Carnitine palmitoyltransferase II (CPT II) deficiency responsible for refractory cardiac arrhythmias, acute multiorgan failure and early fatal outcome.

BACKGROUND: Carnitine palmitoyltransferase II (CPT II) deficiency is a rare inborn error of mitochondrial fatty acid metabolism with autosomal recessive pattern of inheritance. Its phenotype is highly variable (neonatal, infantile, and adult onset) on the base of mutations of the CPT II gene. In affected subjects, long-chain acylcarnitines cannot be subdivided into carnitine and acyl-CoA, leading to their toxic accumulation in different organs. Neonatal form is the most severe, and all the reported patients died within a few days to 6 months after birth. Hereby, we report on a male late-preterm newborn who presented refractory cardiac arrhythmias and acute multiorgan (hepatic, renal, muscular) injury, leading to cerebral hemorrhage, hydrocephalus, cardiovascular failure and early (day 5 of life) to death. Subsequently, extended metabolic screening and target next generation sequencing (NGS) analysis allowed the CPT II deficiency diagnosis. CASE PRESENTATION: The male proband was born at 36+ 4 weeks of gestation by spontaneous vaginal delivery. Parents were healthy and nonconsanguineous, although both coming from Nigeria. Family history was unremarkable. Apgar score was 9/9. At birth, anthropometric measures were as follows: weight 2850 g (47th centile, -0.07 standard deviations, SD), length 50 cm (81st centile, + 0.89 SD) and occipitofrontal circumference (OFC) 35 cm (87th centile, + 1.14 SD). On day 2 of life our newborn showed bradycardia (heart rate around 80 bpm) and hypotonia, and was then transferred to the Neonatal Intensive Care Unit (NICU). There, he subsequently manifested many episodes of ventricular tachycardia, which were treated with pharmacological (magnesium sulfate) and electrical cardioversion. Due to the critical conditions of the baby (hepatic, renal and cardiac dysfunctions) and to guarantee optimal management of the arrythmias, he was transferred to the Pediatric Cardiology Reference Center of our region (Sicily, Italy), where he died 2 days later. Thereafter, the carnitines profile evidenced by the extended metabolic screening resulted compatible with a fatty acid oxidation defect (increased levels of acylcarnitines C16 and C18, and low of C2); afterwards, the targeted next generation sequencing (NGS) analysis revealed the known c.680 C > T p. (Pro227Leu) homozygous missense mutation of the CPTII gene, for diagnosis of CPT II deficiency. Genetic investigations have been, then, extended to the baby's parents, who were identified as heterozygous carriers of the same variant. When we meet again the parents for genetic counseling, the mother was within the first trimester of her second pregnancy. Therefore, we offered to the couple and performed the prenatal target NGS analysis on chorionic villi sample, which did not detect any alterations, excluding thus the CPT II deficiency in their second child. CONCLUSIONS: CPTII deficiency may be suspected in newborns showing cardiac arrhythmias, associated or not with hypertrophic cardiomyopathy, polycystic kidneys, brain malformations, hepatomegaly. Its diagnosis should be even more suspected and investigated in cases of increased plasmatic levels of creatine phosphokinase and acylcarnitines in addition to kidney, heart and liver dysfunctions, as occurred in the present patient. Accurate family history, extended metabolic screening, and multidisciplinary approach are necessary for diagnosis and adequate management of affected subjects. Next generation sequencing (NGS) techniques allow the identification of the CPTII gene mutation, essential to confirm the diagnosis before or after birth, as well as to calculate the recurrence risk for family members. Our report broads the knowledge of the genetic and molecular bases of such rare disease, improving its clinical characterization, and provides useful indications for the treatment of patients.

Diagnostic performance of a rapid immunochromatographic test for the simultaneous detection of antibodies to Theileria equi and Babesia caballi in horses and donkeys.

BACKGROUND: Equine piroplasmosis is caused by two tick-borne protozoan parasites, Theileria equi and Babesia caballi,, which are clinically relevant in susceptible horses, donkeys, and mules. Moreover, equine piroplasmosis significantly constrains international trading and equestrian events. Rapidly diagnosing both parasites in carrier animals is essential for implementing effective control measures. Here, a rapid immunochromatographic test for the simultaneous detection of antibodies to T. equi and B. caballi was evaluated using samples from horses and donkeys collected in Greece, Israel, and Italy. The results were compared with an improved competitive enzyme-linked immunosorbent assay (cELISA) for detecting antibodies to both parasites using the same panel of samples. METHODS: Blood samples were collected from 255 horses and donkeys. The panel consisted of 129 horses sampled at four locations in northern Greece, 105 donkeys sampled at four locations in Sicily, and 21 horses sampled at two locations in Israel. The rapid test and the cELISA were performed according to the manufacturer's instructions, and the results were subjected to a statistical analysis to determine the sensitivity and specificity of both tests and their association. RESULTS: The immunochromatographic test provided a result within 15 min and can be performed in the field, detecting both pathogens simultaneously. The overall coincidence rate between the rapid test and the cELISA for detecting antibodies against T. equi was 93% and 92.9% for B. caballi. The rapid test's sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) for T. equi were above 91.5%. Sixteen samples were positive for both parasites in the rapid test and eight in the cELISA. Either test had no significant association between T. equi and B. caballi detection. The detection rates of both parasites were significantly higher in Italy than in Greece or Israel and in donkeys than in horses. The agreement for T. equi between the results of both tests was high in Greece (93.8%) and Italy (95.2%) and moderate in Israel (76.2%). For B. caballi, the specificity and NPV of the rapid test were high (94.2% and 98.3%, respectively), although the sensitivity and PPV were moderate (69.2% and 39.1%, respectively) due to the small sample size. However, for B. caballi, the sensitivity was higher with the rapid test. CONCLUSIONS: The rapid test detected T. equi and B. caballi simultaneously in the field, potentially replacing laborious cELISA testing and is recommended for import/export purposes. The test can also be helpful for the differential diagnosis of clinical cases, since seropositivity may rule out equine piroplasmosis since it does not indicate current or active infection.

Machine learning-based algorithms applied to drug prescriptions and other healthcare services in the Sicilian claims database to identify acromegaly as a model for the earlier diagnosis of rare diseases.

Acromegaly is a rare disease characterized by a diagnostic delay ranging from 5 to 10 years from the symptoms' onset. The aim of this study was to develop and internally validate machine-learning algorithms to identify a combination of variables for the early diagnosis of acromegaly. This retrospective population-based study was conducted between 2011 and 2018 using data from the claims databases of Sicily Region, in Southern Italy. To identify combinations of potential predictors of acromegaly diagnosis, conditional and unconditional penalized multivariable logistic regression models and three machine learning algorithms (i.e., the Recursive Partitioning and Regression Tree, the Random Forest and the Support Vector Machine) were used, and their performance was evaluated. The random forest (RF) algorithm achieved the highest Area under the ROC Curve value of 0.83 (95% CI 0.79-0.87). The sensitivity in the test set, computed at the optimal threshold of predicted probabilities, ranged from 28% for the unconditional logistic regression model to 69% for the RF. Overall, the only diagnosis predictor selected by all five models and algorithms was the number of immunosuppressants-related pharmacy claims. The other predictors selected by at least two models were eventually combined in an unconditional logistic regression to develop a meta-score that achieved an acceptable discrimination accuracy (AUC = 0.71, 95% CI 0.66-0.75). Findings of this study showed that data-driven machine learning algorithms may play a role in supporting the early diagnosis of rare diseases such as acromegaly.

A new species of the genus Embia Latreille, 1825 (Insecta, Embioptera) from the Madonie Regional Natural Park (Sicily, Italy).

Embia minapalumboi n. sp., a new species of the genus Embia Latreille, 1925, was collected during an excursion in the Madonie Regional Natural Park that took place at the conclusion of the XXVII Italian National Congress of Entomology celebrated in Palermo from 12 to 16 June 2023. In the location of discovery, at an elevation of just under 1400 m and therefore unusual for European Embioptera, the new species was found to be very rare and for this reason most of the few juvenile specimens found were kept in breeding by the author. The new species was compared with all 36 species known to date for the genus and in particular with the 21 species from the Mediterranean area and the 8 known from Europe. The author also presents the general distribution of all species of the genus. Embia tyrrhenica, Stefani, 1953 is also reported for the Madonie and reconfirmed for Sicily.

RP1 Dominant p.Ser740* Pathogenic Variant in 20 Knowingly Unrelated Families Affected by Rod-Cone Dystrophy: Potential Founder Effect in Western Sicily.

Background and Objectives. Retinitis pigmentosa (RP) is the most common inherited rod-cone dystrophy (RCD), resulting in nyctalopia, progressive visual field, and visual acuity decay in the late stages. The autosomal dominant form (ADRP) accounts for about 20% of RPs. Among the over 30 genes found to date related to ADRP, RP1 pathogenic variants have been identified in 5-10% of cases. In a cohort of RCD patients from the Palermo province on the island of Sicily, we identified a prevalent nonsense variant in RP1, which was associated with ADRP. The objective of our study was to analyse the clinical and molecular data of this patient cohort and to evaluate the potential presence of a founder effect. Materials and Methods. From 2005 to January 2023, 84 probands originating from Western Sicily (Italy) with a diagnosis of RCD or RP and their relatives underwent deep phenotyping, which was performed in various Italian clinical institutions. Molecular characterisation of patients and familial segregation of pathogenic variants were carried out in different laboratories using Sanger and/or next-generation sequencing (NGS). Results. Among 84 probands with RCD/RP, we found 28 heterozygotes for the RP1 variant c.2219C>G, p.Ser740* ((NM_006269.2)*, which was therefore significantly prevalent in this patient cohort. After a careful interview process, we ascertained that some of these patients shared the same pedigree. Therefore, we were ultimately able to define 20 independent family groups with no traceable consanguinity. Lastly, analysis of clinical data showed, in our patients, that the p.Ser740* nonsense variant was often associated with a late-onset and relatively mild phenotype. Conclusions. The high prevalence of the p.Ser740* variant in ADRP patients from Western Sicily suggests the presence of a founder effect, which has useful implications for the molecular diagnosis of RCD in patients coming from this Italian region. This variant can be primarily searched for in RP-affected subjects displaying compatible modes of transmission and phenotypes, with an advantage in terms of the required costs and time for analysis. Moreover, given its high prevalence, the RP1 p.Ser740* variant could represent a potential candidate for the development of therapeutic strategies based on gene editing or translational read-through therapy for suppression of nonsense variants.

Characterization of an undocumented CO2 hydrothermal vent system in the Mediterranean Sea: Implications for ocean acidification forecasting.

A previously undocumented shallow water hydrothermal field from Sicily (Southern Tyrrhenian Sea, Italy) is here described, based on a multidisciplinary investigation. The field, covering an area of nearly 8000 m2 and a depth from the surface to -5 m, was explored in June 2021 to characterise the main physico-chemical features of the water column, describe the bottom topography and features, and identify the main megabenthic and nektonic species. Twenty sites were investigated to characterise the carbonate system. Values of pH ranged between 7.84 and 8.04, ΩCa between 3.68 and 5.24 and ΩAr from 2.41 to 3.44. Geochemical analyses of hydrothermal gases revealed a dominance of CO2 (98.1%) together with small amounts of oxygen and reactive gases. Helium isotope ratios (R/Ra = 2.51) and δ13CCO2 suggest an inorganic origin of hydrothermal degassing of CO2 and the ascent of heat and deep-seated magmatic fluids to the surface. Visual census of fishes and megabenthos (mainly sessile organisms) allowed the identification of 64 species, four of which are protected by the SPA/BIO Protocol and two by the International Union for Conservation of Nature. The macroalgae Halopteris scoparia and Jania rubens and the sponge Sarcotragus sp. were the dominant taxa in the area, while among fishes Coris julis and Chromis chromis were the most abundant species. This preliminary investigation of San Giorgio vent field suggests that the site could be of interest and suitable for future experimental studies of ocean acidification.

Identification of a novel intra-genotype reassortant G1P[8] rotavirus in Italy, 2021.

OBJECTIVES: Rotaviruses G1P[8] are epidemiologically relevant and are targeted by vaccines. The introduction of vaccines has altered rotavirus epidemiology. Hospital-based surveillance conducted in Sicily, Italy, showed a progressive decline in rotavirus prevalence since 2014, along with an increasing vaccine coverage (63.8% in 2020), and a marked decrease in circulation of G1P[8] strains. Surprisingly in 2021, G1P[8] viruses accounted for 90.5% (19/21) of rotavirus infections. This study aimed to understand if the increased activity of G1P[8]'s was related to virus-related peculiarities. DESIGN: In 2021, 266 patients <15 years of age were hospitalized with acute gastroenteritis (AGE) and included in rotavirus surveillance. Viral proteins (VP7 and VP4) genotyping and sequence data were generated from all rotavirus-positive samples. The genetic makeup of G1P[8] rotaviruses was investigated by full-genome sequencing. RESULTS: Peculiar G1P[8] rotaviruses, with VP7 and VP4 belonging to novel sub-lineages, circulated in 2021, accounting for 76.2% (16/21) of all rotavirus infections. On full-genome analysis, the novel G1P[8] variant displayed an intra-genotype (Wa-like) reassortant constellation, involving G12 and G1 strains, into a unique arrangement never observed before. The novel G1P[8] variant showed peculiar amino acid substitutions in 8-1 and 8-3 epitopes of the VP4 with respect to the Rotarix strain. CONCLUSIONS: Prompt identification of virus variants circulating in the human population is pivotal to understanding epidemiological trends and assessing vaccine efficacy.

Detection of Echovirus 11 lineage 1 in wastewater samples in Sicily.

We report the presence of Echovirus 11 (E11) in wastewater in Sicily (Southern Italy), since August 2022. Overall, the 5.4 % of sewage samples (7/130) collected in 2022 were positives for E11 and then the percentage of E11-positive sewage samples reached the value of 27.27(18/66) in the first semester of 2023. Phylogenetic analysis of VP1 sequences showed for most E11-positive samples (16/25: 64 %) close genetic correlation (98.4-99.4 % nucleotide identity) to E11 lineage 1 strains involved in recently reported severe neonatal infections.

Response to Genovesi et al.: Ant biosurveillance should come before invasion.

Menchetti et al. respond to the letter of Genovesi et al. and contribute new records of the red imported fire ant in Sicily.

Conservation action should come before publication.

With regard to the recent discovery of the red imported fire ant in Sicily (Menchetti et al. 2023), Genovesi et al. highlight the delay in communicating the observation and call on the scientific communities, scientific journals, and local authorities to ensure that new invasive alien species records are immediately reported for enhancing action.

Blood and hoof biodistibution of some trace element (Lithium, Copper, Zinc, Strontium and, Lead) in horse from two different areas of Sicily.

BACKGROUND AND PURPOSE: Biological monitoring of trace element horses is a well-known tool for investigating potential bioaccumulation in urbanized and industrialized geographical areas. Some biomaterials such as hoof are considered as an important indicator of environmental pollution. Hooves can store trace elements for a long time compared to blood and this represents a scientific key to long-term monitoring of exposure to environmental pollutants. In the present study, samples of equine hoof and blood were taken from an experimental group of horses living in an industrialized area of Sicily (Italy) and from a control group of horses housed in a non-industrialized area of Sicily to evaluate the bioaccumulation of different trace elements in different substrates. METHODS: Hoof and blood mineral concentration of Li- Lithium, Cu- Copper, Zn- Zinc, Sr- Strontium and, Pb- Lead was processed by means of Thermo Scientific iCAP-Q ICP-MS spectrometer. Student T-test was applied to evaluate the differences between groups and substrates. Correlation analysis between substrates and hematological parameters was performed. RESULTS: Statistical analysis showed a significant statistical difference for Li (p < 0.0001) and Pb (p < 0.0001) between blood and hoof in both groups. A statistically significant difference was observed for Li (p < 0.0001), Cu(p < 0.01), Zn (p < 0.0001) and Pb (p < 0.0001) between substrates in the experimental group. The statistical analysis showed a significant difference of blood Zn (p < 0.01) and hoof Pb (p < 0.01) concentration between groups. Our results revealed a statistically higher concentration of minerals in the blood substrate than in the hoof in both groups. Li (p < 0.0001) and Zn (p < 0.001) were positively correlated between control and experimental group in blood substrate. No correlations were found (P > .05) in hoof between groups for other elements. A positive correlation with WBC was observed in Cu concentration for hoof substrate (p < 0.02) in control group and for Zn in experimental group (p < 0.02). A positive correlation with HGB and HCT was observed for Cu hoof concentration (p < 0.02) in experimental group and PLT resulted positively correlated (p < 0.02) with Pb blood substrate in control group. CONCLUSIONS: Horses were not overexposed to these elements in the analyzed groups. The study highlights the role of a new bioaccumulation substrate such as the horse hoof identified as an analytical matrix for the monitoring of heavy metal concentrations in domestic animals.

Transfusion in hemoglobinopathies and red blood cell alloimmunization: data from Sicily, Sardinia and Malta.

BACKGROUND: Hemoglobinopathies are a group of diseases that include those due to globin gene mutations, such as thalassemia major (TM) and thalassemia intermedia (TI) or due to alteration of hemoglobin structure such as sickle cell disease (SCD), as well as a combination of these conditions such as thalasso-drepanocytosis (TD). They constitute the most frequent hereditary anemias requiring blood transfusion. MATERIALS AND METHODS: In April 2022, a questionnaire was sent to the Transfusion Services (TS) of Sicily, Sardinia and the Maltese National Blood Transfusion (MNBT) service. The questionnaire was divided into a generic part including the number of patients followed and the type of hemoglobinopathy, and a section relating to transfusion therapy, including the number of units transfused, whether red blood cells (RBC) were washed and, finally, a section relating to the presence or absence of alloantibodies and their identification. RESULTS: Data was retrieved for 2,574 patients: 68.6% TM, 15.4% TI, 10.3% TD, 4.1% SCD, and 1.6% other hemoglobinopathies (OHA). The number of RBC units transfused was 76,974, equivalent to 24.5% of all the RBCU transfused from the total number of patients followed. The number of washed RBCU was 21.1% of all the units used; 337 patients (37%) were diagnosed with alloantibodies, the majority of which were patients with SCD (20.6%). Of the 485 alloantibodies found, 90.3% were identified. The antibodies found most frequently were related to the Kell system (41.7%) followed by antibodies to the Rhesus system (37.9%); 29.7% of patients had more than one antibody. DISCUSSION: From our study, certain indications can be formulated: complete the National Registry for patients with hemoglobinopathies; create a Registry of alloimmunized patients to ensure transfusion therapy is as safe as possible, considering antibody evanescence; and 3) increase the recruitment of blood donors of diverse ethnicities.

Dental diseases and dental wear as a proxy for dietary patterns in Hellenistic-early Roman Menainon, Sicily.

OBJECTIVE: This paper explores dental diseases and wear as a proxy for dietary patterns in Hellenistic-early Roman Menainon. MATERIALS: This study includes 166 individuals (4th-1st c. BCE). METHODS: Carious lesions, dental calculus, antemortem tooth loss, and dental wear were recorded to explore male-female and adult-juvenile differences, and to position Menainon in the broader Hellenistic/early Roman world through comparisons with published data from other sites. RESULTS: Males and females showed similar rates of dental diseases. Dental wear, in contrast, was systematically greater in males. Caries rates were high in both adults and juveniles, but adults showed more calculus. The population from Menainon had higher frequencies for calculus and carious lesions compared to contemporary Italian and Greek assemblages, and a similar frequency for antemortem tooth loss. CONCLUSION: Some sex-related differences in the dietary patterns of the Menainon population were visible but small. The diet of adults and juveniles must have been similar in terms of carbohydrate consumption but different with regard to protein consumption. The high frequency of carious lesions and calculus compared to other Greco-Roman sites suggests that this population must have had good access to dietary resources (protein and carbohydrates). SIGNIFICANCE: This paper provides insights on gender (sex-related) and age divisions in the Hellenistic/early Roman society through the exploration of food consumption in a Sicilian assemblage. LIMITATIONS: Dividing the assemblage by sex and age group reduced considerably the sample size. SUGGESTIONS FOR FURTHER RESEARCH: Stable isotopes and dental microwear analyses should be used to investigate dietary patterns further.

Genetic screening for hereditary transthyretin amyloidosis with polyneuropathy in western Sicily: Two years of experience in a neurological clinic.

BACKGROUND AND PURPOSE: Hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) is caused by mutations in the TTR gene, leading to misfolded monomers that aggregate generating amyloid fibrils. METHODS: A prospective systematic genetic screening for ATTRv-PN was proposed in patients presenting with a sensory-motor idiopathic polyneuropathy and two or more "red flags" among the following: family history of polyneuropathy or cardiopathy, bilateral carpal tunnel syndrome, cardiac insufficiency, renal amyloidosis, lumbar tract stenosis, autonomic dysfunction, idiopathic gastrointestinal disease, amyloid deposits on biopsy, and vitreous opacities. The detection rate was calculated, and nonparametric analyses were carried out to underline differences among screened positive versus negative patients. RESULTS: In the first step, 145 suspected patients underwent genetic testing, revealing a diagnosis of ATTRv-PN in 14 patients (10%). Then, cascade screening allowed early recognition of 33 additional individuals (seven symptomatic ATTRv-PN patients and 26 presymptomatic carriers) among 84 first-degree relatives. Patients with a positive genetic test presented a higher frequency of unexplained weight loss, gastrointestinal symptoms, and family history of cardiopathy. CONCLUSIONS: A systematic screening for ATTRv-PN yielded an increased recognition of the disease in our neurological clinic. Unexplained weight loss associated with axonal polyneuropathy had the highest predictive value in the guidance of clinical suspicion. A focused approach for the screening of ATTRv-PN could lead to an earlier diagnosis and identification of asymptomatic carriers, who will be promptly treated after a strict follow-up at the clinical onset.

Assessing the effect of Amphistegina lobifera invasion on infralittoral benthic foraminiferal assemblages in the Sicily Channel (Central Mediterranean).

This article documents, through a quantitative approach, the negative effect of the highly invasive species Amphistegina lobifera Larsen, 1976 on native benthic foraminiferal assemblages of coastal areas in the Sicily Channel (Central Mediterranean). A nested sampling design was applied through the comparison of benthic foraminiferal community structure across three areas that are known to be at different stages of invasion (i.e. Maltese Islands - advanced, southern Sicily - medium, and eastern Sicily - early). Results suggested that both diversity and richness of benthic foraminiferal community from the Maltese Islands were strongly modified by increased abundances of A. lobifera. In contrast, this phenomenon is less evident in southern and eastern Sicily, where the invader displayed lower abundances and the community structure was more diversified. Collected data also allowed for predicting what could happen in the near future in the whole Sicily Channel, as well as in the rest of the Mediterranean Sea.

Insights for brucellosis eradication in Italy through a model-based spread evaluation in grazing livestock - Sicily case study.

Brucellosis is one of the world's major zoonotic pathogens and is responsible for enormous economic losses as well as considerable human morbidity in endemic areas. Definitive control of human brucellosis requires control of brucellosis in livestock through practical solutions that can be easily applied to the field. In Italy, brucellosis remains endemic in several southern provinces, particularly in Sicily Region. The purpose of this paper is to describe the developed brucellosis model and its applications, trying to reproduce as faithfully as possible the complex transmission process of brucellosis accounting for the mixing of grazing animals. The model focuses on the contaminated environment rather than on the infected animal, uses real data from the main grazing areas of the Sicily Region, and aims to identify the best control options for minimizing the spread (and the prevalence) and to reach the eradication within the concerned areas. Simulation results confirmed the efficacy of an earlier application of the controls, showed the control should take place 30 days after going to pasture, and the culling time being negligible. Moreover, results highlighted the importance of the timing of both births and grazing pastures (and their interaction) more than other factors. As these factors are region­specific, the study encourages the adoption of different and new eradication tools, tuned on the grazing and commercial behavior of each region. This study will be further extended to improve the model's adaptability to the real world, with the purpose of making the model an operational tool able to help decision makers in accelerating brucellosis eradication in Italy.

Attitudes towards Italian Mafias Scale (AIMS): development and validation.

In two studies we aimed at developing the Attitude towards Italian Mafias Scale (AIMS). In study 1 (N = 292) we used an Exploratory Factor Analysis to reduce the number of the items and explore their latent constructs. In study 2 (N = 393) we performed a Confirmatory Factor Analysis on the resulting 18-item questionnaire, whose latent structure was best identified by a general factor Mafia Attitude and three specific factors related to Behaviors, Cognitions and Emotions-Cognitions towards mafias. Moreover, we showed that the AIMS has (i) discriminant validity compared to a measure of attitudes towards crime, (ii) predictive validity of donation behavior to an association against mafias, (iii) internal consistency, and (iv) invariance for people of the five deep-rooted mafia regions of Southern Italy and those from the rest of Italy. Finally, we observed a difference between the participants from the five deep-rooted mafia regions (i.e., Apulia, Basilicata, Calabria, Campania, Sicily) and the rest of Italy, with the former having surprisingly more negative attitudes towards mafias compared to the latter. The AIMS might help to reliably survey people's sentiment towards Italian mafias and promote targeted and effective law-related education interventions.

Soundscape of a Mediterranean seashore during loggerhead sea turtle (Caretta caretta) spawning season.

The soundscape is an intrinsic property of an ecosystem and influences the species that live in it. Here, we examined for the first time the soundscape of a beach, one of the most dynamic ecosystems on Earth, where every year the loggerhead sea turtle Caretta caretta lays eggs. The aim of this work was to analyze the acoustic components (biophony, anthropophony and geophony) to which turtles embryos were exposed throughout the development and the post-hatching period. The acoustic monitoring was carried out on the volcanic island of Linosa (central Mediterranean Sea, Strait of Sicily), during the months of July and August 2022, close to two turtle nests. Results revealed that all the acoustic levels (octave bands from 4 Hz to 16 kHz, and total 1-24,000 Hz band) showed lower values in July, and during the night. Furthermore, above 1 kHz the levels decreased and had very little variability. Anthropogenic noise was the main component of the soundscape and consisted of marine and land traffic, that affected sound levels directly or via seismic tremors. When the beach was exposed to the breaking waves, the latters were the first contributor to the noise up to 1 kHz. The only recognized biophony was represented by the shearwater choruses in July (at the frequency band 700-1500 Hz), but they had a negligible weight on the soundscape. Finally, human speech contributed to the soundscape at higher frequencies (1-8 kHz). These outcomes show that the embryos and the post-hatching turtles are exposed to a high anthropogenic noise level, which the effects of are still unknown.

Characterization of artisanal saffron ricotta cheese produced in Sicily: Physicochemical, microbiological, sensory, and antioxidant characteristics.

The present study aims to characterize the artisanal saffron ricotta cheese produced from the whey of Piacentinu Ennese protected designation of origin (PDO) cheesemaking, including via technological parameters detected during the production process and by assessment of the main physicochemical, microbial, sensory, and antioxidant characteristics. A survey on the manufacture process of saffron and control ricotta cheese was conducted on 3 farms, located in the production area of the Piacentinu Ennese PDO cheese. pH and temperature followed a specific behavior, characterized by an inverse trend where pH decreased and temperature increased, playing an important role in the production process. All the analytical parameters were affected by the presence of saffron, also showing high between-farm variability, with significantly higher total solids and fat contents in saffron ricotta cheese compared with the control cheese (28.68% vs. 23.86%, and 19.83% vs. 14.22%, respectively). Microbial analysis showed significantly lower values in saffron compared with control ricotta cheese, for coliforms (1.51 vs. 1.91 log10 cfu/g, respectively), yeasts (1.55 vs. 2.06 log10 cfu/g, respectively), and molds (1.03 vs. 1.30 log10 cfu/g, respectively), denoting potential reduction of microbial growth asserted by saffron. Escherichia coli concentration (1.26 log10 cfu/g) in saffron ricotta cheese was in accordance with EU Regulation 2073/2005 and then safe for consumption. The presence of saffron influenced all sensory attributes, particularly color and aroma. Interestingly, high total antioxidant activity was found in saffron ricotta cheese (372 µC) compared with the control cheese. Thus, this artisanal dairy production could be considered a suitable option for functional foods with antimicrobial properties, due to the presence of saffron, which may contribute to extend the shelf life of the product. Further studies need to focus on the bioactive compounds that affect the antioxidant proprieties, characterization of the microbiota of saffron ricotta cheese, and evaluation of consumers' acceptance and perception as well as market demand.

Fluoro-edenite non-neoplastic diseases in Biancavilla (Sicily, Italy): pleural plaques and/or pneumoconiosis?

BACKGROUND: A mesothelioma cluster in Biancavilla (Sicily, Italy), drew attention to fluoro-edenite, a fibre classified by International Agency for Research on Cancer as carcinogenic to humans. Significant excesses in mortality and morbidity were observed for respiratory diseases and a significant excess of pneumoconiosis hospitalizations was reported. OBJECTIVE: Aim of this study is to assess the characters of the lung damage in Biancavilla residents hospitalized with pneumoconiosis or asbestosis diagnoses. METHODOLOGY: Medical records, available radiographs and computed tomography scans were collected. The obtained imaging was reviewed by a panel of three specialists and focused on pleural and parenchymal abnormalities. Cases with an ILO-BIT or ICOERD score equal or greater than 2 were considered positive for a pneumoconiosis-like damage, cases with a score lower than 2 or insufficient quality of imaging were considered inconclusive. If no pneumoconiotic aspects were present the cases were classified as negative. RESULTS: Out of 38 cases, diagnostic imaging for 25 cases were found. Ten cases out of 25 showed asbestosis-like features, nine subjects were considered negative. In six patients' results were inconclusive. CONCLUSIONS: Asbestosis-like features were substantiated in Biancavilla residents without known occupational exposure to asbestos. Further studies to estimate population respiratory health are required. Experimental studies on the fibrogenic potential of fluoro-edenite are needed.