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Italian horror found footage Road to L. - Il mistero di Lovecraft (2005) and mockumentary H.P. Lovecraft - Ipotesi di un viaggio in Italia (2004) revolve around the fictional discovery of a letter by the American supernatural writer, documenting a putative journey to Italy. The films envisage the conception of the HPL mythos stories following the writer's imagined encounters with the gloomy geography, folklore, and cryptic creatures from the Italian river Po delta. Ipotesi is a mockumentary focusing on the fabricated manuscript. Road to L. is Ipotesi's Blair Witch-inspired "backstage", revolving a found VHS and documenting a film crew's Italian journey on the tracks of HPL. The study discusses horror found footage as a sub-set of risemantisation practices characterised by features, such the diegetic camera and a make-believe register, that aim to bridge over regionalist landscape and "folklore" representations and global horror tropes, depicting the Po Delta region as resonating with the accursed geographies of Lovecraftian literature. The films represent examples of how make-believe found footage and mocku-horror formulas have been used to repurpose regional themes to align with global popular canons to attract national and international audiences. [ABSTRACT FROM AUTHOR]

Background & Aims: Obeticholic acid (OCA) is the only licensed second-line therapy for primary biliary cholangitis (PBC). With novel therapeutics in advanced development, clinical tools are needed to tailor the treatment algorithm. We aimed to derive and externally validate the OCA response score (ORS) for predicting the response probability of individuals with PBC to OCA., Methods: We used data from the Italian RECAPITULATE (N = 441) and the IBER-PBC (N = 244) OCA real-world prospective cohorts to derive/validate a score including widely available variables obtained either pre-treatment (ORS) or also after 6 months of treatment (ORS+). Multivariable Cox regressions with backward selection were applied to obtain parsimonious predictive models. The predicted outcomes were biochemical response according to POISE (alkaline phosphatase [ALP]/upper limit of normal [ULN]<1.67 with a reduction of at least 15%, and normal bilirubin), or ALP/ULN<1.67, or normal range criteria (NR: normal ALP, alanine aminotransferase [ALT], and bilirubin) up to 24 months., Results: Depending on the response criteria, ORS included age, pruritus, cirrhosis, ALP/ULN, ALT/ULN, GGT/ULN, and bilirubin. ORS+ also included ALP/ULN and bilirubin after 6 months of OCA therapy. Internally validated c-statistics for ORS were 0.75, 0.78, and 0.72 for POISE, ALP/ULN<1.67, and NR response, which raised to 0.83, 0.88, and 0.81 with ORS+, respectively. The respective performances in validation were 0.70, 0.72, and 0.71 for ORS and 0.80, 0.84, and 0.78 for ORS+. Results were consistent across groups with mild/severe disease., Conclusions: We developed and externally validated a scoring system capable to predict OCA response according to different criteria. This tool will enhance a stratified second-line therapy model to streamline standard care and trial delivery in PBC., (Copyright © 2024 AGA Institute. Published by Elsevier Inc. All rights reserved.)

Background and Aims: Genetic variants influence primary biliary cholangitis (PBC) risk. We established and tested an accurate polygenic risk score (PRS) using these variants., Methods: Data from two Italian cohorts (OldIT 444 cases, 901 controls; NewIT 255 cases, 579 controls) were analysed. The latest international genome-wide meta-analysis provided effect size estimates. The PRS, together with human leukocyte antigen (HLA) status and sex, was included in an integrated risk model., Results: Starting from 46 non-HLA genes, 22 variants were selected. PBC patients in the OldIT cohort showed a higher risk score than controls: -.014 (interquartile range, IQR, -.023, .005) versus -.022 (IQR -.030, -.013) (p < 2.2 × 10 -16 ). For genetic-based prediction, the area under the curve (AUC) was .72; adding sex increased the AUC to .82. Validation in the NewIT cohort confirmed the model's accuracy (.71 without sex, .81 with sex). Individuals in the top group, representing the highest 25%, had a PBC risk approximately 14 times higher than that of the reference group (lowest 25%; p < 10 -6 )., Conclusion: The combination of sex and a novel PRS accurately discriminated between PBC cases and controls. The model identified a subset of individuals at increased risk of PBC who might benefit from tailored monitoring., (© 2024 The Authors. Liver International published by John Wiley & Sons Ltd.)

Background: The economic burden of Primary Biliary Cholangitis (PBC) has not been investigated at population-level. Aim of this study was to estimate the cost of illness of PBC in Lombardy, Italy., Methods: Individuals with PBC were identified through ICD-9-CM code 571.6 and/or medical exemption code 008.571.6, from the Banca Dati Assistito of Lombardy. Only health services (outpatient, inpatient activities and drugs) related to PBC were considered to estimate direct medical costs in 2017., Results: We identified 970 adult patients (83.5% females) with a mean age of 61 years. Global annual costs were equal to € 913,763 (€ 942 per patient), with € 459,506 (50.3%, € 474 per patient) deriving from hospitalizations (mostly due to liver transplantation, 30.5%, and cirrhosis complications, 20.6%). Costs from outpatient activities were € 109,090 (11.9%, € 112 per patient)., Conclusions: This study provides an overview of the costs attributed to PBC care and management, mainly related to hospitalizations for cirrhosis complications, which is necessary for assuring cost-effective introduction of novel therapies. Additional studies focused on indirect cost, e.g. overall loss of productivity, are warranted., Competing Interests: Conflict of interest All the authors do not have conflicts of interests to declare regarding this publication., (Copyright © 2020 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Introduction: Studies on the epidemiology of primary sclerosing cholangitis (PSC) are mainly based on tertiary referral centers; and are retrospective case series susceptible to selection bias . The aim of this study was to estimate incidence; survival and cause of mortality of PSC in Italy; using population-based data. Methods: Data collected from the National Rare Diseases Registry (RNMR) and the National Mortality Database (NMD) were integrated and analyzed. Results: We identified 502 PSC incident cases. The crude incidence rate between 2012 and 2014 was 0.10 per 100,000 individuals. Sixty percent were male; mean age at disease onset and at diagnosis were 33 and 37 years; respectively; highlighting a mean diagnostic delay of 4 years. The rate of interregional mobility was 12%. Ten-year survival was 92%. In 32% of cases the cause of death was biliary-related; 12% died of biliary or gallbladder cancer. Conclusions: For rare diseases such as PSC; population-based cohort's studies are of paramount importance. Incidence rates of PSC in Italy are markedly lower and survival much longer than the ones reported from tertiary; single-centre series. Moreover; the diagnostic delay and the patient interregional mobility highlights the need for increasing awareness on the disease and for resource reallocation among Italian regions within the National Health Service.

Background: Autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis represent the three major autoimmune liver diseases (AILDs). Their management is highly specialized, requires a multidisciplinary approach and often relies on expensive, orphan drugs. Unfortunately, their treatment is often unsatisfactory, and the care pathway heterogeneous across different centers. Disease-specific clinical outcome indicators (COIs) able to evaluate the whole cycle of care are needed to assist both clinicians and administrators in improving quality and value of care. Aim of our study was to generate a set of COIs for the three AILDs. We then prospectively validated these indicators based on a series of consecutive patients recruited at three tertiary clinical centers in Lombardy, Italy., Methods: In phase I using a Delphi method and a RAND 9-point appropriateness scale a set of COIs was generated. In phase II the indicators were applied in a real-life dataset., Results: Two-hundred fourteen patients were enrolled and followed-up for a median time of 54months and the above COIs were recorded using a web-based electronic medical record program. The COIs were easy to collect in the clinical practice environment and their values compared well with the available natural history studies., Conclusions: We have generated a comprehensive set of COIs which sequentially capture different clinical outcome of the three AILDs explored. These indicators represent a critical tool to implement a value-based approach to patients with these conditions, to monitor, compare and improve quality through benchmarking of clinical performance and to assess the significance of novel drugs and technologies. This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen., (Copyright © 2017. Published by Elsevier B.V.)

The main purpose of control measures in urban areas is to retain particulate matter washed out by stormwater over impermeable surfaces. In stormwater control measures, particulate matter removal typically occurs via sedimentation. Settling column tests were performed to examine the settling efficiency of such units using monodisperse and heterodisperse particulate matter (for which the particle size distributions were measured and modelled by the cumulative gamma distribution). To investigate the dependence of settling efficiency from the particulate matter, a variant of the evolutionary polynomial regression (EPR), a Microsoft Excel function based on multi-objective EPR technique (EPR-MOGA), called EPR MOGA XL, was used as a data-mining strategy. The results from this study have shown that settling efficiency is a function of the initial total suspended solids (TSS) concentration and of the median diameter (d50 index), obtained from the particle size distributions (PSDs) of the samples.