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BACKGROUND: Seventy percent of intrahepatic cholangiocarcinoma (ICC) patients are inoperable. Treatment for unresectable patients is essential to improve poor survival. AIMS: We aimed to evaluate the prognostic factors for ICC patients, and investigate the potential treatment strategies for unresectable patients. METHODS: ICC patients were identified in SEER registry in 2004–2013. Univariate and multivariate Cox proportional hazard regression analysis were performed to evaluate the effect of treatment strategies. RESULTS: Of 2248 cases diagnosed in 2010–2013 and staged according to the American Joint Committee on Cancer (AJCC) 7th edition, 1706 (76.13%) did not receive cancer-directed surgery. This portion increased compared to those diagnosed between 2004 and 2009 and staged according to the AJCC 6th edition (72.87%). In addition, the percentage of stage 4 cases increased, while stage 3 cases decreased, because AJCC 7th staging system categorized both T4 and N1 patients into stage IV, which were previously categorized into stage III by AJCC 6th staging system. Patients with radiofrequency ablation (RFA) showed a poorer survival in 2004–2009 (P = .0213), but an almost the same survival as patients with tumor resection in 2010–2013 (P = .51), suggesting that RFA performed better in recent years. Lymphadenectomy showed protective effect for unresectable patients. Radiotherapy improved cancer-specific survival in non-surgery patients (P

Abstract Background Endocan, also known as endothelial cell specific molecule-1 (ESM1), is a 50 kDa soluble proteoglycan which is frequently overexpressed in many cancer types. Whether it is dysregulated in head and neck squamous cell carcinoma (HNSCC) has not been investigated. Methods We analyzed the expression of ESM1 using bioinformatics analysis based on data from The Cancer Genome Atlas (TCGA), and then validated that ESM1 was significantly overexpressed in human HNSCC at the protein level using immunohistochemistry. We also analyzed the genes co-expressed with ESM1 in HNSCC. Results The most correlated gene was angiopoietin-2 (ANGPT2), a molecule which regulates physiological and pathological angiogenesis. Several transcription factor binding motifs including SMAD3, SMAD4, SOX3, SOX4, HIF2A and AP-1 components were significantly enriched in the promoter regions of the genes co-expressed with ESM1. Further analysis based on ChIP-seq data from the ENCODE (Encyclopedia of DNA Elements) project revealed that AP-1 is an important regulator of ESM1 expression. Conclusions Our results revealed a dysregulation of ESM1 and a potential regulatory mechanism for the co-expression network in HNSCC.

Abstract Autosomal recessive nonsyndromic hearing loss (ARNSHL) is a genetically heterogeneous neurosensory disorder, usually characterized by congenital or prelingual hearing loss. We report a Han Chinese male, born to consanguineous parents, presenting with nonsyndromic sensorineural hearing loss, whose clinical phenotype was also consistent with auditory neuropathy spectrum disorder (ANSD). After exome sequencing, a gap junction protein beta 2 gene (GJB2) c.235delC variant in the homozygous state was detected in the patient. Both parents were heterozygous for this variant, as documented by Sanger sequencing. The known pathogenic GJB2 c.235delC variant was not detected in 200 healthy controls. It is predicted to be a disease-causing alteration by generating a truncated protein p.(L79Cfs*3), disturbing the appropriate folding and/or oligomerization of connexins and leading to defective gap junction channels. This study shows that the association of homozygosity of the GJB2 c.235delC variant with ARNSHL and ANSD in a patient.

Abstract Objective To examine the distribution of brain metastases (BM) in relation to the hippocampus, so as to determine the risk of metastasis in the hippocampus, and thus provide experimental evidence for the hippocampal avoidance (HA) in patients with BM during radiotherapy. Methods (1) For the retrospective analysis of 116 patients diagnosed with malignancies, confirmed as BM, from December 2014 to December 2016 at the First Affiliated Hospital of Bengbu Medical College. We obtained the T1-weighted, postcontrast axial, sagittal, and coronal Magnetic Resonance imaging (MRI) images f the patients, in supine position, using the head restraints and head thermoplastic masks to adjust the positioning, with computed tomography (CT) positioning scan ranging from the head to the mandible (layer thickness: 3 mm). CT and MRI images were fused on a Philips Pinnacle v9.8 treatment planning system;(2) Every metastasis of the 565 metastases was contoured;(3) hippocampus were contoured, and hippocampus with 5 mm expansion envelopes were analyzed;(4) Using the SPSS 16.0 software, we analyzed the relation between the distribution and age, sex, Karnofsky performance status (KPS), primary site, aggregate volume of intracranial metastases and the whole brain. The data were analyzed using a binary logistic regression analysis method, with two-sided P 0.05). Conclusion This study showed a low risk for the perihippocampal metastases (PHM) and no significant correlation between PHM and age, sex, KPS, primary site, aggregate volume of intracranial metastases and the whole brain. Accordingly, it is may be acceptable to avoid the perihippocampal region during whole brain radiotherapy.

When sowing with a wide boundary under full rice straw retention in the rice–wheat rotation area of China, conventional fertilization methods have some problems, such as a low fertilizer utilization rate, heap soil around a buried fertilizer device, or blocked fertilizing orifice. Firstly, combined with theoretical analysis, discrete element numerical simulation technology, and central composite test method, the wide-boundary fertilization device for wheat wide-boundary sowing was designed. Then, with the coefficient of variation for particle uniformity (CVPU) as the response value, the central composite experiment was carried out on the key structural parameters (focal length coefficient, lateral span, tilt angle, and ground clearance) of the wide-boundary fertilization device by EDEM software. Finally, the influential rules of core factors of the device on the CVPU were analyzed by Design-Expert software; then, the optimal parameter combination was determined and verified by a field test. The results showed that all factors had significant effects on the CVPU. The primary and secondary factors affecting the CVPU were the tilt angle, lateral span, focal length coefficient, ground clearance, tilt angle × ground clearance, and lateral span × ground clearance, in which there were certain interactions between the tilt angle and ground clearance and lateral span and ground clearance. When the focal length coefficient, lateral span, tilt angle, and ground clearance were 1.5, 60 mm, 30°, and 192 mm, respectively, the lateral was minimum. In this case, the theoretical value and field test value were 14.11% and 17.63%, respectively. The field test value is consistent with the theoretical calculation value. This study could provide references for the design of a fertilizer-spreading device with a wide boundary.

Limb-girdle muscular dystrophies (LGMD) are hereditary genetic disorders characterized by progressive muscle impairment which predominantly include proximal muscle weaknesses in the pelvic and shoulder girdles. This article describes an attempt to identify genetic cause(s) for a LGMD pedigree via a combination of whole exome sequencing and Sanger sequencing. Digenic variants, the titin gene (TTN) c.19481T>G (p.Leu6494Arg) and the trafficking protein particle complex 11 gene (TRAPPC11) c.3092C>G (p.Pro1031Arg), co-segregated with the disease phenotype in the family, suggesting their possible pathogenicity.

Heterotaxy (HTX), a condition characterized by internal organs not being arranged as expected relative to each other and to the left-right axis, is often accompanied with congenital heart disease (CHD). The purpose was to detect the pathogenic variants in a Chinese family with HTX and CHD. A non-consanguineous Han Chinese family with HTX and CHD, and 200 unrelated healthy subjects were enlisted. Exome sequencing and Sanger sequencing were applied to identify the genetic basis of the HTX family. Compound heterozygous variants, c.3426-1G>A and c.4306C>T (p.(Arg1436Trp)), in the dynein axonemal heavy chain 11 gene (DNAH11) were identified in the proband via exome sequencing and further confirmed by Sanger sequencing. Neither c.3426-1G>A nor c.4306C>T variant in the DNAH11 gene was detected in 200 healthy controls. The DNAH11 c.3426-1G>A variant was predicted as altering the acceptor splice site and most likely affecting splicing. The DNAH11 c.4306C>T variant was predicted to be damaging, which may reduce the phenotype severity. The compound heterozygous variants, c.3426-1G>A and c.4306C>T, in the DNAH11 gene might be the pathogenic alterations resulting in HTX and CHD in this family. These findings broaden the variant spectrum of the DNAH11 gene and increase knowledge used in genetic counseling for the HTX family.

Purpose. To identify the molecular etiology of a Chinese family with nonsyndromic macular dystrophy. Methods. Ophthalmic examinations were performed, and genomic DNA was extracted from available family members. Whole exome sequencing of two members (the proband and her unaffected mother) and Sanger sequencing in available family members were performed to screen potential pathogenic variants. Results. Novel compound heterozygous variants, c.1066C>T (p.Pro356Ser) and c.1102+2T>C, in the major facilitator superfamily domain containing 8 gene (MFSD8) were suspected to be involved in this family’s macular dystrophy phenotype. The novel c.1066C>T variant in the MFSD8 gene probably resulted in substitution of serine for proline at the 356th residue and was predicted to be “uncertain significance” through in silico analyses. The novel c.1102+2T>C variant in the MFSD8 gene was likely to affect the splicing form and predicted to be “pathogenic.” Conclusion. The novel compound heterozygous variants, c.1066C>T (p.Pro356Ser) and c.1102+2T>C, in the MFSD8 gene are likely responsible for the isolated macular dystrophy phenotype in this family. This study enlarged the MFSD8 gene mutant spectrum and might provide more accurate genetic counseling for this family.

Although cutting flow peanut-picking is the main peanut harvesting method, it has the problems of a large harvest loss and a high damage rate of peanut shells. The analysis of impact-friction contact characteristics during peanut fruit picking is crucial to illustrate peanut fruit picking damage. A typical peanut variety, “Dabaisha”, was considered in this study. The characteristics of peanut-picking impact-friction were studied using a peanut-picking impact-friction test bench under different conditions. An orthogonal test with three factors and levels was performed after the single-factor condition was determined. The apparent morphologies of peanut shells before and after the collision and friction tests were compared and analyzed using micro-computed tomography, white-light interferometry, and optical microscopy, whereas the impact-friction damage characteristics of peanuts under the influence of various factors were discussed. The results show that the orders of influence of the coefficient of friction of peanuts and wear loss of peanut pods were as follows: invasion depth > moisture content > contact linear velocity and moisture content > invasion depth > contact linear velocity, respectively. The experimental results and discussion in this study can provide a data reference for developing and designing peanut mechanization production equipment.

The traditional hydrophobic solarevaporator is generally obtained through the modification of alkyl or fluoroalkyl on the photothermal membrane. However, the modified groups can easily be oxidized in the long-term use process, resulting in the poor salt resistance and stability of photothermal membrane. In order to solve this problem, a simple polypyrrole/polyvinylidene fluoride membrane, consisting of an intrinsic hydrophobic support (polyvinylidene fluoride) and a photothermal material (polypyrrole), was fabricated by ultrasonically mixing and immersed precipitation. This photothermal membrane showed good self-floating ability in the process of water evaporation. In order to further improve the photothermal conversion efficiency, a micropyramid structure with antireflective ability was formed on the surface of membrane by template method. The micropyramids can enhance the absorption efficiency of incident light. The water evaporation rate reached 1.42 kg m−2 h−1 under 1 sun irradiation, and the photothermal conversion efficiency was 88.7%. The hydrophobic polyvinylidene fluoride ensures that NaCl cannot enter into membrane during the evaporation process of the brine, thus realizing the stability and salt resistance of polypyrrole/polyvinylidene fluoride in 3.5%wt and 10%wt NaCl solution.

This study presents a tangential-axial flow picking device for peanut combine harvesting. The device was designed with reference to the tangential-axial flow threshing mechanism of the grain combine harvesters. The main purpose of this study was to solve the problem of the high rate of loss and damage in the picking operation of the peanut combine harvesters in China. Through the theoretical analysis and design calculation of the key components of the peanut picking device, the structural and working parameters were determined. The orthogonal test was carried out by taking the feeding amount of the peanut plant, the picking clearance, the speed of the tangential cylinder, and the speed of the axial cylinder as the test factors. Additionally, the non-picking loss rate, entrainment loss rate, and damage rate of the peanut pods were used as the test indexes. The test results were analyzed by range analysis and analysis of variance, and the test parameters were optimized by fuzzy comprehensive evaluation method. The optimal parameter combination for the tangential-axial flow picking device was determined as follows. The feeding amount of the peanut plant was 2 kg/s, the picking clearance was 35 mm, the speed of the tangential cylinder was 360 r/min, the speed of the axial cylinder was 425 r/min. At this time, the non-picking loss rate was 0.52%, the entrainment loss rate was 0.54%, and the damage rate was 0.75%. The test results fully met the standard requirements of the peanut picking operation. This research provides a technical basis for the application of the tangential-axial flow picking device in peanut combine harvesters.

Background/Aims: Epidermolytic palmoplantar keratoderma (EPPK) is an autosomal dominant genodermatosis. It is characterized by diffuse yellow keratoses on the palmoplantar epidermis, with an erythematous border. The keratin 9 gene (KRT9) and less frequently the keratin 1 gene (KRT1) are responsible for EPPK. This study aims to identify and analyse genetic defects responsible for EPPK in a Han Chinese pedigree. Methods: A four-generation Han Chinese pedigree containing five individuals affected with EPPK was recruited. Exome sequencing, Sanger sequencing, and bioinformatics tools were conducted to identify the mutation in this pedigree. HaCaT cells were transfected with either wild-type or mutated KRT9. Confocal laser immunofluorescence assay, imaging processing, and statistical analysis were performed to evaluate wild-type and mutant KRT9 groups. Results: A novel heterozygous c.1369C>T transition (p.Leu457Phe) in exon 6 of the KRT9 gene was identified in four patients. It co-segregated with the disorder in the family. Functional analysis showed that withdrawal of the filament network from the cell periphery and particle formation were present in about 10% of Leu457Phe-transfected HaCaT cells, while approximately 3% of cells transfected with wild-type KRT9 showed this phenotype. The particles in mutant group were larger than that in wild-type group (P-value < 0.05). Conclusion: The variant may be the disease-causing missense mutation and produce dominant negative effects by interrupting keratin network formation. This study indicates the pathogenic role of the KRT9 gene mutation in this pedigree with EPPK, and may be helpful in genetic counseling, prenatal diagnosis and gene-targeted therapies of EPPK.

Due to its ability to significantly improve the wireless communication efficiency, the intelligent reflective surface (IRS) has aroused widespread research interest. However, it is a challenge to obtain perfect channel state information (CSI) for IRS-related channels due to the lack of the ability to send, receive, and process signals at IRS. Since most of the existing channel estimation methods are developed to obtain cascaded base station (BS)-IRS-user devices (UDs) channel, this paper studies the problem of computation and communication resource allocation of the IRS-assisted federated learning (FL) system based on the imperfect CSI. Specifically, we take the statistical CSI error model into consideration and formulate the training time minimization problem subject to the rate outage probability constraints. In order to solve this issue, the semi-definite relaxation (SDR) and the constrained concave convex procedure (CCCP) are invoked to transform it into a convex problem. Subsequently, a low-complexity algorithm is proposed to minimize the delay of the FL system. Numerical results show that the proposed algorithm effectively reduces the training time of the FL system base on imperfect CSI.

Reducing operation energy consumption is the development demand of conservation tillage equipment. In order to solve the problems of high power consumption and the easy blockage of the no-tillage drill under full straw retention, the key parameters of the straw back-throwing device were optimized in this study. The Box–Behnken central combination test method was used to analyze the influence of the impeller rotating speed, feed quantity and cross-sectional area of the throwing pipe on the specific power consumption and throwing speed, the mathematical models of which were built with the aid of the least square method. In addition, the mathematical models were optimized by using a multi-objective quantum behaved particle swarm optimization (QPSO) with an improved target weighting coefficient. The optimization results indicated that, when the impeller rotating speed was 2287 r/min, the feed quantity was 1.1 kg/s and the cross-sectional area of the throwing pipe was 506.997 cm2, the specific power consumption and throwing speed by the models were 7528 m2/s2 and 11.73 m/s, respectively. The models were verified by comparing the optimization results with the measured data in the simulation filed tests, which proved that the multi-objective QPSO algorithm was feasible to optimize the working and structural parameters of the straw back-throwing device of the no-tillage drill under full straw retention. The results provide references for the parameter optimization of similar no-tillage drills under straw retention.

The rapid development of peanut mechanization has increased the amount of dust expelled from peanut mechanized operations, which degrades the air quality and endangers the health of agricultural workers. Therefore, the purpose of this study is to figure out the characteristics of dust emission from mechanized peanut harvesting. To this end, the particulate matters of diameters ≤ 2.5 μm and ≤10 μm and the total suspended particles were sampled in real time during peanut harvesting in Henan Province, China, and the airborne particle concentrations and particle size distributions were measured. The dust particles discharged during the mechanized peanut harvesting were concentrated within the 2~30 µm size range. When the wind speed was reduced below the settling velocity of the largest particles, the more massive particles were carried in the downwind. The amount of free silica in the dust samples was determined by X-ray diffraction analysis. Both the total dust and free silica concentrations exceeded the occupational exposure and threshold limits. To improve the characteristics of dust emission, the microstructure and dispersion of the dust were also investigated. Reducing the agricultural operations during periods of high wind speed, low crop-moisture content, and low air humidity is recommended for reducing the dust exposure of workers. The results will provide guidance and technical support for reducing the dust emissions of mechanized harvesting operations, improving air quality, and reducing the health hazards to operators.

Abstract Background This presentation of two cases and literature review discusses the epidemiology, clinical manifestations, pathogenesis, diagnosis, treatment, and prognosis of high-grade glioma with extracranial metastases. Methods A retrospective analysis of the clinical features of two cases of malignant glioma, including metastatic sites, pathological data, and treatment methods, and a literature review was performed. Results Two patients developed extracranial metastases within 1 year after surgery for primary glioma. One patient developed cervical lymph node and bone metastases while the other developed bone metastases, and both patients died within 2 months after the diagnosis of the extracranial metastasis. Conclusion Extracranial metastases may develop from malignant gliomas. According to the literature, the most common extracranial site is intraspinal (along the neural axis), followed by the vertebrae, lungs, liver, and lymph nodes. The complex metastatic mechanism remains unclear, and the prognosis is very poor, with a survival duration of less than 6 months.

Abstract Background Ellis‐van Creveld syndrome (EVC), a very rare genetic skeletal dysplasia, is clinically characterized by a tetrad consisting of chondrodystrophy, polydactyly, ectodermal dysplasia, and cardiac anomalies. The aim of this study was to identify the genetic defect for EVC in a five‐generation consanguineous Han‐Chinese pedigree. Methods A five‐generation, 12‐member Han‐Chinese pedigree was enrolled in this study. Exome sequencing was applied in the proband to screen potential genetic variant(s), and then Sanger sequencing was used to identify the variant in family members and 200 unrelated ethnicity‐matched controls. Results A novel homozygous variant, c.2014C>T, p.(Q672*), in the EvC ciliary complex subunit 1 gene (EVC), was detected in the patient, which was cosegregated with the disease in the family and absent in the controls. Conclusion The identified novel homozygous EVC variant, c.2014C>T, p.(Q672*), was responsible for EVC in this Han‐Chinese pedigree. The findings in this study extend the EVC mutation spectrum and may provide new insights into EVC causation and diagnosis with implications for genetic counseling and clinical management.

Traditional fluorescence enhancement based on a match of the maximum excitation or emission of fluorescence molecule with the spectra of the nanostructure can hardly enhance blue and red fluorescent molecules. Here, an enhanced method which is a new strategy based on the antireflective array has been developed to enhance the emission of blue and red fluorescent molecules. The fluorescence emission is enhanced by increasing the absorption at excitation wavelengths of the fluorescent molecules and reducing the fluorescent energy dissipation with an antireflective array. By introducing the antireflective arrays, the emission enhancement of blue and red fluorescent molecules is, respectively, up to 14 and 18 fold. It is a universal and effective strategy for enhancing fluorescence emission, which could be applied to enhance the intensity of organic LED and imaging.

Background/Aims. Corneal dystrophies (CDs) belong to a group of hereditary heterogeneous corneal diseases which result in visual impairment due to the progressive accumulation of deposits in different corneal layers. So far, mutations in several genes have been responsible for various CDs. The purpose of this study is to identify gene mutations in a three-generation Hui-Chinese family associated with granular corneal dystrophy type I (GCD1). Methods. A three-generation Hui-Chinese pedigree with GCD1 was recruited for this study. Slit-lamp biomicroscopy, optical coherence tomography, and confocal microscopy were performed to determine the clinical features of available members. Whole exome sequencing was performed on two patients to screen for potential disease-causing variants in the family. Sanger sequencing was used to test the variant in the family members. Results. Clinical examinations demonstrated bilaterally abundant multiple grayish-white opacities in the basal epithelial and superficial stroma layers of corneas of the two patients. Whole exome sequencing revealed that a heterozygous missense mutation (c.1663C > T, p.Arg555Trp) in the transforming growth factor beta-induced gene (TGFBI) was shared by the two patients, and it cosegregated with this disease in the family confirmed by Sanger sequencing. Conclusions. The results suggested that the heterozygous TGFBI c.1663C > T (p.Arg555Trp) mutation was responsible for GCD1 in the Hui-Chinese family, which should be of great help in genetic counseling for this family.

Probiotics are commensal or nonpathogenic microbes that confer beneficial effects on the host through several mechanisms such as competitive exclusion, antibacterial effects, and modulation of immune responses. Some probiotics have been found to regulate immune responses via immune regulatory mechanisms. T regulatory (Treg) cells, T helper cell balances, dendritic cells, macrophages, B cells, and natural killer (NK) cells can be considered as the most determinant dysregulated mediators in immunomodulatory status. Recently, fecal microbiota transplantation (FMT) has been defined as the transfer of distal gut microbial communities from a healthy individual to a patient’s intestinal tract to cure some immune disorders (mainly inflammatory bowel diseases). The aim of this review was followed through the recent literature survey on immunomodulatory effects and mechanisms of probiotics and FMT and also efficacy and safety of probiotics and FMT in clinical trials and applications.

Based on the state of the art of big data research, a national security-oriented network big data analysis and application system was proposed, against existing problems of national security systems, such as data fragmentation, nonstandard, difficult to share, and so on.In this system, the current national security requirements and big data technologies were organic combined.It could not only play the advantages of big data technology in multi-source heterogeneous data fusion, deeply mining security information, and breaking information island, but also share the advantages of the existing business architecture and quickly producing the actual effect.Finally, a systematic introduction to the national security-oriented network big data mining and analysis technologies was given.

The poly(ethyleneimine)-functionalized silica has been developed successfully as an effective adsorbent for the adsorption removal of Cu(II) ions from electroplating wastewater. The influences of pH, contact time and initial concentration of Cu(II) ions on the adsorption capacity and the effect of adsorbent dosage on the removal efficiency of Cu(II) ions from electroplating wastewater were investigated. The adsorption behaviors and mechanisms of Cu(II) ions onto poly(ethyleneimine)-functionalized silica were also studied in details. The uptake of Cu(II) ions on poly(ethyleneimine)-functionalized silica was constant in the range of pH 4-8. The poly(ethyleneimine)-functionalized silica offered a fast rate for the adsorption of Cu(II) ions and reached an equilibrium state within 30 min. The results of static batch experiment indicated that the maximum static adsorption capacity of Cu(II) ions on PEI-functionalized silica was 31.8 mg/g. The adsorption process was found to follow a pseudo-second-order rate mechanism. Langmuir adsorption isotherms fitted well in the experimental data. The optimum dosage of poly(ethyleneimine)-functionalized silica for the removal Cu(II) ions from electroplating wastewater was 10 g/L. The removal efficiency of Cu(II) ions from electroplating wastewater was 92.6%.Some scale-up experiments were also investigated to offer the reference for water treatment. The results showed that poly(ethyleneimine)-functionalized silica could be employed as an effective adsorbent for the removal of Cu(II) ions from electroplating wastewater.

With the explosive growth of internet data and the increasing user demand, the traditional search methods has been difficult to meet user's needs in the mobile search field. In order to achieve the intelligent search based on semantic understanding, it is urgent to improve the level of search from word-based to semantic-based. Facing to the open internet data resources, an intelligent search pattern on “dynamic knowledge network + calculation operator”was proposed. Then, dynamic knowledge network was described in detail, which included theoretical basis, structure model, system model and features. Furthermore, it was presented about the basic structure of intelligent search based on dynamic knowledge network, which provided the support about theory and model for intelligent search. Finally, the main problems and challenges in the future were explained.

Colloidal photonic crystal (PC)-based anti-counterfeiting materials have been widely studied due to their inimitable structural colors and tunable photonic band gaps (PBGs) as well as their convenient identification methods. In this review, we summarize recent developments of colloidal PCs in the field of anti-counterfeiting from aspects of security strategies, design, and fabrication principles, and identification means. Firstly, an overview of the strategies for constructing PC anti-counterfeiting materials composed of variable color PC patterns, invisible PC prints, and several other PC anti-counterfeiting materials is presented. Then, the synthesis methods, working principles, security level, and specific identification means of these three types of PC materials are discussed in detail. Finally, the summary of strengths and challenges, as well as development prospects in the attractive research field, are presented.

Cracks and defects, which could result in lower reflectivity and larger full width at half maximum (FWHM), are the major obstacles for obtaining highly ordered structures of colloidal crystals (CCs). The high-quality CCs with high reflectivity (more than 90%) and 9.2 nm narrow FWHM have been successfully fabricated using a fixed proportion of a soft matter system composed of silica particles (SPs), polyethylene glycol diacrylate (PEGDA), and ethanol. The influences of refractivity difference, volume fractions, and particle dimension on FWHM were illuminated. Firstly, we clarified the influences of the planar interface and the bending interface on the self-assembly. The CCs had been successfully fabricated on the planar interface and presented unfavorable results on the bending interface. Secondly, a hard sphere system consisting of SPs, PEGDA, and ethanol was established, and the entropy-driven phase transition mechanism of a polydisperse system was expounded. The FWHM and reflectivity of CCs showed an increasing trend with increasing temperature. Consequently, high-quality CCs were obtained by adjusting temperatures (ordered structure formed at 90 °C and solidified at 0 °C) based on the surface phase rule of the system. We acquired a profound understanding of the principle and process of self-assembly, which is significant for preparation and application of CCs such as optical filters.

Saponin 15%~20% content in the seed cake of Camellia oleifera Abel, from which Camellia oil is squeezed, is a natural nonionic surface active agent and is extensively applied to emulsification, humectation, foaming, medicine, pesticide, and so on. In this paper, the extraction process of saponin was researched through a combining method of alkali solution and acid isolation. A quantitative method for saponin was established by ultraviolet spectrophotometer. The influence of extraction factors was investigated by a single-factor test and a response surface methodology. The results indicated that the optimal extraction conditions of saponin were extraction temperature 68°C, alkali solution pH 9.1, acid isolation pH 4.1, and liquid-solid ratio 15.9 : 1. The extraction rate of saponin was 76.12% at the optimal extraction conditions.

Squamous cell carcinoma of the head and neck (SCCHN) is an aggressive disease with poor survival and is the sixth most common cancer worldwide. Gastroesophageal reflux is a common event in SCCHN patients. GPR4 is a proton-sensing G-protein coupled receptor, which can be activated by acidosis. The objective of this study was to explore the role of GPR4 in acid exposure and tumor angiogenesis in SCCHN. In this study, we confirmed that overexpressing GPR4 in SCCHN cells could increase the expression and secretion of IL6, IL8 and VEGFA at pH 5.9. This effect could be inhibited by SB203580 (a p38 inhibitor). Western blot analysis indicated that phosphorylation of p38 increased in GPR4 infected cells at pH 5.9, which could be inhibited by SB203580. In tube formation assay, HMEC-1 cells were incubated with conditioned medium (CM, pH 5.9, 6.5, 7.4) derived from control and GPR4 infected SCCHN cells. Tube length was significantly increased in HMEC-1 cells incubated with CM from GPR4 infected cells compared with control cells at pH5.9, which indicated the pro-angiogenic effect of GPR4 in acidic pH. The neutralizing antibodies of IL6, IL8 and VEGFA could inhibit tube formation of HMEC-1 cells. In vivo, the effect of GPR4 on angiogenesis was investigated with the chick chorioallantoic membrane (CAM) model. Control and GPR4 infected SCCHN cells were seeded onto the upper CAM surface (n = 5 in each group) and 5 μL DMEM/F12 (pH 5.9, 6.5, 7.4) was added to the surface of the cell every 24 h. Four days later, the upper CAM were harvested and the ratio of the vascular area to the CAM area was quantified using Image-Pro Plus 6.0 software. GPR4 infected cells could recruit more vascular than control cells at pH5.9. In conclusion, we suggested that GPR4 induces angiogenesis via GPR4-induced p38-mediated IL6, IL8 and VEGFA secretion at acidic extracellular pH in SCCHN.

Camptodactyly is a digit deformity characterized by permanent flexion contracture of one or both fifth fingers at the proximal interphalangeal joints. Though over 60 distinct types of syndromic camptodactyly have been described, only one disease locus (3q11.2-q13.12) for nonsyndromic camptodactyly has been identified. To identify the genetic defect for camptodactyly in a four-generation Chinese Han family, exome and Sanger sequencings were conducted and a missense variant, c.1016C>T (p.S339L), in the talin 2 gene (TLN2) was identified. The variant co-segregated with disease in the family and was not observed in 12 unaffected family members or 1,000 normal controls, suggesting that p.S339L is a pathogenic mutation. Two asymptomatic carriers in the family indicated incomplete penetrance or more complicated compensated mechanism. Most of p.S339L carriers also have relatively benign cardiac phenotypes. Expression of wild and mutant TLN2 in HEK293 cells suggested the predominant localization in cytoplasm. Our data suggest a potential molecular link between TLN2 and camptodactyly pathogenesis.

Congenital contractural arachnodactyly (CCA, OMIM 121050), also known as Beals-Hecht syndrome, is an autosomal dominant disorder of connective tissue. CCA is characterized by arachnodactyly, dolichostenomelia, pectus deformities, kyphoscoliosis, congenital contractures and a crumpled appearance of the helix of the ear. The aim of this study is to identify the genetic cause of a 4-generation Chinese family of Tujia ethnicity with congenital contractural arachnodactyly by exome sequencing. The clinical features of patients in this family are consistent with CCA. A novel missense mutation, c.3769T>C (p.C1257R), in the fibrillin 2 gene (FBN2) was identified responsible for the genetic cause of our family with CCA. The p.C1257R mutation occurs in the 19th calcium-binding epidermal growth factor-like (cbEGF) domain. The amino acid residue cysteine in this domain is conserved among different species. Our findings suggest that exome sequencing is a powerful tool to discover mutation(s) in CCA. Our results may also provide new insights into the cause and diagnosis of CCA, and may have implications for genetic counseling and clinical management.

Spondyloepiphyseal dysplasia congenita (SEDC) is an autosomal dominant chondrodysplasia characterized by disproportionate short-trunk dwarfism, skeletal and vertebral deformities. Exome sequencing and Sanger sequencing were performed in a Chinese Han family with typical SEDC, and a novel mutation, c.620G>A (p.Gly207Glu), in the collagen type II alpha-1 gene (COL2A1) was identified. The mutation may impair protein stability, and lead to dysfunction of type II collagen. Family-based study suggested that the mutation is a de novo mutation. Our study extends the mutation spectrum of SEDC and confirms genotype-phenotype relationship between mutations at glycine in the triple helix of the alpha-1(II) chains of the COL2A1 and clinical findings of SEDC, which may be helpful in the genetic counseling of patients with SEDC.

Autosomal recessive nonsyndromic hearing loss (ARNSHL) is a genetically heterogeneous sensorineural disorder, generally manifested with prelingual hearing loss and absence of other clinical manifestations. The aim of this study is to identify the pathogenic gene in a four-generation consanguineous Chinese family with ARNSHL. A novel homozygous variant, c.9316dupC (p.H3106Pfs*2), in the myoxin XVa gene (MYO15A) was identified by exome sequencing and Sanger sequencing. The homozygous MYO15A c.9316dupC variant co-segregated with the phenotypes in the ARNSHL family and was absent in two hundred normal controls. The variant was predicted to interfere with the formation of the Myosin XVa-whirlin-Eps8 complex at the tip of stereocilia, which is indispensable for stereocilia elongation. Our data suggest that the homozygous MYO15A c.9316dupC variant might be the pathogenic mutation, and exome sequencing is a powerful molecular diagnostic strategy for ARNSHL, an extremely heterogeneous disorder. Our findings extend the mutation spectrum of the MYO15A gene and have important implications for genetic counseling for the family.

Three-dimensional photonic crystals of zirconia were prepared by electrodeposition in a colloidal crystals template following calcination at 500 °C. Scanning electron microscopy, thermogravimetric analysis, X-ray diffraction, and reflectance spectroscopy were employed to characterize the photonic crystals of zirconia. It was found that hydrated zirconium ions could penetrate the colloidal crystals template and reach the substrate easily by electrodeposition, which resulted in stronger bonding between the substrate and the as-deposited membrane. Moreover, the electrodeposited membrane had low water content, leading to a low amount of shrinkage during calcination. Both these properties could suppress detachment from the substrate upon removal of the colloidal crystals template. Therefore, the three-dimensional photonic crystals of zirconia synthesized in this study exhibited very good preservation of the ordered structures of the colloidal crystals template with a high density. A peak of reflection higher than 70% was formed in the reflectance spectrum because of the strong diffraction of the ordered structures.