2,333 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
Dalla mitologia del territorio ad una nuova epistemologia del museo: il caso Plymoth Plantation
No full textTecnologie innovative per lo sviluppo di radar e rayload per telecomunicazioni in banda W
No full text
Innovative technologies for the developments of W-band radars and communication payloads
No full textThe present work1,2, that has been performed in the frame of the ASI (Italian Space Agency) program WAVE (W-Band Analysis and VErification), i.e. the identification of innovative technologies to be used for the design and development of on-board W-band radars or telecommunication payloads. The paper will provide and comment on the major results achieved in this investigation. Based on the state of the art of the present technology the possibility of developing low level W band MMICs with many functions integrated on the same chip has been investigated in order to reduce drastically the number of interconnections, the number of measurements and the workmanship with respect to the equivalent hybrid subsystems, which means a drastic reduction in terms of dimensions, weight and costs. As for the power is concerned, an analysis of the performances of MMICs developed using state-of-the-art consolidated monolithic processes has been carried out together with the analysis of the potential performance of new "wide band-gap" semiconductors that start to play an important role in terms of power and efficiency. At W-band, WR-10 waveguide losses play a significant role in the management of the performance of transmitter and receiver chains and of antennas feeds. In order to reduce such losses different technologies have to be explored. One of those is quasi optics. Quasi-optics is the name given to the technique of manipulating mm-wave signals in free-space beams through the use of quasi-optical components such as lenses, mirrors, polarisers and so on. Quasi-optics (QO) has a number of advantages over other transmission techniques (such as waveguide and microstrip), including low loss propagation and wide bandwidth capacity. © 2006 IEEE
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