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 identify the relationship between exposure to mobile phones and other electronic products and the ability development in children.Methods:Retrospective study.A total of 218 children aged 0.5-6.0 years presented to the Department of Health Care, Beijing Children′s Hospital, Capital Medical University form September 2019 to June 2020 for developmental examination were included.Those with nervous system, mental system diseases, endocrine system diseases and premature children were excluded.Their development was evaluated and the information about exposure to mobiles phones and other electronic products were collected. Chi- square test and Fisher′ s exact probability method were used to analyze the relationship between the exposure to mobile phones and other electronic products and the ability development in children.The influencing factors of children′s development in various fields were analyzed by the binary Logistic regression. Results:Abnormal fine movements of children aged 0.5-0.9 years were related to the latest time every night of exposure ( P<0.05). Abnormalities of adaptability, language and social self-care in children aged 1.0-2.9 years and abnormality of language in children aged 3.0-5.9 years were correlated with the number of hours of daily exposure (all P<0.05). Abnormalities of language and social self-care in children aged 1.0-2.9 years and abnormalities of adaptability, language and social self-care in children aged 3.0-5.9 years were correlated with the purposes of exposure (all P<0.05). Binary Logistic regression analysis indicated that the children′s gross motor was affected by the number of hours of daily exposure ( OR=1.868, P<0.05). The children′s fine motor movements were affected by mother′s educational level and the latest time every night of exposure ( OR=1.722, 2.355, all P<0.05). The children′s adaptability was affected by mother′s educational level, child caregivers, the number of hours of daily exposure and the latest time every night of exposure ( OR=1.711, 2.866, 1.895, 1.650, all P<0.05). The children′s speech was affected by the number of hours of daily exposure, the latest time every night of exposure, and the purposes of exposure (telephone or video phone, early education or study)( OR=2.348, 1.806, 0.328/0.350, all P<0.05). The children′s social interaction and self-care delay were affected by mother′s educational level, the number of hours of daily exposure and the purposes of exposure (telephone or video phone, early education or study)( OR=1.647, 2.678, 0.307/0.363, all P<0.05). Conclusions:The adverse effects of exposure to mobile phones and other electronic products on children should be well concerned.The exposure time of developing young children should be strictly controlled to prevent the adverse effects on the nervous system and development in children.For children who were already affected, relevant habits and behaviors should be timely corrected to avoid irreversible damages.

Objective:To explore the clinical efficacy of aspirin plus low molecule heparin for pancreatic thrombosis during simultaneous pancreas and kidney transplantation (SPK).Methods:A total of 129 patients aged 18 years or higher underwent SPK between September 2016 and March 2020.They were divided retrospectively into two groups of aspirin ( n=60) and heparin ( n=69) according to different anticoagulant regimens.The aspirin group received only aspirin 100 mg/d at Day 1 post-operation.The heparin group received subcutaneous injection of enoxaparin 2 000 AxaIU daily for 7 days and followed by aspirin and clopidogrel.Outcomes and complication rates were compared between two groups. Results:All operations were successful without any mortality.In aspirin group, there were 5 cases of pancreatic thrombosis and one patient underwent pancreatectomy.There was no pancreatic thrombosis in heparin group ( P=0.014). There were 8 cases of intestinal anastomotic bleeding in aspirin group and 19 cases in heparin group.Statistically significant inter-group difference existed ( P=0.048). However, no significant inter-group difference existed in delayed recovery or rejection. Conclusions:Heparin anticoagulation can significantly lower the incidence of pancreatic thrombosis after SPK.Despite a higher incidence of intestinal anastomotic bleeding, no serious complication occurs after conservative meaures.

Objective To investigate the safety of young recipients undergoing living donor renal transplantation from elderly relative donors through long-term follow-up of the pathological changes. Methods According to the age of donors, 28 young recipients were divided into the observation group (n=14, elderly donors) and control group (n=14, young and middle-aged donors). The 7-year survival after renal transplantation, the serum creatinine (Scr) levels at various postoperative time points were compared between two groups. The chronic pathological injury scores of renal allograft biopsy at time-zero, postoperative 6-month and 7-year were compared between two groups. The expression levels of renal interstitial fibrosis indicators connective tissue growth factor (CTGF), transforming growth factor (TGF)-β, laminin (LN), fibronectin (FN), cell senescence indicators intercellular connexin (Cx)-43 and mammalian target of rapamycin (mTOR) at postoperative 6-month and 7-year were compared between two groups. Results The 7-year survival rates in the observation and control groups were 78.5% and 92.8% with no statistical significance (P > 0.05). In the observation and control groups, the levels of Scr were 190 and 160 μmol/L at the postoperative 7 d, and 170 and 125 μmol/L at postoperative 1 month. At each postoperative time point, the levels of Scr in the observation group were significantly higher than those in the control group (all P > 0.05). The total chronic pathological injury scores of renal transplant biopsy at time-zero in the observation group was significantly higher than that in the control group (P > 0.05), whereas the total chronic pathological injury scores at postoperative 7-year did not significantly differ between two groups (P > 0.05). Within either group, the total chronic pathological injury scores at postoperative 7-year was remarkably higher than those at time-zero and postoperative 6-month (both P < 0.05). The expression levels of CTGF, TGF-β, LN, FN, mTOR, Cx43 of renal transplant tissue at postoperative 7-year did not significantly differ between two groups (all P > 0.05). Conclusions The long-term follow-up outcomes demonstrate that the pathological changes of young recipients undergoing renal transplantation from elderly donors are similar to those from young and middle-aged donors. It is safe and feasible for young recipients to undergo renal transplantation from elderly donors in the pathological perspective.

Objective To investigate the role of cyclic GMP-AMP synthase (cGAS),a cytosolic DNA sensor,in regulating innate immune responses induced by reverse transcription intermediate of human T cell leukemia virus type 1 (HTLV-1). Methods (1)ssDNA90,the reverse transcription intermediate of HTLV-1,was transfected into HeLa cells to observe changes in the expression pattern of cGAS in transfected-HeLa cells with immunoblot assay. (2) HeLa cells were firstly transfected with cGAS-encoding plasmid and then ssDNA90 24 hours later. Real-time PCR was used to measure the expression of interferon ( IFN)-β, IFN-gamma-inducible protein 10 ( IP-10 ), regulated on activation, normal T cell expressed and secreted (RANTES) and tumor necrosis factor (TNF)-α. Immunoblot assay was performed to measure phosphorylated interferon regulatory factor 3 (IRF3) and p65. (3)cGAS expression was silenced by siRNA in HeLa and phorbol-12-myristate-13-acetate (PMA)-treated THP1 (PMA-THP1) cells and then ssDNA90 was transfect-ed into these cells 24 hours later. Real-time PCR was used to measure the expression of IFN-β,IP-10,RAN-TES and TNF-α. Immunoblot assay was performed to measure phosphorylated IRF3 and p65. Results Ex-pression of cGAS was increased in HeLa cells after ssDNA90 transfection. Compared with control cells, cGAS-transfected HeLa cells showed increased expression of IFN-β, IP-10, RANTES and TNF-α and en-hanced phosphorylation of IRF3 and p65 after ssDNA90 transfection. Compared with control cells,both HeLa and PMA-THP1 cells with silenced expression of cGAS showed impaired production of IFN-β,IP-10,RAN-TES and TNF-α after ssDNA90 transfection. Moreover,ssDNA90-induced phosphorylation of IRF3 and p65 were decreased after cGAS gene-knockdown. Conclusion cGAS might promote HTLV-1 RTI ssDNA90-in-duced innate immune responses.

Objective To investigate the function and the possible mechanism of cyclic GMP-AMP synthase (cGAS), a DNA sensor, in HeLa cells during human T cell leukemia virus type 1 (HTLV-1) in-fection.Methods HeLa cells were co-cultured with MT2 cells (HTLV-1-positive T cells) and then detec-ted by immunoblot assay to analyze the changes in the expression of cGAS .A hemagglutinin ( HA)-tagged cGAS plasmid was constructed and transfected into HeLa cells .Twenty-four hours after transfection , these cells were co-cultured with MT2 cells for another 24 hours.Immunoblot assay was used to detect the expres-sion of HTLV-1 proteins Tax and p19.Real-time PCR was performed to measure the expression of HTLV-1 Tax, p19, Env, HBZ and px at mRNA level .Immunoblot assay was also used to analyze the phosphorylation of interferon regulatory factor 3 (IRF3) and p65.Expression of interferon (IFN)-β, IFN-gamma-inducible protein 10 ( IP-10 ) , RANTES ( regulated on activation , normal T cell expressed and secreted ) and tumor necrosis factor (TNF)-αwas detected by real-time PCR assay.Results Expression of cGAS was enhanced in HeLa cells after co-cultured with MT2 cells.Compared with control cells , the HeLa cells that were trans-fected with cGAS plasmid showed lower levels of Tax and p 19 proteins, suppressed expression of HTLV-1 Tax, p19, Env, HBZ and px at mRNA level , enhanced phosphorylation of IRF 3 and p65, and higher levels of IFN-β, IP-10, RANTES and TNF-αafter co-cultured with MT2 cells.Conclusion cGAS might promote the innate immune response and inhibit HTLV-1 replication in HTLV-1-infected HeLa cells .

OBJECTIVE:To optimize the preparation technology of Sinomenine topical paste. METHODS:Using“initial vis-cous force”,“holding viscous force”and“peeling strength”as index,heating and stirring time (A,h),heating temperature (B,℃) and the sequence of adding composition (softening agent,blank matrix and sinomenine)(C) as influential factors,the preparation technology of Sinomenine topical paste was optimized by orthogonal test and verified. At the same time,the content of sinomenine was determined by HPLC method. RESULTS:The optimal preparation technology of Sinomenine topical pasta was as follows as adding blank matrix and sinomenine,and then adding softening agent,heating at 80 ℃,stirring for 1 h. In verification test,RSD of comprehensive score for 3 batches of samples were 2.09%(n=3);average contents of samples were 6.7 mg/g, which was in line with the requirement of ≥6.0 mg/g. CONCLUSIONS:The optimal preparation technology of Sinomenine topical pasta is reasonable,stable and feasible. The paste shows good adhesiveness and is qualified in content.

Objective To analyze the clinical and imaging characteristics of congenital sensorineural hearing loss (CSNHL)children combined with white matter (WM)lesions in order to provide evidence for clinical practice. Methods With referral to the Department of Pediatrics,Peking University First Hospital from November 201 1 to De-cember 201 5,documents of 78 patients of CSNHL combined with WMlesions were collected and analyzed for the clini-cal and imaging characteristics.Results Bilateral severe -profound hearing loss existed in all 78 cases,48.1 %(25 /52 cases)of the patients exhibited gross motor development delay,98.1 %(51 /52 cases)of them had normal cognition development.One hundred percent (61 /61 cases)of patients had abnormal language development.Infection occurring during pregnancy existed in 21 .2%(1 1 /52 cases)of the patients,the premature and smaller for the gestational age in-fants accounted for 28.9% (1 5 /52 cases).The bilateral multiple WMlesions from the brain MRI were in dot to flake sizes with sharp boundary,the intensity of T1 -weighted imaging decreased,T2 -weighted imaging and fluid attenuated inversion recovery increased.Eighty -two point one percent (64 /78 cases)of the patients were found to have the periventricular and subcortical WM involvement.The most frequently affected periventricular region was the posterior horn (91 .9%,68 /74 cases),followed by the anterior horn and temporal horn,and the least with the body involvement. The former three had a combined lesion tendency (55.4% -68.9%).There was an extensive involvement in the sub-cortical WMof parietal,frontal,temporal and occipital lobes respectively(73.5% -88.2%).Subcortical WM involve-ment of multiple lobes was common (accounted for 67.6% -77.9%).The enlargement of bilateral ventricles existed in 37.2%(29 /78 cases)of the patients and cystic changes in the subcortical WM of anterior temporal lobe could be found in 9.0% (7 /78 cases)patients.Calcification in 2 CT cases was reported.Corpus callosum and basal ganglia of all cases were normal.For cases with MRI scans more than once,WMlesions of 96.0%(24 /25 cases)patients became silent or self -restored.Conclusions The clinical presentations of CSNHL combined with WM lesions are mild,not paralleled with their multiple foci.It is considered as demyelination or a delay of myelination.Due to its benign course, it is probably not the contraindication for the cochlear implantation.

Objective:To evaluate the effect of preoperative panel reactive antibody(PRA)levels on long-term survival after kidney transplantation. Methods:Data on 1 162 patients underwent first kidney transplantation performed between January 2001 and June 2014 were included in our center. According to the preoperative PRA levels,the patients were divided into negative group( PRA≤10%) and positive group( PRA>10%) ,which were retrospectively analyzed. Results: The 1-,5-,10-year patient survival rates of the negative group calculated by Kaplan-Meier were 96. 8%,89. 4%,78. 6%,respectively,while the positive group were 93. 5%,81. 6%, 65. 4%. The 1-,5-,10 -year death-censored graft survival rates of the negative group were 95. 9%,84. 8%,63. 1%,respectively,while the positive group were 92. 3%,74. 1%,51. 9%. The log-rank test revealed that there was significant difference between the patient and graft survival curves (χ2 =9. 623/11. 019, P=0. 002/0. 001 ) . Cox multivariate analysis found that preoperative PRA levels were independent risk factors for reducing the patient or graft survival rates(P<0. 001). Logistic multivariate regression analysis confirmed the significant association between preoperative PRA levels and the risk of acute rejection ( OR=8. 25,95% CI=2. 86-5. 72, P<0. 001). The 5-,10-year creatinine values were significantly lower in the negative group compared to the positive group(all P<0. 05), while there was no difference in the 1-year. In addition, Logistic multivariate regression analysis confirmed the significant association between preoperative PRA levels and the production of donor specific antibody(DSA)(OR=6. 89,95% CI=4. 52-9. 17,P<0. 05). Conclusion: The detection of preoperative PRA is an important indictor predicting the sensitivity status of the recipients. The preoperative PRA positive recipients need careful monitoring and diagnosis of acute rejection and DSA after kidney transplantation.

Objective To study the pathological changes of renal grafts from elderly donor in young recipients and to investigate the safety of kidney transplantation from elderly donors.Methods Fourteen elderly kidney donors (with the age >55 years old)and fourteen young recipients (with the age <30 years old)underwent living related donor renal transplantation at the Department of Transplantation of the Second Affiliated Hospital of Guangzhou Medical University from January 2008 to December 2008 were enrolled as the object of study.Every elderly donor kidney was performed time-zero biopsy and every young recipient was performed routine renal graft biopsy 6 months after transplantation.The pathological and structural changes of kidney tissues after renal transplantation from elderly donors were observed.Results The histopathological changes occurred at 6 months after transplantation from elderly kidney donors to young recipients.Compared with those before transplantation,the severity of renal arteriolar lesion and the index of renal arteriolar sclerosis alleviated after transplantation (P <0.05 ), and the ratio of glomerulosclerosis did not change after transplantation (P >0.05 ).The expression of fibronectin (FN)decreased after transplantation,but the difference had no statistical significance (P >0.05 ).The expression of laminin (LN ) decreased after transplantation (P <0.05).Conclusions The histopathological structure of renal graft from elderly donors in young recipients has improved.