检索结果-南通市图书馆

Inherited Fanconi syndrome

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

World Journal of Pediatrics 2023年第7期19卷 619-634页

作者： Anna Luiza Braga Albuquerque Rafael dos Santos Borges Ana Flavia Conegundes Erika Emmylaine dos Santos Frederico Moreira Man Fu Clara Tavares Araujo Pedro Alves Soares Vaz de Castrol Ana Cristina Simoes e Silva Interdisciplinary Laboratory of Medical Investigation Unit of Pediatric NephrologyFaculty of MedicineFederal University of Minas Gerais(UFMG)Belo HorizonteBrazil Department of Pediatrics Faculty of MedicineUFMGAlfredo Balena Avenue1902Nd FloorRoom#281Belo HorizonteMG 30130-100Brazil

Background Fanconi-Debré-de Toni syndrome(also known as Fanconi renotubular syndrome,or FRST)profoundly increased the understanding of the functions of the proximal convoluted tubule(PCT)and provided important insights into the pathophysiology of several kidney diseases and drug *** sources We searched Pubmed and Scopus databases to find relevant articles about *** review article focuses on the physiology of the PCT,as well as on the physiopathology of FRST in children,its diagnosis,and *** FRST encompasses a wide variety of inherited and acquired PCT alterations that lead to impairment of PCT *** children,FRST often presents as a secondary feature of systemic disorders that impair energy supply,such as Lowe's syndrome,Dent's disease,cystinosis,hereditary fructose intolerance,galactosemia,tyrosinemia,Alport syndrome,and Wilson's *** rare,congenital causes of FRST greatly impact the morbidity and mortality of patients and impose diagnostic ***,its treatment is diverse and considers the ability of the clinician to identify the correct etiology of the *** The early diagnosis and treatment of pediatric patients with FRST improve the prognosis and the quality of life.

关键词： Cystinosis Fanconi syndrome Fanconi renotubular syndrome Fanconi-Debre-de Toni syndrome Proximal tubule Renal tubular transport-Rickets

Zonal activation of molecular carbon dioxide and hydrogen over dual sites Ni-Co-MgO catalyst for CO_(2) methanation:Synergistic catalysis of Ni and Co species

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Journal of Energy Chemistry 2024年第4期91卷 213-225页

作者： Zonglin Li Jianjun Chen Yu Xie Junjie Wen Huiling Weng Mingxue Wang Jingyi Zhang Jinyan Cao Guocai Tian Qiulin Zhang Ping Ning faculty of Environmental Science and Engineering Kunming University of Science and TechnologyKunming 650500YunnanChina National-Regional Engineering Center for Recovery of Waste Gases from Metallurgical and Chemical Industries Kunming University of Science and TechnologyKunming 650500YunnanChina faculty of Metallurgical and Energy Engineering Kunming University of Science and TechnologyKunming 650093YunnanChina

An in-depth mechanism in zonal activation of CO_(2)and H2molecular over dual-active sites has not been revealed ***,Ni-Co-MgO was rationally constructed to elucidate the CO_(2)methanation *** abundant surface nickel and cobalt components as active sites led to strong Ni-Co interaction with charge transfer from nickel to ***,electron-enriched Coδ-species participated in efficient chemisorption and activation of CO_(2)to generate monodentate ***,plentiful available Ni0sites facilitated H2dissociation,thus CO_(2)and H2were smoothly activated at zones of Coδ-species and Ni0,*** in situ DRIFTS,quasi situ XPS,TPSR,and DFT calculations substantiated a new formate evolution mechanism via monodentate carbonate instead of traditional bidentate carbonate based on synergistic catalysis of Coδ-species and *** zonal activation of CO_(2)and H2by tuning electron behaviors of double-center catalysts can boost heterogeneous catalytic hydrogenation performance.

关键词： Zonal activation CO_(2) methanation Dual active sites Synergistic effect

Retinal capillary plexus in Parkinson’s disease using optical coherence tomography angiography

在线全文

学校读者我要写书评

暂无评论

International Journal of Ophthalmology(English edition) 2024年第1期17卷 131-136页

作者： Ioannis Giachos Spyridon Doumazos Anastasia Tsiogka Konstantina Manoli George Tagaris Tryfon Rotsos Vassilios Kozobolis Ioannis Iliopoulos Marilita Moschos First Department of Ophthalmology National and Kapodistrian University of Athens Medical SchoolAthens 11527Greece Department of Neurology General Hospital of Athens“Georgios Gennimatas”Athens 11527Greece Department of Ophthalmology Faculty of MedicineSchool of Health SciencesUniversity of PatrasPatras 26504Greece Neurology Department Democritus University of ThraceAlexandroupolis 68100Greece

AIM:To evaluate the alterations of the retinal microvasculature and foveal avascular zone in patients with Parkinson’s disease(PD)using optical coherence tomography angiography(OCT-A).METHODS:A retrospective study of PD patients examined in the Ophthalmology Department of the General Hospital of Athens,“Georgios Gennimatas”from March 2021 to March 2022 was *** 44 patients with PD were included and 18 healthy controls were examined,hence a total of 124 eyes were enrolled in the *** foveal and parafoveal superficial and deep capillary plexus vascular density(fSCP-VD,fDCP-VD,pSCP-VD,pDCP-CD)and foveal avascular zone(FAZ)were quantified with *** coherence tomography(OCT)was used to measure macular *** statistical analysis was conducted by using a mixed effect linear regression ***:After adjustment for age and gender,the mean parafoveal superficial capillary plexus vascular density(pSCP-VD)and mean parafoveal deep capillary plexus vascular density(pDCP-VD)were significantly decreased in individuals with PD(P2.35(95%CI-3.3,-1.45)and-7.5(95%CI-10.4,-4.6)***-VD and fDCP-VD didn’t approach statistical *** FAZ area and perimeter were significantly decreased(P2)(95%CI-0.13,-0.07)and-0.49 mm^(2)(95%CI-0.66,-0.32)*** didn’t approach statistical *** retinal thickness(CRT)was significantly decreased in individuals with PD(P23.1μm(95%CI-30.2,-16)and temporal retinal thickness(TRT)was decreased(P=0.025)by-11μm(95%CI-22,-1.5)while nasal retinal thickness(NRT)only approached statistical significance(P=0.066).CONCLUSION:The mean pSCP-VD,pDCP-VD,CRT and TRT are significantly decreased and FAZ is altered in individuals with *** findings can be potentially used as biomarkers for the diagnosis and evaluation of early PD.

关键词： Parkinson’s disease optical coherence tomography angiography retinal vascular density foveal avascular zone

Reconfigurable logic and in-sensor encryption operations in an asymmetrically tunable van der Waals heterostructure

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Nano Research 2024年第4期17卷 3113-3119页

作者： Fan Gong Wenjie Deng Yi Wu Fengming Liu Yihao Guo Zelin Che Jingjie Li Jingzhen Li Yang Chai Yongzhe Zhang Key Laboratory of Optoelectronics Technology of Education Ministry of China Faculty of Information TechnologyBeijing University of TechnologyBeijing 100124China Key Laboratory of Advanced Functional Materials Ministry of EducationFaculty of Materials and ManufacturingBeijing University of TechnologyBeijing 100124China Department of Applied Physics Hong Kong Polytechnic UniversityKowloonHong KongChina

Reconfigurable devices can be used to achieve multiple logic operation and intelligent optical sensing with low power consumption,which is promising candidates for new generation electronic and optoelectronic integrated ***,the versatility is still limited and need to be extended by the device architectures ***,we report an asymmetrically gate two-dimensional(2D)van der Waals heterostructure with hybrid dielectric layer SiO_(2)/hexagonal boron nitride(h-BN),which enable rich function including reconfigurable logic operation and in-sensor information encryption enabled by both volatile and non-volatile optoelectrical *** the partial gate is grounded,the non-volatile light assisted electrostatic doping endowed partially reconfigurable doping between n-type and p-type,which allow the switching of logic XOR and not implication(NIMP).When the global gate is grounded,additionally taking the optical signal as another input signal,logic And and OR is realized by combined regulation of the light and localized gate *** on the high on/off current ratio approaching 105 and reliable&switchable logic gate,in-sensor information encryption and decryption is demonstrated by manipulating the logic ***,these results provide strong extension for current reconfigurable electronic and optoelectronic devices.

关键词： in-sensor encryption reconfigurable logic van der Waals heterostructure asymmetrical tunable architecture

Mechanical circulatory support in cardiogenic shock and post-myocardial infarction mechanical complications

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Journal of Geriatric Cardiology 2022年第2期19卷 130-136页

作者： Daniel Rob Jan Bělohlávek nd Department of medicine-Department of Cardiovascular medicine First Faculty of MedicineCharles University and General University HospitalPragueCzech Republic

Despite advanced therapies,the mortality of patients with myocardial infarction(MI)complicated by cardiogenic shock(CS)remains around 50%.Mechanical complications of MI are rare nowadays but associated with high mortality in patients who present with *** treatment strategies and mechanical circulatory support(MCS)devices have been increasingly used to improve the grim prognosis of refractory *** article discusses current evidence regarding the use of MCS in MI complicated by CS,ventricular septal rupture,free wall rupture and acute mitral *** selection should be tailored according to the cause and severity of *** MCS initiation and multidisciplinary team cooperation is mandatory for good *** associated bleeding remains a major complication and an obstacle to better *** prospective randomized trials will improve current knowledge regarding MCS indications,timing,and patient selection in the coming years.

关键词： mortality infarction rupture

Erosion control of Chinese loess using polymer SH and ryegrass

在线全文

学校读者我要写书评

暂无评论

Journal of Mountain Science 2024年第6期21卷 2043-2058页

作者： YING Chunye LI Lanxing MAKEEN Gehad Mohamed Hossam LIU Yabin School of Geological Engineering Qinghai UniversityXining 810016China Key Laboratory of Genozoic Resource&Environment in North Margin of the Tibetan Plateau Xining 810016China faculty of Engineering China University of GeosciencesWuhan 430074China faculty of Science Ain Shams UniversityCairo 11566Egypt

The China Loess Plateau is subjected to severe soil erosion triggered by intense rainfall,resulting in significant harm and losses to both human society and the natural *** this study,a novel technique for managing loess erosion is introduced,which involves the utilization of a combined polymer SH and ryegrass.A comprehensive series of tests were undertaken,including rainfall erosion tests,disintegration experiments,and scanning electron microscopy examinations,to assess the accumulative sediment yield(ASY),disintegration ratio,and microstructural features of both untreated and treated loess *** results showed a significant reduction in ASY with increased dry density of untreated ***,the combined technique effectively controlled erosion,limiting ASY to 266.2 g/cm^(2)in 60 *** was approximately one-sixth,one-ninth,and one-fifteenth of the ASY in SH-treated loess(L-SH),ryegrass-treated loess(L-R),and untreated loess,*** resisted disintegration better than ryegrass alone but slightly less than *** improvement was due to the combined effect of SH and ryegrass,which reduced raindrop impact,improved loess microstructure,and boosted ryegrass *** innovative technique holds the potential to be applied as a field-scale technique in the Loess Plateau region of China.

关键词： Loess Plateau Loess erosion Ryegrass Polymer SH Erosion control Microstructure

Abdominoplasty, Liposuction and Brazilian Butt Lift (BBL) in Kinshasa in the Democratic Republic of Congo. My Experience in This Aesthetic Plastic Surgery with Black African Women in a Low-Income Country

在线全文

学校读者我要写书评

暂无评论

Modern Plastic Surgery 2024年第3期14卷 36-50页

作者： Anatole Kibadi-Kapay Department of Reconstructive and Aesthetic Plastic Surgery & Hand Surgery University Clinics of Kinshasa Faculty of Medicine University of Kinshasa Kinshasa Democratic Republic of Congo

Background: In low-income African countries, the demand and use of plastic surgery operations including abdominoplasty, liposuction and Brazilian butt lift (BBL) are increasing. The goal of this work is to present my experience and the challenges of this aesthetic plastic surgery among black African women. Material and Methods: A retrospective study was conducted about the abdominoplasties, liposuctions and Brazilian butt lift (BBL) which I operated in public and private hospitals at Kinshasa (Democratic Republic of Congo) in black African women. It covers a period of 13 years, going from December 1, 2010 to December 1, 2023. For this study, I had at least 6 months period (to Juin 1, 2024) to assess the occurrence of early and late postoperative complications. Results: I performed 84 abdominoplasties, 144 liposuctions and 23 Brazilian butt lifts (BBL). The average age at the time of abdominoplasty was 44 years with extremes ranging from 26 to 55 years and a concentration of cases (60.7%) in the age group of 40 to 49 years. The age group of 20 to 29 years old represented the lowest rate of requests for abdominoplasty (4.7%). Patients with a BMI of 30 to 2 were the majority (61.9%), followed by those between 25 to 2 (29.7%). 67.8% of patients were obese (BMI ≥ 30 kg/m2). The average age at the time of liposuction was 41 years with extremes ranging from 21 years to 69 years;and more than half of cases (68%) in the age group between 30 and 49 years. As for Brazilian butt lift (BBL), the average age was 33 years with extremes ranging from 24 to 42 years and a concentration of patients (91.3%) between 20 and 39 years. The immediate postoperative complications of abdominoplasties observed were: seroma in 7% of cases, hematoma and partial infection of the surgical site in 5% of cases. Pathological scars (hypertrophic, keloid) after abdominoplasties were observed in 9% of cases. The most common complication of liposuction was contour deformity. I observed 16 patients (11.1%)

关键词： Abdominoplasty Liposuction Brazilian butt lift (BBL) Black African Women Kinshasa Democratic Republic of Congo

FE65:a hub for neurodevelopment

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Neural Regeneration Research 2024年第9期19卷 1883-1884页

作者： Yuqi Zhai Laura Lok-Haang Ng Dennis Dik-Long Chau Kwok-Fai Lau School of Life Sciences Faculty of ScienceThe Chinese University of Hong KongHong Kong Special Administrative RegionChina

FE65,initially identified as a binding partner of amyloid precursor protein(APP),is an adaptor protein enriched in the brain and regulated during ***65 belongs to the FE65 protein *** family is comprised of three memb... 详细信息

关键词： family development partner

Performance improvement in stepped solar still modified by sponge layer

在线全文

学校读者我要写书评

暂无评论

Journal of Central South University 2024年第6期31卷 1973-1982页

作者： Mahtab TARAHOMI Saman RASHIDI Faramarz HORMOZI Shahabeddin ASHTIANI Department of Chemical Petroleumand Gas EngineeringSemnan UniversitySemnanIran Department of Energy Faculty of New Sciences and TechnologiesSemnan UniversitySemnanIran

In this paper,the experimental investigation on the performance improvement of conventional stepped solar still is *** steps are covered by the porous material to improve the performance of the conventional device and increase the evaporation *** the parameters,including the temperature on the glass surface,the water temperature inside the evaporation zone,and the amount of water produced in both conventional and modified stepped solar stills are measured and *** efficiency of two devices and their exergy efficiency have been ***,the economic analysis of both devices has been done to check the economic feasibility of the modified *** amount of freshwater generated during one day was 2244.4 and 3076.2 mL/m^(2),respectively for the conventional and modified stepped solar *** a result,the amount of water produced in one day by modified stepped solar still is 35.5% more than the conventional ***,the costs for the conventional and modified stepped solar stills have been calculated as 0.0359 and 0.029$/(L·m^(-2)),respectively.

关键词： stepped solar still black sponge efficiency exergy economic analysis