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*

Irreducible anteromedial radial head dislocation (IARHD) caused by transposed biceps tendon is rare. Delayed diagnosis and surgical failure often occur. A 46-year-old fisherman presented with 10 days history of painful swelling and restricted movement of his right elbow due to strangulation injury by a fishing boat cable. On examination, the images of the right elbow reveals in a "semi-extended and pronated" elastic fixation position. Radiography and 3-dimensional reconstruction CT reveals an isolated anteromedial radial head dislocation with extreme protonation of the radius and the bicipital tuberosity towards the posterior aspect of the elbow joint, and MRI shows biceps tendon wrapping around the radial neck, similar to umbilical cord wrapping seen in newborns. The Henry approach was applied for the first time to reduce the biceps tendon. The patient achieved a good functional recovery at 26 months, which represents the first reported case of IARHD without fracture caused by biceps tendon in an adult. In treatment of IARHD, attention should be paid to the phenomenon of biceps tendon transposition. Careful clinical examination, comprehensive imaging modalities, and appropriate surgical approach are the keys to successful management.

Objective:To summarize the clinical features of reversible posterior encephalopathy syndrome(PRES)after allogeneic hematopoietic stem cell transplantation(allo-HSCT)in children.Methods:The clinical data of six children who developed PRES after undergoing allo-HSCT in the Department of Hematology of Wuhan Children's Hospital from June 2016 to December 2022 were retrospectively analyzed,and their clinical characteristics,imaging examination,laboratory examination,and treatment regression were summarized.Results:Among 281 children underwent allo-HSCT,6 cases(2.14％)developed PRES,with a median age of 5.1(1.5-9.7)years old.4 cases underwent related haploidentical donor transplantation,and 2 cases underwent sibling allografting and unrelated donor allografting donor transplantation,respectively.All six children had an acute onset of illness,with clinical manifestations of nausea and vomiting,seizures,psychiatric disorders,visual disturbances.The five cases elevated blood pressure.All children with PRES were treated with oral immunosuppressive drugs during seizures,and 3 cases were combined with different degrees of graft-versus-host disease.Most of the children showed effective improvement in clinical symptoms and imaging after adjusting/discontinuing suspected medications(cyclosporine,etc.)and symptomatic supportive treatments(oral antihypertensive,diazepam for antispasmodic,mannitol to lower cranial blood pressure),and one of them relapsed more than 8 months after the first seizure.Conclusion:PRES is rare after hematopoietic stem cell transplantation in children,and its onset may be related to hypertension,cytotoxic drugs,graft-versus-host disease,etc.Most of them can be recovered after active treatment,but not completely reversible,and the prognosis of those who combined with TMA is poor.

This study adopted the policy text analysis method,review the historical background of the enactment,aimed to comparatively analyze the international pharmaceutical trade policies of the BRICS countries.The main objectives of the BRICS countries'international pharmaceutical trade policies included ensuring stable and accessible drug supply,expanding exports of domestic products and creating a favorable political environment.For these purposes,Brazil,Russia,and South Africa all ensure drug supply through substantial imports.However,they have also taken measures such as compulsory patent licensing and promoting localization of production by foreign companies to reduce import dependence.India,on the other hand,protects its domestic industry by resisting drug imports to ensure drug supply while simultaneously promoting the export of pharmaceutical products.China continually optimizes approval and data monitoring procedures to align with international standards,creating a favorable trade environment and expanding exports.China should further refine its international pharmaceutical trade policies while ensuring the autonomy of domestic drug research and supply,fostering stronger collaboration within BRICS nations and promoting global access to public healthcare products.

OBJECTIVE: To investigate the efficacy and safety of VDZ (Vedolizumab) in the salvage treatment of glucocorticoid resistance to gastrointestinal graft-versus-host disease (GR-GI GVHD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children. METHODS: The clinical data of 5 patients with refractory GI GVHD who received allo-HSCT in Wuhan Children's Hospital from December 2020 to December 2021 were retrospectively analyzed with VDZ salvage therapy. RESULTS: Among the 5 children with refractory GI GVHD, there were 1 male and 4 female, including 2 cases of extremely severe aplastic anemia, 1 case of acute myeloid leukemia (M2, high-risk), 1 case of fanconi anemia and 1 case of myelodysplastic syndrome. The median age of transplant recipients was 54.4 (12-164) months. The median treatment time from transplantation to VDZ was 1.4 (0.6-6.8) months. On average, 3.5 (2-5) doses of VDZ were received. After receiving treatment, 2 patients achieved a complete response (CR), 2 patients achieved a very good partial response (VGPR), 1 patient was non-responsive (NR) after a short-term partial response (PR). Compared with that before VDZ treatment, the amount of diarrhea, stool color, blood and traits of the children after medication were effectively improved. The median follow-up time was 9.3 (7.23-12.83) months. No disseminated or severe bacterial/fungal infections occurred during VDZ treatment and follow-up, and 2 children died of leukemia recurrence and pulmonary bronchiolitis obliterans. CONCLUSION VDZ salvage treatment of refractory GI GVHD in children has obvious short-term efficacy and good safety.
Child ; Humans ; Female ; Male ; Child, Preschool ; Salvage Therapy ; Glucocorticoids/therapeutic use* ; Retrospective Studies ; Graft vs Host Disease

In the fifth scientific and technological revolution， information technology is the first productivity， which has a great impact on the supply and demand of medical services. Generally， internet medicine is equivalent to the combination between health industry and information technology. "Healthy China" strategy is China's "priority development strategy"， which adheres to the principle of health equity， emphasizes the integration of health into all policies， and takes co-construction and sharing as the basic path. With implementing internet medicine， "Healthy China" strategy promotes the mobility of medical service with three tools： interconnection， data and artificial intelligence. This enhances the operation efficiency of overall medical and health system， and optimizes the allocation of medical resources. The future development of internet medicine follows the double helix mode driven by technology and policy， and the policy determines the development boundary of the industry. On the basis to ensure medical safety， we should explore the possibility of internet diagnosis and treatment further， and pay attention to the fairness of resource allocation while improving efficiency， so as to realize the co-construction and sharing of health services.

Internet medicine is not a concept of jurisprudence. China's current medical law system mainly focuses on main qualifications of the participants who conduct diagnosis and treatment. This paper focuses on several business models of the core element （diagnosis and treatment） in internet medicine， namely： online health consultation provided by internet medical platform， online diagnosis and treatment by internet hospital， and certain artificial intelligence（AI） medicine products that are specialized for diagnosis and treatment. This paper summarizes the development of these three business models， and analyzes the main problems in their development， including the legitimacy identification of online consultation， the dilemma of the development of internet hospitals， the impact of insufficient sample data on the development of medical AI， and the reverse restriction of regulatory difficulties to the development of internet medicine. The next step of developing internet medicine is to broaden the scope of internet diagnosis and treatment on the premise of medical safety， which will be based on the improvement of current regulatory system. It is urgent to build early risk warning system and supervision/ management mechanism for internet medicine.

The penetration and integration of internet with medical industry is apparently falling behind its interaction with other industries in terms of speed and scale. The main reason is due to the high risk and difficult supervision of internet medicine. Based on the regulatory context， this paper analyzes the endogenous and exogenous risk of internet medicine. The inherent characteristics of the internet （such as openness， diversity， multi subjectivity， virtuality， transparency， and high aggregation） lead to endogenous risks， including jurisdiction disputes， uncertainty of rights and obligations， security of personal information and medical information， monopoly. The lag of specific law in internet medicine， the lack of extensibility and synergy of traditional hierarchical regulatory system， the uncertainty of internet hospital regulatory policy details， the lack of technical specifications/operating procedures and other external system coverage in internet diagnosis and treatment lead to regulatory blind areas and exogenous risks. Based on the risk analysis， this paper discusses further construction of the mechanisms of risk assessment and regulation for internet medicine.

As a new form of medical industry， internet medicine has not only the endogenous risk of internet industry， but also the blind area and exogenous risk because of lagging regulation. With the continuous progress of internet information technology， online diagnosis and treatment will gradually integrate and reconstruct the traditional medical service. In the history of medical development， the emergence of new technologies generally has the characteristics of risk. Therefore， the supervision of internet medicine should adhere to the rule of inclusiveness and prudence， and take into account the cultivation of the market and standardized development. It should also build a regulatory community， coordinate governance from the four dimensions of legitimacy， compliance， rationality and superiority. Taking the law as the bottom line， we should make full use of early online warning monitoring， off-site supervision， online supervision， reputation mechanism and other measures to promote the innovative development of internet medicine under the framework of legal compliance.

Objective:To determine the current situation of food safety risk monitoring ability of centers for disease control and prevention （CDCs） in a province， and provide reference for strengthening the construction of food safety monitoring ability in the future. Methods:A self-administered questionnaire survey was conducted among 14 CDCs who are responsible for food safety risk monitoring in the province in 2018. Results:The ratio of food inspection staff with senior， middle， and primary professional titles was 1∶1∶1. The educational background of food inspection staff at the provincial and municipal levels was mainly bachelor degree or above， while that of county level directly under the administration of the province was mainly college degree. The age composition of the staff at the provincial level tended to be elder； however， there were more staff with less working experience at the county level. The qualification proportion of laboratory area was 50%. The distribution proportion of food inspection equipment in the CDC at all levels was 83.3%， 79.5% and 36.3%， respectively. In total， 14 CDCs completed the monitoring task as required. They were all accredited with laboratory qualifications； however， one CDC did not conduct re-evaluation after the certification of food inspection qualification expired. Each CDC ensured the accuracy and reliability of monitoring data through the supervision in food testing. Conclusion:The food safety risk monitoring ability of the CDCs in the province is continuously improved， which can meet the needs of routine monitoring. However， the composition of professional titles of food inspection staff is not very reasonable. There is still a gap between the equipment configuration and the national requirements. The ability of food inspection needs to be improved， in which quality management and laboratory qualification should be further strengthened.