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Distal renal tubular acidosis:genetic causes and management

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World Journal of Pediatrics 2019年第5期15卷 422-431页

作者： Sílvia Bouissou Morais soares Luiz Alberto Wanderley de Menezes silva Flávia Cristina de Carvalho Mrad Ana Cristina Simoes e silva Interdisciplinary Laboratory of Medical Investigation Faculty of MedicineFederal University of Minas Gerais(UFMG)Av.Prof.Alfredo Balena190Room#281Belo HorizonteMG 30130-100Brazil Pediatric Nephrology Unit Faculty of MedicineUFMGBelo HorizonteBrazil

Background Distal renal tubular acidosis(dRTA)is a kidney tubulopathy that causes a state of normal anion gap metabolic acidosis due to impairment of urine *** review aims to summarize the etiology,pathophysiology,clinical findings,diagnosis and therapeutic approach of dRTA,with emphasis on genetic causes of *** sources Literature reviews and original research articles from databases,including PubMed and Google *** searching was performed to identify additional studies about *** dRTA is characterized as the dysfunction of the distal urinary acidification,leading to metabolic *** pediatric patients,the most frequent etiology of dRTA is the genetic alteration of genes responsible for the codification of distal tubule channels,whereas,in adult patients,dRTA is more commonly secondary to autoimmune diseases,use of medications and *** with dRTA exhibit failure to thrive and important laboratory alterations,which are used to define the *** oral alkali and potassium supplementation can correct the biochemical defects,improve clinical manifestations and avoid nephrolithiasis and *** dRTA is a multifactorial disease leading to several clinical *** and laboratory alterations can be corrected by alkali replacement therapy.

关键词： Renal tubular acidosis Distal tubule physiology Metabolic acidosis Gene mutations Proton pump

Bartter's syndrome:clinical findings,genetic causes and therapeutic approach

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World Journal of Pediatrics 2021年第1期17卷 31-39页

作者： Flavia Cristina Carvalho Mrad Sílvia Bouissou Morais soares Luiz Alberto Wanderley de Menezes silva Pedro Versiani dos Anjos Menezes Ana Cristina Simoes-e-silva Interdisciplinary Laboratory of Medical Investigation Faculty of MedicineFederal University of Minas Gerais(UFMG)Av.Alfredo Balena190Room#281Belo HorizonteMG 30130-100Brazil Pediatric Nephrology Unit Faculty of MedicineUFMGBelo HorizonteBrazil

Backgound Bartter's syndrome(BS)is a rare group of salt losing tubulopathies due to the impairment of transport mecha-nisms at the thick ascending limb of the Henle's *** sources Literature reviews and original research articles were collected from database,including PubMed and *** According to the time of onset and symptoms,BS can be classified into antenatal and classic *** studies have identified different subtypes of *** types Ⅰ,Ⅱ and Ⅲ are caused by mutations on genes encoding the luminal Na^(+)-K^(+)-2Cl^(-) co-transporter,the luminal K+ channel ROMK,and the basolateral chloride channel ClC-Kb(CLCNKB),***-of-function mutations of Barttin CLCNK type accessory beta subunit cause BS type Ⅳ*** mutations of CLCNKB and CLCNKA cause BS type Ⅳ*** type Ⅴ consists in a novel transient form characterized by antenatal presentation due to mutations in the MAGe family member *** gain-of-function mutations of the extracellular calcium sensing receptor gene can result in an autosomal dominant condition of *** clinical and biochemical alterations in BS include polyuria,dehydration,hypokalemia,hypochloremic metabolic alka-losis,hyperreninemia,high levels of prostaglandins,normal or low blood pressure,hypercalciuria and failure to *** focuses mainly at correcting dehydration and electrolyte disturbances and in measures to reduce polyuria,including the use of nonsteroidal anti-inflammatory medications to control excessive renal prostaglandin e2 *** early diagnosis and treatment of BS may prevent long-term consequences such as growth failure,nephrocal-cinosis and end-stage renal disease.

关键词： Bartter's syndrome Hypercalciuria Hypokalemia Metabolic alkalosis Nephrocalcinosis

Transcriptional cofactors Ski and SnoN are major regulatorsof the TGF-β/Smad signaling pathway in health and disease

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Signal Transduction and Targeted Therapy 2018年第1期3卷 184-198页

作者： Angeles C.Tecalco-Cruz Diana G.Rios-Lopez Genaro Vazquez-Victorio Reyna e.Rosales-Alvarez marina Macias-silva Instituto de Investigaciones Biomedicas at Universidad Nacional Autonoma de Mexico Mexico city 04510Mexico Instituto de Fisiologia Celular at Universidad Nacional Autonoma de Mexico Mexico city 04510Mexico Facultad de Ciencias at Universidad Nacional Autonoma de Mexico Mexico city 04510Mexico

The transforming growth factor-β(TGF-β)family plays major pleiotropic roles by regulating many physiological processes in development and tissue *** TGF-βsignaling pathway outcome relies on the control of the spatial and temporal expression of>500 genes,which depend on the functions of the Smad protein along with those of diverse modulators of this signaling pathway,such as transcriptional factors and ***(Sloan-Kettering Institute)and SnoN(Ski novel)are Smadinteracting proteins that negatively regulate the TGF-βsignaling pathway by disrupting the formation of R-Smad/Smad4 complexes,as well as by inhibiting Smad association with the p300/CBP *** Ski and SnoN transcriptional cofactors recruit diverse corepressors and histone deacetylases to repress gene *** TGF-β/Smad pathway and coregulators Ski and SnoN clearly regulate each other through several positive and negative feedback ***,these cross-regulatory processes finely modify the TGF-βsignaling outcome as they control the magnitude and duration of the TGF-β*** a result,any alteration in these regulatory mechanisms may lead to disease ***,the design of targeted therapies to exert tight control of the levels of negative modulators of the TGF-βpathway,such as Ski and SnoN,is critical to restore cell homeostasis under the specific pathological conditions in which these cofactors are deregulated,such as fibrosis and cancer.

关键词： SnoN homeostasis Smad

Waste Tire Rubber-based Refrigerants for Solid-state Cooling Devices

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Chinese Journal of Polymer Science 2020年第7期38卷 769-775,I0007页

作者： Nicolau Molina Born erik Oda Usuda Mariana da silva Gigliotti Denílson JoséMarcolino de Aguiar William Imamura Lucas soares Paixao Alexandre Magnus Gomes Carvalho Catalan Institute of Nanoscience and Nanotechnology(ICN2) 08860BellaterraBarcelonaSpain The Institute of Photonic Sciences(ICFO) 08193CastelldefelsBarcelonaSpain Universidade Federal de Sāo Paulo(UNIFeSP) 00972-270DiademaSPBrazil Instituto de Física"Gleb Wataghin" Universidade Estadual de Campinas(UNICAMP)13083-970CampinasSPBrazil Universidade Tecnológica Federal do Paraná(UTFPR) 84017-220Ponta GrossaPRBrazil Faculdade de engenharia Mec(a)nica Universidade Estadual de Campinas(UNICAMP)13083-860CampinasSPBrazil Departamento de engenharia Mec(a)nica Universidade Estadual de Maringá(UEM)870-900MaringáPRBrazil

Management of discarded tires is a compelling environmental issue *** there are several approaches developed to recycle waste tire rubbers,their application in solid-state cooling is still *** the high barocaloric potential verified for elastomers,the use of waste tire rubber(WTR)as a refrigerant in solid-state cooling devices is very ***,we investigated the barocaloric effects in WTR and polymer blends made of vulcanized natural rubber(VNR)and WTR,to evaluate its feasibility for solid-state cooling *** adiabatic temperature changes and the isothermal entropy changes reach giant values,as well as the performance parameters,being comparable or even better than most barocaloric materials in ***,pure WTR and WTR-based samples also present a faster thermal exchange than VNR,consisting of an additional advantage of using these discarded ***,the present findings evidence the encouraging perspectives of employing waste rubbers in solid-state cooling based on barocaloric effects,contributing to both the recycling of polymers and the sustainable energy technology field.

关键词： Waste tire rubber Solid-state cooling Barocaloric effect Tire recycling Sustainable energy

Basic biological aspects of Tritrichomonas foetus of re-levance to the treatment of bovines suffering of tricho-moniasis

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Open Journal of Animal Sciences 2011年第3期1卷 112-120页

作者： Newton soares da silva Susane Moreira Machado Fernando Costa e silva Filho Cristina Pacheco-soares 不详

Tritrichomonas foetus is a flagellate protozoan and the etiological agent of bovine genital tri-chomoniasis [1], which is an infectious vene- real disease. This parasite is usually found as- sociated with the mucosal surface of the uro- genital tract in females or the male preputial and penile membranes. In females, the clinical ma-nifestations may include abortion, with repe- tition of estrus at irregular intervals, vaginitis, cervicitis, endometritis, and pyometra. Parasi- tized males may have a discharge with small nodules in the preputial membrane. After that, the bulls have no clinical symptoms, and are thus an asymptomatic carrier that may spread the infection. Considering that a bull could cover up to twenty females [2], bovine genital trichomoniasis is a serious medical and veteri- nary problem, with economical repercussion for beef and milk production. As T. foetus is an amitochondrial and aerotolerant organism, en- ergy production under low O2 tension in the protozoan is done via hydrogenosome, which, as the name suggests, is the organelle where H2 is generated [3,4,5]. The molecular machinery of mitochondrial cell death is, therefore, absent in this parasite and the mechanism that activates of cell death program is not clear. This review seeks to understand the characteristics of the protozoan parasite T. foetus in order to propose new therapies for animals suffering from this infectious and contagious agent.

关键词： Reproduction Protozoan Parasite Tritrichomonas foetus

Liquefaction of Kraft Lignin at Atmospheric Pressure

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Journal of Renewable Materials 2019年第6期7卷 527-534页

作者： Silvia Helena Fuentes da silva Patricia soares Bilhalva dos Santos Darci Alberto Gatto Maria Angeles Andres Itziar egüés Chemical and environmental engineering Department University of Basque CountryPlaza Europa 120018Donostia-San SebastiánSpain Faculty of Forestry engineering Federal University of ParáC.JoséPorfirio2515AltamiraBrazil College of Material engineering(PPGCeM) Federal University of PelotasBrazil

Kraft lignin was liquefied using polyethylene glycol#400(PeG)and glycerol(G)in a weight ratio of 80/20(w/w)and sulphuric acid(SA)as catalyst under atmospheric pressure at 160º*** three independent variables:reaction time(60,80 and 100 min),percentage of lignin(15,20 and 25%,w/w),and catalyst concentration(0,3 and 6%,w/w),were varied resulting in 27 experimental *** effect of these reaction conditions on the properties of the polyols was *** statistical analysis showed that only“the percentage of lignin”did not influence the properties of the liquefied products,however,reaction time and catalyst load were important *** resulting liquefied products were characterized by FTIR analysis.

关键词： Biopolyols Kraft lignin liquefaction renewable resource thermochemical conversion

Inherited Fanconi syndrome

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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

Plant growth and metabolism of exotic and native Crotalaria species for mine land rehabilitation in the Amazon

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Journal of Forestry Research 2024年第1期35卷 143-154页

作者： Hemelyn soares das Chagas Rafael silva Guedes Markus Gastauer Paula Godinho Ribeiro Allan Klynger da silva Lobato Cecílio Frois Caldeira Fabrício William deÁvila AndréRodrigues dos Reis Silvio Junio Ramos Instituto Tecnológico Vale Rua Boaventura da Silva955BelémPA 66055-090Brazil Universidade Federal do Sul e Sudeste do Pará XinguaraPABrazil Núcleo de Pesquisa Vegetal Básica e Aplicada Universidade Federal Rural da AmazôniaParagominasPABrazil Departamento de engenharia Florestal Universidade Estadual do Centro-Oeste/UNICENTROIratiPRBrazil School of Sciences and engineering São Paulo State University(UNESP)TupãSP.CEP 17602-496Brazil

Despite its enormous benefits,mining is respon-sible for intense changes to vegetation and soil ***,after extraction,it is necessary to rehabilitate the mined areas,creating better conditions for the establishment of plant species which is *** study evaluated mineral and organic fertilization on the growth,and carbon and nitrogen(N)metabolism of two Crotalaria species[Cro-talaria spectabilis(exotic species)and Crotalaria maypu-rensis(native species from Carajás Mineral Province(CMP)]established on a waste pile from an iron mine in CMP.A control(without fertilizer application)and six fertilization mixtures were tested(i=NPK;ii=NPK+micronutrients;iii=NPK+micronutrients+organic compost;iv=PK;v=PK+micronutrients;vi=PK+micronutrients+organic compost).Fertilization contributed to increased growth of both species,and treatments with NPK and micronutrients had the best results(up to 257%***),while organic fertilization did not show *** Crotalaria had a greater number of nodules,higher nodule dry mass,chlorophyll a and b contents and showed free ammonium as the predominant N form,reflecting greater increments in biomass compared to native *** having lower growth,the use of this native species in the rehabilitation of mining areas should be considered,mainly because it has good development and meets current government legislation as an opportunity to restore local biodiversity.

关键词： Biological nitrogen fixation Mining Nitrogen metabolism Plant nutrition

Terpenes extracted from marine sponges with antioxidant activity:a systematic review

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Natural Products and Bioprospecting 2023年第1期13卷 516-527页

作者： Cintia Cristina Santi Martignago Beatriz soares-silva Julia Risso Parisi Lais Caroline Souza e silva Renata Neves Granito Alessandra Mussi Ribeiro Ana Cláudia Muniz Renno Lorena Ramos Freitas de Sousa Anna Caroline Campos Aguiar Departamento de Biociências Universidade Federal de São Paulo(UNIFESP)Rua Silva Jardim 136Edifício CentralSantosSP 11015-020Brazil Institute of Chemistry Federal University of Catalão(UFCAT)Av.Dr.Lamartine Pinto de Avelar1120 Vila ChaudCatalãoGO 75704-020Brazil

Marine biodiversity has emerged as a very promising resource of bioactive compounds and secondary metabolites from different sea *** sponge’s secondary metabolites demonstrated various bioactivities and potential pharmacological *** systematic review of the literature focuses on the advances achieved in the antioxidant potential of marine sponges in *** review was performed in accordance with PRISMA *** main inclusion criterion for analysis was articles with identification of compounds from terpene classes that demonstrate antioxidant activity in *** in three different databases,two hundred articles were *** screening abstracts,titles and evaluating for eligibility of manuscripts 14 articles were *** most performed analyzes to detect antioxidant activity were scavenging activity 2,2-diphenyl-1-picrylhydrazyl(DPPH)and measurement of intracellular reactive oxygen species(ROS).It was possible to identify 17 compounds of the terpene class with pronounced antioxidant activity in *** evidence of the studies included in this review was accessed by the GRADe *** play an important ecological role,moreover these molecules have a pharmaceutical and industrial application.

关键词： Antioxidant Marine drugs Sponge Free radical