86,630 research outputs found
Leucodistrofia metacromatica: un caso di pubertà precoce
No full textLeucodistrofia metacromatica: un caso di pubertà precoce
Nardecchia F 1
, Poratti E 1
, Dimiccoli P 1
, Di Maggio C 1
, Commone A 1
, Pucci C 1
, Leuzzi V 1
1DAI Neurosc e Sal Ment, Univ Sapienza, Roma
INTRODUZIONE/BACKGROUND: La leucodistrofia metacromatica è una malattia lisosomiale,
autosomica recessiva, caratterizzata
dall'accumulo di solfatidi a livello della mielina del sistema nervoso e, in minor quantità, nei tessuti
a funzione escretoria, tra cui reni, fegato e cistifellea. E’ stato descritto, inoltre, coinvolgimento di
tipo deficitario dell’ipofisi anteriore, surreni, testicoli, ovaie ed endometrio.
METODI / PAZIENTI:Il caso presentato è una bambina di 7,6 anni con esordio tipico intorno ai 2,6
anni, caratterizzato
da rapida regressione psicomotoria e ipotono. La RMN dell’encefalo mostarva diffusa e marcata
iperintensità di segnale della
sostanza bianca biemisferica sovratentoriale, con coinvolgimento del corpo calloso. Il dosaggio dell
dell’attività dell’arilsulfatasi A rivelava una riduzione del 91% e l’indagine genetica una eterozigosi
composta per [p.Tyr201Cys] e [p.Leu428Pro] nel gene ARSA. A 6 mesi dalla diagnosi comparsa di
episodi critici polimorfi. Intorno ai 4 anni osservato telarca
bilaterale isolato e incremento della lanuggine corporea, con successiva comparsa di pubarca.
RISULTATI:Il GnRH test evidenziava un’attivazione dell’asse ipotalamo-ipofisi- gonadi,
definendo un quadro di
pubertà precoce centrale. L’ecografia pelvica mostrava alcuni follicoli bilaterali con diametro di 3
mm. L’Rx polso evidenziava un’età ossea avanzata rispetto a quella cronologica, con uno scarto
superiore ad un anno. La progressione del quadro neurologico ha determinato un quadro di
tetraparesi spastico-distonica, assenza di linguaggio e residuo ed incostante contatto di sguardo.
Assume terapia antiepilettica con crisi plurisettimanali e trattamento ormonale con analoghi del
GnRH.
DISCUSSIONE:A differenza del riscontro di ipofunzionamento dell’asse ipotalamo-ipofisi- gonadi
descritto nelle
leucodistrofie e consensuale al processo degenerativo, si segnala in questo caso l’iperattivazione
dell’asse, finora non descritta in letteratura
Design of an inverted F antenna by using a transmission line model
No full textThis paper presents a transmission line model used to
design an inverted F antenna (IFA) at the frequency of 2.45 GHz.
This model provides a quickly design of a printed F antenna
antenna that can be easily integrated into handheld devices or
printed on PCMCIA cards for WLAN communications in the
ISM band. A simple iterative method which consider inductive or
capacitive loadings was adopted to design the antenna in
conjunction with a 3D EM simulators obtained a good solutions
to design IFA. A prototype of proposed planar inverted F
antenna (PIFA) has been manufactured on a FR4 substrate with
a bandwidth of 230 MHz in order to demonstrate the reliability
of the model validated by the good agreements with the
experimental and simulated measurements
Variations on the Author
No full text“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Synthesis of biodiesel via homogeneous Lewis acid catalyst
No full textNowadays, most biodiesel (fatty acids methyl esters, FAME) is produced by the transesterification of triglycerides (TG) of refined/edible
type oils using methanol and an homogeneous alkaline catalyst. However, production costs are still rather high compared with the ones of
petroleum-based diesel fuel. To lower costs and make biodiesel competitive less-expensive feedstocks such as waste fats or non-edible type
oils could be used. The use of homogeneous alkaline catalysts in the transesterification of such types of fats and oils poses great difficulties
due to the presence of large amounts of free fatty acids (FFA). This paper studies the use of carboxylic salts as a possible alternative, because
these catalysts are active also in the presence of high FFA concentrations.
The most active catalyst (Cd, Mn, Pb, Zn carboxylic salts) have been individuated and a correlation of the activities with the cation acidity
has been found.
© 2005 Elsevier B
[Newspaper Clipping: Author Claims Evidence of Second JFK Assassin #1]
No full textNewspaper article titled "Author Claims Evidence of Second JFK Assassin." The article states that author Richard J. Whalen concluded "that there is circumstantial evidence to support the theory of a second assassin in the shooting of President John F. Kennedy.
Study of quantum electron diffraction for the LISA test-mass charging
No full textThe future Laser Interferometer Space Antenna (LISA) for gravitational wave detection in space and the precursor LISA Pathfinder missions rely on metal test masses (TMs) playing the role of free-falling geodesic-reference and interferometer end mirrors. Charge deposited on the TMs by high-energy particles of galactic and solar origin couples with stray electric fields thus perturbing the free-falling geodesic motion. In previous works it has been shown that sub-keV electrons play a relevant role for TM charging. In particular, electrons with energies below 100 eV show a wave-like behavior when impacting on the gold coatings of the TMs and electrode-housing (EH), the capacitive position sensor/actuator that surrounds the TMs. We aim at studying the effects of quantum diffraction of electrons on LISA Pathfinder and LISA TMs and EH gold lattice. We calculate the quantum diffraction probability of electrons with energies < 100 eV impinging on gold lattice from different directions by using the methods of quantum mechanics. The effects of electron quantum diffraction on LISA TM charging are reported here for the first time. This work is also of interest for the second-generation LISA-like space interferometers and for all space missions whose performance relies on free-falling metal test masses
Also By The Same Author: AKTiveAuthor, a Citation Graph Approach to Name Disambiguation
No full textThe desire for definitive data and the semantic web drive for inference over heterogeneous data sources requires co-reference resolution to be performed on those data. In particular, name disambiguation is required to allow accurate publication lists, citation counts and impact measures to be determined. This paper describes a graph-based approach to author disambiguation on large-scale citation networks. Using self-citation, co-authorship and document source analyses, AKTiveAuthor clusters papers, achieving precision of 0.997 and recall of 0.818 over a test group of eight surname clusters
John F. Kennedy telegram to Roosevelt
No full textJersey Homesteads (later the Borough of Roosevelt) was established in the 1930s as an agro-industrial cooperative community. It was established specifically for urban Jewish garment workers, many of whom had emigrated from Europe. President John F. Kennedy sent a telegram to the citizens of Roosevelt, New Jersey, apologizing for not being able to attend the memorial dedication in honor of former President Franklin Delano Roosevelt. (Jersey Homesteads became Roosevelt in 1945 in honor of the president.) President Kennedy expressed his gratitude to the people of Roosevelt for constructing the memorial, and commented that it will serve as a constant reminder of Roosevelt's good works
Logarithmic variance profiles and the corresponding f-1 spectra of temperature fluctuations in turbulent Rayleigh-Bénard convection
No full textWe report experimental results for the temperature variance 2(z) and the corresponding frequency spectra P(f) in turbulent Rayleigh-Bénard convection (RBC) in a cylindrical sample of aspect ratioT= D/L = 1:00 (D = 1:12 m is the diameter and L = 1:12 m the height). The measurements were conducted in the Rayleigh-number range 1011 < Ra < 1:35 1014 and Pr ' 0:8. For Ra = 1:35x1014, 2(z) could be described well by a logarithmic dependence on the vertical position z in a range of z 1 < z < z 2 with z 1 ' 70 and z 2 = 0:1L. Here L=(2Nu) is the thickness of a thin thermal sublayer adjacent to the horizontal plate where the heat flux (denoted by the Nusselt number Nu) is carried mostly by thermal diffusion. In the log layer, we found that the temperature spectra had a significant frequency range over which P(f) f with close to 1. As Ra decreased, increased so that the log layer became thinner. At Ra = 2:05 1011, z 2 < z 1 and therefore there was no range for a log layer. Correspondingly, the temperature spectrum near the horizontal plate did not have the f1 scaling form either
Maine author Franklin F. Gould recalls his first glimpse of the outside world
No full textMaine author Franklin F. Gould recalls his first glimpse of the outside world as he relates how, as a young farm boy in the late 1800\u27s, he drove his father\u27s horses on an errand to an icebound river
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