Search results for "GALS"
showing 10 items of 24 documents
Candida blood stream infections observed between 2011 and 2016 in a large Italian University Hospital: A time-based retrospective analysis on epidemi…
2019
Candida bloodstream infection (BSI) represents a growing infective problem frequently associated to biofilm production due to the utilization of intravascular devices. Candida species distribution (n = 612 strains), their biofilm production and hospital antifungal drug consumption were evaluated in different wards of a tertiary care academic hospital in Italy during the years 2011–2016. In the considered time window, an increasing number of Candida BSI (p = 0.005) and of biofilm producing strains were observed (p<0.0001). Although C. albicans was the species more frequently isolated in BSI with a major biofilm production, an increased involvement of non-albicans species was reported, partic…
Bridged Epipolythiodiketopiperazines from Penicillium raciborskii, an Endophytic Fungus of Rhododendron tomentosum Harmaja
2016
Three new epithiodiketopiperazine natural products [outovirin A (1), outovirin B (2), and outovirin C (3)] resembling the antifungal natural product gliovirin have been identified in extracts of Penicillium raciborskii, an endophytic fungus isolated from Rhododendron tomentosum. The compounds are unusual for their class in that they possess sulfide bridges between α- and β-carbons rather than the typical α-α bridging. To our knowledge, outovirin A represents the first reported naturally produced epimonothiodiketopiperazine, and antifungal outovirin C is the first reported trisulfide gliovirin-like compound. This report describes the identification and structural elucidation of the compounds…
The effect of galsulfase enzyme replacement therapy on the growth of patients with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome).
2017
Mucopolysaccharidosis (MPS) VI is an autosomal recessive lysosomal storage disorder arising from deficient activity of N-acetylgalactosamine-4-sulfatase (arylsulfatase B) and subsequent intracellular accumulation of the glycosaminoglycans (GAGs) dermatan sulfate and chondroitin-4-sulfate. Manifestations are multi-systemic and include skeletal abnormalities such as dysostosis multiplex and short stature. Reference height-for-age growth charts for treatment-naive MPS VI patients have been published for both the slowly and rapidly progressing populations. Categorization of disease progression for these charts was based on urinary GAG (uGAG) level; high (>200μg/mg creatinine) levels identified …
Analysis of Renal and Cardiac Outcomes in Male Participants in the Fabry Outcome Survey Starting Agalsidase Alfa Enzyme Replacement Therapy Before an…
2020
Rossella Parini,1,2 Guillem Pintos-Morell,3 Julia B Hennermann,4 Ting-Rong Hsu,5 Nesrin Karabul,6 Vasiliki Kalampoki,7 Andrey Gurevich,7 Uma Ramaswami8 On behalf of the FOS Study Group1Rare Metabolic Diseases Unit, MBBM Foundation, San Gerardo Hospital, Reference Centre for Hereditary Metabolic Disorders (MetabERN), Monza, Italy; 2TIGET Institute, IRCCS San Raffaele Hospital, Milan, Italy; 3Division of Rare Diseases, Reference Centre for Hereditary Metabolic Disorders (MetabERN), University Hospital Vall d&rsquo;Hebron, Autonomous University of Barcelona, Barcelona, Spain; 4Villa Metabolica, Department of Pediatric and Adolescent Medicine, University Medical Center, Mainz, Germany; 5Dep…
A 4-year study of the efficacy and tolerability of enzyme replacement therapy with agalsidase alfa in 36 women with Fabry disease
2009
Although Fabry disease is X linked and considered to affect primarily male hemizygotes, female heterozygotes may experience all the signs and symptoms of this metabolic disorder. This prospective, single-center, open-label, clinical trial was performed to evaluate the long-term response of female patients with Fabry disease to enzyme replacement therapy.Symptomatic women (average age = 47 years) enrolled in this 4-year study were treated with agalsidase alfa (Replagal, Shire HGT, Inc.) at a dose of 0.2 mg/kg, every other week for 4 years (N = 36). Clinical and biochemical assessments were conducted at 12-month intervals.The Mainz Severity Score Index, a measure of total disease burden, was …
El monument al mestre Serrano a Sueca per Vicente Beltrán: entusiasme inicial, construcció i problemes legals
2018
Aceites esenciales: productos antimicrobianos y antioxidantes naturales en la industria agroalimentaria
2020
[ES] Los consumidores son conscientes del peligro derivado del uso de antioxidantes y antimicrobianos sintéticos en la industria agroalimentaria, demandando alternativas más seguras y ecológicas. En este estudio, se ha determinado la actividad antioxidante de aceites esenciales comerciales mediante el método DPPH y su efecto antimicrobiano frente a la bacteria Pseudomonas syringae y el hongo fitopatógeno Fusarium oxysporum a través del empleo del método estandarizado de disco. Los aceites esenciales de clavo, ajedrea, canela y orégano, así como carvacrol, mostraron la máxima actividad antioxidante, comparable a antioxidantes establecidos. El aceite esencial de gaulteria fue el más potente i…
Respuesta canonica, y theologica, al memorial que dio al Rey ... Fr. Francisco Manuel de Zuñiga, Obispo de Ciudad Rodrigo ... del Orden de San Agust…
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On the impact of within-die process variation in GALS-Based NoC Performance
2012
[EN] Current integration scales allow designing chip multiprocessors (CMP), where cores are interconnected by means of a network-on-chip (NoC). Unfortunately, the small feature size of current integration scales causes some unpredictability in manufactured devices because of process variation. In NoCs, variability may affect links and routers causing them not to match the parameters established at design time. In this paper, we first analyze the way that manufacturing deviations affect the components of a NoC by applying a new comprehensive and detailed within-die variability model to 200 instances of an 8¿8 mesh NoC synthesized using 45 nm technology. Later, we show that GALS-based NoCs pr…
Triunfo de la razón sobre las funestas ilusiones de estos últimos tiempos : obra en varios discursos polémicos
Fris, filets Text a 1 col Sign.: [ ]6, A-B8, C9