1,362,596 research outputs found
Spectrum of skeletal abnormalities in a complex malformation syndrome with "cutis tricolor" (Ruggieri-Happle syndrome)
No full textBackground: The term cutis tricolor describes the combination of congenital hyper- and hypopigmented skin lesions in close proximity to each other in a background of normal complexion. This phenomenon has been reported: (i) as a purely cutaneous trait; (ii) as a part of a complex malformation syndrome (Ruggieri-Happle syndrome - RHS); (iii) as a distinct type [cutis tricolor parvimaculata]; (iv) in association with other (e.g. vascular) skin disturbances. Objectives: To delineate the spectrum of skeletal defects in cutis tricolor. Methods: Retrospective and prospective analysis of skeletal surveys in 14 subjects (eight men; six women; aged 2-28 years) with cutis tricolor [4 purely cutaneous trait; 10 syndromic (RHS)]. Results: Bone abnormalities were recorded in 71.4% (10/14) of patients [100% (10/10) of cases with (other-than-skeletal) extra-cutaneous manifestations vs. null (0/4) in cases with purely cutaneous traits] and included overall small skull (n = 6); prognathism (n = 6); 'J'-shaped pituitary fossa (n = 1); absence of atlas posterior arch (n = 3); frontal bossing (n = 6); scoliosis (n = 9) with kyphosis (n = 6) and/or lordosis (n = 6); vertebral (n = 9) and ribs (n = 4) defects. Negative ZFHX1B gene analyses excluded overlaps with Mowat-Wilson syndrome. Conclusions: Cutis tricolor may be a marker of underlying skeletal involvement particularly in subjects with a complex syndromic (RHS) phenotype. © 2010 The Author(s)/Acta Pædiatrica © 2010 Foundation Acta Pædiatrica
Italy
No full textIl volume è uno studio comparativo degli ordinamenti giuridici dei 27 Stati membri dell'Unione europea. Il contributo di cui il sottoscritto risulta co-autore insieme a Ruggieri F. espone in inglese, in un formato comune, i tratti essenziali della procedura penale italiana
Egger, Dujardin, Joyce, Microscopia della voce nel monoilogo interiore
No full textPubblicato nella sezione "Piccola Biblioteca Joyciana" della "Collana di Studi Joyciani", a cura di Franca Ruggieri, il volume si avvale di una "Prefazione" di Jcqueline Risset e di una "Introduzione" diPierre Léon
A Cross-Cultural Experience in Tourism Studies
No full textThe present article is the result of a joint teaching experience carried out by Giovanni Ruggieri, lecturer of Tourism Economics at the University of Palermo, and Ninfa Pagano, lecturer of English language, University of Palermo; the experience was addressed to our students of Tourism Studies at the Department of Economics, University of Palermo.
The rationale at the basis of this teaching experience was to provide Italian students with extra language practice applied to more than one of their main fields of study in order to show how two different subjects, i.e. Tourism Economics and the English language, can be linked, thus working as a reinforcement for students in both areas.
Prof. Ruggieri suggested proposing to our students the reading of an article in English which focused on a comparative analysis of tourism in the Mediterranean islands; the article took into consideration aspects such as bed-place capacity and occupancy rates, strategies to develop sustainable tourism and tourism policies.
Students were asked to analyse it and parallelly to deepen the understanding of the English text through a series of language activities aiming to improve comprehension, lexis expansion and aspects of English syntax.
The objectives we set were thus twofold: improving our students’ knowledge about aspects concerning Tourism Economics while increasing their linguistic competence in English. Such cross-cultural experience has recently been introduced in Italian schools as well with the name of CLIL (Content and Language Integrated Learning). However, it had seldom been proposed at university level, so we carried it out on an experimental basis
An aggregate AQI: comparing different standardizations and introducing a variability index
No full textMany studies demonstrate a strong relationship between air pollution and respiratory and cardiovascular
diseases. For this reason, assessing air pollution, and conveying information about its possible adverse health
effects, may encourage population and policy makers to reduce those activities increasing pollution levels. In
this paper a relative index of variability, to be associated with the aggregate Air Quality Index (AQI) among
pollutants proposed by Ruggieri and Plaia (2011), is developed in order to better investigate air pollution
conditions for the whole area of a city/region. The most widely-used and up to date pollution indices,
based mainly on AQI computed by the US Environmental Protection Agency (EPA) and often defined by
the value of the pollutant with the highest (opportunely standardized) concentration on a given day, aim
at warning the people for short term health impact. An aggregate AQI, taking into account the combined effects
of all the considered pollutants, gives emphasis to possible chronic health effects and long term damages
on environment caused by air pollution. The proposed index of variability adds precious information to the
aggregate AQI, as it allows one to know whether the value assumed by the AQI is influenced by one or
more pollutants. The two indices are jointly used on simulated data, considering different possible scenarios.
Applications to real air pollution data are also reported. Before applying the two indices, the effects of different
standardizations on data are evaluated from a theoretical point of view
Complex malformation (Ruggieri-Happle) phenotype with "cutis tricolor" in a 10-year-old girl
No full textThe term cut is tricolor describes the combination of congenital hyper- and hypo-pigmented skin lesions in close proximity to each other in a background of normal complexion. It is currently regarded as a twin-spotting phenomenon and today is clear that not all cases of cutis tricolor represent one single entity. This phenomenon has been reported so far: (a) as an isolated skin manifestation; (b) as a part of a complex malformation syndrome (Ruggieri-Happle syndrome - RHS); (c) as a distinct phenotype [cut is tricolor parvimaculata]; (d) in association with other (e.g., vascular) skin disturbances. We report a novel case of cutis tricolor in a 10-year-old girl who had dysmorphic facial features [alike those seen in cases with syndromic (RHS) cutis tricolor], overall overgrowth [weight, length, and head circumference were >90th percentile; there was increased bone age], mild cognitive delay (current IQ = 55), behavioural disturbances, febrile seizures and (later) partial complex epilepsy (currently under good control), and skeletal defects [i.e., posterior scalloping of the lumbar vertebrae]. We discuss the main similarities and differences between the various phenotypes in the spectrum of cutis tricolor and with other conditions sharing features with the present case. (C) 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved
Ruggieri, Costantino
No full textBiografia di Costantino Ruggieri tipografo attivo a Roma nel XVIII secol
AN ASSOCIATION INDEX FOR RECTANGULAR CONTINGENCY TABLES WITH ORDERED/UNORDERED VARIABLES
No full textIn a previous work, Mineo and Ruggieri (2005) introduce a new index to measure the association in square contingency tables; in this paper such an index is extended to rectangular tables, preserving the same properties. The considered domain includes all the contingency tables with equal n (the total number of observations), since the tables of maximum dependence, useful for computing the denominator of the proposed index, belong to it. The main findings, which prove the effectiveness of the proposed measure, are presented. In particular, the new measure assumes values in the range [-1,+1], taking value zero in tables with distributive independence and positive/negative values if association/dissociation occurs. The importance of the sign for an association index computed on rectangular contingency tables and the need to recognize association from dissociation is explained. It is also shown as the absolute value of the new measure is invariant to permutation of rows and/or columns of the observed table. Finally, some relevant examples and case studies are reported
Psychology of Reflexivity and reflexivity for psychology
No full textA long tradition has produced a misleading interpretation of the categories
of idiographic and nomothetic. Such an interpretation has hindered
the development of psychology and more in general of social science. “Idiographic”
has been treated as a matter of identity, an ideological approach
that continues to be reproduced through the conflict with what-is-outside-it
viii S. Salvatore, G. Marsico, and R. Andrisano-Ruggieri
(i.e., nomothetic mainstream psychology). Far from such a militant ritualization
of the concept, YIS aims at promoting a return to the origin of
what Windelband (1904/1998) proposed, namely the idea of idiographic
and nomothetic as categories depicting two complementary—rather than
alternative - forms of knowledge building
Turismo relazionale
No full textIl turismo, inteso come viaggio di conoscenza e come svago, esiste da
sempre, almeno da quando l’uomo ha manifestato l’esigenza di scoprire
quello che gli stava intorno, di allargare quindi la propria sfera di
conoscenza e di influenza nel territorio circostante.
Con il suo aprirsi “a tutti” e con l’introduzione dell’aspetto simbolico
dello status sociale, il turismo ha assunto una connotazione sempre più
di“moda”, avviandosi in una direzione gestita da logiche industriali di
“massa”2. Logiche economiche centrate sulla ricerca del massimo profitto,
come unico parametro valido per la misura della performance aziendale
che mostrano oggi, quanto mai prima, i loro difetti e le loro profonde
contraddizioni, soprattutto nel confronto con le logiche dello sviluppo
sostenibile. Uno sviluppo che per essere realmente sostenibile deve farsi
promotore della conservazione e della valorizzazione della tipicità e della
diversità di un territorio e quindi allargare il campo all’entrata di nuovi
valori che negano la sola applicazione della logica del profitto.
Una connotazione, quella industrialista, che col passare del tempo è
arrivata a sostituire gli originari scopi del viaggiatore, facendo assumere al
termine turista, una palese accezione negativa, proprio ad indizio
dell’indifferenza ai luoghi e alle culture indotta da quella forma di turismo.
Per nostra fortuna la funzione e gli scopi originari del viaggio non
saranno cancellati da questa ondata di turismo di massa, ma resteranno
relegati per alcuni decenni in una nicchia riservata a pochi specialisti,
studiosi e ricercatori.
Con il diffondersi di una cultura orientata verso la salvaguardia e la
promozione delle risorse locali e, parallelamente, con l’avvento delle
tecnologie della comunicazione, in particolar modo della telematica e
dell’informatica, quindi nel corso dell’ultimo ventennio, si è timidamente
manifestata una nuova inversione di tendenza verso una riappropriazione
dello spirito originario del turismo, ossia: conoscenza, descrizione,
scambio. Il più delle volte percorrendo strade e modalità anche molto
differenti fra loro
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