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Introduction the prevalence of malnutrition in children and its impact on clinical outcomes is underrecognized by clinicians in Italy as well as worldwide. A novel definition of pediatric malnutrition has been recently proposed by a working group of the Academy of Nutrition and Dietetics and American Society for Parenteral and Enteral Nutrition (A.S.P.E.N.), based on the correlation between illness and the use of zscores of anthropometric measurements. Aim to investigate the prevalence of malnutrition and related nutritional support among hospitalized children in Italy, in a nationwide survey performed in a single day (16/4/2015). Methods an open access website (http://nday.biomedia.net) was used to collected data from 73 hospitals and 101 wards in 14 Italian regions (1994 patients). Anonymous information was collected on hospitals' characteristics, patient's anthropometry, admission diagnosis, presence of chronic diseases and use of nutritional support: oral nutritional supplements (ONS), enteral nutrition (EN) or parenteral nutrition (PN). Z-scores of anthropometric measurements, calculated with Epi Info 7.1.5, defined nutritional status: wasting was identified by BMI or Weight-for-Length z-score (

Membranoproliferative GN (MPGN) was recently reclassified as alternative pathway complement–mediated C3 glomerulopathy (C3G) and immune complex–mediated membranoproliferative GN (IC-MPGN). However, genetic and acquired alternative pathway abnormalities are also observed in IC-MPGN. Here, we explored the presence of distinct disease entities characterized by specific pathophysiologic mechanisms. We performed unsupervised hierarchical clustering, a data-driven statistical approach, on histologic, genetic, and clinical data and data regarding serum/plasma complement parameters from 173 patients with C3G/IC-MPGN. This approach divided patients into four clusters, indicating the existence of four different pathogenetic patterns. Specifically, this analysis separated patients with fluid-phase complement activation (clusters 1–3) who had low serum C3 levels and a high prevalence of genetic and acquired alternative pathway abnormalities from patients with solid-phase complement activation (cluster 4) who had normal or mildly altered serum C3, late disease onset, and poor renal survival. In patients with fluid-phase complement activation, those in clusters 1 and 2 had massive activation of the alternative pathway, including activation of the terminal pathway, and the highest prevalence of subendothelial deposits, but those in cluster 2 had additional activation of the classic pathway and the highest prevalence of nephrotic syndrome at disease onset. Patients in cluster 3 had prevalent activation of C3 convertase and highly electron-dense intramembranous deposits. In addition, we provide a simple algorithm to assign patients with C3G/IC-MPGN to specific clusters. These distinct clusters may facilitate clarification of disease etiology, improve risk assessment for ESRD, and pave the way for personalized treatment.

SUMMARYThis essay investigates censorial responses to Jean Bodin's Methodus (1566) in Counter-Reformation Italy, using evidence from Italian libraries and archives to shed new light on the process that led to the inclusion of the work in the Roman Expurgatory Index of 1607. By examining the diverse, and sometimes conflicting, opinions that Catholic censors expressed on Bodin's text and the ‘errors’ it contained, the essay shows that even a relatively cohesive ‘reading community’ such as that of Counter-Reformation censors could nurture fundamental disagreement in evaluating the content and dangerousness of a book, as well as in devising appropriate countermeasures. Censors often made sense of the same texts in utterly different ways, based not only on their own intellectual interests and backgrounds, but also on the different interpretive strategies that they adopted. In light of this fact, the article suggests that early modern censorship should be seen less as a purely repressive practice than as a pecu...

This article offers a re-interpretation of Jean Bodin’s Six livres de la République (1576), a work that deeply transformed European political discourse at the time of the French Wars of Religion and that had important repercussions on the later ‘reason of state’ tradition. Highlighting the ties between Bodin’s definition of sovereignty in Book 1 and his discussion of demographic growth and territorial expansion in Books 4, 5, and 6, the article shows that Bodin’s critical contribution to early modern political thought, far from being limited to his reframing of the juristic concept of souveraineté or maiestas, extends to his novel understanding of the territory as a non-juridical ‘technologie politique’ (Michel Foucault). Through an examination of Bodin’s work and its later reception, the article argues that Bodin’s insights about territorial and demographic matters played a fundamental role in the early modern ‘territorialisation de la politique’ (Romain Descendre), in that they helped redefine the very terms in which the notion of territory would be understood and discussed in the following decades.

The proposed Directive on criminal sanctions for insider dealing and market manipulation aims at harmonising the sanctioning regimes of EU Member States on market abuse, imposing the introduction of criminal sanctions. This proposal represents the first exercise of the competence introduced by Article 83(2) of the Treaty on the Functioning of the European Union and provides an opportunity to critically reflect on its use. This paper analyses the conditions for the approximation of criminal law set by the Article 83(2) and provides an assessment of the respect of these conditions in the proposed Directive. It argues that the proposal raises some concerns in relation to compliance with its legal basis, in view especially of the lack of a clear reliance on empirical data regarding the essential need of introducing criminal sanctions.

The mode of inheritance of Alport syndrome (ATS) has long been controversial. In 1927, the disease was hypothesized as a dominant condition in which males were more severely affected than females. In 1990, it was considered an X-linked (XL) semidominant condition, due to COL4A5 mutations. Later on, a rare autosomal recessive (AR) form due to COL4A3/COL4A4 mutations was identified. An autosomal dominant (AD) form was testified more recently by the description of some large pedigrees but the real existence of this form is still questioned by many and its exact prevalence is unknown. The introduction of next generation sequencing (NGS) allowed us to perform an unbiased simultaneous COL4A3-COL4A4-COL4A5 analysis in 87 Italian families (273 individuals) with clinical suspicion of ATS. In 48 of them (55%), a mutation in one of the three genes was identified: the inheritance was XL semidominant in 65%, recessive in 4% and most interestingly AD in 31% (15 families). The AD form must therefore be seriously taken into account in all pedigrees with affected individuals in each generation. Furthermore, a high frequency of mutations (>50%) was shown in patients with only 1 or 2 clinical criteria, suggesting NGS as first-level analysis in cases with a clinical suspicion of ATS.

SummaryIn this article, I seek to develop a genetic/diachronic approach to the phenomenon of authorial revision, and to the interpretation of texts that exist in multiple versions. In all such cases, the reconstruction of textual meaning cannot be separated from the reconstruction of the process through which the text in its ‘final’ form came into being; furthermore, an understanding of the author's intentions in (re)writing cannot be entirely separated from an understanding of his/her motives for (re)writing. This article is divided into three sections. In the first section, I consider recent trends in editorial and literary theory that aim at characterising texts in terms of processes rather than products, in order to uphold the equal dignity of each version without losing sight of its connectedness to other stages in the history of the text. In the second section, I discuss how Quentin Skinner's views on meaning and context apply to cases of authorial revision, and I suggest that some key aspects of Sk...

Primary distal renal tubular acidosis is a rare genetic disease. Mutations in SLC4A1 , ATP6V0A4, and ATP6V1B1 genes have been described as the cause of the disease, transmitted as either an autosomal dominant or recessive trait. Particular clinical features, such as sensorineural hearing loss, have been mainly described in association with mutations in one gene instead of the others. Nevertheless, the diagnosis of distal renal tubular acidosis is essentially based on clinical and laboratory findings, and the series of patients described so far are usually represented by small cohorts. Therefore, a strict genotype-phenotype correlation is still lacking, and questions about whether clinical and laboratory data should direct the genetic analysis remain open. Here, we applied next-generation sequencing in 89 patients with a clinical diagnosis of distal renal tubular acidosis, analyzing the prevalence of genetic defects in SLC4A1 , ATP6V0A4, and ATP6V1B1 genes and the clinical phenotype. A genetic cause was determined in 71.9% of cases. In our group of sporadic cases, clinical features, including sensorineural hearing loss, are not specific indicators of the causal underlying gene. Mutations in the ATP6V0A4 gene are quite as frequent as mutations in ATP6V1B1 in patients with recessive disease. Chronic kidney disease was frequent in patients with a long history of the disease. Thus, our results suggest that when distal renal tubular acidosis is suspected, complete genetic testing could be considered, irrespective of the clinical phenotype of the patient.

Johann Jakob Scheuchzer’s De ignis seu caloris certa portione Heluetiae adsignata (1708) is one of a series of scientific papers that the prominent Swiss physician and naturalist (1672-1733) sent to the Royal Society in the early 1700s. This particular essay provides an original contribution to physico-theological thought. Unlike most natural-theological works, it emphasises the dangers of human intervention in nature. As an early modern thought-experiment on climate warming and its expected consequences on Alpine and European ecosystems, it seems to anticipate modern anxiety over climate change. But it is also a fine piece of Neo-Latin mountain-writing in the tradition of earlier authors such as Henricus Glareanus (1488-1563) and Conrad Gesner (1516-1565). This article offers the first edition of De ignis seu caloris certa portione, based on Scheuchzer’s autograph in the Royal Society collections in London. Scheuchzer’s text is accompanied by an English translation, a full textual commentary, a short biography of the author, and an appendix providing the details of Scheuchzer’s papers and letters to the Royal Society for 1703-1708.

The mean annual incidence of hemolytic uremic syndrome in persons

Background Alport syndrome is a clinically heterogeneous, progressive nephropathy caused by mutations in collagen IV genes, namely COL4A3 and COL4A4 on chromosome 2 and COL4A5 on chromosome X. The wide phenotypic variability and the presence of incomplete penetrance suggest that a simple Mendelian model cannot completely explain the genetic control of this disease. Therefore, we explored the possibility that Alport syndrome is under digenic control. Methods Using massively parallel sequencing, we identified 11 patients who had pathogenic mutations in two collagen IV genes. For each proband, we ascertained the presence of the same mutations in up to 12 members of the extended family for a total of 56 persons studied. Results Overall, 23 mutations were found. Individuals with two pathogenic mutations in different genes had a mean age of renal function deterioration intermediate with respect to the autosomal-dominant form and the autosomal-recessive one, in line with molecule stoichiometry of the disruption of the type IV collagen triple helix. Conclusions Segregation analysis indicated three possible digenic segregation models: (i) autosomal inheritance with mutations on different chromosomes, resembling recessive inheritance (five families); (ii) autosomal inheritance with mutations on the same chromosome resembling dominant inheritance (two families) and (iii) unlinked autosomal and X-linked inheritance having a peculiar segregation (four families). This pedigree analysis provides evidence for digenic inheritance of Alport syndrome. Clinical geneticists and nephrologists should be aware of this possibility in order to more accurately assess inheritance probabilities, predict prognosis and identify other family members at risk.

This paper examines the evolution of effective exchange rates in nine Central and Eastern European countries in terms of development trends, volatility and cyclicality. Consequently, it provides direct empirical evidence on the nature of the relationship between effective exchange rates and selected macroeconomic fundamentals, addressing a key precondition of numerous exchange rate determination models and theories that attempt to explain the role of exchange rates in the economy. The results suggest that flexible exchange rate arrangements are reflected in both nominal and real effective exchange rates having higher volatility and variability. Furthermore, the results provide mixed evidence in terms of intensity, direction and cyclicality, but show a weak correlation between exchange rates and fundamentals. Sufficiently high coefficients are found only for money supply. Consequently, using fundamentals for the determination of exchange rates and using the exchange rate to explain economic development may be of limited use for the countries analyzed.

Because groups or teams are commonly used in the workplace, organizations need employees who can be effective team members. In this article, the author discusses her use of cooperative small groups in an introductory accounting class and provides guidelines for their formation, orientation, and evaluation. Statistical analysis showed that the groups enhanced student achievement in the accounting class.

The 60 Hz, Frame 7F engine has been in commercial operation for more than two decades now with approximately 800 Frame 7 (F, FA, FA+ and FA+e models) machines existing in North America and a total of over 1100 F-class machines throughout the world. Of importance to operators/users and owners of this gas turbine engine is the ability to not only recondition the turbine “hot-end section” components, in order to support maintenance requirements, but also to reduce the life cycle costs of these components. Stage 1 nozzles are a cast doublet, manufactured from FSX-414 Co-based superalloy. There are 24 nozzles in an engine set. As a result of the extreme turbine inlet temperatures at the 1st stage nozzle, these components experience severe thermal fatigue cracking. On one particular OEM engine set inspected, over 600 cracks were identified on each nozzle after engine operation. As a consequence, the stage 1 nozzles when compared to the other components in the engine exhibits the worst degradation and is therefore a focus point of reconditioning for those in the industry. The technical objective is to describe the development of a repair scheme for the stage 1 nozzle that can successfully address and repair this level of severe cracking and oxidation, with 0% scrap/fallout for a heavy repair, after a normal duty cycle. Special processes have been established for these components repairs, including but not limited to removal of the damaged and oxidized trailing edges by machining, high frequency Gas Tungsten Arc Welding (GTAW) of new trailing edge coupons. This technical paper describes the repair process development and implementation of the different stages of the repair schemes and shows metallurgical and mechanical characteristics of the repaired regions of the component. As a result of successfully executing these special processes, although a heavy repair workscope was implemented; all the nozzles were successfully repaired without any scrap.

Sixty-one adult (age 25 and over) and 95 traditional-age (ages 18 through 24) two-year college students responded to a battery of instruments (Adult Classroom Environment Scale, Adaptive Style Inventory, Principles of Adult Learning Scale, and an Evaluation of Instruction Questionnaire) distributed in five remedial English and five remedial mathematics courses. Data analyses indicated that student age accounts for little variance in student expectations of the classroom environment, learning style, or select course outcomes. Nevertheless, students in reading and mathematics classes with learner-centered activities achieved higher course grades. Adult students in the mathematics sections reported a greater sense of accomplishment and a more positive total course experience than their traditional-age counterparts.