Prediction of late/early arrivals in container terminals - A qualitative approach

Vessel arrival uncertainty in ports has become a very common problem worldwide. Although ship operators have to notify the Estimated Time of Arrival (ETA) at predetermined time intervals, they frequently have to update the latest ETA due to unforeseen circumstances. This causes a series of inconveniences that often impact on the efficiency of terminal operations, especially in the daily planning scenario. Thus, for our study we adopted a machine learning approach in order to provide a qualitative estimate of the vessel delay/advance and to help mitigate the consequences of late/early arrivals in port. Using data on delays/advances at the individual vessel level, a comparative study between two transshipment container terminals is presented and the performance of three algorithmic models is evaluated. Results of the research indicate that when the distribution of the outcome is bimodal the performance of the discrete models is highly relevant for acquiring data characteristics. Therefore, the models are not flexible in representing data when the outcome distribution exhibits unimodal behavior. Moreover, graphical visualisation of the importance-plots made it possible to underline the most significant variables which might explain vessel arrival uncertainty at the two European ports

Planning and design support tools for walkability: a guide for urban analysts

We present a survey of operational methods for walkability analysis and evaluation, which we hold to show promise as decision-support tools for sustainability-oriented planning and urban design. An initial overview of the literature revealed a subdivision of walkability studies into three main lines of research: transport and land use, urban health, and livable cities. A further selection of articles from the Scopus and Web of Science databases focused on scientific papers that deal with walkability evaluation methods and their suitability as planning and decision-support tools. This led to the definition of a taxonomy to systematize and compare the methods with regard to factors of walkability, scale of analysis, attention on profiling, aggregation methods, spatialization and sources of data used for calibration and validation. The proposed systematization aspires to offer to non-specialist but competent urban analysts a guide and an orienteering, to help them integrate walkability analysis and evaluation into their research and practice

Innovative Virtual Lab for Improving Safety and Port Operations

Computer simulation makes it possible to reproduce real systems and processes in a synthetic environment. In this way virtual analysis turn to be possible and it complex scenarios are suitable to be simulated. In the proposed paper is presented a port system where to study the behavior respect operations and accidents and to consider interaction among multiple players. The simulation is applied to create a Virtual Lab able to evaluate and investigate the development of new procedures, contingency plans during crises. The development of models to be used in simulations is clearly a critical aspect, since the consistency of the simulation depend on the quality of the models and their interaction; in this case the authors used their experience in the field to guarantee a successful Verification and Validation. In this case study, models are used for simulations of phenomena related to port accidents and crises with particular attention to dispersion system of liquid contaminant on sea surface and dispersion of toxic gases into atmosphere. These models have been tested in the Alacres2 simulator in order to create as an effective tool to observe and study the evolution and impact of dangerous situations, as well as a decision-making support to define response plans crises

Niemann-Pick disease type C

Niemann-Pick C disease (NP-C) is a neurovisceral atypical lysosomal lipid storage disorder with an estimated minimal incidence of 1/120 000 live births. The broad clinical spectrum ranges from a neonatal rapidly fatal disorder to an adult-onset chronic neurodegenerative disease. The neurological involvement defines the disease severity in most patients but is typically preceded by systemic signs (cholestatic jaundice in the neonatal period or isolated spleno- or hepatosplenomegaly in infancy or childhood). The first neurological symptoms vary with age of onset: delay in developmental motor milestones (early infantile period), gait problems, falls, clumsiness, cataplexy, school problems (late infantile and juvenile period), and ataxia not unfrequently following initial psychiatric disturbances (adult form). The most characteristic sign is vertical supranuclear gaze palsy. The neurological disorder consists mainly of cerebellar ataxia, dysarthria, dysphagia, and progressive dementia. Cataplexy, seizures and dystonia are other common features. NP-C is transmitted in an autosomal recessive manner and is caused by mutations of either the NPC1 (95% of families) or the NPC2 genes. The exact functions of the NPC1 and NPC2 proteins are still unclear. NP-C is currently described as a cellular cholesterol trafficking defect but in the brain, the prominently stored lipids are gangliosides. Clinical examination should include comprehensive neurological and ophthalmological evaluations. The primary laboratory diagnosis requires living skin fibroblasts to demonstrate accumulation of unesterified cholesterol in perinuclear vesicles (lysosomes) after staining with filipin. Pronounced abnormalities are observed in about 80% of the cases, mild to moderate alterations in the remainder ("variant" biochemical phenotype). Genotyping of patients is useful to confirm the diagnosis in the latter patients and essential for future prenatal diagnosis. The differential diagnosis may include other lipidoses; idiopathic neonatal hepatitis and other causes of cholestatic icterus should be considered in neonates, and conditions with cerebellar ataxia, dystonia, cataplexy and supranuclear gaze palsy in older children and adults. Symptomatic management of patients is crucial. A first product, miglustat, has been granted marketing authorization in Europe and several other countries for specific treatment of the neurological manifestations. The prognosis largely correlates with the age at onset of the neurological manifestations

Capability-wise walkability evaluation as an indicator of urban peripherality

Urban peripherality is a multidimensional phenomenon, requiring operational tools for analysis and policy design. In this paper, we explore if and how the concept of walkability can be employed as an indicator of peripherality. For this purpose, we employ the capability-wise walkability score (CAWS) to assess neighbourhoods of two case study cities to classify them into four classes (periphery, semi-periphery, semi-core, core). In comparing neighbourhoods on both walkability and a set of neighbourhood-level socioeconomic variables, we argue that walkability should be incorporated as part of a comprehensive framework for the analysis of processes of peripherilisation, since walkability should be seen as one relevant factor of urban capabilities, and hence the lack thereof fits into the definition of urban periphery

FUNCTIONAL EFFECT OF NOVEL AMINO ACID VARIANTS OF APOLIPOPROTEIN B IN FAMILIAL HYPOBETALIPOPROTEINEMIA

Introduction. Familial Hypobetalipoproteinemia (FHBL) is a codominant disorder characterized by reduced plasma levels of LDL-C and apolipoprotein (apo) B. In 50% of cases FHBL is due to mutations in APOB gene resulting in truncated apoBs of various size. Some mutations in APOB gene resulting in non-conservative amino acid substitutions were reported to cause FHBL. In vitro, these mutations induce the retention of the mutant apoB in the endoplasmic reticulum (ER) and impair the secretion of apoB-containing lipoproteins. In two FHBL subjects we identified two novel amino acid variants (Thr26_27delinsAsn and Tyr102Cys) located in the N-terminal 1000 amino acids of mature apoB. Methods. To investigate the functional effect of these mutations we constructed plasmids containing human apoB-48 cDNAs harbouring the mutations. McA-RH7777 rat hepatoma cells were transiently and stably transfected with wild type or mutant human apoB-48. The secretion efficiency of human apoB-48 was determined by immunoblotting. To evaluate whether the mutant apoB- 48 was able to form apoB-containing lipoproteins, the incubation media were ultracentrifuged to separate the lipoprotein classes. Immunocytochemistry was used to assess the intracellular localization of the mutant proteins. Results. The mutation Thr26_27delinsAsn strongly reduces the secretion of apoB-48 from the transfected cells. The mutant apoB- 48 appears to be retained in ER as demonstrated by the confocal images showing co-localization of the mutant apoB with the ER marker. In stably transfected cells the defect of mutant apoB-48 secretion was confirmed by the absence of apoB-48 containing lipoproteins in the medium. These observations suggest that Thr26_27delinsAsn alters the structure of the beta-barrel of N-terminal domain of apoB (the first 267 amino acids of mature protein) preventing the secretion of apoB-containing lipoproteins. By contrast the mutation Tyr102Cys had no effect on apoB-48 secretion. Conclusions. This finding supports the notion that Thr26_27 delinsAsn is the cause of FHBL

Mutation screening of the Otop1 gene in familial benign positional paroxysmal vertigo

Objectives: Benign paroxysmal positional vertigo (BPPV) is a sporadic disorder in the vast majority of cases, although a familial, benign, recurrent form, in which the disease segregates in an autosomal dominant fashion has been described. After the evidence for a role of a novel murine gene, Otop1, in knock-out tlt (tilted) and mlh (Mergulhador) mice, lacking the perception of gravity and linear motion and showing a vestibular disorder due to non-syndromic agenesis of both utricular and saccular otoconia, we aimed at verifying the role of the human analogue of the Otop1 gene in BPPV pathogenesis in familial cases of BPPV, collected in our tertiary university referral centre. Methods: Starting in 2007, families with at least two living members thought to have BPPV were considered for inclusion in the present study. The cases were both retrospectively and prospectively identified over the following two years. Results: Seven familial aggregations of BPPV were identified and Otop1 mutation screening showed the presence of a heterozygous mutation in one family, c.1013G> C p.Arg338Pro, which was considered possibly deleterious using the prediction software. It was absent in 100 control alleles, but was also found in two as yet unaffected relatives. Conclusions: The results of a mutation screening of the Otop1 gene in familial cases of BPPV do not support a major role of the gene in the pathogenesis of the disease