Objective:To investigate long-term auditory changes and characteristics of Alport syndrome（AS） patients with different degrees of renal injury. Methods:Retrospectively analyzing clinical data of patients diagnosed AS from January 2007 to September 2022, including renal pathology, genetic detection and hearing examination. A long-term follow-up focusing on hearing and renal function was conducted. Results:This study included 70 AS patients, of which 33（25 males, 8 females, aged 3.4-27.8 years） were followed up, resulting in a loss rate of 52.9%.The follow-up period ranged from 1.1to 15.8 years, with 16 patients followed-up for over 10 years. During the follow-up, 10 patients presenting with hearing abnormalities at the time of diagnosis of AS had progressive hearing loss, and 3 patients with new hearing abnormalities were followed up, which appeared at 5-6 years of disease course. All of which were sensorineural deafness. While only 3 patients with hearing abnormalities among 13 patients received hearing aid intervention. Of these patients,7 developed end-stage renal disease（ESRD）, predominantly males （6/7）. The rate of long-term hearing loss was significantly different between ESRD group and non-ESRD group（P=0.013）. There was no correlation between the progression of renal disease and long-term hearing level（P>0.05）. kidney biopsies from 28 patients revealed varying degrees of podocyte lesion and uneven thickness of basement membrane. The severity of podocyte lesion was correlated with the rate of long-term hearing loss（P=0.048）, and there was no correlation with the severity of hearing loss（P>0.05）. Among 11 cases, theCOL4A5mutationwas most common （8 out of 11）, but there was no significant correlation between the mutation type and hearing phenotype（P>0.05）. Conclusion:AS patients exhibit progressive hearing loss with significant heterogeneity over the long-term.. THearing loss is more likely to occur 5-6 years into the disease course. Hearing abnormalities are closely related to renal disease status, kidney tissue pathology, and gene mutations, emphasizing the need for vigilant long-term hearing follow-up and early intervention.
Male ; Child ; Female ; Humans ; Nephritis, Hereditary/pathology* ; Retrospective Studies ; Kidney ; Deafness ; Hearing Loss/genetics* ; Kidney Failure, Chronic/pathology* ; Mutation

BACKGROUND:It was found that the ligands and receptors of Notch are both cell membrane surface proteins,which are important proteins to mediate intercellular communication,and the Notch signaling pathway plays a crucial regulatory role in the proliferation and differentiation of mesenchymal stem cells. OBJECTIVE:To review the regulatory mechanism of the Notch signaling pathway on the proliferation and differentiation of mesenchymal stem cells,summarize and clarify the research advance in how the Notch signaling pathway regulates the proliferation and differentiation of mesenchymal stem cells,and provide theoretical support for the future use of stem cells to treat various related diseases. METHODS:By using the computer,the first author searched the relevant studies involving Notch signaling pathway regulation of mesenchymal stem cell proliferation and differentiation on CNKI,Wanfang,VIP,PubMed,Web of Science,and Nature databases with Chinese search terms"mesenchymal stem cells,Notch,Notch signaling pathway,proliferation,differentiation"and the English search terms"mesenchymal stem cells,MSC,Notch,Notch signaling pathway,proliferation,differentiation".Part of the literature was searched in combination with the literature tracing method.Finally,87 articles were included in the review analysis. RESULTS AND CONCLUSION:(1)Notch signaling pathway is a conserved signaling pathway in multicellular organisms,which plays an important role in regulating cell differentiation,proliferation,apoptosis,and the cell cycle by mediating communication between neighboring cells through receptor-ligand binding.(2)Mesenchymal stem cells are a class of adult stem cells with self-proliferative and multi-directional differentiation potential,which can be regulated by external signaling pathways to affect their proliferation and differentiation.Notch signaling pathway,as one of them,when Notch ligands are activated,the Notch proteins will undergo two protein hydrolysis cleavages to release Notch intracellular structural domain NICD,which then enters the nucleus and thus promotes the transcription of target genes to regulate the proliferation and differentiation of mesenchymal stem cells from different sources,such as bone marrow,adipose,and umbilical cord.However,the specific mechanisms that regulate the proliferation and differentiation of mesenchymal stem cells from different tissue sources of the same species are different.(3)The Notch signaling pathway can regulate the differentiation of mesenchymal stem cells into different target cells,but due to different target cells,the expression levels of receptors or ligands in the Notch signaling pathway vary.(4)Clinical targeting of the Notch signaling pathway to promote mesenchymal stem cells for the treatment of various refractory diseases,such as aplastic anemia,severe joint injuries,ischemic strokes,and myocardial infarctions,has a promising application.(5)By exploring the Notch signaling pathway via regulating the expression levels of its receptors and ligands in bone marrow mesenchymal stem cells from rat,mouse,and human,it can be found that the Notch signaling pathway expression levels in the proliferation and differentiation of mesenchymal stem cells from different species origins are also different.(6)The role of mesenchymal stem cells in tissue engineering has been gradually highlighted due to their advantages of safety,low immune rejection,and wide therapeutic prospects.The Notch signaling pathway regulates the proliferation and differentiation of mesenchymal stem cells with a wide range of influencing factors,and subsequent studies should further optimize the influencing factor variables and explore the standardized studies of regulating the proliferation and differentiation of mesenchymal stem cells.

OBJECTIVE To optimize β-cyclodextrin(β-CD)inclusion process of Cinnamomi Ramulus Formula Granules vola-tile constituents by orthogonal test,based on standard relation and information entropy method.METHODS On the basis of single factor experiments,the ratio of β-CD to aromatic aqueous solution,the inclusion temperature,and the inclusion time were selected as the investigating factors;the inclusion rate,drug loading,and standard relation of cinnamic aldehyde in inclusion complex were used as the evaluation index.The information entropy method was used to determine the weight coefficient of each index,then the comprehen-sive score was calculated,the inclusion process conditions were optimized by orthogonal experiment.The inclusion complex was charac-terized by thin layer chromatography,ultraviolet absorption spectroscopy,Fourier infrared spectroscopy,and X-ray diffraction.RE-SULTS The best inclusion process was that the ratio of β-CD to aromatic aqueous solution was 3∶100(g·mL-1),the inclusion temperature was 50℃,and the inclusion time was 1 h.The average inclusion rate of the obtained inclusion compound was 80.84%,the drug loading was 8.63%,and the standard relation was 0.91.The results of thin-layer chromatography,ultraviolet,infrared spec-troscopy and other characterization experiments showed that the volatile components in the aromatic aqueous solution successfully en-tered the β-CD cavity,and the inclusion complex was successfully prepared.CONCLUSION The optimum inclusion process is sta-ble and feasible,which can provide references for the preparation process of Cinnamomi Ramulus Formula Granules.

Objective To explore the effect of motivational interview in the psychological counseling and management abilities of family caregivers of children with recurrent wheezing.Methods A total of 120 children with recurrent wheezing who were treated in Hangzhou Children's Hospital from May 2023 to May 2024 were selected.The family caregivers of children were randomly divided into control group and observation group,with 60 people in each group.The control group received conventional knowledge training and health education,while the observation group received motivational interviews based on control group.Compare the asthma control level,anxiety and depression levels of family caregivers of children,scores of various dimensions of the family caregivers of children management measurement scale,and satisfaction between two groups before and after intervention.Results After intervention,the frequency of recurrent wheezing in observation group was lower than that in control group,and the difference was statistically significant(P＜0.05).There was a statistically significant difference(P＜0.05)in family management measure scores between two groups before and after intervention.The satisfaction of family caregivers of children in observation group was significantly higher than that in control group,and the difference was statistically significant(P＜0.05).Conclusion Motivational interview intervention can effectively alleviate the wheezing frequency of children with recurrent wheezing,improve the response of family care,reduce anxiety and depression of family caregivers of children.

Gastric cancer with hemorrhage and cerebral infarction is a serious complica-tion with poor prognosis in clinic. Although the incidence rate is extremely low, the fatality and disability rates are very high. In addition, the opposition in treatment between the two complica-tions increases the difficulty of clinical diagnosis and treatment. The authors report the diagnosis and treatment of a gastric cancer patient with hemorrhage and new cerebral infarction, in order to to provide reference for related treatments.

Bacterial infection is the main cause of infectious diseases in children. Antibacterials play an important role in anti infection treatment of children. At present, the treatment of antimicrobial drugs in children is facing a severe situation of bacterial resistance. In January 2020, a children′s specialized hospital carried out the practice of precise management of antibiotics in combination with key performance indicators. Through the multi sectoral linkage of management and technology, eight key performance indicators and assessment methods were set up from three levels of antibiotic use, bacterial resistance and hospital infection, to standardize the clinical application of antibiotics and continue to promote the rational use of antibiotics. This practice had improved the performance indicators of antibacterial management. Among them, the use intensity of antibacterial drugs for inpatients decreased from 40.07 DDD in 2019 to 29.00 DDD in 2021, the use rate of antibacterial drugs for inpatients decreased from 81.32% to 64.40%, the percentage of antibacterial drug expenses in total drug expenses decreased from 35.41% to 26.82%, the use proportion of non restricted antibacterial drugs in antibacterial drugs increased from 71.30% to 82.21%, and the drug resistance rate of Salmonella to β-Lactam/enzyme inhibitors decreased from 4.84% to 0.03%, and the incidence of hospital infection decreased from 1.16% to 0.96%. The precise management of antibiotics in combination with key performance indicators improved the level of rational use of antibiotics, effectively curbed bacterial resistance, achieved phased results, so as to provide a reference for the scientific management of antibiotics in children′s hospitals.

Vinorelbine（NVB）is a semisynthetic vinca alkaloid and can play an anti-tumor role by inhibiting the synthesis of tubulin. Its oral preparation has been used in the treatment of a variety of tumors as its convenience and good clinical response. The blood concentration of NVB is closely related to its curative effect and toxicity. Small variations in blood concentration may reduce the curative effect and even produce serious toxicity. There are some risks in the clinical drug use due to limited clinical data and effective pharmacodynamic monitoring methods. By reviewing the relevant literature at home and abroad ，this paper summarizes the research progress of in vivo pharmacokinetics and toxicity of NVB ，fully understands the pharmacokinetic characteristics and influencing factors of NVB ，the influencing factors of toxicity ，and the application status of pharmacokinetics in the adjustment of administration scheme ，so as to provide reference for its clinical rational use.

Objective:To investigate the efficacy of flexion-lateral curvature-supination reduction combined with primary anterior surgery for the treatment of lower cervical dislocation with unilateral facet inter-locking.Methods:A retrospective analysis was performed in the 32 patients who had been admitted to Department of Spine Surgery, Honghui Hospital for lower cervical dislocation with unilateral facet interlocking from November 2015 to October 2018. According to their treatments, they were divided into 2 groups. In the emergency group treated by flexion-lateral curvature-supination reduction combined with primary anterior surgery, there were 13 males and 3 females, aged from 24 to 63 years. In the traction group treated by cranial traction reduction combined with secondary anterior surgery, there were 12 males and 4 females, aged from 20 to 64 years. The operation time, intraoperative blood loss, hospital stay, bone graft fusion, American Spinal Injury Association (ASIA) grade and Japanese Orthopaedic Association (JOA) score were compared between the 2 groups.Results:There was no significant difference in the preoperative general data between the 2 groups, showing they were comparable ( P<0.05). All patients were followed up from 26 to 40 months. The hospital stay for the emergency group [(7.2±1.2) d] was significantly shorter than that for the traction group[(10.9±1.2) d] ( P<0.05). There was no significant difference in the operation time, blood loss, ASIA grade or JOA score between the 2 groups ( P>0.05). All patients achieved osseous fusion of intervertebral space. Conclusion:Compared with traditional methods, flexion-lateral curvature-supination reduction combined with primay anterior surgery shows no significant difference in the recovery of neurological function but leads to a shorter hospital stay.

Objective:To analyze the incidence and epidemiological characteristics of traumatic spinal cord injury in China in 2018.Methods:Multi-stage stratified cluster sampling was used to randomly select hospitals capable of treating patients with spinal cord injury from 3 regions，9 provinces and 27 cities in China to retrospectively investigate eligible patients with traumatic spinal cord injury admitted in 2018. National and regional incidence rates were calculated. The data of cause of injury，injury level，severity of injury，segment and type of fracture，complications，death and other data were collected by medical record questionnaire，and analyzed according to geographical region，age and gender.Results:Medical records of 4，134 patients were included in this study，with a male-to-female ratio of 2.99∶1. The incidence of traumatic spinal cord injury in China in 2018 was 50.484 / 1 million （95% CI 50.122-50.846）. The highest incidence in the Eastern region was 53.791 / 1 million （95% CI 53.217-54.365）. In the whole country，the main causes of injury were high falls （29.58%），as well as in the Western region （40.68%），while the main causes of injury in the Eastern and Central regions were traffic injuries （31.22%，30.10%）. The main injury level was cervical spinal cord in the whole country （64.49%），and the proportion of cervical spinal cord injury in the Central region was the highest （74.68%），and the proportion of lumbosacral spinal cord injury in the Western region was the highest （32.30%）. The highest proportion of degree of injury was incomplete quadriplegia （55.20%），and the distribution pattern was the same in each region. A total of 65.87% of the patients were complicated with fracture or dislocation，77.95% in the Western region and only 54.77% in the Central region. In the whole country，the head was the main combined injury （37.87%），as well as in the Eastern and Central regions，while the proportion of chest combined injury in the Western region was the highest （38.57%）. A total of 32.90% of the patients were complicated with respiratory complications. There were 23 patients （0.56%） died in hospital，of which 17（73.91%） died of respiratory dysfunction. Conclusions:The Eastern region of China has a high incidence of traumatic spinal cord injury. Other epidemiological features include high fall as the main cause of injury cervical spinal cord injury as the main injury level，incomplete quadriplegia as the main degree of injury，head as the main combined injury，and respiratory complications as the main complication.

Objective:To explore the risk factors of residual back pain (RBP) in patients undergone PVP within 1 month and further analyze the correlation.Methods:Between March 2013 and January 2015, 1 316 patients with OVCF were treated by PVP. RBP after PVP was defined as a visual analogue scale (VAS) score of > 4 both 1 week and 1 month post-operatively. According to the pain relief, the patients were divided into two groups, the satisfied group and the unsatisfied group. All patients were scheduled for follow-up at1 week, 1 month, 3 months, and 1 year post-operatively, during which radiography and magnetic resonance imaging (T1-weighted, T2-weighted, and short time inversion recovery (STIR) sequences) were recommended to detect the existence of secondary OVCF. VAS scores and Oswestry disability index (ODI) were recorded. Demographic data, surgical information, anesthesia method, number of OVCF, injection amount of cement of single vertebral bone, imaging data and other comorbidity informations of patients in the two groups were analyzed by Logistic regression for the factors related to RBP after PVP.Results:Among 1 316 patients, 60 cases complained RBP, and the prevalence was 4.6%. VAS score and ODI of the two groups were significantly different at 1 week, 1 month and 3 months after surgery, suggesting there was a certain degree of residual pain in the lower back of patients in the unsatisfied group, which was more severe than that in the satisfied group. However, the above differences disappeared in the follow-up of 12 months after surgery.Univariate analysesshowed that preoperative bone mineral density (BMD), number of fracture, cement distribution and volume injected per level and lumbodorsal fascia contusion were associated with RBP after PVP ( P< 0.01, retrospectively). Multivariate analysis revealed that the absolute value of pre-operative BMD(odds ratio ( OR)=3.577, P=0.029), combined withlumbodorsal fascia contusion ( OR=3.805, P=0.002), number of fracture ( OR=3.440, P<0.001), satisfactory cement distribution ( OR=3.009, P=0.013) and combined with depression ( OR=3.426, P=0.028) were positively correlated with RBP after PVP, and these were risk factors. The injection amount of cement of single vertebral bone ( OR=0.079, P<0.001) was negatively correlated with RBP after PVP, which was a protective factor. Conclusion:Pre-operative low BMD, lumbodorsal fascial injury, multiple segment OVCF, insufficient cement injected volume, unsatisfactory cement distribution and depression were risk factors associated with RBP after PVP in patients with OVCF.