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Sphingosine phosphate lyase insufficiency syndrome:a systematic review

World Journal of Pediatrics 2023年第5期19卷 425-437页

作者： Zahra Pournasiri Abbas Madani Fatemeh Nazarpack John A.Sayer Zahra Chavoshzadeh Fatemeh Nili Paulina Tran julie d.saba Mahnaz Jamee Pediatric Nephrology Research Center Research Institute for Children’s HealthShahid Beheshti University of Medical SciencesTehranIran department of Pediatric Nephrology Children’s Medical CenterPediatric Chronic Kidney Disease Research CenterTehran University of Medical SciencesTehranIran Translational and Clinical Research Institute Faculty of Medical SciencesNewcastle UniversityCentral ParkwayNewcastle upon TyneNE13BZUK Renal Services Newcastle Upon Tyne Hospitals NHS Foundation TrustNewcastle upon TyneNE77DNUK NIHR Newcastle Biomedical Research Centre Newcastle UniversityNewcastle upon TyneNE45PLTyne and WearUK Immunology and Allergy department Mofid Children’s HospitalShahid Beheshti University of Medical SciencesTehran15514-15468Iran department of Pathology Imam Khomeini Complex HospitalTehran University of Medical SciencesTehranIran Allergy Immunology division Department of PediatricsChildren’s Hospital of PhiladelphiaPhiladelphiaUSA Perelman School of Medicine at the University of Pennsylvania PhiladelphiaUSA division of Hematology/Oncology Department of PediatricsUniversity of CaliforniaSan FranciscoCAUSA

Background Sphingosine-1-phosphate lyase insufficiency syndrome(SPLIS)or nephrotic syndrome type-14 is caused by biallelic mutations in ***,we conducted a systematic review to delineate the characteristics of SPLIS *** A literature search was performed in PubMed,Web of Science,and Scopus databases,and eligible studies were *** all patients,demographic,clinical,laboratory,and molecular data were collected and *** Fifty-five SPLIS patients(54.9%male,45.1%female)were identified in 19 *** consanguinity and positive family history were reported in 70.9%and 52.7%of patients,*** patients(54.9%)primarily manifested within the first year of life,nearly half of whom survived,while all patients with a prenatal diagnosis of SPLIS(27.5%)died at a median[interquartile(IQR)]age of 2(1.4–5.3)months(P=0.003).The most prevalent clinical feature was endocrinopathies,including primary adrenal insufficiency(PAI)(71.2%)and hypothyroidism(32.7%).Kidney disorders(42,80.8%)were mainly in the form of steroid-resistant nephrotic syndrome(SRNS)and progressed to end-stage kidney disease(ESKd)in 19(36.5%)patients at a median(IQR)age of 6(1.4–42.6)*** 30 different mutations in SGPL1,the most common was c.665G>A(***222Gln)in 11(20%)***-six(49.1%)patients with available outcome were deceased at a median(IQR)age of 5(1.5–30.5)months,mostly following ESKd(23%)or sepsis/septic shock(23%).Conclusion In patients with PAI and/or SRNS,SGPL1 should be added to diagnostic genetic panels,which can provide an earlier diagnosis of SPLIS and prevention of ESKd and other life-threatening complications.

关键词： Nephrotic syndrome type 14 Immunodeficiency Lymphopenia Sphingosine-1-phosphate lyase 1 Sphingosine-1-phosphate lyase insufficiency syndrome

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ASLP-dL—A Novel Approach Employing Lightweight deep Learning Framework for Optimizing Accident Severity Level Prediction

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Computers, Materials & Continua 2024年第2期78卷 2535-2555页

作者： saba Awan Zahid Mehmood department of Software Engineering University of Engineering and TechnologyTaxila47050Pakistan department of Computer Engineering University of Engineering and TechnologyTaxila47050Pakistan

Highway safety researchers focus on crash injury severity,utilizing deep learning—specifically,deep neural networks(dNN),deep convolutional neural networks(d-CNN),and deep recurrent neural networks(d-RNN)—as the preferred method for modeling accident *** learning’s strength lies in handling intricate relation-ships within extensive datasets,making it popular for accident severity level(ASL)prediction and *** prior success,there is a need for an efficient system recognizing ASL in diverse road *** address this,we present an innovative Accident Severity Level Prediction deep Learning(ASLP-dL)framework,incorporating dNN,d-CNN,and d-RNN models fine-tuned through iterative hyperparameter selection with Stochastic Gradient *** framework optimizes hidden layers and integrates data augmentation,Gaussian noise,and dropout regularization for improved *** and factor contribution analyses identify influential *** on three diverse crash record databases—NCdB 2018–2019,UK 2015–2020,and US 2016–2021—the d-RNN model excels with an ACC score of 89.0281%,a Roc Area of 0.751,an F-estimate of 0.941,and a Kappa score of 0.0629 over the NCdB *** proposed framework consistently outperforms traditional methods,existing machine learning,and deep learning techniques.

关键词： Injury severity prediction deep learning feature

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Analysis of b→cτν¯_(τ)anomalies using weak effective Hamiltonian with complex couplings and their impact on various physical observables

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Chinese Physics C 2024年第8期48卷 56-75页

作者： Muhammad Arslan Tahira Yasmeen saba Shafaq Ishtiaq Ahmed Muhammad Jamil Aslam department of Physics Quaid-i-Azam UniversityIslamabad 45320Pakistan department of Physics International Islamic UniversityIslamabad 44000Pakistan National Center for Physics Islamabad 44000Pakistan

Recently,the experimental measurements of the branching ratios and different polarization asymmetries for processes occurring through flavor-changing-charged current(b→cτν¯_(τ))transitions by BABAR,Belle,and LHCb have revealed some significant differences from the corresponding Standard Model(SM)*** has triggered an interest to search for physics beyond the SM in the context of various new physics(NP)models and using the model-independent weak effective Hamiltonian(WEH).Assuming left-handed neutrinos,we add the dimension-six vector,(pseudo-)scalar,and tensor operators with complex Wilson coefficients(WCs)to the SM *** 60%,30%,and 10%constraints resulting from the branching ratio of B_(c)→τν¯_(τ),we reassess the parametric space of these new physics WCs accommodating the current anomalies based on the most recent HFLAV data of R_(τ/μ,e)(d)and R_(τ/μ,e)(d^(*))and Belle data of F_(L)(d^(*))and P_(τ)(d^(*)).We find that the allowed parametric region of left-handed scalar couplings strongly depends on the constraints of the B_(c)→τν¯_(τ)branching ratio,and the maximum pull from the SM predictions results from thed the analysis by adding LHCb data of R_(τ/μ)(J/ψ)and R_(τ/ℓ)(Λ_(c)).Furthermore,due to the large uncertainties in the measurements of R_(τ/μ)(J/ψ)and R_(τ/ℓ)(X_(c)),we derive the sum rules which complement them with R_(τ/μ,e)(d)and R_(τ/μ,e)(d^(*)).Using the best-fit points of the new complex WCs along with the latest measurements of R_(τ/μ,e)(d(^(*))),we predict the numerical values of the observable R_(τ/ℓ)(Λ_(c)),R_(τ/μ)(J/ψ),and R_(τ/ℓ)(X_(c))from the sum *** simultaneous dependence of abovementioned physical observables on the NP WCs is established by plotting their correlation with R_(d) and R_(d^(*)),which are useful to discriminate between various NP *** find that the most significant impact of NP results from the WC C^(S)_(L)=4C^(T).Fi

关键词： B meson anomalies new physics complex coupling HFLAV

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Analysis of b→cτ■ anomalies using weak effective Hamiltonian with complex couplings and their impact on various physical observables

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Chinese Physics C 2024年第8期 59-78页

作者： Muhammad Arslan Tahira Yasmeen saba Shafaq Ishtiaq Ahmed Muhammad Jamil Aslam department of Physics Quaid-i-Azam University department of Physics International Islamic University National Center for Physics

Recently,the experimental measurements of the branching ratios and different polarization asymmetries for processes occurring through flavor-changing-charged current（b→cτvτ）transitions by BABAR,Belle,and LHCb have revealed some significant differences from the corresponding Standard Model（SM） *** has triggered an interest to search for physics beyond the SM in the context of various new physics（NP） models and using the model-independent weak effective Hamiltonian（WEH）.Assuming left-handed neutrinos,we add the dimension-six vector,（pseudo-）scalar,and tensor operators with complex Wilson coefficients（WCs） to the SM *** 60%,3 0%,and 10% constraints resulting from the branching ratio of Bc→τvτ,we reassess the parametric space of these new physics WCs accommodating the current anomalies based on the most recent HFLAV data of Rτ/μ,e（d） and Rτ/μ,e（d*） and Belle data of FL（d*） and Pτ（d*）.We find that the allowed parametric region of lefthanded scalar couplings strongly depends on the constraints of the Bc→τvτbranching ratio,and the maximum pull from the SM predictions results from the d the analysis by adding LHCb data of Rτ/μ（J/ψ） and Rτ/l（Λc）.Furthermore,due to the large uncertainties in the measurements of Rτ/μ（J/ψ） and Rτ/l（Xc）,we derive the sum rules which complement them with Rτ/μ,e（d） and Rτ/μ,e（d*）.Using the best-fit points of the new complex WCs along with the latest measurements of Rτ/μ,e(d（*）),we predict the numerical values of the observable Rτ/l（Λc）,Rτ/μ（J/ψ）,and Rτ/l（Xc） from the sum *** simultaneous dependence of abovementioned physical observables on the NP WCs is established by plotting their correlation with Rdand Rd*,which are useful to discriminate between various NP *** find that the most significant impact of NP results from the WC CLS=***,we study the impact of these NP couplings on various angular and CP triple product asym

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Effects of improved amino acid balance diet on lysine mammary utilization, whole body protein turnover and muscle protein breakdown on lactating sows

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Journal of Animal Science and Biotechnology 2024年第5期15卷 2031-2043页

作者： Sai Zhang Juan C.Marini Vengai Mavangira Andrew Claude julie Moore Mahmoud A.Mohammad Nathalie L.Trottier department of Animal Science Michigan State UniversityEast Lansing 48824USA State Key Laboratory of Swine and Poultry Breeding Industry Key Laboratory of Animal Nutrition and Feed Science in South ChinaMinistry of Agriculture and Rural AffairsGuangdong Provincial Key Laboratory of Animal Breeding and NutritionInstitute of Animal ScienceGuangdong Academy of Agricultural SciencesGuangzhou 510640PR China USdA/ARS Children’s Nutrition Research Center and Pediatric Critical Care Medicine Department of PediatricsBaylor College of MedicineHoustonTX 77030USA department of Large Animal Clinical Sciences Michigan State UniversityEast LansingMI 48824USA Present address:department of Animal Science Cornell UniversityFrank Morrison Hall507 Tower RoadIthacaNY 14853‑4801USA

Background The study objective was to test the hypothesis that low crude protein(CP)diet with crystalline amino acids(CAA)supplementation improves Lys utilization efficiency for milk production and reduces protein turnover and muscle protein *** lactating multiparous Yorkshire sows were allotted to 1 of 2 isocaloric diets(10.80 MJ/kg net energy):control(CON;19.24%CP)and reduced CP with“optimal”AA profile(OPT;14.00%CP).Sow body weight and backfat were recorded on d 1 and 21 of lactation and piglets were weighed on d 1,14,18,and 21 of *** d 14 and 18,a subset of 9 sows(CON=4,OPT=5)was infused with a mixed solution of 3-[methyl-2H3]histidine(bolus injection)and[13C]bicarbonate(priming dose)first,then a constant 2-h[13C]bicarbonate infusion followed by a 6-h primed constant[1-13C]lysine *** blood and milk sampling were performed to determine plasma and milk Lys enrichment,Lys oxidation rate,whole body protein turnover,and muscle protein *** Over the 21-d lactation period,compared to CON,sows fed OPT had greater litter growth rate(Pd to CON,sows fed OPT had greater efficiency of Lys(Pd whole-body protein turnover efficiency(Pd to CON,sows fed OPT tended to have lower whole body protein breakdown rate(P=0.069).Muscle protein breakdown rate did not differ between OPT and CON(P=0.197).Conclusion Feeding an improved AA balance diet increased efficiency of Lys and reduced whole-body protein turnover and protein *** results imply that the lower maternal N retention observed in lactating sows fed improved AA balance diets in previous studies may be a result of greater partitioning of AA towards milk rather than greater body protein breakdown.

关键词： Amino acid Efficiency Lactating sows Protein breakdown Protein turnover Reduced protein diet

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Assessment of the Recent Hydromorphological Features of Nokoue Lake and Its Channels (South-East Benin)

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Open Journal of Modern Hydrology 2024年第1期14卷 33-55页

作者： Tètchodiwèï julie-Billard Yonouwinhi Gérard Alfred Franck Assiom d’Almeida Felix Kofi Abagale Akuemaho Virgile Onésime Akowanou Laboratory of Applied Hydrology (LHA) National Institute of water (INE) African Centre of Excellence for Water and Sanitation (C2EA) University of Abomey-Calavi (UAC) Cotonou Benin International Chair in Mathematical Physics and Applications (ICMPA–UNESCO Chair) University of Abomey-Calavi (UAC) Cotonou Benin Laboratory of Geology Mining and Environment (LGME) Faculty of Science and Technology (FAST) University of Abomey-Calavi (UAC) Cotonou Benin West African Centre for Water Irrigation and Sustainable Agriculture (WACWISA) University of Development Studies (UDS) Tamale Ghana Laboratory of Sciences and Technology for Water and Environment (LSTEE) University of Abomey-Calavi (UAC) Cotonou Benin

Based on 2022 and 2023 hydrometric data and satellite images (Sentinel 2022, SPOT 2010), this study aims to present the Nokoué Lake and its channels’ re-cent hydromorphological characteristics. Integrating flow, tributary morphology, and topography data determined specific power values along the axes studied. The values obtained range from 2.69 to 12.92 W/m2 for Ouémé River and 2.46 to 10.99 W/m2 for Sô River. The resulting water erosion on banks and bottoms is of linear, areolar, or gully and claw types. Lake bathymetry varies from -0.5 to -2.6 m (low flow period) and -1 to -4 m;in the Ouémé, Sô, and Totchè rivers, it varies from -5 m to -7 m, reaching -10 m at the Cotonou channel entrance (flood period). Bathymetric profiles reveal varied “U”, “V” and “Intermediate” bottom morphologies, influenced by erosion/sedimentation processes and human activities. The flow facies identified are lentic in the northern tributaries and lotic in the Cotonou and Totchè canals. Spatial analysis identified nine (09) thematic classes. In 2022, the surface area of the water body has increased from 274 km2 at low water to 280 km2 at high water, whereas in 2010 (a recent year of exceptional flooding), the surface area was 270 km2 at low water and 277 km2 at high water. Significant changes in land use are observed between 2010 and 2022. The floodplain area decreased slightly, from 421 km2 in 2010 (year of exceptional flooding) to 419 km2 in 2022. The evolution of land use shows a progressive expansion of the urban environment to the detriment of the natural environment. In the medium to long term, this trend could threaten the hydromorphological balance and even the existence of this important lagoon ecosystem.

关键词： Nokoué Lake Ouémé River Sô River Cotonou Channel Hydromorphology

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Risk factors and outcomes of peptic ulcer bleed in a Pakistani population:A single-center observational study

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World Journal of Gastrointestinal Pharmacology and Therapeutics 2024年第3期15卷 1-9页

作者： Nazish Butt Muhammad Tayyab Usmani Nimrah Mehak saba Mughal Fakhar Ali Qazi-Arisar Ghulam Mohiuddin Gulzar Khan department of Gastroenterology Jinnah Postgraduate Medical CentreKarachi 75505SindhPakistan National Institute of Liver&GI diseases Dow University of Health SciencesKarachi 75330SindhPakistan School of Public Health Dow University of Health SciencesKarachi 75330SindhPakistan

BACKGROUNd Peptic ulcer disease(PUd)remains a significant healthcare burden,contributing to morbidity and mortality *** advancements in therapies,its prevalence persists,particularly in regions with widespread nonsteroidal antiinflammatory drugs(NSAIds)use and Helicobacter pylori *** To comprehensively analyse the risk factors and outcomes of PUd-related upper gastrointestinal(GI)bleeding in Pakistani *** This retrospective cohort study included 142 patients with peptic ulcer bleeding who underwent upper GI endoscopy from January to december *** on demographics,symptoms,length of stay,mortality,re-bleed,and Forrest classification was *** The mean age of patients was 53 years,and the majority was men(68.3%).Hematemesis(82.4%)and epigastric pain(75.4%)were the most common presenting *** patients(73.2%)were discharged within five *** mortality rates at one week and one month were 10.6%and 14.8%,***-bleed within 24 h and seven days occurred in 14.1%and 18.3%of patients,*** ulcers were Forrest class(FC)Ⅲ(72.5%).Antiplatelet use was associated with higher mortality at 7 and 30 d,while alternative medications were linked to higher 24-hour re-bleed *** use was associated with more FCⅢ***-bleed at 24 h and 7 d was strongly associated with one-week or one-month *** Antiplatelet use and rebleeding increase the risk of early mortality in PUd-related upper GI bleeding,while alternative medicines are associated with early rebleeding.

关键词： Non variceal bleed Mortality Re-bleed Forrest classification Antiplatelets Alternative medicines

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Enhancing pharmaceutical potential and oral bioavailability of Allium cepa nanosuspension in male albino rats using response surface methodology

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Asian Pacific Journal of Tropical Biomedicine 2022年第1期12卷 26-38页

作者： Fatiqa Zafar Nazish Jahan Shaukat Ali saba Jamil Riaz Hussain saba Aslam department of Chemistry University of SahiwalSahiwalPakistan department of Chemistry University of AgricultureFaisalabadPakistan department of Chemistry University of OkaraOkaraPakistan Govt.Graduate College for Women Sargodha RoadFaisalabadPakistan

Objective:To enhance the pharmaceutical potential and oral bioavailability of quercetin contents of Allium cepa peel extract by novel nanosuspension ***:Nanoprecipitation approach was successfully used for the formulation of *** obtain pharmaceutical-grade nanosuspension with minimum particle size and polydispersity index,sodium lauryl sulphate was selected as a *** formulation parameters were statistically optimized by the response surface methodology *** optimized nanosuspension was subjected to stability and in vitro dissolution testing and characterized by scanning electron microscopy,atomic force microscopy,Fourier transform infrared spectroscopy,and zeta *** evaluate the preeminence of nanosuspension over coarse suspension,comparative bioavailability studies were carried out in male albino *** pharmaceutical potential of developed nanosuspension was evaluated by antioxidant,antimicrobial,and toxicity ***:The optimized nanosuspension showed an average particle size of 275.5 nm with a polydispersity index and zeta potential value of 0.415 and−48.8 mV,*** force microscopy revealed that the average particle size of nanosuspension was below 100 *** formulated nanosuspension showed better stability under refrigerated *** showed an improved dissolution rate and a 2.14-fold greater plasma concentration of quercetin than coarse ***,the formulated nanosuspension exhibited enhanced antioxidant and antimicrobial potential and was ***:Optimization of nanosuspension effectively improves the pharmaceutical potential and oral bioavailability of Allium cepa extract.

关键词： Allium cepa Nanoprecipitation Pharmaceutical potential Particle size Polydispersity index Stability Toxicity Quercetin

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Assessment of Cu(Ⅱ) removal from an aqueous solution by raw Gundelia tournefortii as a new low-cost biosorbent: Experiments and modelling

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Chinese Journal of Chemical Engineering 2019年第8期27卷 1945-1955页

作者： saba Golshan Shandi Faramarz doulati Ardejani Fereydoun Sharifi School of Mining College of EngineeringUniversity of TehranTehranIran Faculty of Mining Petroleum and GeophysicsShahrood University of TechnologyShahroodIran

Lignocellulosic materials can be used as biosorbent for refinement of the wastewaters when they are available in large quantities. Many studies were conducted to uptake Cu(Ⅱ) ion from aqueous *** this paper, the biosorption efficiency of Cu(Ⅱ) ions from a synthetic aqueous solution was investigated using Gundelia tournefortii(GT), without any pre-treatment. Fourier transform infrared spectroscopy,scanning electron microscopy and determining the point of zero charge were employed to characterise the biosorbent. Batch experiments were performed to study the influence of p H, biosorbent dosage, contact time, temperature and initial Cu(Ⅱ) concentration on Cu(Ⅱ) removal. The biosorption isotherms were investigated using the Langmuir, Freundlich, Temkin and d-R isotherm models. The findings show that the biosorption isotherm was better fitted by the Langmuir equation and the maximum adsorption capacity of GT was found to be 38.7597 mg·g^-1. The kinetics data were analysed by pseudo-first order,pseudo-second order, and intra-particle diffusion equations. The results indicate that the pseudosecond-order model was found to explain the adsorption kinetics most effectively. The values of thermodynamic parameters including Gibbs free energy(△G°), enthalpy(△H°), and entropy(△S°) demonstrate that the biosorption process was exothermic and spontaneous. The multiple nonlinear regression(MnLR)and artificial neural network(ANN) analyses were applied for the prediction of biosorption capacity. A relationship between the predicted and observed data was obtained and the results show that the Mn LR and ANN models provided successful predictions.

关键词： Adsorption Batch experiment Kinetics Isotherms Thermodynamic Multiple nonlinear regression Artificial neural network

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Management of inflammatory bowel disease with Clostridium difficile infection

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World Journal of Gastroenterology 2017年第27期23卷 4986-5003页

作者： julie d’Aoust Robert Battat Talat Bessissow division of Internal Medicine Jewish General Hospital division of Gastroenterology Jewish General Hospital division of Gastroenterology Mc Gill University Health Centre

To address the management of Clostridium difficile (C. difficile) infection (CdI) in the setting of suspected inflammatory bowel disease (IBd)-flare. METHOdSA systematic search of the Ovid MEdLINE and EMBASE databases by independent reviewers identified 70 articles including a total of 932141 IBd patients or IBd-related hospitalizations. RESULTSIn those with IBd, CdI is associated with increased morbidity, including subsequent escalation in IBd medical therapy, urgent colectomy and increased hospitalization, as well as excess mortality. Vancomycin-containing regimens are effective first-line therapies for CdI in IBd inpatients. No prospective data exists with regards to the safety or efficacy of initiating or maintaining corticosteroid, immunomodulator, or biologic therapy to treat IBd in the setting of CdI. Corticosteroid use is a risk factor for the development of CdI, while immunomodulators and biologics are not. CONCLUSIONStrong recommendations regarding when to initiate IBd specific therapy in those with CdI are precluded by a lack of evidence. However, based on expert opinion and observational data, initiation or resumption of immunosuppressive therapy after 48-72 h of targeted antibiotic treatment for CdI may be considered.

关键词： Biologic therapy Clostridium difficile Inflammatory bowel disease Ulcerative colitis Crohn’s disease Corticosteroids

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