The neuronal ceroid lipofuscinosis type 2 - associated variants: An analysis of alterations in the gene and genotype-phenotype correlation in Ukraine.

The neuronal ceroid lipofuscinosis type 2 (CLN2) is a heterogeneous group of neurodegenerative lysosomal storage disorders caused by autosomal recessive inheritance of two pathogenic variants in trans in the gene. Classical late-infantile CLN2 disease has a very well-defined natural history. However, a small number of patients with TPP1 enzyme deficiency present a later onset or protracted disease course within this group there are phenotypic variants.

Extending the phenotypic spectrum assessed by the CDR plus NACC FTLD in genetic frontotemporal dementia.

Introduction: We aimed to expand the range of the frontotemporal dementia (FTD) phenotypes assessed by the Clinical Dementia Rating Dementia Staging Instrument plus National Alzheimer's Coordinating Center Behavior and Language Domains (CDR plus NACC FTLD).

Methods: Neuropsychiatric and motor domains were added to the standard CDR plus NACC FTLD generating a new CDR plus NACC FTLD-NM scale. This was assessed in 522 mutation carriers and 310 mutation-negative controls from the Genetic Frontotemporal dementia Initiative (GENFI).

Comment and integration to Andreone et al. 2022 "Reconnecting research and natural history museums in Italy and the need of a national collection biorepository".

In Andreone et al. (2022), the authors described a background about the Italian Natural History Museums (NHMs) situation, highlighting difficulties regarding the coordination among institutes due to the fragmented landscape, from the past until today. They suggested how having a national institute, the Future National Biodiversity Centre (FCNB), woul d represent the best solution to the problem.

The relationship between executive functions and writing in children: a systematic review.

Writing is a complex task that is acquired in the early primary school years and continues to develop through adolescence and beyond. Studying the cognitive processes that support writing skills during the acquisition phase may be crucial to support this complex skill especially in less-skilled writers. Executive Functions (EF) could have an important role as they are high cognitive control processes that allow individuals to control and plan thoughts and actions in order to achieve a goal.

rasterdiv-An Information Theory tailored R package for measuring ecosystem heterogeneity from space: To the origin and back.

Ecosystem heterogeneity has been widely recognized as a key ecological indicator of several ecological functions, diversity patterns and change, metapopulation dynamics, population connectivity or gene flow.In this paper, we present a new R package-rasterdiv-to calculate heterogeneity indices based on remotely sensed data. We also provide an ecological application at the landscape scale and demonstrate its power in revealing potentially hidden heterogeneity patterns.

Impairment of episodic memory in genetic frontotemporal dementia: A GENFI study.

Introduction: We aimed to assess episodic memory in genetic frontotemporal dementia (FTD) with the Free and Cued Selective Reminding Test (FCSRT).

Methods: The FCSRT was administered in 417 presymptomatic and symptomatic mutation carriers (181 chromosome 9 open reading frame 72 [], 163 progranulin [], and 73 microtubule-associated protein tau []) and 290 controls. Group differences and correlations with other neuropsychological tests were examined.

Liver fibrosis score, physical frailty, and the risk of dementia in older adults: The Italian Longitudinal Study on Aging.

Introduction: Liver fibrosis increases progressively with aging and has been associated with poorer cognitive performance in middle-aged and older adults. We investigated the relationships between a non-invasive score for advanced liver fibrosis (non-alcoholic fatty liver disease [NAFLD] fibrosis score [NFS]) and dementia risk. We also assessed physical frailty, a common geriatric condition which is associated to dementia.

Chemical characterization and antioxidant properties of products and by-products from L.

The products and by-products of L.: olive fruits (primary agricultural product), oils (primary agro-industrial product), pomaces (agro-industrial processing by-product), and leaves (agricultural practices by-product), are promising sources of bioactive compounds. In the present study, qualitative and quantitative analyses of selected bioactive components in olive fruits, oils, and pomaces were performed.

DOTA-Derivatives of Octreotide Dicarba-Analogs with High Affinity for Somatostatin sst Receptors.

somatostatin receptor scintigraphy is a valuable method for the visualization of human endocrine tumors and their metastases. In fact, peptide ligands of somatostatin receptors (sst's) conjugated with chelating agents are in clinical use. We have recently developed octreotide dicarba-analogs, which show interesting binding profiles at sst's.

Green Positive Guidance and Green Positive Life Counseling for Decent Work and Decent Lives: Some Empirical Results.

This article discusses green positive guidance and green positive life counseling for decent work and decent lives. From a green guidance perspective, the connectedness to nature construct is important both in terms of the meaning of work and life construction. The study discussed in this article analyzed the relationship between empathy and connectedness to nature, controlling for the effects of fluid intelligence and personality traits.

Factor Structure and Psychometric Properties of the Anxiety Sensitivity Index-3 in an Italian Community Sample.

Anxiety Sensitivity (AS) is defined as the fear of anxiety and of arousal-related bodily sensations, arising from erroneous beliefs that these sensations will have adverse consequences. AS plays a key role both in the onset and in the maintenance of several disorders, particularly anxiety disorders. To date, only two studies on American samples have examined the bifactor structure of the Anxiety Sensitivity Index-3 (ASI-3); therefore, findings on different cultures are needed.

Workplace Bullying in a Sample of Italian and Spanish Employees and Its Relationship with Job Satisfaction, and Psychological Well-Being.

Purpose - The purpose of this study is to examine the prevalence rate of workplace bullying in a sample of Italian and Spanish employees, and its differential consequences on employees' job satisfaction and psychological well-being. The effects of workplace bullying on job satisfaction and psychological well-being were explored taking into account a contextualized approach. Design/Methodology/approach - Cross-sectional study was adopted, in which a sample of 1,151 employees in Italy and 705 in Spain completed a questionnaire.

Sphingosine 1-phosphate signaling pathway in inner ear biology. New therapeutic strategies for hearing loss?

Hearing loss is one of the most prevalent conditions around the world, in particular among people over 60 years old. Thus, an increase of this affection is predicted as result of the aging process in our population. In this context, it is important to further explore the function of molecular targets involved in the biology of inner ear sensory cells to better individuate new candidates for therapeutic application.

Emotions shape memory suppression in trait anxiety.

The question that motivated this study was to investigate the relation between trait anxiety, emotions and memory control. To this aim, memory suppression was explored in high and low trait anxiety individuals with the Think/No-think paradigm. After learning associations between neutral words and emotional scenes (negative, positive, and neutral), participants were shown a word and were requested either to think about the associated scene or to block it out from mind.

A trial of preventing early- and late-onset Group B streptococcal sepsis with combined intrapartum chemoprophylaxis and universal neonatal screening.

Background: Prevention of early-onset Group B Streptococcal (GBS) infection has been attempted by employing universal maternal screening for GBS, intrapartum chemoprophylaxis, and a single dose of penicillin given to neonates in the first hour of life. This strategy, however, does not seem to prevent the occurrence of late-onset neonatal group B streptococcal disease.

Study Design: We assessed early and late-onset GBS disease with the use of a before-after study designed to evaluate the implementation of intrapartum antimicrobial prophylaxis.