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*

Objective:To investigate whether end-tidal carbon dioxide (etCO 2) is associated with acute kidney injury (AKI) after liver transplantation. Methods:Clinical data of 83 patients undergoing classic allogeneic liver transplantation were retrospectively collected. Patients were divided into AKI group and non AKI group based on changes in serum creatinine levels within 48 hours after surgery, and differences in baseline data, intraoperative etCO 2 decrease rate [ΔetCO 2(%)], and postoperative creatinine values were analyzed between the groups. Results:The incidence of AKI within 48 hours after liver transplantation in 83 patients was 56.6%(47/83). The proportion of male patients in the AKI group was higher ( P=0.003), and the ΔetCO 2(%) in the anhepatic phase of AKI group patients was higher than that of non AKI patients [(0.26±0.08)% vs (0.22±0.07)%, P=0.024]. There was no statistically significant difference in ΔetCO 2(%) between the two groups during pre liver, inferior vena cava (IVC) occlusion, and new liver phase (all P>0.05). On the 7th day after surgery, the creatinine value in the AKI group was significantly higher than that in the non AKI group [68.7(55.4, 92.6)]μmol/L vs 52.7(44.1, 69.3)μmol/L, P<0.001], at a 3-month follow-up, creatinine levels in 43 patients (91.5%) in the AKI group returned to normal. Conclusions:The decrease in ΔetCO 2(%) during the anhepatic phase of liver transplantation reflects to some extent the severe fluctuations in circulation during the operation, which may be related to the occurrence of AKI within 48 hours after surgery.

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.

Objective To investigate the morbidity, diagnostic profile and perinatal outcome of pregestational diabetes mellitus (PGDM) in 15 hospitals in Guangdong province. Methods A total of 41338 women delivered in the 15 hospitals during the 6 months,195 women with PGDM(PGDM group) and 195 women with normal glucose test result(control group)were recruited from these tertiary hospitals in Guangdong province from January 2016 to June 2016. The morbidity and diagnostic profile of PGDM were analyzed. The complications during pregnancy and perinatal outcomes were compared between the two groups. In the PGDM group, pregnancy outcomes were analyzed in women who used insulin treatment (n=91) and women who did not (n=104). Results (1)The incidence of PGDM was 0.472%(195/41338). Diabetes mellitus were diagnosed in 59 women (30.3%, 59/195) before pregnancy, and 136 women (69.7%,136/195) were diagnosed as PGDM after conceptions. Forty-six women (33.8%) were diagnosed by fasting glucose and glycohemoglobin (HbA1c) screening. (2) The maternal age, pre-pregnancy body mass index (BMI), prenatal BMI, percentage of family history of diabetes, incidence of macrosomia, concentration of low density lipoprotein were significantly higher in PGDM group than those in control group (all P<0.05). Women in PGDM group had significantly higher HbA1c concentration((6.3±1.3)% vs (5.2±0.4)%), fasting glucose [(6.3±2.3) vs (4.8±1.1) mmol/L], oral glucose tolerance test(OGTT)-1 h glucose((12.6±2.9) vs (7.1± 1.3) mmol/L)and OGTT-2 h glucose [(12.0±3.0) vs (6.4±1.0) mmol/L] than those in control group (P<0.01). (3)The morbidity of preterm births was significantly higher (11.3% vs 1.0%, P<0.01), and the gestational age at delivery in PGDM group was significantly smaller [(37.6±2.3) vs (39.2±1.2) weeks, P<0.01]. Cesarean delivery rate in the PGDM group (70.8% vs 29.7%) was significantly higher than the control group (P<0.01). There was significantly difference between PGDM group and control in the neonatal male/female ratio (98/97 vs 111/84, P=0.033). The neonatal birth weight in PGDM group was significantly higher((3159±700) vs (3451±423) g, P<0.01). And the incidence of neonatal hypoglycemia in the PGDM group was higher than the control group (7.7% vs 2.6%, P=0.036).(4)In the PGDM group, women who were treated with insulin had a smaller gestational age at delivery [(36.9±2.9) vs (37.9±2.5) weeks, P<0.01], and the neonates had a higher neonatal ICU(NICU)admission rate (24.2% vs 9.6% , P<0.01). Conclusions The morbidity of PGDM in the 15 hospitals in Guangdong province is 0.472%. The majority of PGDM was diagnosed during pregnancy; HbA1c and fasting glucose are reliable parameters for PGDM screening. Women with PGDM have obvious family history of diabetes and repeated pregnancy may accelerate the process of diabetes mellitus. Women with PGDM have higher risk for preterm delivery and neonatal hypoglycemia. Unsatisfied glucose control followed by insulin treatment may increase the need for NICU admission.

Objective To analyze the risk factors for traumatic cerebral venous sinus occlusion (CVSO)and to investigate the strategies of early diagnosis of traumatic CVSO. Methods The clinical data of 212 patients with moderate to severe closed traumatic brain injury from January 2012 to December 2015 were analyzed retrospectively. Logistic regression analysis was used to evaluate the risk factors for traumatic CVSO. Results Of the 212 patients with traumatic brain injury, 16.5%(35/212) patients had CVSO. Ten patients had CVSO of thrombotic type (typeⅠ), 16 patients had CVSO of compression type (typeⅡ), and 9 patients had CVSO of mixed type (typeⅢ). Logistic regression analysis showed that skull fracture (OR = 8.141; 95%CI: 3.224-20.840) and epidural hematoma of crossing venous sinus (OR = 3.179; 95%CI: 1.470-7.037) were the risk factors for CVSO, and the former was more significantly correlated with CVSO. Female gender was the risk factor for CVSO typeⅠ(OR =10.425; 95%CI: 1.831-30.053), epidural hematoma of crossing venous sinus was the risk factor for CVSO typeⅡ(OR = 5.766; 95%CI: 1.885-18.197), and skull fracture, epidural hematoma of crossing venous sinus, and the previous history of vein thrombosis was the risk factors for CVSO type Ⅲ(OR =18.005, 4.596, 11.394; 95%CI: 2.021-58.836, 1.144-19.525, 1.436-46.558). Conclusions In the early diagnosis of traumatic CVSO, the crossing venous sinus fracture line and epidural hematoma should be given attention. Attention should be paid to the history of venous thrombosis. MR venography and CT venography contributes to early diagnosis of CVSO.

Non-governmental organizations ( NGOs) are playing an increasingly significant role in global health governance. This study selected ten key NGOs that play an important role in global health affairs and summarized the tools employed by NGOs participating in global health governance, including nine dimensions:“Generating informa-tion and evidence/intelligence”, “Cooperation ( Partnerships )”, “Participation”, “Consultation”, “Transparen-cy”,“Organizational adequacy/system design”, “Formulating policy / strategic direction”, “Responsibility” and“Regulation”. Four types of NGOs including Operational, Supportive, Advocacy and Integrated ones presented com-monness and their priorities in the selection of tools to participate in global health governance. Meanwhile, China should strive to nurture local NGOs, which should pay attention to“Transparency”,“Participation” and“Cooperation ( Partnerships)”.

The abnormal expression (level and status) of the key molecular targets of tumors is related to molecular targeted therapy response, effect, and prognosis. Therefore, the expression level and status of key molecular targets of tumors must be accurately evalu-ated, regardless of the status before, during, and after receiving targeted therapy. Molecular imaging is a non-invasive method used for qualitative and quantitative research on key molecular targets of tumor in vivo and in real-time. This technique is also employed to screen treatment beneficiaries, guide therapy, and evaluate prognosis. This paper reviews the application progress of molecular imag-ing using various probes in cancer targeted therapy. The clinical value of molecular imaging in tumor targeted therapy is further ana-lyzed to promote the development of novel targeted therapy for tumors.

OBJECTIVE: To analyze the curative effect of CI in children with GJB2-associated NSSNHL. METHOD: The evaluations of curative effect with CI include auditory threshold, IT-MAIS/MAIS, CAP, SIR. MESP. The outcomes of 40 cases with GJB2-associated NSSNHI, were compared 80 patients with negative results of screening of gene mutation (control group). RESULT: In comparison with control group the auditory threshold in children with GJB2-associated NSSNIL is better, however had no significant difference in other tests (P > 0.05). CONCLUSION CI could he performed on children with GJB2-associated NSSNHL. Postoperative outcomes of hearing and speech were satisfied.
Child ; Child, Preschool ; Cochlear Implantation ; Connexin 26 ; Connexins ; genetics ; Female ; Hearing Loss, Sensorineural ; genetics ; surgery ; Humans ; Infant ; Male ; Mutation ; Treatment Outcome

A number of biosimilars will be soon marketing due to the expiration of patent protection of their originators.Unlike classical chemical drugs,biotherapeutics are proteins with large and complex molecules produced by biological high-tech with sophisticated manufactural procedures.This leads to the difficulties of the copy of biosimilars from their originators,and the almost inevitable distinctions between the two products.Thus biosimilars are only similar but not identical to their reference drugs in terms of stracture,action,and safety.It is important for physicians to understand these differences before using biosimilars.Optimal management by a national regulatory authority with rigorous standards is very important in all aspects of authorization of the development,production,marketing,and postmarketing surveillances for biosimilars.

OBJECTIVE: To study the molecular pathogenesis of SLC26A4 mutations associated with inner ear malformations including large vestibular aqueduct syndrome (LVAS), Mondini dysplasia and inner ear malformations but not accompanied with LVAS. METHOD: DNA sample and clinical material were obtained from 14 sporadic LVAS probands, six Mondini dysplasia probands and seven inner ear malformations excluding IVAS probands. SLC26A4 gene mutation was analyzed by direct sequencing for its 20 coding exons. GJB2 gene and also mt12SrRNA were analyzed by direct sequencing. RESULT: In 14 cases of LVAS, two mutations were detected in 12 patients (85.7%, either homozygous or compound heterozygous mutations), and one mutation was found in two patients (14.3%). In six cases of Mondini dysplasia, two mutations were detected in all of patients (100%). No mutation could be found in the seven cases of other inner ear abnormalities not accompanied with LVAS. No pathogenic mutation was detected in all of these 27 probands in GJB2 gene and mt12SrRNA 1555/1494T. CONCLUSION We have shown that LVAS and Mondini dysplasia closely correlate with SLC26A4 gene. No mutation was detected in seven probands of inner ear malformations not accompanied with LVAS. We should study the molecular pathogenesis of this disease in depth.
Adolescent ; Adult ; Child ; Child, Preschool ; Connexins ; Ear, Inner ; abnormalities ; Exons ; Female ; Genome ; Humans ; Infant ; Male ; Membrane Transport Proteins ; genetics ; Mutation ; Sulfate Transporters ; Syndrome ; Vestibular Aqueduct ; abnormalities ; Young Adult