检索结果-南通市图书馆

Sturge–Weber syndrome:an update for the pediatrician

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 2024年第5期20卷 435-443页

作者： Emilie Dingenen damien Segers Hannelore De Maeseneer Dirk Van Gysel Ghent University Faculty of Medicine and Health Sciences GhentBelgium Department of Pediatrics O.L.Vrouw Hospital AalstMoorselbaan 1649300AalstBelgium Interdisciplinary Unit of Pediatric Dermatology Vrije Universiteit Brussel(VUB)Universitair Ziekenhuis Brussel(UZ Brussel)BrusselsBelgium

Sturge–Weber syndrome(SWS)is a rare congenital neurocutaneous disorder characterized by the simultaneous presence of both cutaneous and extracutaneous capillary malformations.SWS usually presents as a facial port-wine birthmark,with a varying presence of leptomeningeal capillary malformations and ocular vascular abnormalities.The latter may lead to significant neurological and ocular morbidity such as epilepsy and glaucoma.SWS is most often caused by a somatic mutation involving the G protein subunit alpha Q or G protein subunit alpha 11 gene causing various alterations in downstream signaling pathways.We specifically conducted a comprehensive review focusing on the current knowledge of clinical practices,the latest pathophysiological insights,and the potential novel therapeutic avenues they provide.Data sources A narrative,non-systematic review of the literature was conducted,combining expert opinion with a balanced review of the available literature.A search of PubMed,Google Scholar and Embase was conducted,using keywords“Sturge–Weber Syndrome”OR“SWS”,“Capillary malformations”,“G protein subunit alpha 11”OR“G protein subunit alpha Q”.Results One of the hallmark features of SWS is the presence of a port-wine birthmark at birth,and forehead involvement is most indicative for SWS.The most common ocular manifestations of SWS are glaucoma and choroidal hemangioma.Glaucoma presents in either in infancy(0–3 years of age)or later in life.Neurological complications are common in SWS,occurring in about 70%–80%of patients,with seizures being the most common one.SWS significantly impacts the quality of life for patients and their families,and requires a multidisciplinary approach for diagnosis and treatment.Currently,no disease-modifying therapies exist,and treatment is mostly focused on symptoms or complications as they arise.Conclusions SWS remains a complex and heterogeneous disorder.Further research is needed to optimize diagnostic and therapeutic strategies,and to translate insights from molecular pathogenesis to clin

关键词： Capillary malformations G-protein Gq-G11 Port-wine birthmark Neurocutaneous disorder Sturge-Weber syndrome

Multiple origin of the lichenicolous life habit in Helotiales,based on nuclear ribosomal sequences

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Fungal Diversity 2015年第1期70卷 55-72页

作者： Ave Suija damien ertz James D.Lawrey Paul Diederich Institute of Ecology and Earth Sciences University of Tartu40 Lai st.EE-51005 TartuEstonia Département Bryophytes et Thallophytes Jardin botanique national de BelgiqueDomaine de BouchoutB-1860 MeiseBelgium Department of Environmental Science and Policy George Mason UniversityMSN 5G810900 University BoulevardManassasVA 20110-2203USA Musée national d’historie naturelle 25 rue MunsterL-2160 LuxembourgLuxembourg

The Helotiales are an ecologically and morphologically highly diverse group of ascomycetes that also includes lichen-inhabiting(lichenicolous)species.We generated sequence data of three rDNA regions(nuSSU,nuLSU,5.8S of ITS)from 28 lichenicolous specimens representing nine genera in order to determine their phylogenetic placement.Based on the most complete dataset of helotialean fungi to date,the analyses were performed using Maximum Likelihood(ML)and Bayesian approaches.Our results suggest that 1)the lichen-inhabiting life-style in Helotiales was subjected to gains or losses at least three times;2)Thamnogalla,previously tentatively included in Ostropales,is shown to belong to Helotiales;3)ascomata found intermixed with pycnidia of the asexual Diplolaeviopsis ranula and possessing the same pigments are tentatively considered as the sexual morph of this taxon,and are shown to belong to Helotiales;4)the lichenicolous species of cf.Diplolaeviopsis,Llimoniella,Rhymbocarpus,Skyttea,Thamnogalla and Unguiculariopsis form a well-supported clade together with non-lichenicolous encoelioid fungi from the genera Ionomidotis,Cordierites and Encoelia;5)Geltingia associata forms a highly supported clade with the fungicolous asexual fungus Eleutheromyces subulatus,whereas Pezizella epithallina clusters with an aquatic asexual fungus Tetracladium sp.;6)Phaeopyxis punctum belongs to Ostropomycetidae(Lecanoromycetes),but its deep relationships to other groups remain unresolved based on rDNA sequences.

关键词： Convergence Helotiales rDNA Taxonomy Diplolaeviopsis Geltingia Llimoniella Pezizella Phaeopyxis Rhymbocarpus Skyttea Thamnogalla Unguiculariopsis

Antimicrobial stewardship and targeted therapies in the changing landscape of maternal sepsis

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 Intensive Medicine 2024年第1期4卷 46-61页

作者： Nishel M Shah Esmita Charani damien Ming Fook-Choe Cheah Mark R Johnson Department of Metabolism Digestion and ReproductionImperial College LondonChelsea and Westminster HospitalLondonUK Health Protection Research Unit in Healthcare Associated Infection and Antimicrobial Resistance Imperial College LondonLondonUK Division of Infectious Diseases and HIV Medicine Department of MedicineGroote Schuur HospitalUniversity of Cape TownCape TownSouth Africa Department of Infectious Diseases Imperial College LondonChelsea and Westminster HospitalLondonUK Department of Paediatrics Faculty of MedicineUniversiti Kebangsaan Malaysia Medical CentreKuala LumpurMalaysia

Pregnant and postnatal women are a high-risk population particularly prone to rapid progression to sepsis with significant morbidity and mortality worldwide.Moreover,severe maternal infections can have a serious detrimental impact on neonates with almost 1 million neonatal deaths annually attributed to maternal infection or sepsis.In this review we discuss the susceptibility of pregnant women and their specific physiological and immunological adaptations that contribute to their vulnerability to sepsis,the implications for the neonate,as well as the issues with antimicrobial stewardship and the challenges this poses when attempting to reach a balance between clinical care and urgent treatment.Finally,we review advancements in the development of pregnancy-specific diagnostic and therapeutic approaches and how these can be used to optimize the care of pregnant women and neonates.

关键词： Sepsis Pregnancy Immunology Cardiovascular Personalised medicine Antibiotic stewardship

Molecular data resolve a new order of Arthoniomycetes sister to the primarily lichenized Arthoniales and composed of black yeasts, lichenicolous and rock-inhabiting 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

Fungal Diversity 2014年第3期66卷 113-137页

作者： damien ertz James D.Lawrey Ralph S.Common Paul Diederich Jardin botanique national de Belgique Domaine de BouchoutB-1860 MeiseBelgium Department of Environmental Science and Policy George Mason University4400 University DriveFairfaxVA 22030-4444USA 534 Fenton St LansingMI 48910USA P.Diederich Musée national d’histoire naturelle 25 rue MunsterL-2160 LuxembourgLuxembourg

Lichenicolous fungi belonging to the anamorphtypified genus Phaeosporobolus and to the teleomorph-typified genus Lichenostigma were isolated in pure culture or sequenced directly,with nuLSU and mtSSU sequences obtained.Phylogenetic analyses place the species of Phaeosporobolusin a strongly supported clade with the generic type of Lichenostigma(L.maureri),the genus Phaeococcomyces and several melanized rock-inhabiting isolates.This strongly supported nonlichenized lineage is sister to the primarily lichenized Arthoniales in the Arthoniomycetes and is here described as the Lichenostigmatales.The new order is characterized by cells multiplying by budding,either representing black yeasts,or species in which conidiomata and ascomata are entirely made of an organised agglomeration of spherical yeast-like cells.This way of life is not only very different from all other Arthoniomycetes that exist only in the mycelial stage,but ascomata and conidiomata representing a dense and organised agglomeration of yeast cells might be unique amongst fungi.A further difference with the Arthoniales is the absence of paraphysoids.Phylogenetic results suggest that Phaeosporobolus usneae is the asexual stage of Lichenostigma maureri.Most species of Phaeosporobolus are transferred to the genus Lichenostigma except P.trypethelii,for which the new genus Etayoa is described.The genus Diederimyces is reduced into synonymy with Lichenostigma.Several other members of Lichenostigma are placed in the Dothideomycetes and are intermixed with Lichenothelia species.

关键词： Anamorphic fungi Arthoniomycetes Dothideomycetes Lichenostigmatales Phaeococcomycetaceae Yeasts

Molecular data place the hyphomycetous lichenicolous genus Sclerococcum close to Dactylospora (Eurotiomycetes) and S. parmeliae in Cladophialophora (Chaetothyriales)

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Fungal Diversity 2013年第1期58卷 61-72页

作者： Paul Diederich damien ertz James D.Lawrey Masoumeh Sikaroodi Wendy A.Untereiner Musée national d’histoire naturelle 25 rue MunsterL-2160LuxembourgLuxembourg Jardin botanique national de Belgique Département bryophytes-thallophytesDomaine de BouchoutB-1860MeiseBelgium Department of Environmental Science and Policy George Mason University4400 University DriveFairfaxVA 22030-4444USA Department of Biology Brandon UniversityBrandonMB R7A 6A9Canada

The lichenicolous anamorphic fungus Sclerococcum parmeliae was isolated in pure culture,and ITS,nuLSU and mtSSU sequences were obtained from these isolates.For comparison,sequences from S.sphaerale,the generic type,were obtained directly from freshly collected specimens.Phylogenetic analyses place S.sphaerale with species of Dactylospora and an unidentified lichen-inhabiting isolate in a strongly supported clade that is sister to a lineage comprising members of the Chaetothyriales and Pyrenulales.In contrast,S.parmeliae is inferred as a member of the Herpotrichiellaceae(Chaetothyriales)and belongs to a robustly supported clade that also includes species of Cladophialophora,Capronia semiimmersa,and Phialophora verrucosa.Within the Herpotrichiellaceae,S.parmeliae most closely resembles members of the anamorph genus Cladophialophora.Accordingly,we propose the transfer of S.parmeliae and the morphologically similar species S.cladoniae,S.hawksworthii and S.normandinae to Cladophialophora.A new lichenicolous species,Clad.megalosporae,collected twice on Megalospora in Florida and Papua New Guinea,is also described.

关键词： Anamorphic fungi Capronia Conidial fungi Hyphomycetes Parasitic fungi

A large-scale phylogenetic revision of Roccellaceae(Arthoniales)reveals eight new genera

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Fungal Diversity 2015年第1期70卷 31-53页

作者： damien ertz Anders Tehler Martin Irestedt Andreas Frisch Göran Thor Pieter van den Boom Jardin Botanique National de Belgique Département Bryophytes-ThallophytesDomaine de Bouchout1860 MeiseBelgium Naturhistoriska riksmuseet Enheten för botanikBox 5000710405 StockholmSweden Naturhistoriska riksmuseet Enheten för bioinformatik och genetikBox 5000710405 StockholmSweden Department of Botany National Museum of Nature and Science4-1-1 AmakuboTsukuba-CityIbarakiJapan 305-0005 Department of Ecology Swedish University of Agricultural SciencesP.O.Box 704475007 UppsalaSweden Arafura 16 5691 JA SonThe Netherlands

A two-locus phylogenetic hypothesis of the family Roccellaceae is presented based on data from the nuclear ribosomal large subunit(nucLSU)and the second largest subunit of RNA polymerase II(RPB2).This analysis includes 341 sequences(166 newly generated)and 180 specimens representing about 114 species.The genera Lecanactis,Roccellina,Schismatomma and Sigridea were found to be paraphyletic/polyphyletic.In order to make these groups monophyletic,the new genera Crocellina,Diromma,Gyrographa,Gyronactis,Ocellomma,Pseudoschismatomma,Psoronactis and Vigneronia are described.The genus Sagenidium is placed in synonymy with Lecanactis.The new species Enterographa incognita,Gyronactis asiatica and Lecanactis submollis are described.Several species of Opegrapha are transferred to the Roccellaceae.Sorediate morphs are recorded for the first time in the genus Syncesia.

关键词： Arthoniomycetes Lichenized fungi Molecular phylogenetics New genera Taxonomy

Briancoppinsia,a new coelomycetous genus of Arthoniaceae(Arthoniales)for the lichenicolous Phoma cytospora,with a key to this and similar taxa

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Fungal Diversity 2012年第1期52卷 1-12页

作者： Paul Diederich James D.Lawrey Masoumeh Sikaroodi Pieter P.Gvan den Boom damien ertz Musée national d’histoire naturelle 25 rue Munster2160 LuxembourgLuxembourg Department of Environmental Science and Policy George Mason University4400 University DriveFairfaxVA 22030-4444USA Arafura 16 5691 JA Sonthe Netherlands Jardin Botanique National de Belgique Domaine de Bouchout1860 MeiseBelgium

Morphological,anatomical,chemical and molecular data suggest that a relatively common lichenicolous coelomycete on Lecanora conizaeoides is conspecific with Phoma cytospora,previously known only from parmelioid lichens,and that further populations on Cladonia and Pertusaria belong to the same species.This species is distinguished from Phoma by several taxonomically important characters and obviously represents a previously unrecognized genus,for which the name Briancoppinsia is introduced.Phylogenetic analyses using nuLSU and mtSSU sequences of isolates obtained in pure culture suggest that the new genus belongs to the Arthoniaceae(Arthoniales).This is the first obligate lichenicolous,non-lichenized anamorph confirmed to belong to the Arthoniales based on molecular data.

关键词： Anamorphic fungi Arthonia Conidial fungi Mitosporic fungi

Phylogenetic placement of lichenicolous Phoma species in the Phaeosphaeriaceae(Pleosporales,Dothideomycetes)

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Fungal Diversity 2012年第4期55卷 195-213页

作者： James D.Lawrey Paul Diederich Matthew P.Nelsen Colin Freebury Dries Van den Broeck Masoumeh Sikaroodi damien ertz Department of Environmental Science and Policy George Mason University4400 University DriveFairfaxVA 22030-4444USA Musée national d’histoire naturelle 25 rue MunsterL-2160 LuxembourgLuxembourg Committee on Evolutionary Biology University of Chicago1025 E.57th StreetChicagoIL 60637USA Department of Botany The Field Museum1400 S.Lake Shore DriveChicagoIL 60605USA Canadian Museum of Nature P.O.Box 3443Station DOttawaON K1P 6P4Canada Jardin Botanique National de Belgique Domaine de BouchoutB–1860 MeiseBelgium

More than twenty species of lichenicolous fungi have been described in Phoma,a large anamorphic genus of primarily plant-associated pathogens with broad geographic distributions.We obtained nuclear and mitochondrial rDNA sequences from 19 fungal cultures isolated from specimens representing four described and two undescribed lichenicolous species in the genus.Our multilocus phylogeny indicates that lichenicolous Phoma species represent at least two phylogenetically distinct clades in the Phaeosphaeriaceae,one including a new species,Phoma puncteliae,isolated from a specimen of Punctelia rudecta collected inMaryland,USA,and another group of primarily lichenicolous species.This latter group includes four described lichenicolous Phoma species,an unidentified melanized rock fungus,and a new lichenicolous Phoma species isolated from Xanthomendoza species collected in Canada that we are naming P.xanthomendozae.Some specimens in this clade collected from different lichen genera and species were found to be very similar genetically,which calls into question the recent practice of recognizing lichenicolous Phoma species mainly by differences in host preference.

关键词： Anamorphic fungi Coelomycetes Conidial fungi Parasitic fungi

Primordial black hole mass functions as a probe of cosmic origin

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Science China(Physics,Mechanics & Astronomy) 2024年第5期67卷 164-171页

作者： Yi-Fu Cai Chengfeng Tang Geyu Mo Sheng-Feng Yan Chao Chen Xiao-Han Ma Bo Wang Wentao Luo damien A.Easson Antonino Marcianò Deep Space Exploration Laboratory/School of Physical Sciences University of Science and Technology of ChinaHefei 230026China CAS Key Laboratory for Researches in Galaxies and Cosmology/Department of Astronomy School of Astronomy and Space ScienceUniversity of Science and Technology of ChinaHefei 230026China Istituto Nazionale di Fisica Nucleare(INFN) Sezione di MilanoMilano 20146Italy DiSAT Universitàdegli Studi dell'InsubriaComo 22100Italy Jockey Club Institute for Advanced Study The Hong Kong University of Science and TechnologyHong KongChina Department of Physics Arizona State UniversityTempe 85287-1504USA Department of Physics&Center for Field Theory and Particle Physics Fudan UniversityShanghai 200433China Laboratori Nazionali di Frascati INFN Via Enrico Fermi 541 Frascati(Roma)00044Italy INFN sezione Roma Tor Vergata Rome I-00133Italy School of Physics University of Electronic Science and Technology of ChinaChengdu11731China

We discuss a novel window to probe the origin of our universe via the mass functions of primordial black holes(PBHs).The mass functions of PBHs are simply estimated using the conventional Press-Schechter formalism for two paradigms of cosmic origin,including inflationaryΛCDM and bounce cosmology.The standard inflationaryΛCDM model cannot generate an appreciable number of massive PBHs;however,non-trivial inflation models with blue-tilted power spectra at small scales and matter bounce cosmology provide formation mechanisms for heavy PBHs,which in turn,may seed the observed supermassive black holes(SMBHs).By fitting the SMBH mass functions at high redshift(z~6)derived from Sloan Digital Sky Survey(SDSS)and Canada-France High-z Quasar Survey(CFHQS)quasars,for two paradigms of cosmic origin,we derive constraints on the PBH density fraction fPBHat z~6 and the characteristic mass M_(★),with the prior assumption that all SMBHs stem from PBHs.We demonstrate that this newly proposed procedure,relying on astronomical measurements that utilize deep-field surveys of SMBHs at high redshift,can be used to constrain models of cosmic origin.Additionally,although not the main focus of this paper,we evolve the mass function from z~6 to z~0 through an assumption of 3×10^(8)-year Eddington’s accretion,and give a rough estimation of fPBHat z~0.

关键词： origin cosmic massive