检索结果-南通市图书馆

Genetics of congenital and infantile nephrotic syndrome

World Journal of Pediatrics 2019年第2期15卷 198-203页

作者： sara Nawfal sharief Nada Abdullatif Hefni Walaa Ali Alzahrani Iman Ibrahim Nazer Marwa Abdullah Bayazeed Khalid A.Alhasan Osama Y.Safdar Sherif M.El-Desoky Jameela Abdulaziz Kari King Abdulaziz University JeddahKingdom of Saudi Arabia College of Medicine Pediatric DepartmentKing Saud UniversityRiyadhKingdom of Saudi Arabia Department of Pediatrics Faculty of MedicinePediatric Nephrology Center of ExcellenceKing Abdulaziz UniversityPO Box 80215Jeddah 21589Kingdom of Saudi Arabia

Background Congenital and infantile nephrotic syndrome (CNS and INS) are rare inherited defects in glomerular filtration involving a variety of gene *** study aimed to analyze all genetic mutations associated with congenital and infantile nephrotic syndrome treated at our *** also discussed our different approach secondary to culture and *** A retrospective single-center study of all children diagnosed as NS before the age of l year over a duration of over one *** Twenty-nine children (12 boys) were included in the *** median age (range) was 2.4 (0.1-12) months (20 CNS and 9 INS).Consanguinity was present in 90% of *** genetic analysis' results were only available for 20 *** underlying causative homozygous mutation was detected in 18 children (90%):NPHS1 (9),NPHS2(2),LAMB2(3),PLCE1(1),WT1(1),and ITSN1 novel mutation (2).One child had heterozygous mutation of NPHS2 and another child had heterozygous mutation of NPHS1 which could not explain the *** CNS cases were all managed with intermittent intravenous albumin infusion,ACEi,diuretics,and *** of the children were managed by nephrectomy followed by peritoneal dialysis (PD) because of limited *** one child achieved partial remission,while 15 children died at a median (range) age of 5.8 (1.25-29) *** remaining 14 children were followed up for an average of 36 (3.9-120) *** children progressed to end-stage kidney disease and PD was performed in only two *** NPHS1 is the main underlying cause of CNS and INS in our study *** and INS were associated with high morbidity and mortality.

关键词： Congenital nephrotic Infantile nephrotic Genetics Outcome

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Urinary neutrophil gelatinase-associated lipocalin(NGAL)and serum cystatin C measurements for early diagnosis of acute kidney injury in children admitted to PICU

引用

World Journal of Pediatrics 2018年第2期14卷 134-142页

作者： Jameela Abdulaziz Kari Mohamed Ahmed Shalaby Kholoud Sofyani Ahmad Saleh Sanad Albaraa Fuad Ossra Rayan Smeer Halabi Maha Hassan Aljuhani Wael Mohammad Toffaha Feras Aymen Moria Samar Sabry Hanan AbdelAziz Ahmed Khalid Abdulaziz Alhasan sara sharief Osama Safdar Pediatric Nephrology Center of Excellence and Department of Pediatrics King Abdulaziz University80215Jeddah 21589Kingdom of Saudi Arabia Pediatric Intensive Care Unit Department of PediatricsKing Abdulaziz UniversityJeddahKingdom of Saudi Arabia Pediatric Department Cairo UniversityGizaEgypt Department of Pediatrics College of MedicineKing Khalid University HospitalKing Saud UniversityRiyadhKingdom of Saudi Arabia

Background Acute kidney injury(AKI)is common in critically ill children with significant mortality and *** creatinine is an insensitive and late biomarker compared to newly proposed AKI *** Prospective study in pediatric intensive care unit(PICU)over three months to compare between serum cystatin-C(s-Cys-C)and urinary neutrophil gelatinase-associated lipocalin(uNGAL)as AKI biomarkers at multiple time points with pediatric risk,injury,failure,loss,end-stage renal disease(pRIFLE)classification in diagnosing *** Forty children were *** these 40 children,22 developed AKI according to pRIFLE *** was no significant difference between AKI and non-AKI in age(P=0.29).Post cardiac surgery,renal insult was the main cause of AKI(27.3%).There was a twofold increased risk of incident AKI in those patients with high baseline uNGAL at PICU admission and almost a fourfold increased risk in patients with high baseline s-Cys-C at PICU *** levels were highly predictive of AKI during the follow-up period[area under the curve(AUC)=0.76,95%confidence interval(CI)0.61-0.92].The cutoff point with the highest correctly classified proportion was 223 ng/mL(≥12 centiles)which correctly predict 80.0%patients with AKI,with a corresponding sensitivity of 72.7%and a specificity of 89.9%.AUC for s-Cys-C was 0.86(95%CI 0.75-0.97),and the highest correctly classified proportion was 1009μg/L(≥13 centiles);75%of patients with AKI,with a corresponding sensitivity of 63.6%and a specificity of 88.9%.Conclusion uNGAL and s-Cys-C predicts AKI early in critically ill children.

关键词： Acute kidney injury Neutrophil gelatinase-associated lipocalin Serum cystatin C

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Synthesis and Properties of Biomimetic Self-Assembling Structures from Poultry Feather Keratin

引用

Journal of Renewable Materials 2025年第1期13卷 1-19页

作者： sara Mattiello Carlo Santulli School of Science and Technology Universitàdi Camerinovia Gentile III da Varano 7Camerino62032Italy

Taking a widely contaminated yet abundant waste,such as poultry feathers,and extracting keratin from this struc-ture appears to be a real challenge whenever the preservation of the secondary structure of the protein is *** process would allow exploiting it in ways(e.g.,in the biomedicalﬁeld)that are inspired by a structure that is primarily designed forﬂight,therefore capable speciﬁcally of withstandingﬂexure and lateral buckling,also with very low *** preservation of the structure is based on disulﬁde crosslinks,and it is offered with pre-ference by some chemical treatments,mainly those based on ionic liquid and on a reduction ***,the degree of preservation cannot always be precisely assessed;however,beyond chemical characterization,the forma-tion of homogeneous gels can also suggest that the process was successful in this *** extraction respectful of nature’s intentions,considering that the secondary structure builds up according to the very function of the feath-ers in the animal,can be deemed to be *** particular,biomimetic extractions comply with the very characteristics the protein was designed for to serve in the speciﬁc environmental and mechanical situation in which it is *** review tries to elucidate in which cases this aim is achieved and for which speciﬁc appli-cations a chicken feather keratin that has preserved its secondary structure can be suited.

关键词： Keratin extraction secondary structure self-assembly chicken feathers

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

The complex role of protocadherin-19 in brain function:a focus on the oxytocin system

引用

Neural Regeneration Research 2025年第11期20卷 3211-3212页

作者： sara Mazzoleni Marta Busnelli Silvia Bassani CNR Institute of Neuroscience Vedano al LambroItaly NeuroMi Milan Center for Neuroscience MilanItaly

Mutations in the protocadherin-19(PCDH19)gene(Xq22.1)cause the X-linked syndrome known as developmental and epileptic encephalopathy 9(DEE9,OMIM#300088)(Dibbens et al.,2008).DEE9 is characterized by early-onset clustering epilepsy associated with intellectual disability ranging from mild to profound,autism spectrum disorder,and other neuropsychiatric features including schizophrenia,anxiety,attentiondeficit/hyperactivity,and obsessive or aggressive *** seizures may become less frequent in adolescence,psychiatric comorbidities persist and often worsen with age(Dibbens et al.,2008;Kolc et al.,2020).

关键词： epilepsy function system

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Shear strength characteristics of mixing slag-stone ballast reinforcement with tire geo-scrap using large-scale direct shear tests

引用

Railway Engineering Science 2025年第1期33卷 94-107页

作者： Morteza Esmaeili Hamidreza Heydari Maziar Mokhtari sara Darvishi School of Railway Engineering Iran University of Science and TechnologyTehranIran

Utilizing the ballast layer with more durable and stable characteristics can help avoid significant expenses due to decreased maintenance *** the ballast layer with different types of reinforcements or substituting the stone aggregates with the appropriate granular materials could potentially help to achieve this goal by reducing the ballast *** of the exquisite and most effective solutions to eliminate these challenges is to use waste materials such as steel slag aggregates and useless *** these waste materials in the ballasted railway track will contribute to sustainable development,an eco-friendly system,and green *** in a state-of-the-art insightful,the ballast aggregates,including a mixture of steel slag and stone aggregates,are reinforced with a novel kind of geo-grid made of waste tire strips known as *** laboratory research tried to explain the shear strength behavior of the introduced mixing slag-stone ballast reinforced with tire *** achieve this goal,a series of large-scale direct shear tests were performed on the ballast which is reinforced by tire geo-scrap and included various combinations of slag and stone *** concluded results indicate that the optimal mixing ratio is attained by a combination of 75%slag and 25%stone aggregates which is reinforced by tire geo-scrap at a placing level of 120 *** this case,the shear strength,internal friction angle,vertical displacement,and dilatancy angle of stone–slag ballast reinforced with geo-scraps exhibited average changes of+28%,+9%,-28%,and-15%,respectively.

关键词： Ballast deterioration Ballast stabilization Steel slag aggregates Large-scale direct shear test Waste tire geoscrap

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Effect of Modulen vs budesonide on clinical response and mucosal healing in Crohn’s patients

引用

World Journal of Gastroenterology 2025年第5期31卷 84-93页

作者： Baruch Ovadia Eva Niv sara Stern Katie Elisabeth Mahajna Oren Gal Yael Kopelman Department of Gastroenterology Hillel Yaffe Medical CenterHadera 38100HaifaIsrael Faculty of Medicine Technion-Israel Institute of TechnologyHadera 38100HaifaIsrael Department of Gastroenterology Meuhedet Health ServicesHadera 38100HaifaIsrael

BACKGROUND Mucosal healing has become an important goal of Crohn’s disease(CD)***,enriched with transforming growth factor-beta 2,and budeso-nide are commonly accepted treatments for mild-moderate ***,their effects on the small bowel(SB)mucosa remain *** To prospectively assess clinical and mucosal responses to Modulen vs budesonide in adults with CD,using SB capsule *** Thirty patients were divided into two groups:Modulen+home-based diet(21 patients)and budesonide(9 patients)for an eight-week intervention followed by four weeks of ***,laboratory,and endoscopic responses were *** mucosal changes were assessed through SB capsule *** Results indicated significant clinical improvement in the Modulen group with reduced CD activity index(P=0.041)and improved inflammatory bowel disease questionnaire score(P=0.016).Moreover,Modulen was associated with a signifi-cant SB mucosal improvement,evidenced by a decrease in Lewis score(P=0.027).No significant changes were observed in calprotectin or other laboratory ***,budesonide exhibited more modest clinical effects,but it improved calprotectin,hemoglobin,and C-reactive protein levels(P=0.051,P=0.014,and P=0.038,respectively).The capsule endoscopy did not reveal a significant mucosal response in the budesonide *** Both interventions have a role in CD ***,their effects differ and may complement each other:Modulen yields clinical and mucosal improvements,while budesonide primarily leads mainly to laboratory improvements.

关键词： Crohn’s disease Modulen oral polymeric diet Transforming growth factor-beta 2 Budesonide Mucosal healing Clinical response Capsule endoscopy Small bowel capsule

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Advances and challenges in diagnosing and managing adult autoimmune enteropathy

引用

World Journal of Gastroenterology 2025年第2期31卷 128-131页

作者： Grigorios Christodoulidis sara E Agko Marina N Kouliou Konstantinos E Koumarelas Dimitris Zacharoulis Department of General Surgery University Hospital of LarissaLarissa 41110Greece Department of Intensive Care Unit Asklepios Paulinen Clinic WiesbadenWiesbaden 65197Germany Department of Emergency Medicine General Hospital of LarissaLarissa 41221Greece Department of Surgery University Hospital of LarissaLarissa 41334Greece

Autoimmune enteropathy(AIE)is a rare immune mediated disorder primarily affecting children,characterized by chronic diarrhea,malabsorption,vomiting,weight loss and villous *** has also been observed in adults presenting diagnostic and treatment challenges due to its overlap with other gastrointestinal disorders such as celiac *** diagnostic criteria for AIE include small bowel villous atrophy,lack of response to dietary restrictions,presence of anti-enterocyte antibodies,and predisposition to autoimmunity without severe *** criteria emphasize characteristic histological findings and exclusion of other causes of villous *** is associated with various autoimmune disorders and can present with overlapping features with Celiac disease,including villous atrophy but without significant intraepithelial *** primarily involves immunosuppression using corticosteroids,calcineurin inhibitors,and anti-tumor necrosis factor therapy,alongside nutri-tional *** the challenges,understanding AIE’s diverse manifest-ations and improving diagnostic criteria are essential for effective management and improved patient *** research is needed to elucidate the pathogenesis,disease progression and long-term outcomes of AIE.

关键词： Autoimmune enteropathy Diagnosis Prognosis Adult autoimmune enter-opathy and challenges Autoimmune enteropathy and treatment Autoimmune enteropathy and diagnostic criteria Pathophysiology of autoimmune enteropathy

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Productive and metabolomic consequences of arginine supplementation in sows during different gestation periods in two different seasons

引用

Journal of Animal Science and Biotechnology 2025年第1期16卷 218-237页

作者： sara Virdis Diana Luise Federico Correa Luca Laghi Norma Arrigoni Roxana Elena Amarie Andrea Serra Giacomo Biagi Clara Negrini Francesco Palumbo Paolo Trevisi Department of Agricultural and Food Sciences University of BolognaViale G.Fanin 4440127 BolognaItaly Istituto Zooprofilattico Sperimentale Della Lombardia E Dell’Emilia Romagna(IZSLER)“Bruno Ubertini” Via Bianchi925124 BresciaItaly Department of Agriculture Food and EnvironmentUniversity of PisaVia del Borghetto 8056124 PisaItaly Department of Veterinary Medicine University of BolognaVia Tolara Di Sopra 5040064 Ozzano Dell’Emilia(BO)Italy.

Background The prolificacy of sows(litter size at birth)has markedly increased,leading to higher post-natal *** stress can exacerbate this *** plays an important role in several physiological pathways;its effect on gestating sows can depend on the period of *** study evaluated the effects of arginine supplementation on the productive performance and physiological status of sows during different gestation periods and seasons,using a multi-omics *** A total of 320 sows were divided into 4 groups over 2 seasons(warm/cold);a control group(CO)received a standard diet(including 16.5 g/d of arginine)and 3 other groups received the standard diet supplemented with 21.8 g/d of arginine(38.3 g/d of arginine)either during the first 35 d(Early35),the last 45 d(Late45)or through-out the entire gestation period(COM).The colostrum was analyzed for nutritional composition,immunoglobulins and metabolomic *** and feces were analyzed on d 35 and 106 for the metabolomic and microbial *** body weight and mortality were recorded at birth,d 6,d 26,and on d 14 *** Interactions between arginine and season were never *** Early35 group had a lower percent-age of stillborn(P<0.001),mummified(P=0.002)and low birthweight(LBW)piglets(P=0.02)than the CO *** Late45 group had a lower percentage of stillborn piglets(P=0.029)and a higher percentage of high birthweight piglets(HBW;P<0.001)than the CO *** COM group had a higher percentage of LBW(P=0.004)and crushed piglets(P<0.001)than the CO *** supplementation modifies the metabolome characterization of colos-trum,urine,and *** and nitric oxide pathways,as well as metabolites related to microbial activity,were influenced in all matrices.A slight trend in the beta diversity index was observed in the microbiome profile on d 35(P=0.064).Conclusions Arginine supplementation during early gestation reduced the percentage o

关键词： Colostrum Feces Microbiota Piglets’birth weight Stillborn Urine

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Patients admitted in the intensive care unit after solid organ or bone marrow transplantation:Retrospective cohort study

引用

World Journal of Transplantation 2025年第1期15卷 95-104页

作者： Ana Vujaklija Brajkovic Iva Kosuta Lucija Batur sara Sundalic Marijana Medic Andro Vujevic Luka Bielen Jaksa Babel Department of Internal Medicine University Hospital Centre ZagrebZagreb 10000Croatia School of Medicine University of ZagrebZagreb 10000Croatia

BACKGROUND Solid organ transplantation(SOT)and hematopoietic stem cell transplantation(HSCT)revolutionized the survival and quality of life of patients with malignant diseases,various immunologic,and metabolic disorders or those associated with a significant impairment in a patient's quality of *** To investigate admission causes and treatment outcomes of patients after SOT or HSCT treated in a medical intensive care unit(ICU).METHODS We conducted a single-center,retrospective epidemiological study in the medical ICU at the University Hospital Centre Zagreb,Croatia covering the period from January 1,2018 to December 31,*** The study included 91 patients with either SOT[28 patients(30.8%)]or HSCT[63 patients(69.2%)].The median age was 56(43.2-64.7)years,and 60.4%of the patients were *** with SOT had more comorbidities than patients after HSCT[χ^(2)(5,n=141)=18.513,P<0.001].Sepsis and septic shock were the most frequent reasons for admission,followed by acute respiratory insufficiency in patients following *** rate significantly differed between SOT and HSCT[χ^(2)(1,n=91)=21.767,P<0.001].ICU survival was 57%in the SOT and 12.7%in the HSCT *** need for mechanical ventilation[χ^(2)(1,n=91)=17.081,P<0.001]and vasopressor therapy[χ^(2)(1,n=91)=36.803,P<0.001]was associated with *** necessity for acute renal replacement therapy did not influence patients'survival[χ^(2)(1,n=91)=0.376,P=0.54].In the subgroup of patients with infection,90%had septic shock,and the majority had positive microbiological samples,mostly Gram-negative *** ICU survival of patients with sepsis/septic shock cumulatively was 15%.The survival of SOT patients with sepsis/shock was 45%.CONCLUSION Patients with SOT or HSCT are frequently admitted to the ICU due to sepsis and septic *** advancements in critical care,the mortality rate of patients with refractory septic shock and multiorgan failure in this patient population is extremely hig

关键词： Liver transplantation Kidney transplantation Hematopoietic stem cell transplantation Critical care Sepsis Septic shock Mortality

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

An illustrated guide to:Distributed and integrated fibreoptic sensing in seismology

引用

Earthquake Science 2025年第1期38卷 67-77页

作者： Andreas Fichtner Fabian Walter Patrick Paitz sara Klaasen Daniel C.Bowden Sebastian Noe Nils Müller Dominik Husmann Jacques Morel Department of Earth Sciences ETH ZürichZürich 8049Switzerland Swiss Federal Institute for Forest Snow and Landscape ResearchBirmensdorf 8903Switzerland Swiss Federal Institute of Metrology METAS Bern 3003Switzerland

The properties of laser signals are affected by deformation of the optical fibre through which they are *** this deformation dependence is undesirable in telecommunication,it can be exploited for the construction of novel seismic sensors that fill a niche in data acquisition where traditional seismometer arrays would be difficult to *** includes densely populated urban centers,the oceans,volcanoes and the Earth’s polar *** notes complement a presentation on recent methodological developments and applications in fibre-optic *** first part is focused on the use of distributed fibreoptic sensing in cryosphere research,and specifically the investigation of the internal structure and seismicity of glaciers and ice *** second part is dedicated to recent advances in integrated fibre-optic sensing,with emphasis on novel measurement principles and sensitivity.

关键词： seismology fibre-optics cryosphere earthquakes

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

欢迎您,

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案：

请选择收藏分类：

通借通还

欢迎您,

建议与咨询 留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案： 新增检索档案 确定 取消

请选择收藏分类： 新增自定义分类 确定 取消

通借通还

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

请选择保存的检索档案：

请选择收藏分类：