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

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

In recent years, non-invasive positive pressure ventilation (NIPPV) has been widely used in clinical practice to improve the therapeutic effect of patients with chronic obstructive pulmonary disease (COPD) . However, there is non-compliance in patients during the application of the ventilator. The patients cannot tolerate NIPPV and terminates treatment in advance, resulting in unsatisfactory NIPPV treatment. This article reviews the application of non-invasive ventilator in COPD patients, the factors that affect the compliance of NIPPV treatment and nursing countermeasures, in order to provide references for improving NIPPV compliance as well as the therapeutic effect of COPD patients.

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 Using questionnaires to evaluate the audiological benefit and satisfaction of bilateral aural atresia patients with bone-anchored hearing aid (Baha).Methods Implanted 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.Results The 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.Conclusion Baha can significantly improve hearing ability and quality of life of patients with bilateral aural atresia.

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 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 effect of stepwise nursing in postoperative rehabilitation of patients with spinal cord injury.Methods:From January 2020 to June 2021, convenience sampling was used to select 91 patients with spinal cord injury admitted to the Henan Third Provincial People's Hospital as the research object. The patients were divided into the observation group ( n=46) and the control group ( n=45) by random number table method. The control group received routine nursing, while the observation group received stepwise nursing on the basis of the control group. The postoperative functional independence and quality of life were compared between the two groups. Results:After six months of intervention, the scores of the Spinal Cord Independence Measure Ⅲ and the scores of each dimension of the Generic Quality of Life Inventory 74 in the observation group were higher than those in the control group, and the differences were statistically significant ( P<0.05) . Conclusions:Stepwise nursing can promote postoperative functional recovery and improve the quality of life of patients with spinal cord injury.

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