Twenty years later: the new perspectives of the Palermo Convention

Questo saggio illustra la modernità e le prospettive della Convenzione delle Nazioni Unite contro il crimine organizzato transnazionale a venti anni dalla sua adozione. L’autore si sofferma sul potenziale contributo che la cosiddetta “Risoluzione Falcone”potrebbe apportare al rafforzamento della cooperazione internazionale in relazione alle misure di prevenzione patrimoniali, alla cooperazione giudiziaria e nelle indagini transnazionali, in particolare grazie a nuovi strumenti quali i “joint investigative bodies”, i magistrati di collegamento e l’uso di tecniche investigative speciali.This essay illustrates the modernity and prospects of the United Nations Convention against Transnational Organized Crime twenty years after its adoption. The author focuses on the potential contribution that the so-called "Falcone Resolution" could bring to the strengthening of international cooperation in the field of preventive measures on assets, judicial cooperation, and transnational investigations, in particular thanks to new tools such as joint investigative bodies, liaison magistrates and the use of special investigative techniques

Reproductive system of the genus Crasiella (Gastrotricha, Macrodasyida)

Crasiella diplura from Sweden and Crasiella sp. from Italy were studied alive and with transmission electron microscopy (TEM). The two species are simultaneous hermaphrodites and share the same reproductive system lay-out: paired ovaries extend along the posterior part of the intestine and join mid-dorsally, while bilateral,club-shaped testes lie at the sides of the anterior gut, extending as deferentia that fuse on the mid-ventral plane and open into a single pore; gametes mature in a caudocephalicand centripetal direction. The bulky, sac-like, frontal organ is lined by a simple epithelium and lies dorsolaterally to the intestine, on the left side of the body.The spindle-shaped caudal organ is musculo-glandular and is located ventrolaterally to the gut on the right side. It is characterized by the presence of a roughly Y-shapedinternal channel that opens into two pores close to each other, which function for the intake and outlet of the (auto)sperm, respectively. The spermatozoa, which arepeculiar and similar in the two species, are characterized by a long and complex acrosome consisting of four ultrastructurally distinct regions, three of which findequivalence in other gastrotrich species. The flagellum lacks a striated cylinder. Anatomy and ultrastructure enable us to hypothesize a modality of sperm transfer in Crasiellathat is similar to that observed in Macrodasys

Faunistics and zoogeographical overview of the Mediterranean and Black Sea marine Gastrotricha.

The paper provides an updated overview of the knowledge regarding the marine gastrotrich fauna of 10 Mediterranean and 2 Black Sea countries. Taxonomic account and species distribution come from published records, including electronically disseminated information, as well as original data from 18 localities (16 western and 2 levantine), investigated by the authors between 1990 and 2002. Since the description of the first marine Mediterranean gastrotrich, Emydasys agaso Claparede, 1867, discovered in the Gulf of Naples (Italy), 273 additional species, from 417 locations have been recorded in the basin so far. Of the gastrotrichs found, 143 species, in 24 genera and 6 families, belong to the order Macrodasyida and 131 species, in 11 genera and 3 families, belong to the order Chaetonotida; these statistics include several species as of yet not described. The number of species per location is variable, ranging from 1-37, with a global mean of 8.04 \uf0b1 6.69 spp/location. Egypt and Israel show mean values well above the average, with 12.8 and 10.8 spp/loc. respectively, whereas mean values for Algeria, Tunisia, Bulgaria, Croatia, and France are below average (1- 5.43 spp /location); data for Cyprus, Greece, Romania and Italy are within the average value. Acanthodasys aculeatus, recorded in 28% of the investigated location is the most frequently found macrodasyidan; Halichaetonotus aculifer, also recorded in 28% of the investigated localities, is the most common chaetonotidan whereas. Data analysis indicated substantial differences among countries, regarding sampling effort and, consequently, faunistic knowledge. In comparison with the generally good information concerning the Italian fauna (177 species from 246 localities), gastrotrichs from other Mediterranean nations are much less known; along with Italy, only few other Nations have been investigated to a sufficient extent (i.e., Greece: 44 sampled localities, 77 recorded species; France: 37 L, 70 spp; Egypt: 28 L, 81 spp; Israel: 15 L, 55 spp; Cyprus: 9 L, 41 spp), whereas investigation carried out in several other countries can be considered, to a variable degree, incomplete (i.e., Algeria: 16 investigated localities and 11 species found; Romania: 7 L, 30 spp; Tunisia: 5 L, 2 spp; Bulgaria: 3 L, 11 spp; Croatia: 1 L, 12 spp, Spain: 1 L, 3 sp, Malta; 2L, 2 spp.), or nil (e.g., Morocco, Libya, Turkey, Albania). All marine gastrotrich families and most marine genera have representatives in the Mediterranean fauna, notable absences regard the genera Desmodasys, Dinodasys, Planodasys, Prostobuccantia and perhaps Pseudoturbanella; on the other hand representatives of two genera, Emydasys and Dendropodola, are unknown elsewhere in the world except the western Mediterranean. At species level, about 140 taxa have so far been found only in the Mediterranean area, whereas the remainder have some north-Atlantic connections, including several considered as regional cosmopolitans (i.e., reported from at least two Oceans). Within the Mediterranean, the species geographic distribution does not appear to be homogeneous, with several taxa restricted to either the western or to the levantine basin; yet, Italy, Egypt and Israel show the highest numbers of endemic species, 70, 24 and 14 respectively, whereas only one species seems restricted to the Black Sea. A cluster analysis using the Bray-Curtis presence-absence similarity value to examine the relationship between 11 geographic areas, shows a clear separation between the Black Sea and the Mediterranean regions and within this, the Algerian-Tunisian region is separated from and the remaining 9 areas. The latter appear arranged in two main clusters, one including the southern Levantine regions (i.e., Egypt, Israel and Cyprus), the other containing regions of the north-western basin, the Adriatic sea and the two Greek sub-regions. In this cluster, the region comprising coastal France and Formentera (Spain) appears separated from the one containing, arranged into two distinct subsets, the Italian and the Greek areas. However, due to the paucity of information, data from key-areas is urged to confirm or disprove our current perception about numbers and distribution of gastrotrich taxa within the Mediterranean and the Black Sea

Energy, environment and sustainable development of the belt and road initiative: The Chinese scenario and Western contributions

Abstract The Belt and Road Initiative has tremendously increased the interaction of China with the countries involved, pushing forward the integration and comparative phase, based on the main factors affecting the energy, environmental and development scenarios. The indicators of this process are strictly related to the environmental sustainability of projects and infrastructural initiatives which entail aspects regarding climate change, environmental impact, transport management, urbanization and effective utilization of energy. Through this osmotic program, which the Chinese government said in 2013 would be executed from east to west, the contributions Western countries may make to maximize the common efforts are foreseeably very important for the success of the whole BRI and, indirectly, for the harmonization of the very rapid Chinese growth. China held the first position in 2014 for electricity generation (5388 billion kwh/h) and coal production (4.27 billion short tons/year), as well as the second position for petroleum consumption. On the other hand, carbon dioxide emissions were 1.8 times those of the USA in 2015, and transport, urbanization and energy intensity still struggle to attain optimal levels. The quality of productive sectors, research and universities is still low in the world ranking, despite the huge efforts of the Chinese, due to a still slow and cumbersome internationalization process. This article aims to integrate the numerous excellent studies published in the last few years on sustainable development and energy effectiveness within the BRI (Table 1 provides some specific references), by carrying out a systematic analysis of the Chinese energy, environmental and sustainable landscape from a Western perspective, breaking the main areas of the Chinese scenario down into its components and identifying those where the contributions of Western countries can support the gaps coverage. In this respect, the change of viewpoint provided by this study may be beneficial to properly balance the BRI perspective along the east-west axis

Growing Up with Type 1 narcolepsy: Its anthropometric and endocrine features

Study Objectives: To evaluate the effect of type 1 narcolepsy (NT1) on anthropometric and endocrine features in childhood/adolescence, focusing on patterns and correlates of weight, pubertal development, and growth in treated and untreated patients. Methods: We collected anthropometric (height, weight, body mass index (BMI) z-scores), pubertal, metabolic, and endocrine data from 72 NT1 patients at diagnosis and all available premorbid anthropometric parameters of patients from their pediatric files (n = 30). New measurements at 1-y reassessment in patients undergoing different treatments were compared with baseline data. Results: We detected a high prevalence of overweight (29.2%), obesity (25%), metabolic syndrome (18.8%), and precocious puberty (16.1%), but no signs of linear growth alterations at diagnosis. According to anthropometric records, weight gain started soon after NT1 onset. At 1-y follow-up reassessment, sodium oxybate treatment was associated with a significant BMI z-score reduction (-1.29 \ub1 0.30, p < 0.0005) after adjusting for baseline age, sex, sleepiness, and BMI. Conclusions: NT1 onset in children/adolescents is associated with rapid weight gain up to overweight/obesity and precocious puberty without affecting growth. In our study, sodium oxybate treatment resulted in a significant weight reduction in NT1 overweight/obese patients at 1-y follow-up

Simulation of forest harvesting alternative processes and concept design of an innovative skidding winch focused on productivity improvement

In contexts in which mechanized harvesting is limited, such as in the northwestern Black Sea region of Turkey, it is important to improve timber harvesting productivity while preserving operators' safety and reducing environmental damage. This study aims to introduce a methodology in which the harvesting process is simulated with discrete-event simulation (DES) software in order to identify bottlenecks. An alternative process is compared to the original within the DES software, carrying out further steps oriented to the generation of new innovative product concepts. As a case study, the design of an innovative skidding winch is proposed. The development of the product was focused towards customer satisfaction by collecting customer requirements and identifying quality characteristics with a quality function deployment approach. Contradictions identified in the design phase were solved using the TRIZ contradiction toolkit, generating different product concepts. Inventive solutions provided by TRIZ were designed within parametric CAD software. The concepts were compared in a virtual environment, eventually selecting an optimal solution. The results showed that, with the concept adopted, it is possible to achieve a substantial increase in productivity, from 121% to 133%, in terms of kilograms of logs per hour deposited on the landing

46, XX DSD due to androgen excess in monogenic disorders of steroidogenesis: Genetic, biochemical, and clinical features

The term ‘differences of sex development’ (DSD) refers to a group of congenital conditions that are associated with atypical development of chromosomal, gonadal, or anatomical sex. Disorders of steroidogenesis comprise autosomal recessive conditions that affect adrenal and gonadal enzymes and are responsible for some conditions of 46, XX DSD where hyperandrogenism interferes with chromosomal and gonadal sex development. Congenital adrenal hyperplasias (CAHs) are disorders of steroidogenesis that mainly involve the adrenals (21-hydroxylase and 11-hydroxylase deficiencies) and sometimes the gonads (3-beta-hydroxysteroidodehydrogenase and P450-oxidoreductase); in contrast, aromatase deficiency mainly involves the steroidogenetic activity of the gonads. This review describes the main genetic, biochemical, and clinical features that apply to the abovementioned conditions. The activities of the steroidogenetic enzymes are modulated by post-translational modifications and cofactors, particularly electron-donating redox partners. The incidences of the rare forms of CAH vary with ethnicity and geography. The elucidation of the precise roles of these enzymes and cofactors has been significantly facilitated by the identification of the genetic bases of rare disorders of steroidogenesis. Understanding steroidogenesis is important to our comprehension of differences in sexual development and other processes that are related to human reproduction and fertility, particularly those that involve androgen excess as consequence of their impairment

Novel associations in disorders of sex development: findings from the I-DSD registry

Context: The focus of care in disorders of sex development (DSD) is often directed to issues related to sex and gender development. In addition, the molecular etiology remains unclear in the majority of cases.<p></p> Objective: To report the range of associated conditions identified in the international DSD (I-DSD) Registry.<p></p> Design, Setting, and Patients: Anonymized data were extracted from the I-DSD Registry for diagnosis, karyotype, sex of rearing, genetic investigations, and associated anomalies. If necessary, clarification was sought from the reporting clinician.<p></p> Results: Of 649 accessible cases, associated conditions occurred in 168 (26%); 103 (61%) cases had one condition, 31 (18%) had two conditions, 20 (12%) had three conditions, and 14 (8%) had four or more conditions. Karyotypes with most frequently reported associations included 45,X with 6 of 8 affected cases (75%), 45,X/46,XY with 19 of 42 cases (45%), 46,XY with 112 of 460 cases (24%), and 46,XX with 27 of 121 cases (22%). In the 112 cases of 46,XY DSD, the commonest conditions included small for gestational age in 26 (23%), cardiac anomalies in 22 (20%), and central nervous system disorders in 22 (20%), whereas in the 27 cases of 46,XX DSD, skeletal and renal anomalies were commonest at 12 (44%) and 8 (30%), respectively. Of 170 cases of suspected androgen insensitivity syndrome, 19 (11%) had reported anomalies and 9 of these had confirmed androgen receptor mutations.<p></p> Conclusions: Over a quarter of the cases in the I-DSD Registry have an additional condition. These associations can direct investigators toward novel genetic etiology and also highlight the need for more holistic care of the affected person.<p></p&gt