The Importance of Place Names in the Sustainable Tourist Development of the Inland Areas of Tuscany : Toponyms along the Via Francigena

The study of place names, through appropriate both linguistic and geographical tools (topographic I.G.M. maps; Environmental Information System of Regione Toscana, etc.) makes come up hidden identities in geographical areas that can be exploited because of their cultural heritage or their landscape. Sometimes the discovery of old linguistic remains is essential to understand the meaning and the role of places and of buildings linked to a past living of some ethnic groups who unconsciously let somewhere their marks. The Italian Peninsula is rich in particular place names, due to the passage of people both from the North and from the Mediterranean Sea in different periods and for different purposes. In Tuscany, too, some areas do exist which have a cultural heritage depending on ancestral presences that can be still revealed: the study of toponyms, in fact, is an effective way to understand the origins and the role of places, the interaction between man and the territories where he lived, the historical and geographical changes. Toponyms are able to let us understand the identity and the uniqueness of a place, reveal us the sense of place and build a cultural base in order to make local institutions awere of it, bringing them to apply for a valorization of economically weak regions. In particular we will deal with the path of Via Francigena in Tuscany, between Lunigiana and Val d’Orcia, where we find some economic important areas since their origins, rich in resources and cultural quality; some other ones considered “minor” but vital enough thanks to the new rural policy; and, at last, some other marginal areas

The Experience-Oriented Tourism in Tuscan Rural Areas

Despite the present scenario of general economic uncertainty, Tuscan rural spaces are still appealing to tourists from Italy and abroad. The changes which took place in the early 1990’s are still evolving in these rural places, where farmers are adapting their supply to the demand of this changing market. In fact, nowadays, there still is a tourist demand for an old-style holiday in rural spaces which is based on the attraction towards natural places and genuine food, while a demand for a more stunning lamorous-style holiday is increasing. The widespread presence of farms involved in tourism is sizable, with more than 4,000 farms (and about 52,000 beds) recorded in 2011, which were dispersed around places with valued cultural landscape and places with valued cultural landscape and linked to apreciated and certified products which in some areas are the pivotal resources. Rethinking rural tourism and agritourism through a wider range of integrated and customized services and looking at rural areas from a different perspective represent two important strategies in order to implement the experience-oriented tourism in the countryside too

Different somatic alterations of the HRPT2 gene in a patient with recurrent sporadic primary hyperparathyroidism carrying an HRPT2 germline mutation

Early onset of primary hyperparathyroidism (PHPT) and multiglandular involvement suggest a familial form in which germline mutation of a PHPT-related gene(s) and a somatic event at the same locus can be often demonstrated. We investigated the involvement of multiple endocrine neoplasia type 1 (MEN1) and HRPT2 genes in a 39-year-old man with recurrent PHPT. PHPT was firstly diagnosed at the age of 21 and the patient had two recurrences separated by extended periods of normocalcemia. This unusual history prompted us to investigate other family members and study the MEN1 and HRPT2 genes. An HRPT2 germline missense mutation in exon 3 (R91P) was found in the index case, which was associated with different HRPT2 somatic alterations in each of the three examined parathyroid tumors. These findings are consistent with Knudson's 'two hit' concept of biallelic inactivation of classical tumor suppressor genes. Screening of 15 asymptomatic relatives was negative for the R91P germline mutation. All the three abnormal parathyroid specimens showed cystic features at histology and were negative for parafibromin immunostaining. In one specimen, diffuse parafibromin staining was evident in a rim of normal parathyroid tissue surrounding the adenomatous lesion. Our study shows that different somatic genetic events at the HRPT2 locus are responsible for the asynchronous occurrence of multiple adenomas in a patient carrying an HRPT2 germline mutation. The finding of diffuse parafibromin staining in a rim of normal parathyroid tissue, but not in the contiguous adenomatous lesion, reinforces the concept that loss of parafibromin expression is responsible for the development of parathyroid tumors in this setting

Identification and functional characterization of loss-of-function mutations of the calcium-sensing receptor in four italian kindreds with familial hypocalciuric hypercalcemia

Objective: Identification and characterization of calcium-sensing receptor (CASR) mutations in four unrelated Italian kindreds with familial hypocalciuric hypercalcemia. Design: Clinical evaluation and genetic analysis of CASR gene. Functional characterization of mutated CASRs. Methods: Direct sequencing of CASR gene in genomic DNA. Studies of CASR-mediated increases in cytosolic calcium concentration [Ca2C]i in CASR-transfected COS-7 cells in vitro. Results: Four unreported heterozygous CASR mutations were identified, including three missense (H595Y, P748H, and C765W) and one splice site (IVS2C1GOC) mutation. The H595Y, P748H, and C765W mutant receptors, although expressed at normal levels on the cell surface, showed a reduced response in [Ca2C]i relative to the wildtype (WT) CASR to increasing extracellular calcium concentrations. Cotransfection experiments showed that the H595Y and P748H mutants did not affect the apparent affinity of the WT CASR for calcium, suggesting that they do not exert a dominant-negative effect. On the other hand, the co-transfected C765W mutant decreased the maximum response of the WT CASR to calcium, suggesting that it may reduce the effective concentration of the normal CASR on the cell surface or impair its maximal signaling capacity. Conclusions: Four CASR mutations were identified. The reduced functional responses to extracellular calcium and normal expression of the mutant receptors suggest that conformational changes account for altered CASR activity. Moreover, a reduced complement of normal CASRs in these heterozygous patients, perhaps combined with a mutant receptor-induced decrease in maximal activity of the WT receptor, may contribute to defective calcium-sensing in vivo.L'articolo è disponibile sul sito dell'editore http://www.euro-endo.org/default.asp

Charge transport and trapping in Cs-doped poly(dialkoxy-p-phenylene vinylene) light-emitting diodes

Al/Cs/MDMO-PPV/ITO (where MDMO-PPV stands for poly[2-methoxy-5-(3'-7'-dimethyloctyloxy)-1,4phenylene vinylene] and ITO is indium tin oxide) light-emitting diode (LED) structures, made by physical vapor deposition of Cs on the emissive polymer layer, have been characterized by electroluminescence, current-voltage, and admittance spectroscopy. Deposition of Cs is found to improve the balance between electron and hole currents, enhancing the external electroluminescence efficiency from 0.01 cd A-1 for the bare Al cathode to a maximum of 1.3 cd A-1 for a Cs coverage of only 1.5×1014 atoms/cm2. By combining I-V and admittance spectra with model calculations, in which Cs diffusion profiles are explicitly taken into account, this effect could be attributed to a potential drop at the cathode interface due to a Cs-induced electron donor level 0.61 eV below the lowest unoccupied molecular orbital. In addition, the admittance spectra in the hole-dominated regime are shown to result from space-charge-limited conduction combined with charge relaxation in trap levels. This description allows us to directly determine the carrier mobility, even in the presence of traps. In contrast to recent literature, we demonstrate that there is no need to include dispersive transport in the description of the carrier mobility to explain the excess capacitance that is typically observed in admittance spectra of p-conjugated materials

Small Area Estimation of Latent Economic Well-being

© The Author(s) 2019. Small area estimation (SAE) plays a crucial role in the social sciences due to the growing need for reliable and accurate estimates for small domains. In the study of well-being, for example, policy makers need detailed information about the geographical distribution of a range of social indicators. We investigate data dimensionality reduction using factor analysis models and implement SAE on the factor scores under the empirical best linear unbiased prediction approach. We contrast this approach with the standard approach of providing a dashboard of indicators or a weighted average of indicators at the local level. We demonstrate the approach in a simulation study and a real data application based on the European Union Statistics for Income and Living Conditions for the municipalities of Tuscany

Association of kidney disease measures with risk of renal function worsening in patients with type 1 diabetes

Background: Albuminuria has been classically considered a marker of kidney damage progression in diabetic patients and it is routinely assessed to monitor kidney function. However, the role of a mild GFR reduction on the development of stage 653 CKD has been less explored in type 1 diabetes mellitus (T1DM) patients. Aim of the present study was to evaluate the prognostic role of kidney disease measures, namely albuminuria and reduced GFR, on the development of stage 653 CKD in a large cohort of patients affected by T1DM. Methods: A total of 4284 patients affected by T1DM followed-up at 76 diabetes centers participating to the Italian Association of Clinical Diabetologists (Associazione Medici Diabetologi, AMD) initiative constitutes the study population. Urinary albumin excretion (ACR) and estimated GFR (eGFR) were retrieved and analyzed. The incidence of stage 653 CKD (eGFR < 60 mL/min/1.73 m2) or eGFR reduction > 30% from baseline was evaluated. Results: The mean estimated GFR was 98 \ub1 17 mL/min/1.73m2 and the proportion of patients with albuminuria was 15.3% (n = 654) at baseline. About 8% (n = 337) of patients developed one of the two renal endpoints during the 4-year follow-up period. Age, albuminuria (micro or macro) and baseline eGFR < 90 ml/min/m2 were independent risk factors for stage 653 CKD and renal function worsening. When compared to patients with eGFR > 90 ml/min/1.73m2 and normoalbuminuria, those with albuminuria at baseline had a 1.69 greater risk of reaching stage 3 CKD, while patients with mild eGFR reduction (i.e. eGFR between 90 and 60 mL/min/1.73 m2) show a 3.81 greater risk that rose to 8.24 for those patients with albuminuria and mild eGFR reduction at baseline. Conclusions: Albuminuria and eGFR reduction represent independent risk factors for incident stage 653 CKD in T1DM patients. The simultaneous occurrence of reduced eGFR and albuminuria have a synergistic effect on renal function worsening