PROFILO CLINICO DELL' IPERPARATIROIDISMO PRIMARIO SPORADICO NEI GIOVANI ADULTI

L’Iperparatiroidismo Primario (PHPT) è la condizione morbosa che deriva da un’eccessiva e parzialmente incontrollata secrezione di PTH. Nella maggior parte dei casi (circa il 90%) è sporadico con una prevalenza di 3/1000 abitanti ed età di insorgenza in V/VI decade di vita. In meno del 10% dei casi il PHPT è familiare con età di insorgenza più precoce. L’iperparatiroidismo primario sporadico (s-PHPT) giovanile è una malattia rara la cui reale incidenza non è ben nota, si ritiene che tale squilibrio ormonale e metabolico nei giovani abbia una presentazione diversa da quella più comunemente conosciuta dell’adulto. Lo scopo del lavoro è stato la realizzazione di un’analisi retrospettiva che permettesse di descrivere le caratteristiche cliniche di un campione di 92 pazienti con età minore o uguale a 40 anni e diagnosi di s-PHPT, seguiti presso il reparto di Endocrinologia dell’Azienda Ospedaliera Universitaria Pisana dal Gennaio 1994 al Maggio 2013. In tutti i pazienti non vi era familiarità per PHPT o tumori endocrini ed in tutti i familiari di I grado la calcemia era normale. Il gruppo di studio includeva 72 femmine (78,3%) e 20 maschi (21,7%), con rapporto M:F=1:3,6. L’età media alla diagnosi era 32 anni (range 14-40). Il sintomo di presentazione più frequente era la litiasi renale in 50/92 pazienti (54,3%); altri sintomi erano quelli aspecifici e neuropsichici in 80/92 pazienti (86,9%). Assenza completa di sintomi è stata riscontrata in soli 4 (4.3%) pazienti. Tutti i pazienti presentavano ipercalcemia, il valore medio della calcemia totale era di 11.01±1.09 mg/dl [9.09-16.60]; il calcio ionizzato, alto in tutti i pazienti, era in media 1.49±0.16 mmol/l, [1.31-2.04]). I valori di PTH erano superiori alla norma o inappropriatamente normali per i rispettivi valori della calcemia (media 188 ±173.8 pg/ml; [34-1450]). Settantasei (82,6%) pazienti erano stati sottoposti ad intervento di paratiroidectomia. L’esame istologico evidenziava un singolo adenoma paratiroideo in 71 pazienti (93,5%); malattia multi-ghiandolare/iperplasia in 2 (2,6%); carcinoma in 2 (2.6%) ed in 1 paziente non veniva evidenziato tessuto paratiroideo patologico (1.3%). Sedici (17,4%) pazienti avevano preferito procrastinare l’intervento chirurgico seppur consigliato. Il follow-up (mediana 2 anni) era disponibile in 69/76 pazienti sottoposti a paratiroidectomia, mentre 7 venivano persi. In 62/69 (89,9%) pazienti chirurgici si assisteva ad una normalizzazione della calcemia e del PTH (calcio ione medio 1.25±0.10 mmol/l; PTH medio 46,95±26,08 pg/ml) mentre in 7 (10,1%) pazienti era presente una persistenza/recidiva di malattia. I pazienti non sottoposti ad intervento chirurgico, di cui si disponeva del follow-up, erano 11/16. In questi pazienti si osservava una stazionarietà di malattia con calcio ione medio pari a 1,40±0,10 mmol/L ed un PTH medio di 75,82±26,24 pg/ml. In conclusione, il s-PHPT giovanile non presenta caratteristiche fenotipiche diverse dalla forma dell’adulto, tranne per la presentazione clinica che è sintomatica nella maggior parte dei casi, e rappresentata spesso dalla nefrolitiasi, aspetto meno comune nella popolazione adulta

Enforcement condiviso in contesti multilivello. Italia e Unione Europea nella Politica comune della pesca. (Shared Enforcement of the European Regulation Facing Political Accountability:Italy and the Common Fisheries Policy. With English summary.)

Studies on the effectiveness of regulation are increasingly focusing their attention on all phases of decisionmaking. With regard to European regulation, most gaps in effectiveness occur during implementation, so far the competence of Member States. To strengthen its own intervention and to provide European regulation with more effectiveness, EU institutions are increasingly acquiring direct enforcement powers. Regarding the effects of this growing shared enforcement, a preliminary question is whether such mechanisms raise any problems with regard to democratic accountability, which is, as a matter of common knowledge, a long-standing concern for European institutions and governance. The CFP is a suitable example of this trend towards more direct action by the EU in enforcing regulation, where, though, mechanisms of accountability have not undergone changes accordingly. The paper takes mechanisms of shared enforcement within the CFP into account, to assess whether they rely on adequate mechanisms of political accountability, therefore displaying gaps that cast some shadow on the legitimacy itself of the way shared enforcement policies are set up, and on the system of European citizens' protection

An Integrated Approach for Evaluating the Restoration of the Salinity Gradient in Transitional Waters: Monitoring and Numerical Modeling in the Life Lagoon Refresh Case Study

Large lagoons usually show a salinity gradient due to fresh water tributaries with inner areas characterized by lower mean values and higher fluctuation of salinity than seawaterdominated areas. In the Venice Lagoon, this ecotonal environment, characterized in the past by oligo‐mesohaline waters and large intertidal areas vegetated by reedbeds, was greatly reduced by historical human environmental modifications, including the diversion of main rivers outside the Venice Lagoon. The reduction of the fresh water inputs caused a marinization of the lagoon, with an increase in salinity and the loss of the related habitats, biodiversity, and ecosystem services. To counteract this issue, conservation actions, such as the construction of hydraulic infrastructures for the introduction and the regulation of a fresh water flow, can be implemented. The effectiveness of these actions can be preliminarily investigated and then verified through the combined implementation of environmental monitoring and numerical modeling. Through the results of the monitoring activity carried out in Venice Lagoon in the framework of the Life Lagoon Refresh (LIFE16NAT/IT/000663) project, the study of salinity is shown to be a successful and robust combination of different types of monitoring techniques. In particular, the characterization of salinity is obtained by the acquisition of continuous data, field campaigns, and numerical modeling

Aquatic Angiosperm Transplantation: A Tool for Environmental Management and Restoring in Transitional Water Systems

Since the 1960s, the Venice Lagoon has suffered a sharp aquatic plant constriction due to eutrophication, pollution, and clam fishing. Those anthropogenic impacts began to decline during the 2010s, and since then the ecological status of the lagoon has improved, but in many choked areas no plant recolonization has been recorded due to the lack of seeds. The project funded by the European Union (LIFE12 NAT/IT/000331-SeResto) allowed to recolonize one of these areas, which is situated in the northern lagoon, by widespread transplantation of small sods and individual rhizomes. In-field activities were supported by fishermen, hunters, and sport associations; the interested surface measured approximately 36.6 km2. In the 35 stations of the chosen area, 24,261 rhizomes were transplanted during the first year, accounting for 693 rhizomes per station. About 37% of them took root in 31 stations forming several patches that joined together to form extensive meadows. Plant rooting was successful where the waters were clear and the trophic status low. But, near the outflows of freshwater rich in nutrients and suspended particulate matter, the action failed. Results demonstrate the effectiveness of small, widespread interventions and the importance of engaging the population in the recovery of the environment, which makes the action economically cheap and replicable in other similar environments

Diagnostic and Therapeutic Challenges of Malignant Pleural Mesothelioma

Malignant pleural mesothelioma is a rare cancer characterized by a very poor prognosis. Exposure to asbestos is the leading cause of malignant pleural mesothelioma. The preinvasive lesions, the mesothelial hyperplasia and its possible evolution are the focus of the majority of the studies aiming to identify the treatable phase of the disease. The role of BAP-1 and MTAP in the diagnosis of mesothelioma in situ and in the prognosis of malignant pleural mesothelioma is the main topic of recent studies. The management of preinvasive lesions in mesothelioma is still unclear and many aspects are the subject of debate. The diagnosis, the disease staging and the accurate, comprehensive assessment of patients are three key instants for an appropriate management of patients/the disease