Objective The purpose of writing the"expert consensus on humanistic care for patients in hospice care"(hereinafter referred to as the"consensus")aims to standardize the practice of humanistic care in the field of hospice care,ensuring that humanistic care is integrated throughout the entire service process for hospice care patients and their families.Methods A systematic search was conducted in domestic and foreign databases for literature related to hospice care and humanistic care,including guidelines,expert consensuses,systematic reviews or Meta-analyses,and evidence summaries.High-quality evidence was evaluated,extracted,and summarized to form the initial draft of the"consensus".From June to October 2024,20 experts from the fields of hospice care,nursing humanities,and evidence-based nursing were invited to participate in 1 round of expert consultation.Among them,13 experts were selected for 2 rounds of expert demonstration meetings.After collating and analyzing the experts' opinions,the initial draft was revised and refined,ultimately resulting in the final version of the"consensus".Results The effective response rate of the consultation questionnaire was 100％,with expert authority coefficient of 0.880,judgment coefficient of 0.935,and familiarity level of 0.825.The Kendall harmony coefficient of the expert consultation was 0.134(P＜0.05).The"consensus"consisted of 13 aspects,including the targets and objectives,principles,institutional guarantees,environmental requirements,etc.Conclusion This"consensus"possesses strong scientific rigor and practicality,which can provide guidance and references for the practice of humanistic care in the field of hospice care,promoting the standardization and humanization of hospice care services.

Objective:To explore the perioperative precautions, rehabilitation effect, and affecting factors in cochlear implantation (CI) among patients with hereditary syndromic hearing loss.Methods:This was a retrospective cohort study. 47 patients diagnosed as hereditary syndromic deafness were treated in the Department of Otolaryngology-Head and Neck Surgery of the Chinese PLA General Hospital from 2010 to 2021, including 26 males and 21 females, aged 0.9-25 years. All patients received unilateral or bilateral CI. Clinical manifestation combined with genetic testing was used to diagnose syndromic hearing loss. The risks and precautions of CI in these patients were summarized from preoperative imaging, intraoperative observations, and postoperative complications. Single factor linear regression and multiple linear regression models in SPSS 26.0 software were used to evaluate the effects of various factors on auditory and speech rehabilitation after CI for syndromic hearing loss. The postoperative outcomes were analyzed through aided hearing thresholds, categories of auditory performance (CAP) scale, and speech intelligibility rate (SIR) scale.Results:Thirteen kinds of syndromes, totally 47 cases, including CHARGE (20 cases), Waardenburg (9 cases), Autosomal dominant deafness-onychodystrophy (DDOD, 4 cases), Pendred (3 cases), Noonan Syndrome with Multiple Lentigines (NSML, 2 cases), Branchio-Oto-Renal (BOR, 2 cases), Bart-Pumphery (1 case), Perrault (1 case), Kabuki (1 case), Frontometaphyseal dysplasia type 2 (FMD 2, 1 case), Mandibulofacial dysostosis Guion-Almeida type (MFDGA, 1 case), Coffin-Siris (1 case), and 10q26.12-q26.3 del (1 case), were enrolled. The perioperative special management included the following measures. For patients with cardiac and/or cartilage development issues, preoperative assessments of cardiac function and/or laryngeal cartilage development were performed to minimize anesthetic risks. For patients with mild intellectual disability and/or an auditory neuropathy phenotype, preoperative communication with the patients′ families was conducted to explain the limitations of CI and assist in setting reasonable expectations. For syndromic hearing loss patients who commonly present with inner ear malformations, facial nerve anomalies, and/or intraoperative cerebrospinal fluid leakage, appropriate electrodes were selected prior to surgery, intraoperative facial nerve monitoring and careful cerebrospinal fluid leak repair were conducted, respectively. For patients with NSML accompanied by coagulation issues, the postoperative compression bandaging duration was extended to reduce the risk of hematoma formation. The daily duration of cochlear implant use, the presence of cochlear malformation, and developmental delay were independent factors influencing postoperative CAP scores. The daily duration of cochlear implant use, developmental delay, and unilateral or bilateral CI were independent factors influencing postoperative SIR scores.Conclusions:Hereditary syndrome deafness is a rare disease that affects multiple organs and causes extensive functional impairment. Before CI, a comprehensive evaluation of major affected organ functions is required to assess anesthetic and surgical risks. Genetic diagnosis not only identifies the molecular etiology of patients with syndromic hearing loss and reveals rare phenotypes, but also aids in prognostic evaluation. The main factors affecting CI outcomes in patients with syndromic hearing loss include the presence of cochlear malformations, developmental delays, daily duration of cochlear implant use, and bilateral implantation status.

The extracellular matrix（ECM）is a complex network structure composed of collagen，glycoproteins，and proteoglycans.It not only provides structural support and viscoelasticity to tissues but also participates in cell signaling，responding to environmental forces and signals to mediate tissue remodeling in response to environmental cues. Due to the intricate and precise functions of the inner ear，the perception and transmission of sound rely on the complex interactions between cochlear cell structures and the ECM. In the inner ear，the ECM not only constitutes key structures such as the basilar membranes（BM）and tectorial membranes（TM），which are essential for sound perception，but also regulates cell shape，adhesion，and migration.Certain ECM components interact with cell surface receptors to activate signaling pathways that regulate gene expression.Additionally，the ECM modulates the storage and diffusion of ions and secreted factors, creating concentration gradients.These functions are critical for inner ear development，repair，and function.Thus，the ECM plays a vital role in auditory processes，and abnormalities in ECM are a cause of certain hereditary hearing loss.This review primarily summarizes the ECM genes that lead to hearing loss.
Humans ; Extracellular Matrix/genetics* ; Hearing Loss/genetics* ; Mutation ; Cochlea ; Extracellular Matrix Proteins/genetics*

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*

Objective:To evaluate the perioperative safety and long-term complications of simultaneous bilateral cochlear implantation（BCI） in young infants, providing reference data for clinical BCI in young children. Methods:Seventy-four infants aged 6-23 months with congenital severe to profound sensorineural hearing loss who were candidates for cochlear implantation at the Department of Otolaryngology, Chinese PLA General Hospital between August 2018 and August 2019 were consecutively enrolled. Parents made the decision to implant either unilaterally or bilaterally. Participants were divided into unilateral cochlear implantation（UCI） group（before and after 12 months of age） and simultaneous BCI group（before and after 12 months of age）. Safety indicators, including perioperative risk variables, complications, and other postoperative adverse events were monitored, with complications followed up for 5-6 years. Comparisons were made between the BCI and UCI, as well as between implantation before and after 12 months of age regarding perioperative safety and long-term complications. Results:A total of 40 BCI patients（23 before 12 months, 17 after 12 months） and 34 UCI patients（20 before 12 months, 14 after 12 months） were included in the study. Regarding perioperative risk variables, the BCI group showed significantly longer anesthesia duration, operative time, and greater blood loss compared to the UCI group, though less than twice that of the UCI group; no anesthetic complications occurred in either group; and there was no significant difference in postoperative hospital stay between the groups. Regarding surgical complications during the 5-year follow-up period, the BCI group experienced 7 complications（2 major, 5 minor）, while the UCI group had 7 complications（1 major, 6 minor）, with no statistical differences between groups. Regarding other postoperative adverse events, the BCI group demonstrated significantly higher total adverse event rates than the UCI group（80.0% vs 38.2%）, with higher rates of moderate to severe anemia（60.0% vs 20.6%） and lower mean hemoglobin levels[（92.35±12.14） g/L vs（102.39±13.09） g/L]. No significant differences were found in postoperative fever rates（50.0% vs 52.9%） or C-reactive protein levels between groups. Within the BCI group, patients implanted before 12 months indicated notably higher rates of total adverse events（91.3% vs 64.7%）, high fever（26.1% vs 0）, and moderate to severe anemia（78.3% vs 35.3%） compared to those implanted after 12 months. Conclusion:Simultaneous BCI in young children under 2 years of age demonstrates controllable overall risks. Compared to UCI, while it shows no increase in anesthetic or surgical complications, it presents higher perioperative risks and adverse event rates, especially in patients implanted before 12 months of age, warranting special attention from medical staff.
Humans ; Cochlear Implantation/methods* ; Infant ; Postoperative Complications ; Hearing Loss, Sensorineural/surgery* ; Follow-Up Studies ; Male ; Perioperative Period ; Female ; Cochlear Implants

Objective The purpose of writing the"expert consensus on humanistic care for patients in hospice care"(hereinafter referred to as the"consensus")aims to standardize the practice of humanistic care in the field of hospice care,ensuring that humanistic care is integrated throughout the entire service process for hospice care patients and their families.Methods A systematic search was conducted in domestic and foreign databases for literature related to hospice care and humanistic care,including guidelines,expert consensuses,systematic reviews or Meta-analyses,and evidence summaries.High-quality evidence was evaluated,extracted,and summarized to form the initial draft of the"consensus".From June to October 2024,20 experts from the fields of hospice care,nursing humanities,and evidence-based nursing were invited to participate in 1 round of expert consultation.Among them,13 experts were selected for 2 rounds of expert demonstration meetings.After collating and analyzing the experts' opinions,the initial draft was revised and refined,ultimately resulting in the final version of the"consensus".Results The effective response rate of the consultation questionnaire was 100％,with expert authority coefficient of 0.880,judgment coefficient of 0.935,and familiarity level of 0.825.The Kendall harmony coefficient of the expert consultation was 0.134(P＜0.05).The"consensus"consisted of 13 aspects,including the targets and objectives,principles,institutional guarantees,environmental requirements,etc.Conclusion This"consensus"possesses strong scientific rigor and practicality,which can provide guidance and references for the practice of humanistic care in the field of hospice care,promoting the standardization and humanization of hospice care services.

Objective:To explore the perioperative precautions, rehabilitation effect, and affecting factors in cochlear implantation (CI) among patients with hereditary syndromic hearing loss.Methods:This was a retrospective cohort study. 47 patients diagnosed as hereditary syndromic deafness were treated in the Department of Otolaryngology-Head and Neck Surgery of the Chinese PLA General Hospital from 2010 to 2021, including 26 males and 21 females, aged 0.9-25 years. All patients received unilateral or bilateral CI. Clinical manifestation combined with genetic testing was used to diagnose syndromic hearing loss. The risks and precautions of CI in these patients were summarized from preoperative imaging, intraoperative observations, and postoperative complications. Single factor linear regression and multiple linear regression models in SPSS 26.0 software were used to evaluate the effects of various factors on auditory and speech rehabilitation after CI for syndromic hearing loss. The postoperative outcomes were analyzed through aided hearing thresholds, categories of auditory performance (CAP) scale, and speech intelligibility rate (SIR) scale.Results:Thirteen kinds of syndromes, totally 47 cases, including CHARGE (20 cases), Waardenburg (9 cases), Autosomal dominant deafness-onychodystrophy (DDOD, 4 cases), Pendred (3 cases), Noonan Syndrome with Multiple Lentigines (NSML, 2 cases), Branchio-Oto-Renal (BOR, 2 cases), Bart-Pumphery (1 case), Perrault (1 case), Kabuki (1 case), Frontometaphyseal dysplasia type 2 (FMD 2, 1 case), Mandibulofacial dysostosis Guion-Almeida type (MFDGA, 1 case), Coffin-Siris (1 case), and 10q26.12-q26.3 del (1 case), were enrolled. The perioperative special management included the following measures. For patients with cardiac and/or cartilage development issues, preoperative assessments of cardiac function and/or laryngeal cartilage development were performed to minimize anesthetic risks. For patients with mild intellectual disability and/or an auditory neuropathy phenotype, preoperative communication with the patients′ families was conducted to explain the limitations of CI and assist in setting reasonable expectations. For syndromic hearing loss patients who commonly present with inner ear malformations, facial nerve anomalies, and/or intraoperative cerebrospinal fluid leakage, appropriate electrodes were selected prior to surgery, intraoperative facial nerve monitoring and careful cerebrospinal fluid leak repair were conducted, respectively. For patients with NSML accompanied by coagulation issues, the postoperative compression bandaging duration was extended to reduce the risk of hematoma formation. The daily duration of cochlear implant use, the presence of cochlear malformation, and developmental delay were independent factors influencing postoperative CAP scores. The daily duration of cochlear implant use, developmental delay, and unilateral or bilateral CI were independent factors influencing postoperative SIR scores.Conclusions:Hereditary syndrome deafness is a rare disease that affects multiple organs and causes extensive functional impairment. Before CI, a comprehensive evaluation of major affected organ functions is required to assess anesthetic and surgical risks. Genetic diagnosis not only identifies the molecular etiology of patients with syndromic hearing loss and reveals rare phenotypes, but also aids in prognostic evaluation. The main factors affecting CI outcomes in patients with syndromic hearing loss include the presence of cochlear malformations, developmental delays, daily duration of cochlear implant use, and bilateral implantation status.

Patients with severe trauma require an extremely timely treatment and transfusion plays an irreplaceable role in the emergency treatment of such patients. An increasing number of evidence-based medicinal evidences and clinical practices suggest that patients with severe traumatic bleeding benefit from early transfusion of low-titer group O whole blood or hemostatic resuscitation with red blood cells, plasma and platelet of a balanced ratio. However, the current domestic mode of blood supply cannot fully meet the requirements of timely and effective blood transfusion for emergency treatment of patients with severe trauma in clinical practice. In order to solve the key problems in blood supply and blood transfusion strategies for emergency treatment of severe trauma, Branch of Clinical Transfusion Medicine of Chinese Medical Association, Group for Trauma Emergency Care and Multiple Injuries of Trauma Branch of Chinese Medical Association, Young Scholar Group of Disaster Medicine Branch of Chinese Medical Association organized domestic experts of blood transfusion medicine and trauma treatment to jointly formulate Chinese expert consensus on blood support mode and blood transfusion strategies for emergency treatment of severe trauma patients ( version 2024). Based on the evidence-based medical evidence and Delphi method of expert consultation and voting, 10 recommendations were put forward from two aspects of blood support mode and transfusion strategies, aiming to provide a reference for transfusion resuscitation in the emergency treatment of severe trauma and further improve the success rate of treatment of patients with severe trauma.