Single-cell transcriptomic profiling of the mouse cochlea: An atlas for targeted therapies.

Functional molecular characterization of the cochlea has mainly been driven by the deciphering of the genetic architecture of sensorineural deafness. As a result, the search for curative treatments, which are sorely lacking in the hearing field, has become a potentially achievable objective, particularly via cochlear gene and cell therapies. To this end, a complete inventory of cochlear cell types, with an in-depth characterization of their gene expression profiles right up to their final differentiation, is indispensable. We therefore generated a single-cell transcriptomic atlas of the mouse cochlea based on an analysis of more than 120,000 cells on postnatal day 8 (P8), during the prehearing period, P12, corresponding to hearing onset, and P20, when cochlear maturation is almost complete. By combining whole-cell and nuclear transcript analyses with extensive in situ RNA hybridization assays, we characterized the transcriptomic signatures covering nearly all cochlear cell types and developed cell type-specific markers. Three cell types were discovered; two of them contribute to the modiolus which houses the primary auditory neurons and blood vessels, and the third one consists in cells lining the scala vestibuli. The results also shed light on the molecular basis of the tonotopic gradient of the biophysical characteristics of the basilar membrane that critically underlies cochlear passive sound frequency analysis. Finally, overlooked expression of deafness genes in several cochlear cell types was also unveiled. This atlas paves the way for the deciphering of the gene regulatory networks controlling cochlear cell differentiation and maturation, essential for the development of effective targeted treatments.

3D ZnII-Based coordination polymer: Synthesis, structure and fluorescent sensing property for nitroaromatic compounds.

A water-stable ZnII-based coordination polymer (CP) with excellent photophysical behavior, namely [Zn2L(atez)(H2O)2] (compound 1; H3L = 4-(2',3'-dicarboxylphenoxy); atez = 5-aminotetrazole), was successfully prepared by the solvothermal reaction of Zn ions with a π-conjugated and semi-rigid multicarboxylate ligand H3L in the presence of N-containing linker atez. Compound 1 displays a hierarchically pillared three-dimensional (3D) (3,4,5)-connected (4·62) (42·64) (43·64·83) net which is based on two-dimensional (2D) multicarboxylate- ZnII layers strutted by the atez ligands. Sensing investigations of compound 1 reveal that this material can selectively and sensitively detect nitroaromatic compounds in water suspension through fluorescence quenching effect. In particular, it is worth noting that it shows highly specific detection of nitrobenzene (NB) and 2,4,6-trinitrophenol (TNP) with remarkable quenching constants (KSV = 7.5 × 104 M-1 for NB and KSV = 1.9 × 105 M-1 for TNP) and low limit of detection (LOD = 0.93 µM for NB and LOD = 0.36 µM for TNP). Investigations reveal that the probable mechanisms for such sensing processes are the concurrent presence of fluorescence resonance energy transfer (FRET) as well as photoinduced electron transfer (PET) between the CP and nitroaromatic molecules. This work not only offers an effective route to improve the application of fluorescent CPs but also provide one novel probable fluorescence probe for nitroaromatic compounds.

Multifunctional Cd-CP for fluorescence sensing of Cr(VI), MnO4-, acetylacetone and ascorbic acid in aqueous solutions.

The development of multifunctional fluorescent chemosensors for the detection of multiple targets remains challenging but of great importance. In this paper, one novel coordination polymer (CP), denoted as [Cd2(edda)(phen)2]∙H2O (compound 1, H4edda = 5,5' (ethane-1,2-diylbis(oxy)) diisophthalic acid, phen = 1,10-phenanthroline) is successfully designed and prepared under hydrothermal conditions. Structural analysis indicates that compound 1 possesses a one-dimensional (1D) double chain structure, then self-assembles into a three-dimensional (3D) supramolecular framework via π…π interactions between phen molecules. Interestingly, compound 1 is found to be tolerant in wide range of acidic to alkaline aqueous solutions (pH = 2-13). Fluorescent spectral investigations reveal that compound 1 exhibits highly selective and sensitive fluorescence responses toward MnO4-, Cr(VI) ions, acetylacetone (acac) and ascorbic acid (AA) by fluorescence quenching in the aqueous phase. The detection limits are in the very low range, reaching µM level for the detection of MnO4-, Cr(VI) ions, nM for AA and ppm for acac detection. The distinguished multi-responsive performance suggests compound 1 to be a potential multifunctional probe. Furthermore, the possible quenching mechanisms have also been systematically investigated in this work.

Cognitive and Brain Gray Matter Changes in Children with Obstructive Sleep Apnea: A Voxel-Based Morphological Study.

BACKGROUND: To explore the neural difference between children with obstructive sleep apnea (OSA) and healthy controls, together with the relation between this difference and clinical severity indicator of children with OSA. METHODS: Twenty-seven children with OSA (7.6 ± 2.5 years, apnea hypopnea index [AHI]: 9.7 ± 5.3 events/h) and 30 healthy controls (7.8 ± 2.6 years, AHI: 1.7 ± 1.2 events/h) were recruited and matched with age, gender, and handedness. All children underwent 3.0 T magnetic resonance imaging of the brain and cognitive testing evaluating. Volumetric segmentation of cortical and subcortical structures and voxel-based morphometry were performed. Pearson's correlation analysis was performed between these features of gray matter volume (GMV) and obstructive apnea index (OAI) among children with OSA. RESULTS: In the comparison of children's Wechsler test scores of full-scale intelligence quotient and verbal intelligence quotient, the OSA group was significantly lower than the control group (p < 0.05). Compared with the control group, the GMV of many brain regions in the OSA group was significantly decreased (p < 0.05). In the correlation analysis of GMV and OAI in OSA group, right inferior frontal gyrus volume was significantly negatively correlated with OAI (r = - 0.49, p = 0.02). CONCLUSION: Children with OSA presented abnormal neural activities in some brain regions and impaired cognitive functions. This finding suggests an association between the OSA and decreased GMV in children.

Accuracy and capability of tri-ponderal mass index in assessing cardio-metabolic risk factors in Chinese children and adolescents aged 3 to 17 years, compared with body mass index / 中华医学杂志(英文版)

BACKGROUND@#Tri-ponderal mass index (TMI) has been reported to be a more accurate estimate of body fat than body mass index (BMI). This study aims to compare the effectiveness of TMI and BMI in identifying hypertension, dyslipidemia, impaired fasting glucose (IFG), abdominal obesity, and clustered cardio-metabolic risk factors (CMRFs) in 3- to 17-year-old children.@*METHODS@#A total of 1587 children aged 3 to 17 years were included. Logistic regression was used to evaluate correlations between BMI and TMI. Area under the curves (AUCs) were used to compare discriminative capability among indicators. BMI was converted to BMI- z scores, and accuracy was compared by false-positive rate, false-negative rate, and total misclassification rate.@*RESULTS@#Among children aged 3 to 17 years, the mean TMI was 13.57 ± 2.50 kg/m 3 for boys and 13.3 ± 2.33 kg/m 3 for girls. Odds ratios (ORs) of TMI for hypertension, dyslipidemia, abdominal obesity, and clustered CMRFs ranged from 1.13 to 3.15, higher than BMI, whose ORs ranged from 1.08 to 2.98. AUCs showed similar ability of TMI (AUC: 0.83) and BMI (AUC: 0.85) in identifying clustered CMRFs. For abdominal obesity and hypertension, the AUC of TMI was 0.92 and 0.64, respectively, which was significantly better than that of BMI, 0.85 and 0.61. AUCs of TMI for dyslipidemia and IFG were 0.58 and 0.49. When 85th and 95th of TMI were set as thresholds, total misclassification rates of TMI for clustered CMRFs ranged from 6.5% to 16.4%, which was not significantly different from that of BMI- z scores standardized according to World Health Organization criteria.@*CONCLUSIONS@#TMI was found to have equal or even better effectiveness in comparison with BMI in identifying hypertension, abdominal obesity, and clustered CMRFs TMI was more stable than BMI in 3- to 17-year-old children, while it failed to identify dyslipidemia and IFG. It is worth considering the use of TMI for screening CMRFs in children and adolescents.

S1PR1 serves as a viable drug target against pulmonary fibrosis by increasing the integrity of the endothelial barrier of the lung

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with unclear etiology and limited treatment options. The median survival time for IPF patients is approximately 2-3 years and there is no effective intervention to treat IPF other than lung transplantation. As important components of lung tissue, endothelial cells (ECs) are associated with pulmonary diseases. However, the role of endothelial dysfunction in pulmonary fibrosis (PF) is incompletely understood. Sphingosine-1-phosphate receptor 1 (S1PR1) is a G protein-coupled receptor highly expressed in lung ECs. Its expression is markedly reduced in patients with IPF. Herein, we generated an endothelial-conditional S1pr1 knockout mouse model which exhibited inflammation and fibrosis with or without bleomycin (BLM) challenge. Selective activation of S1PR1 with an S1PR1 agonist, IMMH002, exerted a potent therapeutic effect in mice with bleomycin-induced fibrosis by protecting the integrity of the endothelial barrier. These results suggest that S1PR1 might be a promising drug target for IPF therapy.

Current situation and strategy analysis of research ward construction in Beijing municipal hospitals / 中华医院管理杂志

Objective:To understand the current situation and problems in the construction of research wards in Beijing municipal hospitals, and put forward corresponding strategies and suggestions to explore the path for the construction of research hospitals.Methods:From March 2020 to May 2021, through the method of questionnaire and expert interview, the bed and personnel allocation, projects undertaken, review efficiency and problems faced by the research wards of 22 Beijing municipal hospitals were analyzed in depth.Results:82%(18/22)of the municipal hospitals explored the construction of research wards, and 27%(6/22) were Beijing Demonstration research wards construction units. In 2020, the overall performance of the construction of research ward was as follows: the investment of clinical research resources was increased, the ability of clinical research was enhanced, the ability to serve enterprises in Beijing was improved, the efficiency of examination was improved, but the enrollment rate of clinical research(including clinical trials) was reduced. At present, the main problems were focused on investment, personnel and informatization.Conclusions:The construction of research wards in Beijing municipal hospitals is developing steadily, but there are still some problems, such as lack of clinical research talents, performance incentive and guarantee to be strengthened. In the future, we should strengthen the training of clinical research talents, improve the incentive and guarantee mechanism, promote the integration of industry, university and research, accelerate the construction of clinical research information system, further improve the hospital′s clinical research ability and level, and accumulate experience for the construction of research hospitals.

[Characteristics of Industrial Volatile Organic Compounds(VOCs) Emission in China from 2011 to 2019].

In order to better understand the industrial volatile organic compounds(VOCs) emissions in China in recent years, an industrial VOCs emission inventory was developed from 2011 to 2019, based on the dynamic emission factors method and the comprehensive source classification system. The results showed that VOCs emissions increased first from 11122.7 kt in 2011 to 13397.9 kt in 2017, and then decreased to 13247.0 kt in 2019. The emission structure of the four source categories changed. The contribution from basic organic chemical industries, gasoline storage and transportation, manufacturing(i.e., coatings, inks, pigments, and similar products), and industrial protective coatings continued to increase. On the contrary, the contributions of oil and natural gas processing, automobile, and container manufacturing industries declined over the study period. Among the industrial emissions in China in 2019, industrial coating, printing, and basic organic chemical industries emitted large amounts of VOCs(accounting for 39.2% of the total emission), and because their contribution became increasingly prominent since 2011, these sectors will be the key emission sources in the future. With respect to the spatial distribution in 2019, East China and South China had the largest VOCs emissions. Shandong, Guangdong, Jiangsu, and Zhejiang were the four provinces that contributed the most, accounting for 40.6% of the total VOCs emissions.

Single-cell transcriptomic atlas of individuals receiving inactivated COVID-19 vaccines reveals distinct immunological responses between vaccine and natural SARS-CoV-2 infection

To control the ongoing COVID-19 pandemic, CoronaVac (Sinovac), an inactivated vaccine, has been granted emergency use authorization by many countries. However, the underlying mechanisms of the inactivated COVID-19 vaccine-induced immune response remain unclear, and little is known about its features compared to SARS-CoV-2 infection. Here, we implemented single-cell RNA sequencing (scRNA-seq) to profile longitudinally collected PBMCs (peripheral blood mononuclear cells) in six individuals immunized with CoronaVac and compared these to the profiles of COVID-19 infected patients from a Single Cell Consortium. Both inactivated vaccines and SARS-CoV-2 infection drove changes in immune cell type proportions, caused B cell activation and differentiation, and induced the expression of genes associated with antibody production in the plasma. The inactivated vaccine and SARS-COV-2 infection also caused alterations in peripheral immune activity such as interferon response, inflammatory cytokine expression, innate immune cell apoptosis and migration, effector T cell exhaustion and cytotoxicity, however, the magnitude of change was greater in COVID-19 patients, especially those with severe disease, than in immunized individuals. Further analyses revealed a distinct peripheral immune cell phenotype associated with CoronaVac immunization (HLA class II upregulation and IL21R upregulation in naive B cells) versus SARS-CoV-2 infection (HLA class II downregulation and IL21R downregulation in naive B cells severe disease). There were also differences in the expression of important genes associated with proinflammatory cytokines and thrombosis. In conclusion, this study provides a single-cell atlas of the systemic immune response to CoronaVac immunization and reveals distinct immune responses between inactivated vaccines and SARS-CoV-2 infection.

Proteomic and metabolomic signatures associated with the immune response in healthy individuals immunized with an inactivated SARS-CoV-2 vaccine

SummeryCoronaVac (Sinovac), an inactivated vaccine for SARS-CoV-2, has been widely used for immunization. However, analysis of the underlying molecular mechanisms driving CoronaVac-induced immunity is still limited. Here, we applied a systems biology approach to understand the mechanisms behind the adaptive immune response to CoronaVac in a cohort of 50 volunteers immunized with 2 doses of CoronaVac. Vaccination with CoronaVac led to an integrated immune response that included several effector arms of the adaptive immune system including specific IgM/IgG, humoral response and other immune response, as well as the innate immune system as shown by complement activation. Metabolites associated with immunity were also identified implicating the role of metabolites in the humoral response, complement activation and other immune response. Networks associated with the TCA cycle and amino acids metabolic pathways, such as phenylalanine metabolism, phenylalanine, tyrosine and tryptophan biosynthesis, and glycine, serine and threonine metabolism were tightly coupled with immunity. Critically, we constructed a multifactorial response network (MRN) to analyze the underlying interactions and compared the signatures affected by CoronaVac immunization and SARS-CoV-2 infection to further identify immune signatures and related metabolic pathways altered by CoronaVac immunization. These results suggest that protective immunity against SARS-CoV-2 can be achieved via multiple mechanisms and highlights the utility of a systems biology approach in defining molecular correlates of protection to vaccination.

Practice of collaborative development model of pediatrics′s medical practice, education and research at Beijing municipal hospitals / 中华医院管理杂志

Collaborative development among medical practice, education and research is a strategic decision of the country in disciplinary development guided by the innovation-driven strategy. In October 2017, Beijing Hospitals Authority organized 18 tertiary hospitals with pediatrics discipline and founded a collaborative development center for pediatrics. This center operated in a model featuring both leadership of due authorities and autonomous administration. Two of the specialized pediatrics hospitals work as leading units, and existing high quality pediatrics resources of the member hospitals were pooled to establish an academics committee and an executive committee. A development system was established with disciplinary construction as the focus, collaborative development as the goal and horizontal collaboration as the means. It was designed to explore a new model featuring overall planning and standardized management of the discipline, building of a shared platform for clinical capacity development, joint development of continued medical education and talent cultivation, as well as diversified and multi-centered research and platform resources sharing. This model can effectively promote the overall development level of pediatrics in Beijing municipal hospitals.

Central Sleep Apnea Alters Neuronal Excitability and Increases the Randomness in Sleep-Wake Transitions.

OBJECTIVE: While most studies on Central Sleep Apnea (CSA) have focused on breathing and metabolic disorders, the neuronal dysfunction that causes CSA remains largely unknown. Here, we investigate the underlying neuronal mechanism of CSA by studying the sleep-wake dynamics as derived from hypnograms. METHODS: We analyze sleep data of seven groups of subjects: healthy adults (n = 48), adults with obstructive sleep apnea (OSA) (n = 29), adults with CSA (n = 25), healthy children (n = 40), children with OSA (n = 18), children with CSA (n = 73) and CSA children treated with CPAP (n = 10). We calculate sleep-wake parameters based on the probability distributions of wake-bout durations and sleep-bout durations. We compare these parameters with results obtained from a neuronal model that simulates the interplay between sleep- and wake-promoting neurons. RESULTS: We find that sleep arousals of CSA patients show a characteristic time scale (i.e., exponential distribution) in contrast to the scale-invariant (i.e., power-law) distribution that has been reported for arousals in healthy sleep. Furthermore, we show that this change in arousal statistics is caused by triggering more arousals of similar durations, which through our model can be related to a higher excitability threshold in sleep-promoting neurons in CSA patients. CONCLUSIONS: We propose a neuronal mechanism to shed light on CSA pathophysiology and a method to discriminate between CSA and OSA. We show that higher neuronal excitability thresholds can lead to complex reorganization of sleep-wake dynamics. SIGNIFICANCE: The derived sleep parameters enable a more specific evaluation of CSA severity and can be used for CSA diagnosis and monitor CSA treatment.

Status analysis and management reflection of the development of pediatric young scientists / 中华医学科研管理杂志

Objective:Through analysis of the development status of pediatric and the problems faced by pediatric young scientists, combined with the situation of research management, this paper puts forward some suggestions.Methods:Collect the identified problems of pediatric development, summarize the common ones and propose methodological strategies for scientific research management.Results:The development of pediatrics faces problems such as insufficient resources, unreasonable resource allocation and talents shortage.Conclusions:It is recommended to support the development of pediatric according to optimize the scientific research management model, establish a reasonable evaluation and incentive mechanism, establish a pediatric talent training model and team, improve the pediatric research level, and obtain policy support for salary, talent introduction and training, promotion of professional titles, research resources and so on.

DNABarcodeCompatibility: an R-package for optimizing DNA-barcode combinations in multiplex sequencing experiments.

SUMMARY: Using adequate DNA barcodes is essential to unambiguously identify each DNA library within a multiplexed set of libraries sequenced using next-generation sequencers. We introduce DNABarcodeCompatibility, an R-package that allows one to design single or dual-barcoding multiplex experiments by imposing desired constraints on the barcodes (including sequencer chemistry, barcode pairwise minimal distance and nucleotide content), while optimizing barcode frequency usage, thereby allowing one to both facilitate the demultiplexing step and spare expensive library-preparation kits. The package comes with a user-friendly interface and a web app developed in Java and Shiny (https://dnabarcodecompatibility.pasteur.fr), respectively, with the aim to help bridge the expertise of core facilities with the experimental needs of non-experienced users. AVAILABILITY AND IMPLEMENTATION: DNABarcodeCompatibility can be easily extended to fulfil specific project needs. The source codes of the R-package and its user interfaces are publicly available along with documentation at [https://github.com/comoto-pasteur-fr] under the GPL-2 licence. SUPPLEMENTARY INFORMATION: Supplementary data are available at Bioinformatics online.

Diagnosis and treatment of lymphatic malformations / 中华耳鼻咽喉头颈外科杂志

More than seventy percent of lymphatic malformations (LMs) occur in head and neck region. The management of head and neck LMs is challenging,especially for diffuse and infiltrative cases.It can cause deformity and death in severe patients. Surgical resection is the main method to treat LMs in the past and sclerotherapy has also greatly improved the treatment out come of LMs over the past 20 years. However, it is still hardly to achieve satisfactory prognosis for the patients with extensively infiltrative lesions, regardless of surgical resection or sclerotherapy. In the past five years, some scholars have made a preliminary exploration of new treatment methods, such as oral medicine, to diversify the treatment options of head and neck LMs. This article will review the general situation, biological properties, clinical characteristics, diagnostic methods, and current treatment strategies for LMs as well as trends in management of LMs.

Analysis of sleep quality and related factors among children in Beijing / 中华耳鼻咽喉头颈外科杂志

Objective@#To investigate the sleep quality of children in Beijing and to analyze the related factors.@*Methods@#The data were collected from the survey of 3-14 years old children in 7 urban districts of Beijing in 2015. By using multi-stage stratified cluster random sampling method, 26 kindergartens and primary and secondary schools in 7 districts and counties, including Xicheng, Chaoyang, Changping, Shunyi, Fangshan, Huairou and Mentougou, were randomly selected, with a total of 11 420 children. Children′s sleep status was investigated with Children′s Sleep Questionnaire (PSQ), and the proportion of children with sleep quality problems when the PSQ score was greater than 7. Various sleep related factors were investigated with self-made questionnaire. A multilevel model was used to analyze the relationship between PSQ score and related factors.@*Results@#The average PSQ score of the children was 3.60±2.69. The proportion of children with sleep quality problems was 8.87%(816/9 198). Multilevel model analysis showed that the younger the children, the higher the PSQ score (<6 years old vs. 6~12 years old vs. >12 years old: 3.94±2.58 vs. 3.58±2.66 vs. 3.30±2.84, F=33.015, P<0.001); male PSQ score higher than female (3.89±2.75 vs. 3.30±2.60, t=10.560, P<0.001); and snoring, obesity, father/mother snoring, playing games before bed, surfing the internet, eating and other factors were statistically related to PSQ.@*Conclusions@#Sleep quality of children in Beijing should not be neglected, especially preschool children with high PSQ scores. Parents should pay attention to children′s snoring problems and try to reduce some pre-sleep behaviors that may affect sleep quality.

Exploring an proactive model for pediatric integration medical service in Beijing-Tianjin-Hebei region / 中华医院管理杂志

Beijing-Tianjin-Hebei region is the " Capital Circle Area" in China, holding a strategic position and importance. The authors analyzed the medical and health service bottlenecks problems incurred by the shortage and unequal distribution of pediatric medical resources to be solved urgently. On such basis, this article introduced the experience of Beijing Children′s Hospital in a top-level design targeting at building a collaborative integrated health service system to meet children′s health requirements. The hospital created four types of proactive medical collaborative innovation, including a managed type, collaborative development type, technical support type and medical alliance type, aiming at providing a diversified Beijing-Tianjin-Hebei Integration proactive medical service model. These efforts are designed to relieve the structural setback of supply and demand of pediatric medical services and better children′s health and families′ welfare.

The interpretation of Management Guidelines for Children with Thyroid Nodules and Differentiated Thyroid Cancer / 中华耳鼻咽喉头颈外科杂志

For a long time, the diagnosis and treatment of pediatric thyroid nodules and cancer (PTNC) are mainly referred to adults. In recent years, it has been found that there are great differences between PTNC and TNC in adults. In 2015, the American Thyroid Association released the first Management Guidelines for Children with Thyroid Nodules and Differentiated Thyroid Cancer. In the guidelines, the characteristics of PTNC were described, and clear management strategies were provided. In this paper, we mainly interpret the surgical part of the guidelines and also review the associated research progress in recent years.

The prevalence of snoring and its related family factors of children from 3 to 14 years old in Beijing / 中华耳鼻咽喉头颈外科杂志

Objective@#To investigate the snoring status and related family factors of children from 3 to 14 years old in Beijing.@*Methods@#From May to July, 2015, data of children from 3 to 14 years old were obtained from a status survey from 7 districts(Xicheng, Chaoyang, Changping, Shunyi, Fangshan, Huairou and Mentougou) in Beijing. A total of 11 420 children from 25 primary and middle schools were randomly selected. Pediatric Sleep Questionnaire (PSQ) and a self-administered questionnaire were carried out for the adopted children. Self-administered questionnaire included the snoring related family factors. Logistic regression was used to estimate the odd ratio(OR) with 95% confidence intervals for variables.@*Results@#A total of 9 198 children meet the inclusion criteria and are analyzed in the study, of whom 901 (9.80%) were found with snoring behavior. The incidence of boys is higher than girls. Obese children take higher risk of snoring. Compared with younger children (≤6 years old), older children (≥12 years old) have a significantly lower risk of snoring (OR=0.464, 95%CI 0.368-0.585). There is no statistical association between full-term infants, infant feeding pattern, parental cigarette smoking and child snoring.The children with family history of snoring have a significantly higher risk of snoring occurrence. The educational background of mother is statistically related to children snoring (OR=1.241, 95%CI 1.058-1.457).@*Conclusions@#The incidence of children snoring in Beijing is 9.80%, male gender, obesity, and young age are all risk factors for children snoring. There is a significantly statistical relationship between snoring and related family factors, such as family snoring history and education experience.

Clinical study on 48 children with head and neck rhabdomyosarcoma treated by multi-disciplinary therapy / 中华实用儿科临床杂志

Objective To explore the short-term efficacy in children with head and neck rhabdomyosarcoma (HN-RMS) treated by multidisciplinary therapy,and to analyze the prognostic factors,so as to guide the diagnosis and treatment.Methods Patients with HN-RMS admitted at Hematology Oncology Center of Beijing Children's Hospital (BCH),Capital Medical University between December 2012 and May 2017,were included in this case-observation study.The clinical characteristics were analyzed and the treatment effect and prognostic factors were summarized.Results A total of 48 cases were collected,including 36 boys and 12 girls,with a median age of 4.6 years.Primarysite parameningeal RMS(PM-RMS) (34 cases,70.8％),orbital (2 cases,4.2％) and non-orbital,non-parameningeal region(12 cases,25.0％) were found.Twenty cases belonged to alveolar type(41.7％),and 28 cases were of embryonaltype(58.3％).The diameter of the tumor was ＞5 cm(n =25,52.1％),and ≤5 cm(n =23,47.9％).IRS staging:there were 29 cases(60.4％) of stage Ⅱ-Ⅲ,19 cases (39.6％) of stage Ⅳ;29 cases (60.4％) of low-medium risk,and 19 cases (39.6％) of high risk.Twenty-three patients (47.9％) received surgery,and 25 cases (52.1％) received biopsy only.All patients (48 cases) received systemic chemotherapy.Twenty patients (41.6％) received external radiation,15 cases (31.3％) received 125I particle implantation,6 cases (12.5％) received proton therapy,but 3 cases (6.2％)did not receive radiation.The follow-up time lasted 13-57 months[(24.1 ± 12.3) months].The 2-year overall survival(OS) rate was (66.4 ± 7.2)％,and 2-year event free survival (EFS) rate was (59.9 ± 7.5) ％.Patients with tumor diameter ≤ 5 cm had higher OS and EFS than patients with tumor diameter ＞5 cm [2-year OS (87.4±6.8)％ vs.(42.9 ±6.8)％,2-year EFS (78.8 ±8.6％) vs.(38.5 ±10.8)％],and the differences were statistically significant (all P =0.006).Patients with orbital and non-orbital,non-parameningeal RMS had higher OS and EFS than PM-RMS [2-year OS 100％ vs.(87.5％ ± 11.7) ％ vs.(57.0 ± 8.8) ％;2-year EFS 100％ vs.(88.9 ± 10.5)％ vs.(51.1 ± 8.9)％],and the differences were statistically significant (P =0.008,P =0.030).Patients who received surgery had higher OS and EFS than those who did not received surgery [2-year OS (80.7±8.8)％ vs.(53.3 ± 10.4)％;2-year EFS (71.1 ±10.1)％ vs.(49.5±10.4)％],and the differences were statistically significant (P =0.008,P =0.026).COX regression analysis showed tumor diameter ＞ 5 cm was an adverse prognostic factor (OR =4.124,95％ CI:1.213-14.025,P =0.023).Conclusions PM-RMS accounted for a high proportion in RMS patients.The primary site and the size of the tumor are the main prognostic factors.Intensive therapy is expected to improve the prognosis of HN-RMS with meningeal invasion.