Hearing loss is the most prevalent disabling disease. Cochlear implantation（CI） serves as the primary intervention for severe to profound hearing loss. This consensus systematically explores the value of genetic diagnosis in the pre-operative assessment and efficacy prognosis for CI. Drawing upon domestic and international research and clinical experience, it proposes an evidence-based medicine three-tiered prognostic classification system（Favorable, Marginal, Poor）. The consensus focuses on common hereditary non-syndromic hearing loss（such as that caused by mutations in genes like GJB2, SLC26A4, OTOF, LOXHD1） and syndromic hereditary hearing loss（such as Jervell & Lange-Nielsen syndrome and Waardenburg syndrome）, which are closely associated with congenital hearing loss, analyzing the impact of their pathological mechanisms on CI outcomes. The consensus provides recommendations based on multiple round of expert discussion and voting. It emphasizes that genetic diagnosis can optimize patient selection, predict prognosis, guide post-operative rehabilitation, offer stratified management strategies for patients with different genotypes, and advance the application of precision medicine in the field of CI.
Humans ; Cochlear Implantation ; Prognosis ; Hearing Loss/surgery* ; Consensus ; Connexin 26 ; Mutation ; Sulfate Transporters ; Connexins/genetics*

Arsenic is a semi-metal,and lipid-soluble arsenic compounds are one of the widespread forms in the environment and food chain,but there is a lack of standards for lipid-soluble arsenic compounds,which is one of the bottlenecks in the current analytical detection and toxicological studies of organic arsenic.In this study,four saturated arsenic-containing hydrocarbons,AsHC 318,AsHC 332,AsHC 346,and AsHC 374(The number is relative molecular mass),were successfully synthesized in three steps by using dimethylarsinic acid,potassium iodide,sodium hydroxide,and four brominated alkanes(1-Bromotetradecane,1-bromopentadecane,1-bromohexadecane,and 1-bromooctadecane)as raw materials.The structures of these four saturated arsenic-containing hydrocarbons were characterized by proton nuclear magnetic resonance(1H NMR)spectroscopy,13C nuclear magnetic resonance(13C NMR)spectroscopy,and high-resolution mass spectrometry(HR-MS).The yields of the method were 8%-10%,and the synthesized compounds could be used in subsequent toxicity evaluation experiments to assess the toxic effects and mechanisms of action of arsenic-containing hydrocarbons.This study provided an effective method for synthesis of arsenic-containing hydrocarbons,enriching the synthesis methods of arsenic-containing hydrocarbons,and provided raw materials for the subsequent toxicological studies of arsenic-containing hydrocarbons.

Abdominal aortic balloon occlusion is an emerging interventional treatment method,which has been used to control the risk of postpartum hemorrhage caused by pernicious placenta previa(PPP).Numerous studies have shown that abdominal aortic balloon occlusion can not only significantly reduce the difficulty and risk of surgery,shorten the time spent for surgery,but also further increase the success rate of surgery,which undoubtedly has a positive impact on both the delivery woman and neonate.This paper aims to make a brief introduction of the development history of abdominal aortic balloon occlusion and its technical principles,and to expound the timing of using abdominal aortic balloon occlusion and the clinical application of the balloon with different properties,focusing on the advantages of using abdominal aortic balloon in patients with PPP.It is expected that this paper can provide a reference for formulating clinical treatment strategies.

Nipah virus (NiV) and related viruses form a distinct henipavirus genus within the Paramyxoviridae family. NiV continues to spillover into the humans causing deadly outbreaks with increasing human-bat interaction. NiV encodes the large protein (L) and phosphoprotein (P) to form the viral RNA polymerase machinery. Their sequences show limited homologies to those of non-henipavirus paramyxoviruses. We report two cryo-electron microscopy (cryo-EM) structures of the Nipah virus (NiV) polymerase L-P complex, expressed and purified in either its full-length or truncated form. The structures resolve the RNA-dependent RNA polymerase (RdRp) and polyribonucleotidyl transferase (PRNTase) domains of the L protein, as well as a tetrameric P protein bundle bound to the L-RdRp domain. L-protein C-terminal regions are unresolved, indicating flexibility. Two PRNTase domain zinc-binding sites, conserved in most Mononegavirales, are confirmed essential for NiV polymerase activity. The structures further reveal anchoring of the P protein bundle and P protein X domain (XD) linkers on L, via an interaction pattern distinct among Paramyxoviridae. These interactions facilitate binding of a P protein XD linker in the nucleotide entry channel and distinct positioning of other XD linkers. We show that the disruption of the L-P interactions reduces NiV polymerase activity. The reported structures should facilitate rational antiviral-drug discovery and provide a guide for the functional study of NiV polymerase.
Nipah Virus/chemistry* ; Cryoelectron Microscopy ; Viral Proteins/genetics* ; RNA-Dependent RNA Polymerase/genetics* ; Phosphoproteins/genetics* ; Humans ; Models, Molecular ; Protein Binding

Objective:To evaluate the safety and efficacy of accelerator-based boron neutron capture therapy (AB-BNCT) in the treatment of recurrent and refractory malignant tumors.Methods:The data of 14 patients admitted to Xiamen Humanity Hospital from September 2022 to April 2023 were prospectively collected, including 7 patients with primary brain malignancies and 7 patients with locally recurrent inoperable head and neck malignancies. All patients received intravenous infusion of boron drug (NBB-001, p-dihydroxyborylphe nylalanine, a patented freeze-dried formulation) at a total nominal dosage of 500 mg/kg (11 patients) or 750 mg/kg (3 patients), and were irradiated with neutrons (operating with NeuPex system). Adverse events after treatment were recorded and assessed. The primary efficacy endpoint was the 90 d objective response rate (ORR), while the secondary endpoints included progression-free survival (PFS) and complete response rate (CRR). Data were compiled and analyzed by SAS 9.4 software. The rate and 95% CI were calculated using Clopper-Pearson method. Results:The median dose delivered to 80% of the target volume (D 80%) was 16.80 GyE (range: 8.93-23.79 GyE). The most common adverse reactions were hyperamylasemia, alopecia, and hyperprolactinemia. Five patients experienced 8 cases of grade 3 or above adverse events, including 1 case of grade 4 acute kidney injury and 7 cases of grade 3 adverse events. All adverse events were recovered after observation or treatment. At 90 d after treatment, the ORR of all patients was 9/14 (64%, 95% CI: 35%-87%), disease control rate (DCR) was 10/14 (71%, 95% CI: 42%-92%), CRR was 2/14 (14%, 95% CI: 2%-42%); and the best overall response during the entire course included an ORR of 10/14 (71% ,95% CI: 42%-92%), DCR of 13/14 (93%, 95% CI: 66%-100%), and CRR of 3/14 (21% ,95% CI: 5%-51%). The 1-year survival rate for head and neck malignancies was 71.4%, and the 2-year survival rate was 42.8%. The 1-year survival rate for recurrent brain malignancies was 42.8%. Conclusion:AB-BNCT demonstrates favorable safety and promising efficacy in treating primary brain malignancies and recurrent/refractory head and neck malignancies, representing a potential therapeutic option.

Objective To investigate the longitudinal relationship between sleep duration(SD)and positive youth development(PYD)among primary and secondary school students in Chengdu city using a cross-lagged model,and to provide scientific evidence for enhancing sleep management practices for students.Methods A total of 4061 students of grades 3 through 9 from the Chengdu Child Positive Development Cohort were included in this three-wave longitudinal study.There was a one-year interval between one survey and the following round of survey,and the time points for the baseline,12-month follow-up,and 24-month follow-up surveys were designated T0,T1,and T2.The PYD of the participants was assessed using the Chinese version of the Positive Youth Development Scale.The demographic data and the average daily SD over the past month were collected.Spearman correlation analysis was performed to examine the associations between SD and PYD,and a cross-lagged model was used to investigate the longitudinal relationship between them.Results The average daily SD for the 3 rounds of surveys conducted at T0,T1,and T2 was 9.00(8.04,10.00)hours,10.44(9.67,11.11)hours,and 10.39(9.83,11.00)hours,respectively,while the PYD scores were 5.30(4.73,5.71),5.27(4.73,5.73),and 5.39(4.89,5.77),respectively.Statistical significance was found in the differences of SD and PYD scores across the 3 rounds(P＜0.05).Spearman correlation analysis revealed synchronous correlations between SD and PYD at all three time points(r=0.10 at T0,r=0.18 at T1,and r=0.21 at T2,P＜0.05)and significant lagged correlations(e.g.,r=0.10 for T1-PYD and T2-SD,and likewise,significant correlation was found for the 3 other cross-lagged paths).The cross-lagged model demonstrated that PYD at T0 and T1 positively predicted SD at T1 and T2,respectively(β0-1=0.116[95％CI,0.083-0.150],β1-2=0.097(95％CI,0.067-0.127),P＜0.05),and that SD at T0 and T1 also positively predicted PYD at T1 and T2(β0-1=0.028[95％CI,0-0.056],β1-2=0.042[95％CI,0.010-0.074],P＜0.05).According to these findings,a bidirectional predictive relationship between SD and PYD across different time points was observed in primary and secondary school students.Furthermore,PYD demonstrated better performance for predicting SD than SD did for PYD.Subgroup analysis by sex confirmed the robustness of the predictive power of PYD for SD.Conclusion This study reveals a positive bidirectional predictive relationship between SD and PYD among primary and secondary school students,suggesting that higher levels of PYD may contribute to adequate sleep.These findings provide critical scientific evidence for schools and families to strengthen sleep management and promote the holistic development and well-being of adolescents.

Objective To explore the application value of musculoskeletal ultrasound(MSUS)in grading acute gastrocnemius muscle injuries.Methods A retrospective analysis was conducted on ultrasound images of 291 patients who presented with sudden calf pain and suspected acute gastrocnemius muscle injury in the General Hospital of Central Theater Command from March 2019 to July 2024.The images were independently reviewed and assessed by three ultrasound doctors with different qualifications to determine the presence and grade of gastrocnemius muscle injury.The consistency of grading results among three doctors was compared.The diagnostic results of three doctors were summarized.Then,the diagnostic results of 29 patients who underwent routine MRI scans were compared with those of MSUS,and the agreement between the two imaging modalities was assessed using the Kappa test.Results Among the 291 patients,171 cases(58.8%)were diagnosed with gastrocnemius muscle injury,including 55 cases(32.2%)with grade Ⅰ,109 cases(63.7%)with grade Ⅱ,and 7 cases(4.1%)with grade Ⅲ.There were 159 cases(93.0%)of unilateral medial head injury,10 cases(5.8%)of unilateral lateral head injury,and 2 cases(1.2%)of bilateral medial and lateral head injury.Compared with patients without gastrocnemius muscle injury,patients with gastrocnemius muscle injury were older(P<0.05),with no significant difference in gender and laterality(P>0.05).No significant differences in baseline characteristics were found among patients with different grades of injury(P>0.05).The three doctors diagnosed 173(59.5%),171(58.8%),and 171(58.8%)cases of injury,respectively,with an inter-class correlation coefficient(ICC)of 0.947(P<0.001).Among 29 patients who underwent MRI,the diagnostic agreement between MRI and ultrasound for grade Ⅰ,Ⅱ and Ⅲ injury was 8(27.6%),18(62.1%)and 3(10.3%)for MRI;and 9(31.0%),17(58.6%)and 3(10.3%)for MSUS,respectively,with a Kappa value of 0.808(P<0.001).Conclusions MSUS is effective for assessing the grade of acute gastrocnemius muscle injury,and shows high diagnostic consistency among doctors with different qualifications.It is recommended as the preferred method for diagnosing gastrocnemius muscle injury.

Objective To construct a symptom network in patients with acute type A aortic dissection(ATAAD),so as to provide theoretical basis for the screening of dissection ATAAD during emergency pre-screening triage.Methods There were 433 patients diagnosed with ATAAD during 2019 to 2023 in an emergency department of a tertiary hospital in Wuhan.Their basic information and medical records were reviewed by self-designed data questionnaire.UCINET6.0 software was used to construct a symptom network,analyze the centrality index and determine the core symptoms.Symptom distribution of patients with positive and negative blood pressure in extremities was analyzed in the further.Results The most common symptoms in patients with type A aortic dissection were chest pain(77.37％),back pain(42.96％),and sweating(29.79％).In the symptom network,chest pain had the highest degree(rs=659).The closeness of chest pain,chest tightness,shortness of breath,back pain,nausea and vomiting,limb numbness and fatigue were same(rc=93.33).Fatigue has the highest betweenness(rb=13.69).Patients with positive limbs blood pressure mainly reported chest pain(70.17％),back pain(44.96％),and nausea and vomiting(19.33％),while those with negative limb blood pressure mainly reported chest pain(63.64％),back pain(63.64％),and orosphyalgia(39.40％).Orosphyalgia had the highest degree(rs=20).Conclusion The symptoms of ATAAD are complex and varied in patients.During triage,nurses should measure the limb blood pressure when patients complained chest pain alone or when combined with other hypoperfusion symptoms,such as back pain,chest tightness,sweating,near-death sensation,and shortness of breath.Aortic dissection cannot be ruled out in patients with negative blood pressure when they had chest pain,back pain or orosphyalgia.

Objective To construct a symptom network in patients with acute type A aortic dissection(ATAAD),so as to provide theoretical basis for the screening of dissection ATAAD during emergency pre-screening triage.Methods There were 433 patients diagnosed with ATAAD during 2019 to 2023 in an emergency department of a tertiary hospital in Wuhan.Their basic information and medical records were reviewed by self-designed data questionnaire.UCINET6.0 software was used to construct a symptom network,analyze the centrality index and determine the core symptoms.Symptom distribution of patients with positive and negative blood pressure in extremities was analyzed in the further.Results The most common symptoms in patients with type A aortic dissection were chest pain(77.37％),back pain(42.96％),and sweating(29.79％).In the symptom network,chest pain had the highest degree(rs=659).The closeness of chest pain,chest tightness,shortness of breath,back pain,nausea and vomiting,limb numbness and fatigue were same(rc=93.33).Fatigue has the highest betweenness(rb=13.69).Patients with positive limbs blood pressure mainly reported chest pain(70.17％),back pain(44.96％),and nausea and vomiting(19.33％),while those with negative limb blood pressure mainly reported chest pain(63.64％),back pain(63.64％),and orosphyalgia(39.40％).Orosphyalgia had the highest degree(rs=20).Conclusion The symptoms of ATAAD are complex and varied in patients.During triage,nurses should measure the limb blood pressure when patients complained chest pain alone or when combined with other hypoperfusion symptoms,such as back pain,chest tightness,sweating,near-death sensation,and shortness of breath.Aortic dissection cannot be ruled out in patients with negative blood pressure when they had chest pain,back pain or orosphyalgia.

Objective:To evaluate the safety and efficacy of accelerator-based boron neutron capture therapy (AB-BNCT) in the treatment of recurrent and refractory malignant tumors.Methods:The data of 14 patients admitted to Xiamen Humanity Hospital from September 2022 to April 2023 were prospectively collected, including 7 patients with primary brain malignancies and 7 patients with locally recurrent inoperable head and neck malignancies. All patients received intravenous infusion of boron drug (NBB-001, p-dihydroxyborylphe nylalanine, a patented freeze-dried formulation) at a total nominal dosage of 500 mg/kg (11 patients) or 750 mg/kg (3 patients), and were irradiated with neutrons (operating with NeuPex system). Adverse events after treatment were recorded and assessed. The primary efficacy endpoint was the 90 d objective response rate (ORR), while the secondary endpoints included progression-free survival (PFS) and complete response rate (CRR). Data were compiled and analyzed by SAS 9.4 software. The rate and 95% CI were calculated using Clopper-Pearson method. Results:The median dose delivered to 80% of the target volume (D 80%) was 16.80 GyE (range: 8.93-23.79 GyE). The most common adverse reactions were hyperamylasemia, alopecia, and hyperprolactinemia. Five patients experienced 8 cases of grade 3 or above adverse events, including 1 case of grade 4 acute kidney injury and 7 cases of grade 3 adverse events. All adverse events were recovered after observation or treatment. At 90 d after treatment, the ORR of all patients was 9/14 (64%, 95% CI: 35%-87%), disease control rate (DCR) was 10/14 (71%, 95% CI: 42%-92%), CRR was 2/14 (14%, 95% CI: 2%-42%); and the best overall response during the entire course included an ORR of 10/14 (71% ,95% CI: 42%-92%), DCR of 13/14 (93%, 95% CI: 66%-100%), and CRR of 3/14 (21% ,95% CI: 5%-51%). The 1-year survival rate for head and neck malignancies was 71.4%, and the 2-year survival rate was 42.8%. The 1-year survival rate for recurrent brain malignancies was 42.8%. Conclusion:AB-BNCT demonstrates favorable safety and promising efficacy in treating primary brain malignancies and recurrent/refractory head and neck malignancies, representing a potential therapeutic option.