Auriculocondylar syndrome (ARCND) is a genetic and rare craniofacial condition caused by abnormal development of the first and second pharyngeal arches during the embryonic stage and is characterized by peculiar auricular malformations (question mark ears), mandibular condyle hypoplasia, micrognathia and other less-frequent features. GNAI3, PLCB4 and EDN1 have been identified as pathogenic genes in this syndrome so far, all of which are implicated in the EDN1-EDNRA signal pathway. Therefore, ARCND is genetically classified as ARCND1, ARCND2 and ARCND3 based on the mutations in GNAI3, PLCB4 and EDN1, respectively. ARCND is inherited in an autosomal dominant or recessive mode with significant intra- and interfamilial phenotypic variation and incomplete penetrance, rendering its diagnosis difficult and therapies individualized. To raise clinicians’ awareness of the rare syndrome, we focused on the currently known pathogenesis, pathogenic genes, clinical manifestations and surgical therapies in this review.

Cooling tower is an important part of circulating cooling water system. This paper studies the counter-flow wet seawater shower cooling tower, establishes and verifies the detailed thermal performance calculation model. The influences of salinity, inlet velocity and droplet diameter on cooling efficiency, heat dissipation, gas-phase thermal efficiency and exergy efficiency were analyzed. The results showed that the cooling efficiency, exergy efficiency, gas-phase thermal efficiency and heat dissipation of the upper jet column were 15.59%, 4.89%, 8.62% and 34.58% higher than those of the lower jet column, respectively. With the increase of droplet diameter, decreased by 38.76%, Q decreased by 78.11%, decreased by 26.30% and decreased by 33.53% under standard salinity conditions. Under high salinity conditions, is reduced by 35.1%, is reduced by 17.16% and is reduced by 30.55%.With the increase of air velocity, increased by 13.76%, Q increased by 22.44%, decreased by 6.66%and decreased by less than 2% under the standard salt condition.Under high salinity conditions, compared with the standard salinity, the average reduction of is 2.96%, the average reduction of is 1.63% and the average reduction of is 2.73%. Through the optimization analysis of seawater cooling tower, it is obvious that selecting the best operating conditions can improve the heat and moisture exchange characteristics of seawater cooling tower.

The deployment of scientific and reasonable cultivated land quality (CLQ) monitoring points can provide timely and accurate information on the current situation and changes in CLQ, which is highly important to protect national food security. The conventional methods of selecting CLQ monitoring points are based on the CLQ of land use patches. As there may be different grades of large patches, being selected as monitoring points reduces the reliability of monitoring CLQ. Moreover, the conventional monitoring point deployment method mainly considers only CLQ and ignores road accessibility and terrain as factors, resulting in the inaccessibility of some monitoring points. Therefore, to improve the reliability of CLQ monitoring, this study presented a novel approach for deploying CLQ monitoring points. First, the pixel-scale CLQ was estimated using the genetic algorithm-back propagation neural network (GA-BPNN) model based on the Landstat8 data with 30 m spatial resolution. Second, the stratified sampling model was used to determine the optimal sample points. Finally, the improved spatial simulated annealing algorithm (ISSA), considering both slope and road accessibility, was applied to optimize the location of monitoring points. This study was conducted in the Conghua District of Guangzhou, Guangdong Province, China. The results highlighted that (1) compared to the accuracy of measured CLQ, the accuracy (R 2  =  0.63, RMSE = 79.32, and NRMSE = 13.77%) of CLQ estimated using the remote sensing technique was reliable, and the pixel-scale CLQ data was more reasonable than the patch-scale CLQ data with different grades. (2) A total of 132 monitoring points were finally identified in the study area based on the stratified sampling model. (3) When compared with those of the spatial simulated annealing algorithm (SSA) and the standard grid method, the approach proposed in this study had a higher total score (F = 94.61). Moreover, the obtained sample points were mainly located near roads and flat terrain. This can effectively avoid the inaccessible places. Thus, the results based on the novel approach proposed in this study provide a scientific basis and technical support for obtaining the optimal CLQ monitoring points.

Mucormycosis was an acute and invasive fungal infection with a high mortality rate. The treatment of mucormycosis was challenging. And the incidence of the mucormycosis seems to be increasing. The key to Mucormycosis therapy not only depend on anti-Mucor infection, but also included the management of some risk factors. Liposomal amphotericin B (L-AMB) was the first line drug to treat mucormycosis. The dosage regimen recommended by the guideline was often associated with higher nephrotoxicity and morbidity. A pharmacokinetic/pharmacodynamic (PKPD) model was conducted to evaluate suitable dosage regimens in Mucormycosis patients. 10mg/kg/day LAMB recommended in the guidelines might not be needed. 5mg/kg/day LAMB might be sufficient to achieve the target value of PKPD and indicated a good anti-Mucor effect. Successful management of mucormycosis was also based on suitable management of several risk factors which played a very important role in the progression of mucormycosis, such as iron factor, diabetes mellitus with acidosis and thrombosis. Application of deferasirox, statins and keep platelet level might be promising approaches in mucormycosis therapy.

Key points: (no abstract required) • Cochlear implant effectiveness in asymmetric hearing loss and single-sided deafness is well established, but rarely covered by health care systems. • Continuation of wearing the implant beyond the first year could be considered as an indirect indicator of subjective benefit. • Percentage of cochlear implant abandon at 5-years post-implantation was very low in patients with asymmetric hearing loss and single-sided deafness. • High education level, low audibility and good unaided speech perceptions scores of the contralateral acoustic-hearing ear were positive prognostic factors of CI use. • The low percentage of non-users is an additional strong argument to recommend cochlear implantation in patients with asymmetric hearing loss and single-sided deafness, on the ear with a profound hearing loss, especially in case of frail contralateral acoustic-hearing ear.

The PHO workforce landscape has been evolving over the past decades, with concern for waning interest in the subspecialty. In this study, we aimed to evaluate perceptions of the PHO workforce (both fellows and faculty), the experience of seeking an initial faculty position, and its impact on fellow stress and anxiety. An anonymous survey consisting of demographics, job search experiences including barriers and helpful tools, and emotional health impacts such as stress and anxiety was developed and distributed to all PHO fellowship programs. Seventy-nine fellows and 83 faculty responded. Faculty were less likely than fellows to perceive that fellows are struggling to find jobs ( P=0.0198). However, faculty were more likely than fellows to perceive that fellows are either “extremely stressed” or “stressed” due to job search ( P=0.0003). By June 2021, 30 of 44 (68%) candidates had been offered a position, and 80% of those had accepted. There were no significant associations between fellow stress level and job offering, proximity to their ideal goals, or change in career type. Common barriers to the job search included geographic constraints and partner employment. Respondents identified centralized job listings, formal training on career development strategies, introduction to various career paths, and more transparency about the job search as needed interventions. The perception of difficulty and stress regarding the job search is endorsed by most, identifying a need for more interventions to improve the job search experience. These data also highlight unmet mentorship and educational needs among PHO fellows in preparing for the job search.

Background Acute Lymphoblastic Leukemia (ALL) represents around 70% of pediatric leukemia. In high-income countries, 5-year survival is above 90% but survival in low-and middle-income countries is inferior. Objective: To document the treatment outcome and prognostic factors of Pediatric ALL in Pakistan. Materials and methods: in this descriptive study, all newly diagnosed cases of ALL/LBL from one to 16 years of age enrolled between 1 st January 2012 to 31 st December 2021 were included. The treatment was based on the standard arm of the UKALL2011 protocol. Results: Data of 945 cases of ALL, including 597 males (63.2%) was analyzed. The mean age at diagnosis was 5.73 ± 3.51 years. Pallor was the commonest presentation in 95.2% followed by fever in 84.2% cases. The mean white blood cells count was 56.6 ± 103.4 x10 9/L. Neutropenic fever followed by myopathy was the most common complication during induction. In univariate analysis, high white blood cells count (P=<0.001), intensive chemotherapy (P=<0.001), malnutrition (P=0.007), poor response to induction chemotherapy (P=0.001), delay presentation (P= 0.004), use of steroids before chemotherapy (P=0.023) significantly adversely affected overall survival (OS). Delayed presentation was the most significant prognostic factor in the multivariate analysis (P = <.002). After a median follow-up of 54.64 ± 33.80 months, the 5-year OS and DFS was 69.9% and 67.8%, respectively. Conclusion. In this largest cohort of childhood ALL from Pakistan, a high WBC count, malnutrition, delayed presentation, prior steroids use, intensive chemotherapy and poor response to the induction chemotherapy were associated with decreased OS and DFS rates.

In a new pacemaker implantation the surgical absorbable suture material usually doesn't cause problem to patient. We report a new pacemaker implant case complicated by a granulomatous reaction to non absorbable suture. The literature doesn't describe cases of reaction to suture material in the setting of pacemaker implantation.

Metabolic reprogramming has been coined as a hallmark of cancer, accompanied by which the alterations in metabolite levels have profound effects on gene expression, cellular differentiation and the tumor environment. Yet a systematic evaluation of quenching and extraction procedures for quantitative metabolome profiling of tumor cells is currently lacking. To achieve this, this study is aimed at establishing an unbiased and leakage-free metabolome preparation protocol for Hela carcinoma cell. We evaluated 12 combinations of quenching and extraction methods from three quenchers (liquid nitrogen, -40°C 50% methanol, 0.5°C normal saline) and four extractants (80% methanol, methanol: chloroform: water (1:1:1, v/v/v), 50% acetonitrile, 75°C 70% ethanol) for global metabolite profiling of adherent Hela carcinoma cells. Based on the isotope dilution mass spectrometry (IDMS) method, gas/liquid chromatography in tandem with mass spectrometry was used to quantitatively determine 43 metabolites including sugar phosphates, organic acids, amino acids, adenosine nucleotides and coenzymes involved in central carbon metabolism. Among 12 combinations, cells that washed twice with phosphate buffered saline (PBS), quenched with liquid nitrogen, and then extracted with 50% acetonitrile was found to be the most optimal method to acquire intracellular metabolites with minimal loss during sample preparation. Furthermore, a case study was carried out to evaluate the effect of doxorubicin (DOX) on both adherent cells and 3D tumor spheroids using quantitative metabolite profiling. Based on this, quantitative time-resolved metabolite data can serve to the generation of hypotheses on metabolic reprogramming to reveal its important role in tumor development and treatment.

Lake has an essential role in Earth’s hydrologic cycle. The changing of lake water areas shows the ecological health of lakes by instant feedback. Remote sensing monitoring reflects the changes in lake water intuitively. This study, based on remote sensing, combined with the hydrological data, meteorological data, and land use survey, investigated the Poyang lake water area changes from 1977 to 2021. Examined the relationship between the lake water area and the impact factor and analyzed the influence of river lake interaction on the lake water area. The results showed that: (1) From 1977 to 2017, the water area of Poyang Lake in flood season (June to September) and dry season (November to Next February) showed a significant downward trend by years. (2) In flood season, runoff, sediment, and precipitation significantly correlate with a water area. In the dry season, sediment significantly correlated with a water area. (3) The Three Gorges Dam water impoundment at the upper reaches of the Yangtze River weakened and eliminated the block and reverse flow of the Yangtze River on Poyang lake, causing the water area to decrease in advance at the end of the flood season after 2005, and decrease rapidly in the dry season after 2003. (4) The change in lakebed elevation caused by sedimentation and human activities also accelerated the shrinkage of the water area. Results demonstrated that the variation of river lake interaction is the primary cause of the Poyang Lake water area’s dramatic change.

Pakistan is primarily dependent on water supplies from the Upper Indus basin for irrigated agriculture. Any changes in available resources due to climate, consequently has the potential to have a significant impact on the environment. Coupled model inter-comparison project phase 6 (CMIP6) based global climate models (GCMs) under shared socioeconomic pathways (SSP245) scenario were assessed to evaluate the study area for climate change effect on river runoff using Soil and water assessment tool (SWAT). Temperature fluctuations have a significant effect on stream flow, since the primary sources of river runoff in the Upper Regions of Indus Basin (URIB) are snow and glacier melting. The temperature (min & max) will likely increase by almost 18% in the future, the projected precipitation pattern will increase by 13-17 %, and the stream flow will increase by 19-30 % in the future due to the warmer temperature. Temperature (min & max), precipitation and stream flow have had different effects in each season, while their variability in the projected annual changes are increasing for mid and late 21 st century. Hydroelectricity generation, irrigation, flood prevention, and storage reservoir will be required in the strategies and action plans for the effective water resources management.

Objective To assess the safety and efficacy of novel pulsed field ablation (PFA) as a treatment option for atrial fibrillation. Methods and results PubMed, Ovid, Google Scholar, Web of Science, and Scopus databases and various major scientific conferences were searched for studies reporting outcomes about pulsed-field ablation technique. Sixteen studies were included reporting 485 with atrial fibrillation who underwent pulsed field operation. Most of the patients had an average age of 60 years. Their total procedure time was 94 min. Their mean time of Fluoro procedure is 17 min. The overall proportion of successful pulmonary vein isolation is 100% with 95% CI (p =1.00). The overall recurrence rate of arrhythmia in the participants is 0.0284 with 95%-CI (p =0.79). During or after the PFA surgery, the overall proportion of complications was 0.0223 (p <0.01) with 95%-CI indicating high safety of the PFA procedure. Conclusion The novel technique of pulsed-field ablation has shown very safe outcomes and high efficacy as a novel treatment for patients with atrial fibrillation

Introduction: His bundle pacing (HBP) was developed as a physiological conduction system pacing to complement the problem of conventional right ventricular pacing (RVP) related to dyssynchrony. Recently, left bundle branch area pacing (LBBAP), which overcomes the shortcomings of HBP, has been implemented. Most researches on initial experiences with LBBAP have reported that it was achieved through a lumen-less pacing lead (LLL) with a fixed helix design; however, there are situations in which LLL cannot be used. The purpose of present research is to evaluate the initial experience and learning curve of LBBAP using a standard stylet-driven lead with an extendable helix design. Methods: 265 patients who underwent LBBAP or conventional RVP performed by operators without previous LBBAP experience at Yonsei University Severance Hospital in Korea between December 2020 and October 2021 were enrolled. LBBAP was performed using a stylet‐driven pacing lead with an extendable helix. The learning curve was evaluated by analyzing fluoroscopy and procedure times. Results: LBBAP was successful in 65 of 69 (94.2%) patients during the observation period. In 65 patients who underwent LBBAP, mean fluoroscopy and procedural times were 17.1 ± 17.2 minutes and 64.2 ± 33.5 minutes, respectively. The learning curve for achieving LBBAP plateaued after the 24th case, with a gradually shortened in procedure time. Conclusion: During the initial experience with LBBAP, fluoroscopy and procedural times improved with increasing operator experience. For operators who were experienced in cardiac pacemaker implantation, the steepest part of the learning curve was over the first 20-25 cases.

Carbon dioxide (CO2) capture and storage (CCS) processes could benefit from thermostable carbonic anhydrases (CAs), which can significantly increase the rate of CO2 absorption into solvents. Since conventional CO2 absorption techniques are usually performed under high-temperature conditions in industrial plants, implementing highly thermostable CAs is very important to introduce a robust and economical process. The present study employed a combination of in silico tools to rationally engineer a thermostable CA (SspCA) originating from a thermostable bacterium Sulfurihydrogenibium yellowstonense. Based on the results, while the general folding of the enzyme and its catalytic efficiency was retained in the K100G mutant, the melting temperature increased by 3°C, and the CO2 hydration activity half-life at 85°C increased by two times. Molecular dynamics (MD) simulations were performed to determine the underlying causes of the thermostability improvement. The K100G mutation was shown to cause a reduction in protein local flexibility mainly by confining the flexible parts due to rearrangement of salt bridges and hydrogen interactions network. In sum, while strengthening the importance of rational design in thermostable enzymes, the present study showed the capability of merely one rationally-designed mutation which indirectly resulted in CA thermostability improvement by exerting local structural rigidification in its neighboring part.

The umbral operational techniques offer sturdy mechanism in the studies of special functions and special polynomials. The techniques of the umbral calculus are employed to derive the properties of families of exponential like functions and their hyperbolic forms. The generalized forms of Mittag-Leffler functions are used to solve the technical problems concerning the transport of a charged beam in a solenoid magnet. The proposed method is flexible and has many advantages over the standard computational techniques.

Background: The present study aimed to explore the relationship between serum anion gap (AG) and long-term mortality in patients undergoing coronary artery bypass grafting (CABG). Methods: Clinical variables were extracted among patients undergoing CABG from Medical Information Mart for Intensive Care III (MIMIC III) database. The primary outcome was four-year mortality following CABG. An optimal cut-off value of AG was determined by receiver operating characteristic (ROC) curve. The Kaplan-Meier (K-M) analysis and multivariate Cox hazard analysis were performed to investigate the prognostic value of AG in long-term mortality after CABG. In order to eliminate the bias between different groups, propensity score matching (PSM) was conducted to validate the findings. Results: The optimal cut-off value of AG was 17.00 mmol/L. Then a total of 3,162 eligible patients enrolled in this study were divided into a high AG group (≥17.00, n=1,022) and a low AG group (<17.00, n=2,140). A lower survival rate was identified in the high AG group based on K-M curve (p<0.001). Compared with patients in the low AG group, patients in the high AG group had an increased risk of long-term mortality [One-year: HR 2.309, 95% CI (1.672-3.187), P<0.001; two-year: HR 1.813, 95% CI (1.401-2.346), P<0.001; three-year: HR 1.667, 95% CI (1.341-2.097), P<0.001; four-year: HR 1.710, 95% CI (1.401-2.087), P<0.001] according to multivariate Cox hazard analysis. And further validation of above results were consistent in the matched cohort after PSM. Conclusions: The AG is an independent predictive factor for long-term all-cause mortality in patients following CABG, where a high AG value is associated with an increased mortality.

A new fractional difference equation 2D-TFCDM based on Caputo derivative is proposed. Using the bifurcation diagram, the maximum Lyapunov exponent and the phase diagram, the numerical solutions of the fractional difference equations are obtained, and the chaotic behavior is observed numerically. After encrypting the key with elliptic curve cryptosystem, the fractional map is developed as an encryption algorithm and applied to color image encryption. Finally, the proposed encryption system is systematically analyzed from five main aspects, and the results show that the proposed encryption system has a good encryption effect. In respective of application, we apply the proposed discrete fractional map into color image encryption with the secret keys ciphered by Menezes-Vanstone Elliptic Curve Cryptosystem (MVECC). Finally, the image encryption algorithm is analysed in 4 aspects that indicates the proposed algorithm is superior to others.

In this paper, for the bounded solution of the non-densely defined non-autonomous evolution equation, we present the conditions for asymptotic periodicity by using the spectral theory of functions on the half line and the extrapolation theory of nondensely defined evolution equation.

The principal purpose of this work is to ‎present ‎a‎ numerical technique for the fractional vibration equation of large ‎membranes.‎ This method uses the Chebyshev cardinal functions ‎and ‎the required approximate solution as the elements of Chebyshev cardinal functions. Using the operational matrix ‎o‎f derivative, the time fractional vibration ‎equation‎ is reduced to a set of algebraic equations. ‎Mea‎nwhile‎, an estimation of the error bound for this algorithm ‎is ‎given‎ on the basis ‎of‎ some ‎theorems. ‎‎Two‎ numerical examples are included ‎by ‎taking‎ different initial conditions ‎to demonstrate the efficiency and applicability of ‎this ‎approach‎. ‎‎T‎o examine ‎the ‎‎accuracy ‎of the ‎suggested ‎method, ‎the ‎n‎umerical results are compared with the existing analytical ‎methods.‎

Specific background information on cancer patients affected more severely by crisis and disasters is lacking. We experienced an advanced breast cancer patient whose hospital visits were interrupted for two years due to multiple internal and external factors. These factors need to be taken more into account for breast cancer management.

Data on two deceased individuals with COVID-19 and comorbidities such as hepatitis C, chronic kidney disease, diabetes mellitus type 2 and hypertension are discussed. Changes in laboratory signatures with impact on COVID-19 severity in both cases indicate the need for extensive monitoring of comorbid individuals to reduce morbidity and mortality.

The emergent BPA, effective for even a central-type CTEPH, can lead the patient under fatal conditions to pulmonary endarterectomy.

RA-UIP has a poor prognosis, and how to reverse or stabilize the lesions remains challenging. Based on researches finding that tofacitinib and IGU might be effective in pulmonary fibrosis, we present 3 cases that treated with the combined strategy with significant improvement of DAS28-CRP score and reversals in HRCT.

This paper analyzes the convergence rate of sample matrix inversion (SMI) filter used by array receivers in pulse interference environment. Firstly, the convergence characteristics of SMI filter under pulse interference are deduced, and the relationship between the convergence speed and length of training samples and duty of interference is given. Then, the results of signal simulation are consistent with those of numerical analysis, which verifies the correctness of theoretical analysis. The results show that the convergence speed of SMI filter decreases under pulse interference, and the SMI filter needs more training samples to suppress the pulse interference effectively.