerotherapy has no curative effect.

BACKGROUND:Angiotensin-converting enzyme as a key enzyme of the renin-angiotensin system, through the degradation effects of substance P mechanism, is involved in the occurrence and development of Alzheimer’s disease.
OBJECTIVE:To research the relationship between angiotensin-converting enzyme gene polymorphism and Alzheimer’s disease in Jiamusi region, as wel as the effect of gender and hypertension on the relationship.
METHODS:This case-control study included 96 Alzheimer’s disease patients. Another 102 subjects served as controls coming from the same area and in the same environmental condition. DNA segments were amplified using PCR in 20 g/L agarose gel electrophoresis and observed under ultraviolet lamp. II, ID, DD genotypes and genotype frequencies were calculated for statistical analysis. On this basis, according to clinical data col ected, we investigated association of Alzheimer’s disease with hypertension and gender.
RESULTS AND CONCLUSION:There was significant difference between Alzheimer’s disease patients and controls in angiotensin-converting enzyme genotypes and al ele frequency. There was statistical y significant difference between Alzheimer’s patients with hypertension and controls in angiotensin-converting enzyme genotypes and al ele frequency. There was no statistical difference between Alzheimer’s disease patients with different genders and controls in angiotensin-converting enzyme genotypes and al ele frequency. These findings indicate that there are some relationships between angiotensin-converting enzyme polymorphism and Alzheimer’s disease. II genotype is a risk factor for Alzheimer’s disease, angiotensin-converting enzyme II genotype is a risk factor for Alzheimer’s disease with hypertension.

OBJECTIVE: To discuss the clinical characteristics, imaging findings, surgical treatment and effectiveness of faciocervical lipomas in children. METHOD: Twenty children with lipomas who were performed surgical resection under the tracheal intubation anesthesia, of which 3 cases located in face and 17 cases located in neck were retrospectively analyzed. Computed tomography (CT) was used in 8 cases and magnetic resonance imaging (MRI) in 6 cases for differential diagnosis. CT and MRI were used to diagnose 6 patients pre-operatively. RESULT: The image of computed tomography (CT) revealed that 15 cases showed oval and flat circular area and 5 cases showed lobulated lesion. The CT value was negative typically. The signal was inhomogeneous in MRI. The patients' magnetic resonance imaging (MRI) presented a high signal intensity on T1 and T2WI. The hyperintense signal can be suppressed in fat-suppression T2WI sequence. The hypointense of tumor signals fell in tandem surrounding adipose tissue. All the patients got satisfied curative effect after operation. There were no complications such as trachyphonia, bucking and dyspnea. Following up for 6 months to 2 years,no recurrence case occurred. CONCLUSION Lipomas generally occur in adult patients, quite rare in children. Imaging examination is helpful in the differential diagnosis of lipoma. Surgical resection is the only effective treatment for lipomas at present.
Adult ; Child ; Diagnosis, Differential ; Head and Neck Neoplasms ; diagnosis ; surgery ; Humans ; Lipoma ; diagnosis ; surgery ; Magnetic Resonance Imaging ; Neoplasm Recurrence, Local ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome