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*

Probiotics have shown excellent application prospects in preventing and treating many diseases. However, their sensitivity to the harsh environment in vivo always leads to a massive loss of viability and insufficient therapeutic effect. Fortunately, modified probiotics have emerged and provide multiple possibilities for their use in various diseases. Modification not only endows probiotics with extra capacity to resist severe environments but also gives them exogenous characteristics, such as prolonged retention time and improved therapeutic effects. Modified probiotics could combine with other therapies, which has opened up new avenues to enhance the efficacy of probiotic-based therapy. In this review, we have summarized the current physicochemical and biological modification strategies of probiotics. In addition, the progress of research on probiotic-based combination therapy has also been extensively reviewed, which contributes to the enhanced delivery of probiotics or other active constituents and provides new ideas for disease treatment, bioimaging, and diagnosis.

Metachromatic leukodystrophy (MLD) is an inherited disease caused by a deficiency of the enzyme arylsulfatase A (ARSA). Lentivirus-modified autologous hematopoietic stem cell gene therapy (HSCGT) has recently been approved for clinical use in pre and early symptomatic children with MLD to increase ARSA activity. Unfortunately, this advanced therapy is not available for most patients with MLD who have progressed to more advanced symptomatic stages at diagnosis. Patients with late-onset juvenile MLD typically present with a slower neurological progression of symptoms and represent a significant burden to the economy and healthcare system, whereas those with early onset infantile MLD die within a few years of symptom onset. We conducted a pilot study to determine the safety and benefit of HSCGT in patients with postsymptomatic juvenile MLD and report preliminary results. The safety profile of HSCGT was favorable in this long-term follow-up over 9 years. The most common adverse events (AEs) within 2 months of HSCGT were related to busulfan conditioning, and all AEs resolved. No HSCGT-related AEs and no evidence of distorted hematopoietic differentiation during long-term follow-up for up to 9.6 years. Importantly, to date, patients have maintained remarkably improved ARSA activity with a stable disease state, including increased Functional Independence Measure (FIM) score and decreased magnetic resonance imaging (MRI) lesion score. This long-term follow-up pilot study suggests that HSCGT is safe and provides clinical benefit to patients with postsymptomatic juvenile MLD.
Humans ; Leukodystrophy, Metachromatic/genetics* ; Pilot Projects ; Genetic Therapy/methods* ; Hematopoietic Stem Cell Transplantation ; Male ; Follow-Up Studies ; Female ; Lentivirus/genetics* ; Child ; Child, Preschool ; Hematopoietic Stem Cells/metabolism* ; Cerebroside-Sulfatase/metabolism* ; Adolescent

This research study focuses on addressing the limitations of current neuropathic pain (NP) treatments by developing a novel dual-target modulator, E0199, targeting both Na_V1.7, Na_V1.8, and Na_V1.9 and K_V7 channels, a crucial regulator in controlling NP symptoms. The objective of the study was to synthesize a compound capable of modulating these channels to alleviate NP. Through an experimental design involving both in vitro and in vivo methods, E0199 was tested for its efficacy on ion channels and its therapeutic potential in a chronic constriction injury (CCI) mouse model. The results demonstrated that E0199 significantly inhibited Na_V1.7, Na_V1.8, and Na_V1.9 channels with a particularly low half maximal inhibitory concentration (IC₅₀) for Na_V1.9 by promoting sodium channel inactivation, and also effectively increased K_V7.2/7.3, K_V7.2, and K_V7.5 channels, excluding K_V7.1 by promoting potassium channel activation. This dual action significantly reduced the excitability of dorsal root ganglion neurons and alleviated pain hypersensitivity in mice at low doses, indicating a potent analgesic effect without affecting heart and skeletal muscle ion channels critically. The safety of E0199 was supported by neurobehavioral evaluations. Conclusively, E0199 represents a ground-breaking approach in NP treatment, showcasing the potential of dual-target small-molecule compounds in providing a more effective and safe therapeutic option for NP. This study introduces a promising direction for the future development of NP therapeutics.

Objective:To explore the adverse outcomes of pregnant women with overt diabetes mellitus(ODM).Methods:A retrospective analysis was performed on 1321 pregnant women delivered in Peking Universi-ty International Hospital.Pregnant women were divided into normal blood glucose group(NGDM),gestational di-abetes mellitus group(GDM)and overt diabetes mellitus group(ODM).Maternal and neonatal adverse out-comes were compared.Results:The age,early pregnancy glycosylated hemoglobin,uric acid,triglycerides and late pregnancy glycosylated hemoglobin levels of women in ODM group were significantly higher than those in NGDM group,and the differences were statistically significant(P＜0.01).The risk of developing gestation hyper-tension(OR 6.32,P＜0.01)and cesarean section(OR 1.87,P＜0.05)in the ODM group was significantly high-er than that in the NGDM group.The rate of preterm birth(OR 2.73,P＜0.05)and macrosomia(OR 3.45,P＜0.01)in the ODM group was significantly higher than that in the NGDM group.Compared with the GDM group,the ODM group did not significantly increase the risk of hypertension,eclampsia or preeclampsia,shoulder dysto-cia,premature rupture of placenta,cesarean section,preterm birth,macrosomia,and low body mass infants(P＞0.05).Conclusion:Pregnant women with ODM increase the risk of gestational hypertension,cesarean section,preterm birth and macrosomia.Active management is needed in pregnant women with ODM.

Objective:To explore the adverse outcomes of pregnant women with overt diabetes mellitus(ODM).Methods:A retrospective analysis was performed on 1321 pregnant women delivered in Peking Universi-ty International Hospital.Pregnant women were divided into normal blood glucose group(NGDM),gestational di-abetes mellitus group(GDM)and overt diabetes mellitus group(ODM).Maternal and neonatal adverse out-comes were compared.Results:The age,early pregnancy glycosylated hemoglobin,uric acid,triglycerides and late pregnancy glycosylated hemoglobin levels of women in ODM group were significantly higher than those in NGDM group,and the differences were statistically significant(P＜0.01).The risk of developing gestation hyper-tension(OR 6.32,P＜0.01)and cesarean section(OR 1.87,P＜0.05)in the ODM group was significantly high-er than that in the NGDM group.The rate of preterm birth(OR 2.73,P＜0.05)and macrosomia(OR 3.45,P＜0.01)in the ODM group was significantly higher than that in the NGDM group.Compared with the GDM group,the ODM group did not significantly increase the risk of hypertension,eclampsia or preeclampsia,shoulder dysto-cia,premature rupture of placenta,cesarean section,preterm birth,macrosomia,and low body mass infants(P＞0.05).Conclusion:Pregnant women with ODM increase the risk of gestational hypertension,cesarean section,preterm birth and macrosomia.Active management is needed in pregnant women with ODM.

Objective:To explore the adverse outcomes of pregnant women with overt diabetes mellitus(ODM).Methods:A retrospective analysis was performed on 1321 pregnant women delivered in Peking Universi-ty International Hospital.Pregnant women were divided into normal blood glucose group(NGDM),gestational di-abetes mellitus group(GDM)and overt diabetes mellitus group(ODM).Maternal and neonatal adverse out-comes were compared.Results:The age,early pregnancy glycosylated hemoglobin,uric acid,triglycerides and late pregnancy glycosylated hemoglobin levels of women in ODM group were significantly higher than those in NGDM group,and the differences were statistically significant(P＜0.01).The risk of developing gestation hyper-tension(OR 6.32,P＜0.01)and cesarean section(OR 1.87,P＜0.05)in the ODM group was significantly high-er than that in the NGDM group.The rate of preterm birth(OR 2.73,P＜0.05)and macrosomia(OR 3.45,P＜0.01)in the ODM group was significantly higher than that in the NGDM group.Compared with the GDM group,the ODM group did not significantly increase the risk of hypertension,eclampsia or preeclampsia,shoulder dysto-cia,premature rupture of placenta,cesarean section,preterm birth,macrosomia,and low body mass infants(P＞0.05).Conclusion:Pregnant women with ODM increase the risk of gestational hypertension,cesarean section,preterm birth and macrosomia.Active management is needed in pregnant women with ODM.

Critically ill patients are at high risk for hospital acquired infections, which can significantly increase the mortality rate and treatment costs for these patients. Therefore, in the process of treating the primary disease, strict prevention and control of new hospital infections is an essential component of the treatment for critically ill patients. The treatment of critically ill patients involves multiple steps and requires a concerted effort from various aspects such as theory, management, education, standards, and supervision to achieve effective prevention and control of hospital infections. However, there is currently a lack of unified understanding and standards for hospital infection prevention and control. To address this, in March 2024, a group of experts in critical care medicine, infectious diseases, and hospital infection from China discussed the current situation and issues of hospital infection control in the intensive care unit together. Based on a review of the latest evidence-based medical evidence from both domestic and international sources, 2024 Expert Consensus on Hospital Acquired Infection Control Principles in the Department of Critical Care Medicine was formed, aiming to provide a basis for the development of hospital infection prevention and control strategies in the field of critical care medicine.

Objective:To explore the adverse outcomes of pregnant women with overt diabetes mellitus(ODM).Methods:A retrospective analysis was performed on 1321 pregnant women delivered in Peking Universi-ty International Hospital.Pregnant women were divided into normal blood glucose group(NGDM),gestational di-abetes mellitus group(GDM)and overt diabetes mellitus group(ODM).Maternal and neonatal adverse out-comes were compared.Results:The age,early pregnancy glycosylated hemoglobin,uric acid,triglycerides and late pregnancy glycosylated hemoglobin levels of women in ODM group were significantly higher than those in NGDM group,and the differences were statistically significant(P＜0.01).The risk of developing gestation hyper-tension(OR 6.32,P＜0.01)and cesarean section(OR 1.87,P＜0.05)in the ODM group was significantly high-er than that in the NGDM group.The rate of preterm birth(OR 2.73,P＜0.05)and macrosomia(OR 3.45,P＜0.01)in the ODM group was significantly higher than that in the NGDM group.Compared with the GDM group,the ODM group did not significantly increase the risk of hypertension,eclampsia or preeclampsia,shoulder dysto-cia,premature rupture of placenta,cesarean section,preterm birth,macrosomia,and low body mass infants(P＞0.05).Conclusion:Pregnant women with ODM increase the risk of gestational hypertension,cesarean section,preterm birth and macrosomia.Active management is needed in pregnant women with ODM.

Malignant tumors in children are one of the most important diseases that threaten the health and quality of life of children and are the second most common cause of death in children.With the continuous improvement and progress of treatment technology, the long-term survival rate of children with tumor has been significantly improved, but both the disease itself and the treatment can impair the immune function of children, which makes them vulnerable to various infectious diseases and secondary serious complications, and even become a source of infection, endangering the health of others. Vaccination is the most cost-effective measure to prevent infectious diseases. For children with normal immune functions, the benefits of vaccination usually outweigh the disadvantages. However, there is a lack of detailed data on the vaccination situation, efficacy and safety of vaccine use for such immunocompromised tumor survivors, and there are no authoritative and uniform vaccination recommendations. This article reviewed and summarized the literature and consensus of some domestic and foreign scholars on current status of post-treatment vaccination status, efficacy and safety of vaccination for children with tumors after treatment, with the aim of providing a reference for the practice in this field in China.