575 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
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
Fortified settlements role in the western side of Gran Sasso d'Italia. The study case of Prata's Castle survey
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GR-2009-1606736 - Effect on professional practice and health care outcomes of computerized evidence-based information system and clinical decision support: a randomized controlled trial in orthopedics
No full textL’implementazione dei sistemi di supporto decisionale nella pratica clinica:
leve e ostacoli percepiti dai professionisti
Il progetto di ricerca dal titolo“L’implementazione dei sistemi di supporto decisionale nella pratica clinica: leve e ostacoli percepiti dai professionisti”è uno studio qualitativo promosso dall’IRCCS Istituto Ortopedico Galeazzi di Milano che ha l’obiettivo di indagare barriere e facilitatori all’adozione dei sistemi di supporto decisionale computerizzati percepiti dai potenziali utilizzatori delle tecnologie in questione, cioè i professionisti sanitari.
La ricerca è orientata da una metodologia qualitativa e consisterà nella somministrazione di interviste al personale clinico (medici e infermieri) e ad alcuni ruoli dirigenziali.
Lo studio verrà svolto presso tre centri: IRCCS Istituto Ortopedico Galeazzi di Milano; IRCCS IstitutoOrtopedico Rizzoli di Bologna; IRST Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori di Meldola. Le interviste saranno terminate entro il 31 maggio 2014.
Questa ricerca qualitativa fa parte dello studio CODES GR-2009-1606736, un progetto di ricerca finalizzata con finanziato dal Ministero della Salute e da Regione Lombardia, che vede come unità capofila l’IRCCS Istituto Ortopedico Galeazzi di Milano e tra i partner l’Università degli Studi del capoluogo lombardo,l’Istituto di Ricerche Farmacologiche Mario Negri, l’IRCCS Istituto Ortopedico Rizzoli di Bologna e l’IRCCS IRTS di Meldola
A fast, secure, reliable, and resilient data transfer framework for pervasive IoT applications
No full textThe number of online devices with on board sensors is growing steadily in the last years, leading to many solutions depending on data crowd-sourcing. In these scenarios, usually the collected data need to be transferred on a cloud infrastructure, where can later be aggregated and analyzed using specialized data processing algorithms. The simple task of data transfer becomes a problem in stressed environments such as space communication systems, drone monitoring or marine ecosystems, where the network connectivity is unstable and not available for long period of times. In this paper, we propose a novel Internet of Things (IoT) data transfer framework for cloud-based applications in delay tolerant environments. The framework implements a protocol that is i) elastic, taking advantage of short connection times; ii) lightweight, which makes it suitable for IoT devices; and iii) secure, which makes it a good choice also for applications with sensitive data. We evaluate the framework in a real system with a marine navigation application, demonstrating its benefits in terms of performance, resiliency, and security
Ricchi e poveri nel processo
No full textcommento ad uno del capitolo del volume "processo e garanzie della persona
FORTIFIED SETTLEMENTS ROLE IN THE WESTERN SIDE OF GRAN SASSO D’ITALIA LANDSCAPE STRUCTURE. THE STUDY CASE OF PRATA’S CASTLE SURVEY
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