Oculo-auriculo-vertebral spectrum (OAVS), also known as Goldenhar syndrome, hemifacial microsomia, oculo-auriculo-vertebral dysplasia and facio-auriculo-vertebral spectrum, is a developmental disorder associated with the first and second branchial arches. Most cases are sporadic, while some familial instances observed suggested that the etiology of OAVS heterogeneous. In this review, we summarize the OAVS epidemiology, classification and mainlyheterogeneous etiology.
Goldenhar Syndrome ; pathology ; Humans

OBJECTIVEUsing questionnaires to evaluate the audiological benefit and satisfaction of bilateral aural atresia patients with bone-anchored hearing aid (Baha).

METHODSImplanted Baha user questionnaire was applied to 19 patients suffering bilateral aural atresia, and 15 of the patients were evaluated with abbreviated profile of hearing aid benefit (APHAB). Glasgow children's benefit inventory (GCBI) was used to measure subjective benefit of patients under the age of 18.

RESULTSThe Baha user questionnaire demonstrated great satisfaction. The mean Baha scores for the subdomains of ease of communication (EC), backgroud noise (BN) and revereration (RV) were decreased by 54.6 ± 10.2 (t = 20.6, P < 0.05), 46.9 ± 11.1 (t = 16.4, P < 0.05) and 58.8 ±15.4 (t = 21.4, P < 0.05) compared to the unaided scores. For the subdomain of aversiveness (AV), the Baha score was 56.7 ± 9.9 (t = 10.8, P < 0.05) higher than the unaided score. The general benefit score GCBI was 41.1 ± 13.0.

CONCLUSIONBaha can significantly improve hearing ability and quality of life of patients with bilateral aural atresia.

Objective:To explore the tumor microenvironment (TME) module associated with pancreatic ductal adenocarcinoma (PDAC) and identify prognostic biomarkers and potential immunotherapeutic targets.Methods:The genetic expression profile data were collected and selected from a dataset of 142 PDAC patients in The Cancer Genome Altas (TCGA) database and 2 microarray datasets (GSE2150, GSE62452) of 168 PDAC patients in Gene Expression Omnibus (GEO) database, and the cell type enrichment analysis of PDAC gene expression data was analyzed by xCell network tool. According to the median cell enrichment score, 142 patients from TCGA were divided into high score group and low score group, and the cell types with prognostic value were determined by univariate survival analysis and validated by GEO datasets. According to the cell type, the differential expression gene analysis and univariate survival analysis were performed to determine the prognosis related differential expression genes (DEGs), and the prognostic DEGs were analyzed by function enrichment analysis and protein-protein interaction (PPI) network analysis. At the same time, GEO dataset was used to verify the prognosis related DEGs of TCGA datasets. Finally, TISIDB database was searched for the common DEGs of TCGA and GEO database, and its correlation with immune system was analyzed.Results:Cell type enrichment analysis showed that Th1 cell and keratinocyte had the same prognostic value in both TCGA and GEO dataset; the overall survival rate of patients with high score was lower than that of those with low score, and the differences were statistically significant (all P values <0.05). 216 prognosis related DEGs were identified, and the results of functional enrichment showed that 9 of the 21 biological process items were closely related to the immune process, and 4 of the 5 KEGG (Kyoto Encyclopedia Of Genes and Genomes) pathways were closely related to the immune process. Through PPI network analysis, CCR7, CD 27, CD 5, CXCL13, ZAP70, MS4A1 and CCL19 were proved to be possibly closely associated with central genes. Through the validation of GEO datasets, there were 15 DEGs with similar prognostic value in GEO and TCGA datasets, which was searched in TISIDB dataset, and the result showed that GIMAP7 was closely related with the immune process of PDAC. Conclusions:A group of 216 TME genes and 7 central genes related to the prognosis of PDAC were identified, and 5 potential targets for immunotherapy of PDAC were provided, including CCR7, CCL19, CD 27, CXCL13 and GIMAP7.

OBJECTIVE: To explore the prevention and treatment of common complications of ear reconstruction. METHOD: To summarize complications of 46 patients of microtia whom underwent auricular reconstructions using soft tissue skin expander and autogenous rib cartilage framework by two stages from November 2011 to October 2012. RESULT: One patient had flap necrosis resulting to exposures of the expanders in the first surgical stage. Three cases of flap necrosis occurred in the second surgical stage. After repair the defect with pedicled flap, the reconstructed auricles of patients with complications were aesthetically satisfied. CONCLUSION Treating every step of the reconstruction seriously and correctly could prevent the happening of complications, and when confronting the complications, apply the right treatment such as using pedicled flaps repair the defect to reconstruct the lifelike auricles.
Adolescent ; Adult ; Child ; Ear Auricle ; surgery ; Humans ; Postoperative Complications ; prevention & control ; Reconstructive Surgical Procedures ; adverse effects ; methods ; Young Adult

Objective:To evaluate the methodological quality and reporting quality of clinical practice guidelines or consensuses in the field of Chinese pancreatic diseases from 2016 to 2021, and provide reference for formulating clinical practice guidelines and consensuses in this field.Methods:VIP, Wanfang , CNKI and CBM databases were searched for articles published from January 2016 to March 2021. The main index terms were " pancreas" , " guideline" , " consensus" , and the supplementary index terms were " pancreatitis" , " pancreatic cancer" , " pancreatic head cancer" . Two researchers independently selected the literature. The appraisal of guidelines for research and evaluation (AGREE-China) was utilized to assess the methodological quality of the guidelines or consensuses, and the reporting items for practice guidelines in healthcare (RIGHT) tool was used to assess the reporting quality.Results:A total of 14 literature were included, including 7 literature on pancreatic cancer, 3 literature on acute pancreatitis, 1 literature on chronic pancreatitis and 3 literature on others. The results of the assessment by the AGREE-China tool showed that there were no document with a total score greater than or equal to 60.0 points, two with 40.0 to 59.9 points, eleven with 20.0 to 39.9 points, and one with less than 20.0 points. Among the results of RIGHT list, basic information was reported the highest(72.62%) and funding and declaration was the lowest(0).Conclusions:The methodological and reporting quality of the guidelines or consensuses on pancreatic disease in China from 2016 to 2021 are generally not high. In the process of developing domestic guidelines or consensuses on pancreatic diseases, the guideline developer should refer to AGREE-China and RIGHT to improve the quality of clinical practice guidelines or consensuses.

Objective:To investigate the specific types of lateral meniscus injury in Schatzker type II tibial plateau fractures and its potential correlation with CT features of the lateral plateau.Methods:The data of 213 patients with Schatzker II tibial plateau fractures from August 2014 to June 2021 were retrospectively analyzed, including 132 males and 81 females, aged from 29 to 61 years, with an average of 44.9 years. All patients underwent arthroscopic evaluation of fracture reduction immediately after open reduction and internal fixation (ORIF). According to the actual situation during the operation, the types and locations of lateral meniscus injury were determined and the patients were divided into the meniscus injury group and non-injury group. By measuring lateral plateau depression (LPD) and lateral plateau widening (LPW) of the lateral tibial plateau on CT images, the correlation of which and lateral meniscus injury was analyzed. The optimal critical values of LPD and LPW for predicting lateral meniscus injury were obtained by drawing the relevant receiver operating characteristic (ROC) curves.Results:The meniscus injury group (109 patients) mainly showed injuries involving the mid-body and posterior horn of lateral meniscus (98.2%, 107/109) and LPD was 13.1±3.2 mm; while the LPD of 104 patients without meniscus injury was 9.1±3.0 mm with a statistical difference ( t=3.98, P<0.001). The LPW of meniscus injury group and non-injury groups was 8.0±1.3 mm and 6.7±1.6 mm, respectively, and the difference was statistically significant ( t=2.68, P=0.011). The optimal predictive critical point of LPD and LPW was 7.6 mm (sensitivity 90.3%, specificity 64.7%, area under the curve 0.834) and 7.3 mm (sensitivity 80.5%, specificity 58.8%, area under the curve 0.722). Conclusion:Schatzker II tibial plateau fractures combined with lateral meniscus injury is usually characterized by meniscus-joint capsule separation, rupture and longitudinal fracture. The mid-body and posterior horn of lateral meniscus injury is more likely to occur when LPD> 7.6 mm and/or LPW> 7.3 mm on coronal CT images.

Objective Based on network pharmacology,molecular docking and animal experiment,this study explored the mechanism of Icariin combined with Prednisone in the treatment of steroid-resistant nephrotic syndrome(SRNS)in rats.Methods The targets of Icariin active components were determined by TCMSP,GeneCards and CTD databases.The targets of SRNS were screened from GeneCards,OMIM,CTD and DRUGBANK databases,Remove and integrate.Venn graph was drawn to obtain the intersection target.A protein-protein interaction network was constructed using STRING database,and core targets were screened by topological analysis using Cytoscape 3.7.2.DAVID 6.8 was employed for GO term enrichment and KEGG pathway enrichment.And AutoDockTools Vina,PyMOL for molecular docking.SRNS rat model was constructed.The body weight of rats was measured,The 24-hour urinary protein quantity was detected,and the pathological morphology of renal tissue was observed by HE staining.,and the expression of core target proteins was de tected by Western blot.Results Network pharmacologic analysis showed that 139 targets corresponding to Icariin active ingredients were obtained,and 58 common targets were obtained by intersection with 1476 SRNS related targets.In PPI network,the top three degree values were Akt,JUN and IL6.KEGG enrichment analysis showed that Icariin played a therapeutic role in SRNS mainly by PI3K/AKT singnaling pathway.Molecular docking verification showed that Akt,PI3K and autophagy-related protein LC3-II had good binding activity with Icariin.Animal experiment result showed that Icariin combined with Prednisone significantly increased the body weight of SRNS rats(P<0.05),decreased the 24 hour urine protein quantity(P<0.01),and improved the glomerular morphological changes.The results of Western-blotting showed that Icariin combined with Prednisone could significantly improve the abnormal decrease of LC3-II protein and the abnormal increase of Akt and PI3K protein expression in renal tissue of SRNS rats(P<0.01).Conclusion Icariin can play a role in the treatment of SRNS through a multi-target and multi-pathway,regulate the PI3K-Akt signaling pathway,and increase the autophagy activity of kidney tissue in SRNS rats,which may be one of its main mechanisms.

Objective To explore the incidence and risk factors of gout in Jinchang cohort and provide scientific evidence for the prevention and control of gout.Methods People without gout detected by baseline survey in Jinchang cohort were selected as study subjects.All the subjects were followed up through questionnaire interview,physical examination as well as laboratory test from January 24,2013 to November 24,2015.Cox regression model was used to analyze the risk factors for gout in Jinchang cohort.In addition,log-linear model was used to analyze the interaction between risk factors.Results A total of 33 153 subjects were followed up,and there were 277 newly diagnosed gout cases in the cohort.The overall incidence of gout was 0.8％.The incidence of gout in males was higher than that in the females,but the incidence of gout in males and females was similar after the age of 60 years.Cox regression analysis showed that age ＞40 years (at age 40 to 59 years:HR=2.982,95％CI:1.503-5.981;at age 60 to 91 years:HR=2.588,95％CI:1.107-6.049),alcohol abuse (HR=2.234,95％CI:1.128-4.427),obesity (HR=2.204,95％CI:1.216-3.997),diabetes (HR=2.725,95％CI:1.500-4.950) and high uric acid (HR=5.963,95％CI:3.577-9.943) were risk factors for gout,while weekly beans intake ≥0.25 kg (HR=0.528,95％CI:0.345-0.808) and regular physical exercise (HR=0.499,95％ CI:0.286-0.869) were protective factors for gout.The analysis with log-linear model showed that there were two order effects between the risk factors.Conclusions Age,beans intake,alcohol abuse,physical exercises,obesity,diabetes and high uric acid were important factors influencing the incidence of gout.It is important to have healthy lifestyle and dietary habits,receive regular health examination to prevent and control the incidence of gout in this cohort.

OBJECTIVE: To investigate the surgical technique of the pericanal electrode insertion technique for ies cochlear implantation. METHOD: Forty cases of sensorineural deafness were subjected to the ies cochlear implants. Cochleostomy was performed through the external auditory canal with a microdrill anterior to the round window. The electrode impedance and electrically auditory brainstem responses(EABR) were tested during the operation. The X-ray photographs were taken after the operation. The cochlear implant was activated in all 40 cases 4 weeks following surgery. RESULT: All of the electrodes were inserted and all of the implants worked well. No electrode extrusions or serious surgical complications happened during postoperative observation for 6 months. CONCLUSION The pericanal electrode insertion technique is a safe approach for ies cochlear implantation.
Child, Preschool ; Cochlear Implantation ; methods ; Cochlear Implants ; Ear Canal ; surgery ; Female ; Hearing Loss, Sensorineural ; surgery ; Humans ; Infant ; Male ; Otologic Surgical Procedures ; methods ; Subcutaneous Tissue ; surgery

Background Air pollutants PM_2.5 and its adsorbed metal elements are important factors affecting public health. Objective To explore the distribution characteristics and sources of metal elements in atmospheric PM_2.5 in Lanzhou from 2019 to 2020, and to assess the health risks of metal elements to different groups of residents through inhalation. Methods From January 2019 to December 2020 in two districts of Lanzhou City (Chengguan District and Xigu District), regular PM_2.5 and metal elements [antimony (Sb), aluminum (Al), arsenic (As), beryllium (Be), cadmium (Cd), chromium (Cr), mercury (Hg), lead (Pb), manganese (Mn), nickel (Ni), selenium (Se), and thallium (Tl)] were regularly monitored, and their concentrations were described by the median (M) and 25^th and 75^th percentiles (P₂₅, P₇₅) as not following a normal distribution (because the detection rates of the five elements Be, Cr, Hg, Ni, and Se were less than 70%, the five elements were not included in subsequent analysis), and then compared with the secondary concentration limits in the Ambient Air Quality Standards (GB 3095-2012). The differences between the medians of the two groups were compared by the Mann-Whitney U rank sum test, and the differences among the medians of multiple groups were compared by the Kruskal-Wallis H rank sum test; the enrichment factor (EF) method and principal component analysis were used to evaluate the pollution degree of the metals and their sources; the health risks of five non-carcinogenic metals (Sb, Al, Pb, Mn, and Tl) and two carcinogenic metals (As and Cd) in PM_2.5 were evaluated by hazard index (HI) and hazard quotient (HQ) using the incremental lifetime cancer risk (LCR) model and the non-carcinogenic risk assessment model, respectively. Results The PM_2.5 concentrations [M (P₂₅, P₇₅)] in Lanzhou City were 38.50 (26.00, 65.00) and 41.00 (29.00, 63.10) μg·m⁻³ in 2019 and 2020, respectively, and the difference was not statistically significant (Z=−0.989, P > 0.05). The average levels of the metal elements from high to low were: Al > Pb > Mn > As > Cd > Sb > Tl, and the annual average concentration of each metal element in 2019 was higher than that in 2020 (P<0.05). The M ( P₂₅, P₇₅) of PM_2.5 concentrations in Chengguan and Xigu districts were 52.98 (17.00, 61.00) and 55.40 (17.00, 67.00) μg·m⁻³, respectively, with no statistically significant differences (P<0.05); the concentrations of Sb and Al in Chengguan District were lower than those in Xigu District (P<0.05), and the concentrations of other metal elements were not different between the two areas (P>0.05). There were seasonal differences in the concentrations of PM_2.5 and seven metal elements in Lanzhou City (except P_Al=0.007, the other P_s < 0.001). The results of the enrichment factor method showed that the EF values of the six metals (Sb, Al, As, Cd, Pb and Tl) were all greater than 1. Among them, except As, the EF values of other metal elements were all greater than 10, and the EF values of Al and Cd were both greater than 100. The results of principal component analysis showed that the variance contributions of the three principal components were 45.61%, 24.22%, and 14.42%, and the cumulative contribution reached 84.25%. The principal component 1 included Pb, As, Cd, and Sb, the principal component 2 included Al and Mn, and the principal component 3 contained Tl. The non-carcinogenic risks of the five metals were, in descending order, Al > Mn > Pb > Tl > Sb, among which the HQ values of the remaining four metals were less than 1 for adults and children, except the HQ value of Al for adults, which was greater than 1. The ILC values of carcinogenic metal As for adult males, adult females, and children were 2.68×10⁻⁵, 2.51×10⁻⁵, and 1.45×10⁻⁵, respectively; the ILC values of carcinogenic metal Cd for adult males, adult females, and children were 1.53×10⁻⁶, 1.43×10⁻⁶, and 8.26×10⁻⁷, respectively. Conclusion There is pollution of atmospheric PM_2.5 and its adsorbed metal elements in Lanzhou. As and Cd elements may pose potential carcinogenic risks to the residents.