Guidelines for the Development of Comprehensive Care Centers for Congenital Adrenal Hyperplasia: Guidance from the CARES Foundation Initiative

Patients with rare and complex diseases such as congenital adrenal hyperplasia (CAH) often receive fragmented and inadequate care unless efforts are coordinated among providers. Translating the concepts of the medical home and comprehensive health care for individuals with CAH offers many benefits for the affected individuals and their families. This manuscript represents the recommendations of a 1.5 day meeting held in September 2009 to discuss the ideal goals for comprehensive care centers for newborns, infants, children, adolescents, and adults with CAH. Participants included pediatric endocrinologists, internal medicine and reproductive endocrinologists, pediatric urologists, pediatric surgeons, psychologists, and pediatric endocrine nurse educators. One unique aspect of this meeting was the active participation of individuals personally affected by CAH as patients or parents of patients. Representatives of Health Research and Services Administration (HRSA), New York-Mid-Atlantic Consortium for Genetics and Newborn Screening Services (NYMAC), and National Newborn Screening and Genetics Resource Center (NNSGRC) also participated. Thus, this document should serve as a "roadmap" for the development phases of comprehensive care centers (CCC) for individuals and families affected by CAH.

Prediction of sediments toxicity using consensus-based sediment quality guidelines and benthic foraminifera in the Ria de Aveiro(Portugal)

Ria de Aveiro is a large and shallow lagoon on the west coast of Portugal (40º38’N, 8º45´W), characterized by a complex geometry. It includes large areas of intertidal flats and a network of narrow channels which are connected to the Atlantic by an artificial inlet. Tides are the main forcing of the hydrology and physical processes of the lagoon. The deeper areas near the inlet are characterized by strong marine influence through tidal inflow, with high values of current velocity (>1m/s) and tidal range (2–3 m at spring tides), while in remote shallow areas, the circulation and the sea water inflow are reduced. These remote areas are more influenced by fresh waters received from several rivers and several small streams. The Aveiro lagoon is a very important ecosystem but as been used as recipient for various kinds of anthropogenic wastes resulting from the high population density, urban activities and industrial development. One of the most important Portuguese industrial centre is located in the lagoon margins. Ria de Aveiro is a coastal lagoon under huge direct antropization. This system also suffers strong diffuse antropization. This work is related with diffuse antropization linked with chemical pollution which may lead to biological stress and collapse.

Dimensions of the author citation potential

[EN] The citation potential is a measure of the probability of being cited. Obviously, it is different among fields of science because of systematic differences in publication and citation behaviour across disciplines. In the past, the citation potential was studied at journal level considering the average number of references in established groups of journals. In this paper, some characterizations of the author’s scientific research through three different research dimensions are proposed: production (journal papers), impact (journal citations), and reference (bibliographical sources). An empirical application, in a set of 120 randomly selected authors in four subject areas, shows that the ratio between production and impact dimensions is a normalized measure of the citation potential at the level of individual authors.

Author stance in doctoral dissertations of native and non-native speakers of English: a corpus-based study on epistemic adverbs

[EN]Our study concentrates on the epistemic adverbs used in conveying author stance in academic English. The Contrastive Interlanguage Analysis (Granger, 1996) was run to three sets of corpora comprising doctoral dissertations written by native and non-native academic authors of English. Epistemic adverbs occurring in the dissertations were identified through a computer programme and their frequencies were separately computed for each corpus. Lastly, a log-likelihood test was administered to see whether there is a statistically significant difference across the groups in concern concerning the use of these adverbs.

Metamorfosi temporali e spaziali: dal “Revisore” di Nikolaj Gogol’ alla “Morte accidentale di un anarchico” di Dario Fo.

La tesi si propone di tracciare un confronto tra due autori molto distanti tra loro sia nel tempo che nello spazio: Nikolaj Gogol’ e Dario Fo; in particolare si analizzano le commedie Il Revisore di Gogol’ (1836) e Morte accidentale di un anarchico di Fo (1970). Il nesso tra le due opere è stato individuato dalla critica italiana (Franco Quadri, Ferdinando Taviani, Paolo Puppa). Tuttavia gli spunti interpretativi e comparativi non sono mai stati sviluppati appieno della critica. Nonostante le grandi distanze temporali e spaziali, l’analisi si propone dunque di evidenziare i numerosi motivi di consonanza tra le due opere e i due autori, che peraltro testimoniano anche della grande fortuna arrisa all’estero all’autore ucraino, attraverso la mediazione del teatro russo-sovietico del XX secolo. L’approccio metodologico adottato si fonda sui testi dei formalisti russi, di Lotman, sulla Stilkritik di Aurbach, sugli studi di Frye sulla Bibbia intesa come “grande canone” della letteratura occidentale, e soprattutto sull’analisi della cultura carnevalesca e popolare condotta da Bachtin. Lo studio è indirizzato su alcuni elementi precisi: la trama, lo scambio della personalità, l’impostura, la paura e il riso. Per ciascun di questi elementi è stata svolta l’analisi comparativa delle due opere, situandole in un contesto letterario e culturale che va all’antichità e al Medio Evo, dal quale entrambi gli autori mutuano molte suggestioni.

Development of guidelines for microbiological control in microbrewery

Over the past 15 years the Italian brewing scene showed interesting changes, especially with regard to the creation of many breweries with an annual production of less than 10,000 hectoliters. The beers produced by microbreweries are very susceptible to attack by spoilage micro-organisms that cause the deterioration of beer quality characteristics. In addition, most of the microbreweries do not practice heat treatments of stabilization and do not carry out quality checks on the product. The high presence of beer spoilage bacteria is an economic problem for the brewing industry because it can damage the brand and it causes high costs of product retrieval. This thesis project was aimed to study the management of the production process in the Italian microbreweries within a production less than 10,000 hl. In particular, the annual production, type of plant, yeast management, process management, cleaning and sanitizing of a representative sample of microbreweries were investigated. Furthermore was made a collection of samples in order to identify, with simple methods, what are spoilage bacteria more present in the Italian craft beers. 21% of the beers analysed were positive at the presence of lactic acid bacteria. These analytical data show the importance of understanding what are the weak points of the production process that cause the development of spoilage bacteria. Finally, the thesis examined the actual production of two microbreweries in order to understand the process management that can promote the growth of spoilage bacteria in beer and production plant. The analysis of the data for the two case studies was helpful to understand what are the critical points where the microorganisms are most frequently in contact with the product. The hygiene practices are crucial to ensure the quality of the finished product, especially in the case of non-pasteurized beer.

Flessibilità e controllo sindacale nel mercato del lavoro contemporaneo: il caso del contratto a tempo determinato

Il lavoro di ricerca che si presenta è suddiviso in tre capitoli nei quali, da altrettanti punti di osservazione, è analizzato il tema della instabilità del lavoro. Nel primo capitolo, il candidato evidenzia le cause che hanno determinato il vorticoso aumento di utilizzo dei contratti di lavoro flessibili e, a tal proposito, da un prospettiva extra-nazionale, analizza le direttive europee e, i principi comuni e le guidelines che, nel percorso di sviluppo della strategia europea per l’occupazione, hanno posto la flexicurity come modello di mercato europeo tipico; dalla medesima prospettiva, prendendo spunto dai cambiamenti del mercato globale, si pone attenzione all’analisi economica del diritto del lavoro e, in particolare, alle conseguenze che le trasformazioni economiche generano sulla capacità di questa materia di tenere elevato il grado di sicurezza occupazionale connesso alla stipula dei contratti di lavoro. Il secondo capitolo è dedicato al ruolo svolto in Italia dai sindacati sul tema della flessibilità. In tal senso, l’autore evidenzia come la funzione “istituzionale” cui sono chiamate anche le organizzazioni dei lavoratori abbia caratterizzato le scelte di politica sindacale in materia di lavori temporanei; è così preso in esame il concetto di flessibilità “contrattata”, come già emerso in dottrina, e si teorizza la differenza tra rinvii “aperti” e rinvii “chiusi”, quali differenti forme di delega di potere normativo alle parti sociali in materia di flessibilità. Nel terzo ed ultimo capitolo l’autore tenta di evidenziare i vantaggi e le funzioni che derivano dall’utilizzo del contratto a termine, quale principale forma di impiego flessibile del nostro mercato del lavoro. Premessi tali benefici viene formulato un giudizio critico rispetto al grado di liberalizzazione che, con le ultime riforme, è stato ammesso per questo istituto sempre più strumento di arbitrio del datore di lavoro nella scelta della durata e delle condizioni di svolgimento della prestazione di lavoro.

Guidelines of Special Care Dentistry in Patients with Chromosomal and Genetic syndromes

Chromosomal and genetic syndromes are frequently associated with dental and cranio-facial alterations. The aim of our study is to identify and describe the dental and craniofacial alterations typical of six genetic and chromosomal syndromes examined. Materials and Methods- A dental visit was performed to 195 patients referred from Sant’Orsola Hospital of Bologna, University of Bologna, to Service of Special Need Dentistry, Dental Clinic, Department of Biomedical and Neuromotor Science, University of Bologna. The patients recruited were 137 females and 58 males, in an age range of 3-49 years (mean age of 13.8±7.4). The total sample consisted of subjects affected with Down Syndrome (n=133), Familiar Hypophosphatemic Ricket (n=10), Muscular Dystrophies (n=12), Noonan Syndrome (n=13), Turner Syndrome (n=17), Williams Syndrome(n=10). A questionnaire regarding detailed medical and dental history, oral health and dietary habits, was filled by parents/caregivers, or patients themselves when possible. The intra-oral and extra-oral examination valued the presence of facial asymmetries, oral habits, dental and skeletal malocclusions, dental formula, dental anomalies, Plaque Index (Silness&LÖe Index), caries prevalence (dmft/DMFT index), gingivitis and periodontal disease, and mucosal lesions. Radiographic examinations (Intraoral radiographies, Orthopanoramic, Skull teleradiography) were executed according to patient’s age and treatment planning. A review of literature about each syndrome and its dental and cranio-facial characteristics and about caries, hygiene status and malocclusion prevalence on syndromic and non-syndromic population was performed. Results - The data of all the patients were collected in the “Data Collection Tables” created for each syndrome. General anamnesis information, oral hygiene habits and dmft/DMFT, PI, malocclusion prevalence were calculated and compared to syndromic and non-syndromic population results found in literature. Discussions and conclusions - Guidelines of Special Care dentistry were indicated for each syndrome, in relation to each syndrome features and individual patient characteristics.

Raccontare la storia al tempo delle crisi

La ricerca analizza il tema della relazione tra storia e narrazione nella letteratura degli ultimi quindici anni in tre contesti nazionali: Italia, Spagna e Portogalo. Per indagare un campo così vasto si sono identificate tre direttrici principali connesse tra loro, coincidenti con tre "crisi": la crisi del rapporto tra letteratura e mercato, la crisi del concetto di verità e la crisi dello stato nazione. Attraverso le riflessioni sul postmoderno (Lyotard, Jameson Hutcheon) e l’analisi di Bourdieu si indaga il rapporto tra mercato e autore letterario, facendo particolare riferimento ai percorsi letterari di Rafael Chirbes, Mia Couto e Wu Ming. Il tema della forma letteraria è invece letto atttraverso le analisi di Hutcheon e analizzando i testi di Helder Macedo (Pedro e Paula), Isaac Rosa (¡Otra maldita novela sobre la guerra civil!) e Tommaso De Lorenzis-Guido Favale (L’aspra stagione). La crisi del concetto di verità viene analizzata alla luce del dibattito sulla storiografia nella seconda metà del Novecento. In particolare si evidenzia la tensione tra Hayden White e Carlo Ginzuburg. Per evidenziare come le relazioni di potere influenzino la narrazione della storia si fa inoltre riferimento alle analisi di Michel Foucault, Michel de Certeau, Stephen Greenbaltt e Gayatri Spivak. Si analizzano quindi Anatomía de un instante, di Javier Cercas, Romanzo criminale, di Giancarlo de Cataldo e As três vidas, di João Tordo. Infine ci si riferisce alla crisi dello stato-nazione individuando una tensione tra le analisi di György Lukács e Franco Moretti, e allargando la riflessione agli studi sociologici di Immanuel Wallerstein e Saskia Sassen. Inoltre, attraverso i testi di Benedict Anderson, Homi B. Bhabha, José Saramao e Eduardo Lourenço si articola una riflessione sull’immaginario politico nazionale. I testi analizzati sono Victus, di Albert Sánchez Piñol, Pro Patria, di Ascanio Celestini e A voz da terra di Miguel Real.

Das deutsche Kriegsgefangenenwesen 1939 - 1945

Zusammenfassung: Die vorliegende Untersuchung zum deutschen Kriegsgefangenenwesen (KGW) im Zweiten Weltkrieg schließt eine wichtige Lücke innerhalb der geschichtswissenschaftlichen Forschungen zum Themenkreis der Kriegsgefangenschaft in deutschem Gewahrsam. Bisherige Studien (bis einschließlich 1997) behandeln vor allem sozial- und kulturgeschichtliche Aspekte der Kriegsgefangenen (Kgf.), der Lagergesellschaft und dem Alltag von Soldaten in Kriegsgefangenschaft. Der Verfasser indes legt mit seiner Magisterarbeit erstmals eine Organisations- und Strukturgeschichte des deutschen Kriegsgefangenenwesens von 1939 bis 1945 vor, welche fundamentale Grundlagen der deutschen militärischen Lagerorganisation und Verwaltung dokumentiert. So wird die Entwicklung von den Vorkriegsplanungen bis zum Kriegsende anhand der zentralen Dienststellen herausgearbeitet und im Kontext des Genfer Kriegsgefangenenabkommens von 1929 und völkerrechtlicher Implikationen gewichtet. Hiermit untrennbar verbundene Einflußnahmen nichtmilitärischer Stellen in die Entscheidungsgewalt der Streitkräfte im Heimatkriegsgebiet und in den Wehrmachtbefehlshaberbereichen werden nicht zuletzt auch anhand mehrerer Organigramme veranschaulicht. Zudem dokumentiert und analysiert die Untersuchung die im Kriegsverlauf stetig verschärften Maßnahmen zur Fluchtprävention und der konzertierten Fahndung nach geflohenen Kriegsgefangenen: Die Machterosion des Oberkommandos der Wehrmacht (OKW) zugunsten des Reichsführers-SS, des Reichssicherheitshauptamts und nicht zuletzt der Parteikanzlei der NSDAP wird so augenfällig. Trotz eminenter Schriftgutverluste kann der Verfasser vor allem anhand einer nahezu vollständig erhaltenen Schlüsselquelle die Stellenbesetzung und Organisationsstruktur der mit Kriegsgefangenenfragen befassten Stellen im OKW rekonstruieren. Die Auswertung dieser Sammelmitteilungen / Befehlssammlung für das Kriegsgefangenenwesen sowie an anderer Stelle überlieferter Organisationsbefehle ermöglicht wichtige Änderungen am derzeitigen Forschungsstand. Darüber hinaus beschäftigt sich die vorliegende Untersuchung mit dem Arbeitseinsatz Kriegsgefangener in der deutschen Wirtschaft als in der zweiten Kriegshälfte zentralem Element der Kriegsgefangenschaft. Außerdem wird beleuchtet, welche politischen, (rassen)ideologischen oder reziprok konnotierten Faktoren den Stellenwert gefangener Soldaten unterschiedlicher Nationalität innerhalb der Gefangenenhierarchie im deutschen Kriegsgefangenenwesen bestimmten. Inhalt: 1. Einführung; 2. Die Entwicklung des Kriegsvölkerrechts und das Genfer Kriegsgefangenenabkommen von 1929; 3. Einleitende Bemerkungen zum deutschen Kriegsgefangenenwesen: Quellenlage, Grundlagen; 4. Organisationsstruktur und Aufgaben des KGW: Zuständigkeiten für Kgf. in OKW und OKH, Abt. Wehrmachtverluste und Kriegsgefangene, der General z.b.V. für das KGW 1939 bis Ende 1941, Allgemeine und Organisationsabteilung seit Januar 1942, Generalinspekteur und Inspekteur des KGW von Juli 1943 bis Oktober 1944, das Kriegsgefangenenwesen unter Himmler seit Oktober 1944; 5. Die Kriegsgefangenenlager: Lagertypen, Anzahl und Verwendung, die Gesamtzahl Kgf. und Belegstärken ausgewählter Lager; 6. Richtlinien für KGL: Die Sammelmitteilungen / Befehlssammlung für das KGW, Lagerorganisation und Behandlung Kriegsgefangener; 7. Die Post der Kriegsgefangenen: Tätigkeit von Auslandsbriefprüfstelle, Abwehr III Referat Kgf. und Abwehrstellen der Wehrkreise, Vorgaben für Postüberwachung und Stimmungsberichte der Asten, Befehle zur Kgf-Post und Kooperation mit Hilfsorganisationen und Schutzmächten; 8. Fluchtprävention: Bestimmungen und Maßnahmen zur Fluchtvereitelung, der Fluchterlass vom 22.09.1942, der Sonderfahndungsplan der Sicherheitspolizei und des SD vom 28.09.1942, Erlass zur Kriegsfahndung vom 5.12.1942, der Fluchterlaß vom 02.07.1943, der Erlaß zur Mitarbeit NSDAP bei Groß- und Kriegsfahndungen vom 10.07.1943, Schulung zur Fluchtprävention auf Wehrkreisebene 1944, Preisausschreiben "Wie verhindere ich Fluchten?" vom 09.04.1945, Anwerbung von V-Leuten durch die Abwehr; 9. Arbeitseinsatz Kriegsgefangener in der deutschen Wirtschaft und beteiligte Stellen; 10. Der Status Kriegsgefangener unterschiedlicher Nationalitäten im Vergleich; 11. Schluss

Prevention, diagnosis, therapy and follow-up care of sepsis: 1st revision of S-2k guidelines of the German Sepsis Society (Deutsche Sepsis-Gesellschaft e.V. (DSG)) and the German Interdisciplinary Association of Intensive Care and Emergency Medicine (Deutsche Interdisziplinäre Vereinigung für Intensiv- und Notfallmedizin (DIVI))

Practice guidelines are systematically developed statements and recommendations that assist the physicians and patients in making decisions about appropriate health care measures for specific clinical circumstances taking into account specific national health care structures. The 1(st) revision of the S-2k guideline of the German Sepsis Society in collaboration with 17 German medical scientific societies and one self-help group provides state-of-the-art information (results of controlled clinical trials and expert knowledge) on the effective and appropriate medical care (prevention, diagnosis, therapy and follow-up care) of critically ill patients with severe sepsis or septic shock. The guideline had been developed according to the "German Instrument for Methodological Guideline Appraisal" of the Association of the Scientific Medical Societies (AWMF). In view of the inevitable advancements in scientific knowledge and technical expertise, revisions, updates and amendments must be periodically initiated. The guideline recommendations may not be applied under all circumstances. It rests with the clinician to decide whether a certain recommendation should be adopted or not, taking into consideration the unique set of clinical facts presented in connection with each individual patient as well as the available resources.