Prenatal ultrasound diagnosis of a case of Pfeiffer syndrome without cloverleaf skull and review of the literature

Pfeiffer syndrome is characterized by bilateral coronal craniosynostosis, midface hypoplasia, beaked nasal tip, broad and medially deviated thumbs and great toes. Originally, it was described in eight persons from three generations in a pedigree consistent with an autosomal dominant transmission. Since then, several reports have documented its high clinical and genetic heterogeneity. The condition is usually detected in the newborn period or later, and very few prenatal ultrasound diagnoses have been reported. We present a case of Pfeiffer syndrome prenatally diagnosed at 20 weeks' gestation, in which the sonographic features of craniosynostosis, hypertelorism associated with an extreme proptosis, and broad thumb led to the diagnosis, confirmed after termination of pregnancy by dysmorphological, pathological and radiological evaluation. DNA analysis of the fibroblast growth factor receptor 2 (FGFR2) showed a missense mutation consisting in a transversion G → C at nucleotide 870. This led to a Trp290Cys amino acidic substitution. We discuss the relevant findings of our and previously published cases. Our report demonstrates that a careful sonographic examination can lead to an early prenatal diagnosis of Pfeiffer syndrome also in cases without cloverleaf skull. Copyright © 2004 John Wiley & Sons, Ltd.     

Exploratory field trial of motorcycle autonomous emergency braking (MAEB): Considerations on the acceptability of unexpected automatic decelerations

Objective: Autonomous emergency braking (AEB) acts to slow down a vehicle when an unavoidable impending collision is detected. In addition to documented benefits when applied to passenger cars, AEB has also shown potential when applied to motorcycles (MAEB). However, the feasibility of MAEB as practically applied to motorcycles in the real world is not well understood.

Methods: In this study we performed a field trial involving 16 riders on a test motorcycle subjected to automatic decelerations, thus simulating MAEB activation. The tests were conducted along a rectilinear path at nominal speed of 40 km/h and with mean deceleration of 0.15 g (15% of full braking) deployed at random times. Riders were also exposed to one final undeclared brake activation with the aim of providing genuinely unexpected automatic braking events.

Results: Participants were consistently able to manage automatic decelerations of the vehicle with minor to moderate effort. Results of undeclared activations were consistent with those of standard runs.

Conclusions: This study demonstrated the feasibility of a moderate automatic deceleration in a scenario of motorcycle travelling in a straight path, supporting the notion that the application of AEB on motorcycles is practicable. Furthermore, the proposed field trial can be used as a reference for future regulation or consumer tests in order to address safety and acceptability of unexpected automatic decelerations on a motorcycle.     

Mapping forest ecosystem functions for landscape planning in a mountain Natura2000 site,Central Italy

In last decades, numerous efforts have been carried out by the scientific community to assess the multifunctional role of forests in supporting the forest decision-making processes. Recently developed in Italy, the Forest Landscape Management Planning seems to be the most suitable tool to maintain the sustainability balance between forests and local populations. This research identifies and maps different forest ecosystem functions in a mountain Natura2000 Network site by performing a spatial estimation of forest inventory parameters, and implementing a multi-criteria and multi-level approach. Final results on mapping of forest functions show acceptable values of classification accuracy.     

Siting landfills and incinerators in areas of historic unpopularity: Surveying the views of the next generation

The Campania Region in Southern Italy has suffered many problems with municipal solid waste management since the mid-1990s, leading to significant public disturbances and subsequent media coverage. This paper reports on the current views and knowledge of young people (university students) in this region about waste management operations and facilities, specifically the siting of landfills and incinerators. By means of a structured questionnaire, opinion and knowledge were systematically examined by degree type and course year. The study took place in 2011 at the University of Salerno campus. A sample of 900 students, comprising 100 students for each of the nine considered faculties, and 20 students for every academic course year, was randomly selected. Only about a quarter of respondents were not opposed to the siting of a landfill or an incinerator in their city. This clearly highlights that historic opposition to the construction of waste facilities is difficult to overcome and that distrust for previous poor management or indiscretions is long-lived and transcends generations. Students from technical faculties expressed the most reasonable opinion; opinion and knowledge were statistically related (Chi-square test, p < 0.05) to the attended faculty, and the knowledge grew linearly with progression through the university. This suggests that awareness of waste management practices develops with experience and understanding of environmental issues. There is general acceptance that many stakeholders – technicians, politicians and citizens – all have to be part of the decision process when siting a new waste management facility. The opinions of the young respondents were significantly influenced by their level of environmental knowledge.     

Spatial distribution of lindane in topsoil of Northern France

Lindane is a persistent organochlorine insecticide and the use of this insecticide in agriculture was banned in France in 1998. In this study we investigated the concentrations of lindane in topsoil in Northern France and used robust geostatistics to map the geographical distribution of lindane. The study was based on a 16 km × 16 km grid covering an area of ca 25 000 km². Lindane was found in all soils, even those from non-agricultural-application areas. Very low ratios of α-/γ-HCH and δ-/γ-HCH suggested that a long time had passed since technical HCH was used in the studied area, or that emission sources of lindane were still present. A strong gradient in lindane concentration was observed, with the highest lindane concentrations in an area located in the northern region. Results suggested that some of the lindane observed in the high concentration area may have come from volatilization of old lindane applied to intensively cultivated areas, which was then transported by prevailing winds coming from the south-west and deposited in a densely inhabited depression.