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Purpose: Myelofibrosis (MF) is a chronic myeloproliferative neoplasm characterised by an aggressive clinical course, with disabling symptoms and reduced survival. Patients experience a severely impaired quality of life and their families face the upheaval of daily routines and high disease-related financial costs. The aim of this study was to investigate the perceptions of Italian patients and their caregivers about living with MF and the burden of illness associated with MF.Methods: A quali-quantitative questionnaire and a prompted written narrative survey were administered to patients affected by primary or post-essential thrombocythemia/post-polycythaemia vera MF and their primary caregiver in 35 Italian haematological centres.Results: In total, 287 questionnaires were returned by patients and 98 by caregivers, with 215 and 62, respectively, including the narrative. At the time of diagnosis, the most commonly expressed emotional states of patients were fear, distress and anger, confirming the difficulty of this phase. A high level of emotional distress was also reported by caregivers. Along the pathway of care, the ability to cope with the disease differed according to the quality of care received. The mean cost to each patient attributable to MF was estimated as €12,466 per year, with an estimated average annual cost of loss of income of €7774 per patient and €4692 per caregiver.Conclusions: Better understanding of the personal life of MF patients and their families could improve the relationships between health workers and patients, resulting in better focused healthcare pathways and more effective financial support to maintain patients in their social roles. [ABSTRACT FROM AUTHOR]

Background: Even if the food supplement industry is one of the fastest growing worldwide, the inappropriate use of these products can potentially cause harms. Considering the key role played by pharmacists in advising citizens on the purchase of food supplements, this cross-sectional observational study evaluates pharmacists’ knowledge, attitudes and practice on this topic. Methods: From September 2020 to February 2021, an online questionnaire implemented through the EU-Survey tool, was administered to pharmacists of both public and private authorized pharmacies of the Friuli-Venezia Giulia (FVG) Region (Italy). The 47 multiple-choice questions about pharmacists’ knowledge (20), attitudes and practice (27), were followed by a sociodemographic section (7 items) collecting data about sex, age, years of experience, degree, role within pharmacy (owner, employee). Descriptive analyses, Chi-square, Shapiro-Wilk and Mann Whitney tests were used and a p-value<0.05 was considered as statistically significant. Results: A sample of 232 questionnaires was collected, 70.7% of which from women. Most responders (77.2%) had a Pharmacy degree, with owners representing the 34.5%. The 61.2% of pharmacists had at least 10 years of experience. Knowledge was generally poor with just 49.2% of the sample scoring above the median value of 11 but higher among pharmacists with more than 11 years of experience. Lesser-known aspects were the possible link between cancer and multivitamin’s abuse and presence of non-labeled agents in food supplements. Responders<=10 years of experience felt more unprepared about vitamin’s dosage and in counselling particular groups of patients (p<0.05). Employees more frequently reported the attitude to purchase food supplements under patient specifical request (p<0.05). Conclusions: Pharmacists’ knowledge about food supplements is scarce and young professionals in particular need further education to ensure a safe and effective purchase of these products to patients. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to achieve a consensus on the minimum set of outcome measures and predictors to be used in the neurosurgical practice and on the timing of outcome assessment., Methods: A consensus building approach was employed. All neurosurgical departments in Lombardy (Italy) were invited to participate by the Carlo Besta Neurologic Institute IRCCS Foundation. Three workshops were organized during which a multidisciplinary group called Neurosurgical Outcome Network (NEON) was created and the methodology to select outcome measures, predictors, and timing of outcome assessment was established. Eight working groups were created for the different neurosurgical diseases (neuro-oncological, skull base, vascular, traumatic, spinal, peripheral nervous system, malformation, functional) and 8 workshops were organized to identify the outcome measures and predictors specific for each of the neurosurgical diseases based on the experts' clinical practice and the existing literature., Results: A total of 20 neurosurgical departments participated in this study. Specific outcome measures, predictors and the timing of outcome assessment were identified for each of the 8 neurosurgical diseases. Moreover, a list of variables common to all pathologies were identified by the NEON group as further data to be collected., Conclusions: A consensus on the minimum set of outcome measures and predictors and the timing of outcome assessments for 8 neurosurgical diseases was achieved by a group of neurosurgeons of the Lombardy region, called NEON. These sets could be used in future studies for a more homogeneous data collection and as a starting point to reach further agreement also at national and international level.

In Italy, few epidemiologic studies have been conducted by tracing melanoma reports directly in the electronic registers of the operating units of pathologic anatomy. The Cancer Registers of the Italian regions receive only partial and incomplete data on the diagnoses of melanoma, for this reason, the incidences are usually underestimated. Our work offers a precise picture of the epidemiologic situation of melanoma in a homogenous sample of patients residing in a geographic area traditionally considered to have a high incidence of melanoma., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Medical students and residents play an important role in patient care and ward activities, thus they should follow hospital procedures and ensure best practices and patient safety. A survey concerning staff on training was conducted to assess the perceived quality of healthcare from healthcare workers (HCWs), residents, medical students and patients in Udine Academic Hospital, Italy. Between December, 2018 and March, 2019, a 5-point Likert-scale questionnaire was administered in 21 units, covering four thematic areas: patients and medical staff satisfaction with the quality of care provided by residents and students, patient privacy, clinical risk management, patient perception of staff on training. Data analysis included descriptive analysis and ordered logistic regressions. A total of 596/1,863 questionnaires were collected from: HCWs (165/772), residents (110/355), students (121/389), and patients (200/347). Residents were rated high both by patients (median = 5, IQR = 4-5, OR 0.49, 95%CI 0.26-0.93) and HCWs (median = 4, IQR = 3-5, OR 0.14, 95%CI 0.08-0.26), with a lower score for medical students on the same topic, both by patients (median = 4, IQR = 3-5, OR 2.94, 95%CI 1.49-5.78) and HCWs (median = 3, IQR = 2-3, OR 0.41, 95%CI 0.25-0.67). Therefore, the role of staff on training in quality and safety of healthcare deserves integrated regular evaluation, since direct interaction with patients contributes to patients' perception of healthcare., Competing Interests: The authors have declared that no competing interests exist.

HLA molecules are important for immunoreactivity in allogeneic hematopoietic stem cell transplantation (HSCT). The Gruppo Italiano Trapianto di Cellule Staminali e Terapie Cellulari, Italian Bone Marrow Donor Registry, and Associazione Italiana di Immunogenetica e Biologia dei Trapianti promoted a retrospective observational study to evaluate HLA matching and the impact of allelic HLA mismatching and non-HLA factors on unrelated Italian HSCT outcomes. From 2012 to 2015, 1788 patients were enrolled in the study. The average donor age was 29 years and the average recipient age was 49 years. As a conditioning regimen, 71% of the patients received myeloablative conditioning. For GVHD prophylaxis, 76% received either antithymocyte or anti-T lymphocyte globulin, cyclosporine A, and methotrexate. Peripheral blood was the stem cell source in 80%. The median duration of follow-up was 53 months. Regarding HLA matching, 50% of donor-recipient pairs were 10/10 matched, 38% had 1 mismatch, and 12% had 2 or more mismatches. A total of 302 pairs shared Italian origin. Four-year overall survival (OS), progression-free survival, GVHD-free relapse-free survival, and relapse rates were 49%, 40%, 22%, and 34%, respectively. The 4-year NRM was 27%, and the 100-day cumulative incidence of grade ≥II acute GVHD (aGVHD) was 26%. In multivariate analysis, 9/10 and ≤8/10 HLA allele-matched pairs were associated with worse OS (P = .04 and .007, respectively), NRM (P = .007 and P < .0001, respectively), and grade III-IV aGVHD (P = .0001 and .01, respectively). Moreover, the incidences of grade II-IV aGVHD (P = .001) and chronic GVHD (P = .002) were significantly lower in Italian pairs. In conclusion, 10/10 HLA matching is a favorable prognostic factor for unrelated HSCT outcome in the Italian population. Moreover, the presence of 2 HLA-mismatched loci was associated with a higher NRM (P < .0001) and grade II-IV aGVHD (P = .006) and a poorer OS (P = .001) compared with 1 HLA-mismatched locus in early or intermediate disease phases. Finally, we found that Italian donor and recipient origin is a favorable prognostic factor for GVHD occurrence., (Copyright © 2020 The American Society for Transplantation and Cellular Therapy. Published by Elsevier Inc. All rights reserved.)

Background: In Italy vaccine hesitancy worsened after a failure to vaccinate episode that took place in Friuli Venezia-Giulia Region until early 2017 which undermined herd immunity by leaving unprotected more than 5,444 children., Methods: Between May and June 2017, 2,557 parents were surveyed at the local vaccination clinic where they were invited within the subsequent extraordinary vaccination campaign. The aim of the survey was to evaluate whether the multi-channel extraordinary vaccination campaign had reached the target population and to know parental beliefs and trusted sources of information after the failure to vaccinate event., Results: While 279 parents were non-hesitant (10.9%) and 1,491 hesitant acceptors (58.3%), just 38 (1.5%) refused to have their children revaccinated. Overall, the most consulted sources of information were print media (18.8%), physicians (16.0%), relatives and friends (12.1%). The majority of parents considered vaccination as a fundamental practice (73.9%), but many were worried about potential side effects (38.8%) or doubtful about the effectiveness of some vaccines (11.0%). According to parents, 19.7% of them (57) changed their opinion about vaccines after the Codroipo case., Conclusions: After the Codroipo case, most parents chose to have their children re-vaccinated and just a little proportion refused the re-administration of vaccines. More studies are needed to confirm the importance of a coherent multi-channel communication strategy using both traditional and new media in order to counteract vaccine hesitancy.

With more widespread prolonged survival, Duchenne muscular dystrophy patients progressively experience multisystem complications. We retrospectively reviewed the charts of 132 Duchenne patients (112 alive/20 dead, age 3.5÷32.3 years) with the aims: 1) to provide a comprehensive description of the clinical status considering different aspects of the disease; 2) to propose a new scoring tool able to consider and pool together heterogeneous different functional. Five functions were analyzed: cardiac, respiratory, nutritional, ambulation and scoliosis. For each function, different items were considered and classified according to clinical severity (as indicated by international guidelines) and an incremental scoring was assigned. In addition, a global score incorporating all functions was defined. The scoring system confirmed that despite the significant protective role of steroids, all functions deteriorated with age. The severity of the global score became significantly higher since the age of 13 years. The severity of cardiac, respiratory and nutritional dysfunction was higher since 18 years. Deceased patients were characterized by significantly worse cardiac function, absence of steroid therapy and later use of respiratory assistive devices. The index proposed in this pilot study is a promising tool able to aggregate and correlate heterogeneous functions. It could become either an individual prognostic indicator of decline or a global score to evaluate changes in clinical trials therefore allowing multicenter studies, optimizing the management of both the primary and the secondary complications of the disease and understanding their relative impact.

Background: Malnutrition in Intensive Care Unit patients has been associated with worse clinical outcomes such as mortality and length of stay (LOS) in Intensive Care Unit (ICU), and nutritional status of Intensive Care Unit patients in particular seemed to be a significant predictor of mortality. Promptness of clinical nutrition administration is a key of nutritional support whenever volitional intake is unfeasible. Early enteral nutrition is associated with better clinical outcomes (reduced complications, LOS in ICU and in Hospital). The aim of this study is to investigate the nutrition therapy management in a large Academic Hospital, evaluating its effects on mortality and LOS in ICU and in the Hospital., Study Design: Data were collected retrospectively from clinical records. Six physicians were trained on the data collection protocol and they reviewed every clinical record of patients included in the survey., Methods: Data of 426 patients admitted to ICUs between November 2016, 1st and April 2017, 30th were collected. A multivariate logistic adjusted regression, with backward variables selection method, was performed in order to identify predictors of enteral and parenteral nutrition conducted within 48 hours after admission to the ICU. The relation between medical nutrition therapy, mortality and LOS in ICU and in the Hospital were also evaluated., Results: Patients were given prompt parenteral and enteral nutrition in 25.12% and 27.46% of cases, respectively. No association was found between medical nutrition therapy and ICU or hospital mortality. Predictors of early enteral nutrition were type of admission and surgery before admission; early parenteral nutrition predictors were gender, ICU (A vs B), impaired immunity status and Central Venous Catheter presence at admission., Conclusions: Our study stresses the need of monitoring nutrition prescribing behaviors in acute hospitals in order to better set up tailored interventions to standardize clinicians' practices and to focus on specific training targets.

The purpose of the present paper is to review the origin and development of the epidemiology of mesothelioma in Italy, starting with the detection and investigation of the major outbreak of the disease observed in Casale Monferrato, Piedmont Region. Over the last four decades, mortality among the cohort of ex-Eternit workers has been measured at three points in time. More recently, population based case-control studies in the area of Casale Monferrato have provided new light on the dose-response curve of the relationship between asbestos exposure and mesotheliomas. The publication of the first Casale Monferrato study had a major impact in the country and contributed to the decision of the Italian Parliament to ban the use of asbestos. The experience of Casale Monferrato represents a lesson in several terms, from the epidemiological surveillance to the health care of the victims and the relationship between epidemiologists, victims, their relatives and residents in contaminated areas.

Objectives: Facioscapulohumeral muscular dystrophy type 1 (FSHD1) has been genetically linked to reduced numbers (≤ 8) of D4Z4 repeats at 4q35. Particularly severe FSHD cases, characterised by an infantile onset and presence of additional extra-muscular features, have been associated with the shortest D4Z4 reduced alleles with 1-3 repeats (1-3 DRA). We searched for signs of perinatal onset and evaluated disease outcome through the systematic collection of clinical and anamnestic records of de novo and familial index cases and their relatives, carrying 1-3 DRA., Setting: Italy., Participants: 66 index cases and 33 relatives carrying 1-3 DRA., Outcomes: The clinical examination was performed using the standardised FSHD evaluation form with validated inter-rater reliability. To investigate the earliest signs of disease, we designed the Infantile Anamnestic Questionnaire (IAQ). Comparison of age at onset was performed using the non-parametric Wilcoxon rank-sum or Kruskal-Wallis test. Comparison of the FSHD score was performed using a general linear model and Wald test. Kaplan-Meier survival analysis was used to estimate the age-specific cumulative motor impairment risk., Results: No patients had perinatal onset. Among index cases, 36 (54.5%) showed the first signs by 10 years of age. The large majority of patients with early disease onset (26 out of 36, 72.2%) were de novo; whereas the majority of patients with disease onset after 10 years of age were familial (16, 53.3%). Comparison of the disease severity outcome between index cases with age at onset before and over 10 years of age, failed to detect statistical significance (Wald test p value=0.064). Of 61 index cases, only 17 (27.9%) presented extra-muscular conditions. Relatives carrying 1-3 DRA showed a large clinical variability ranging from healthy subjects, to patients with severe motor impairment., Conclusions: The size of the D4Z4 allele is not always predictive of severe clinical outcome. The high degree of clinical variability suggests that additional factors contribute to the phenotype complexity., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Introduction: The prevalence of cognitive impairment may be increased in adults with end-stage kidney disease compared with the general population. However, the specific patterns of cognitive impairment and association of cognitive dysfunction with activities of daily living and clinical outcomes (including withdrawal from treatment) among haemodialysis patients remain incompletely understood. The COGNITIVE impairment in adults with end-stage kidney disease treated with HemoDialysis (COGNITIVE-HD) study aims to characterise the age-adjusted and education-adjusted patterns of cognitive impairment (using comprehensive testing for executive function, perceptual-motor function, language, learning and memory, and complex attention) in patients on haemodialysis and association with clinical outcomes., Methods and Analysis: A prospective, longitudinal, cohort study of 750 adults with end-stage kidney disease treated with long-term haemodialysis has been recruited within haemodialysis centres in Italy (July 2013 to April 2014). Testing for neurocognitive function was carried out by a trained psychologist at baseline to assess cognitive functioning. The primary study factor is cognitive impairment and secondary study factors will be specific domains of cognitive function. The primary outcome will be total mortality. Secondary outcomes will be cause-specific mortality, major cardiovascular events, fatal and non-fatal myocardial infarction and stroke, institutionalisation, and withdrawal from treatment at 12 months., Ethics and Dissemination: This protocol was approved before study conduct by the following responsible ethics committees: Catania (approval reference 186/BE; 26/09/2013), Agrigento (protocol numbers 61-62; 28/6/2013), USL Roma C (CE 39217; 24/6/2013), USL Roma F (protocol number 0041708; 23/7/2013), USL Latina (protocol number 20090/A001/2011; 12/7/2013), Trapani (protocol number 3413; 16/7/2013) and Brindisi (protocol number 40259; 6/6/2013). All participants have provided written and informed consent and can withdraw from the study at any time. The findings of the study will be disseminated through peer-reviewed journals and national and international conference presentations and to the participants through communication within the dialysis network in which this study is conducted., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

A study was conducted on olive oils extracted from olives collected in South West Calabria (Southern Italy) over three harvest years 2010-2011-2012. Three autochthonous cultivars were considered: Cassanese, Ottobratica and Sinopolese and seven allochtonous cultivars: Coratina, Itrana, Leccino, Nocellara Messinese, Nociara, Pendolino and Picholine. Thin Layer Chromatography - Gas Chromatograph (TLC-GC) technique permitted the separation and analysis of the fatty alcohol compounds. A general decline in fatty alcohol content was found during the three months of sampling, most evident in hexacosanol. Pendolino showed the greatest decline. A less evident decrease was measured in the odd chained fatty alcohols, mainly in heptacosanol. Both harvest date and cultivar significantly influenced the fatty alcohol content. This is the first report about the fatty alcohol variation during ripening in olive oil produced in South West Calabria (Southern Italy).

Purpose: In patients with myeloma, thalidomide significantly improves outcomes but increases the risk of thromboembolic events. In this randomized, open-label, multicenter trial, we compared aspirin (ASA) or fixed low-dose warfarin (WAR) versus low molecular weight heparin (LMWH) for preventing thromboembolism in patients with myeloma treated with thalidomide-based regimens., Patients and Methods: A total of 667 patients with previously untreated myeloma who received thalidomide-containing regimens and had no clinical indication or contraindication for a specific antiplatelet or anticoagulant therapy were randomly assigned to receive ASA (100 mg/d), WAR (1.25 mg/d), or LMWH (enoxaparin 40 mg/d). A composite primary end point included serious thromboembolic events, acute cardiovascular events, or sudden deaths during the first 6 months of treatment., Results: Of 659 analyzed patients, 43 (6.5%) had serious thromboembolic events, acute cardiovascular events, or sudden death during the first 6 months (6.4% in the ASA group, 8.2% in the WAR group, and 5.0% in the LMWH group). Compared with LMWH, the absolute differences were +1.3% (95% CI, -3.0% to 5.7%; P = .544) in the ASA group and +3.2% (95% CI, -1.5% to 7.8%; P = .183) in the WAR group. The risk of thromboembolism was 1.38 times higher in patients treated with thalidomide without bortezomib. Three major (0.5%) and 10 minor (1.5%) bleeding episodes were recorded., Conclusion: In patients with myeloma treated with thalidomide-based regimens, ASA and WAR showed similar efficacy in reducing serious thromboembolic events, acute cardiovascular events, and sudden deaths compared with LMWH, except in elderly patients where WAR showed less efficacy than LMWH.

Background: Mutations in GDAP1 associate with demyelinating (CMT4A) and axonal (CMT2K) forms of CMT. While CMT4A shows recessive inheritance, CMT2K can present with either recessive (AR-CMT2K) or dominant segregation pattern (AD-CMT2K), the latter being characterised by milder phenotypes and later onset. The majority of the GDAP1 mutations are associated with CMT4A and AR-CMT2K, with only four heterozygous mutations identified in AD-CMT2K., Methods: We screened GDAP1 gene in a series of 43 index patients, 39 with CMT2 and 4 with intermediate CMT, with sporadic and familial occurrence of the disease., Results: Three novel mutations were identified in three families with dominant segregation of the disease: two missense changes, p.Arg226Ser and p.Ser34Cys, affecting the GST domain of the GDAP1 protein and a novel deletion (c.23delAG) leading to early truncation of the protein upstream the GST domain. Wide variability in clinical presentation is shared by all three families mostly in terms of age at onset and disease severity. A rare variant p.Gly269Arg, located within the GST domain, apparently acts as phenotype modulator in the family carrying the deletion., Conclusion: The results obtained reveal a GDAP1 mutation frequency of 27% in the dominant families analysed, a figure still unreported for this gene, thus suggesting that GDAP1 involvement in dominant CMT2 might be higher than expected.

Even though the natural history of cervical and oral human papillomavirus (HPV) infection has been investigated intensely, the possibility that HPV may infect both sites in the same subject is not well documented. This study investigated the frequency of concurrent oral and cervical HPV infection in southern Italian women, in the light of some selected socio-behavioral variables. One hundred forty women (mean age: 36 years), with known cervical HPV status, were analyzed for oral HPV. Age, smoking/drinking habits, clinical and socio-behavioral history were assessed by personal interviews. Oral mucosal cells were collected by oral brushing and HPV DNA was sought by the use of nested PCR amplification followed by direct DNA sequencing and the commercial assay INNOLiPA HPV Genotyping (Innogenetics N.V., Ghent, Belgium). The data were analyzed by using the chi-square test and a logistic regression (logit) model (P < 0.05 statistically significant). Oral HPV infection was detected in 2/140 (1.4%) cases, being present in 2/76 (2.6%) women with cervical HPV infection and 0/64 uninfected women (P = 0.19). A lack of type-specific concordance in the two patients with concurrent infection was observed. In the sample of population examined, HPV cervical infection does not seem to predispose to oral transmission, even in the presence of oral-genital sexual habits, thus suggesting the independence of infection at the two mucosal sites., (2009 Wiley-Liss, Inc.)

In this paper we attempt to identify behavioral differences between public and private not-for-profit hospitals, by exploiting the introduction of the DRG-based payment system in the Italian NHS during the second half of the 1990s. We estimate the technical efficiency of a sample of hospitals for the period 1995-2000 considering an output distance function, and adopting both parametric (COLS and SF) and nonparametric (DEA) approaches. Our results show a convergence of mean efficiency scores between not-for-profit and public hospitals, and seem to suggest that differences in economic performances between competing ownership forms are more the result of the institutional settings in which they operate than the effect of the incentive structures embedded in the different proprietary forms. We also observe a decline in technical efficiency, probably due to policies aimed at reducing hospitalization rates., (Copyright (c) 2006 John Wiley & Sons, Ltd.)

A group of subgingival isolates of C. albicans recovered from Italian HIV-positive (HIV+) subjects were characterized both phenotypically and genotypically. Phenotyping of the isolates was carried out by a biotyping method based on the enzyme profiles, carbohydrate assimilation patterns and boric acid resistance of the yeasts. Genotyping was performed through randomly amplified polymorphic DNA (RAPD) analysis. Five biotypes were found among the 29 subgingival C. albicans strains examined. The predominant biotypes were A1R (55.17%), A1S (24.14%), and A2R (13.79%), while the biotypes A11R and A13R were represented by a single isolate each. RAPD profiles identified 15 genotypes among the 29 isolates. Almost every individual harboured his/her own specific isolate and in three out of the six subjects with multiple isolates (two to six each) more than one genotype (two to six) was found. The biotype distribution we found is consistent with previous reports on C. albicans isolates from other oral sources, whereas the resistance to boric acid was highly frequent in subgingival strains. RAPD analysis showed high genetic heterogeneity within subgingival isolates, also when isolates were phenotypically identical. These findings, obtained from HIV+ subjects living in Southern Italy, may be useful as baseline information on subgingival C. albicans colonization in the Mediterranean area.

The characteristics of R plasmids in strains of Salmonella typhimurium isolated in Sicily during 1983 from apparently isolated cases of human infection have been determined. A plasmid of the Inc group 10-B-0, characterized by ApSm resistance markers and with a molecular weight of 70 Md, was found in strains belonging to different phage types (2, 3, 117, 135, NT). The data obtained suggest that strains of the same phage type harboring such plasmids come from the same bacterial clone which has continued to be endemic through interhuman contagion. Instead, the presence of the same plasmid in strains of different phage type could indicate its spreading in different bacterial clones.