Inflammation in Fabry disease: stages, molecular pathways, and therapeutic implications.

Fabry disease, a multisystem X-linked disorder caused by mutations in the alpha-galactosidase gene. This leads to the accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (Lyso-Gb3), culminating in various clinical signs and symptoms that significantly impact quality of life. Although treatments such as enzyme replacement, oral chaperone, and emerging therapies like gene therapy exist; delayed diagnosis often curtails their effectiveness.

Real-world evidence study on tolerance and growth in infants fed an infant formula with two human milk oligosaccharides vs mixed fed and exclusively breastfed infants.

Introduction: Human milk oligosaccharides (HMOs) are important components of human milk having diverse functions in the development of infants. Randomized controlled trials (RCTs) have demonstrated that infant formulas with the HMOs 2'-fucosyllactose (2'FL) and lacto-N-neotetraose (LNnT) are safe, well-tolerated, and support normal growth. This study aimed to generate real-world evidence (RWE) on growth and gastrointestinal (GI) tolerance in infants consuming a formula with 1 g/L 2'FL and 0.

subsp. Reduces Perceived Psychological Stress in Healthy Adults: An Exploratory Clinical Trial.

Emerging science shows that probiotic intake may impact stress and mental health. We investigated the effect of a 6-week intervention with (1 × 10 CFU/daily) on stress-related psychological and physiological parameters in 45 healthy adults with mild-to-moderate stress using a randomized, placebo-controlled, two-arm, parallel, double-blind design. The main results showed that supplementation with the probiotic significantly reduced the perceived stress and improved the subjective sleep quality score compared to placebo.

The neural signature of reality-monitoring: A meta-analysis of functional neuroimaging studies.

Distinguishing imagination and thoughts from information we perceived from the environment, a process called reality-monitoring, is important in everyday situations. Although reality monitoring seems to overlap with the concept of self-monitoring, which allows one to distinguish self-generated actions or thoughts from those generated by others, the two concepts remain largely separate cognitive domains and their common brain substrates have received little attention. We investigated the brain regions involved in these two cognitive processes and explored the common brain regions they share.

A Probiotic Intervention With Bifidobacterium longum NCC3001 on Perinatal Mood Outcomes (PROMOTE Study): Protocol for a Decentralized Randomized Controlled Trial.

Background: Perinatal mood disorders such as depression and anxiety are common, with subclinical symptomology manifesting as perinatal mood disturbances being even more prevalent. These could potentially affect breastfeeding practices and infant development. Pregnant and lactating women usually limit their exposure to medications, including those for psychological symptoms.

DSM 17938-Containing Infant Formulas and the Associations with Gastrointestinal Tolerance: A Cross-Sectional Observational Study.

(.; previously ) has been shown to influence gastrointestinal (GI) tolerance. This study was a secondary analysis of GI tolerance data from a multi-country, cross-sectional, observational study in healthy infants using the validated Infant Gastrointestinal Symptom Questionnaire (IGSQ) and a gut comfort questionnaire.

The effect of modified optic flow gain on quiet stance.

Visual feedback provides critical information to support postural stability. Previous work has shown that magnifying visual feedback, such as by presenting individuals with biofeedback during balance tasks, can improve postural control. When studies manipulate the availability of optic flow directly, the conditions are often restricted to include an absence of visual feedback or sway referenced paradigms.

Infant feeding practice and gastrointestinal tolerance: a real-world, multi-country, cross-sectional observational study.

Background: Signs of feeding intolerance, such as gastrointestinal (GI) symptoms, are frequently observed in otherwise healthy formula-fed infants in the first months of life. The primary objective of this observational study was to examine GI tolerance in formula-fed infants (FFI) vs. breastfed infants (BFI) in a real-world setting with a secondary objective being the comparison of infants fed formula with pre- and/or probiotics (FFI_PP) and those fed formula without any pre- and/or probiotics (FFI_noPP) as well as BFI.

Continuous subcortical monitoring of motor pathways during glioma surgery with ultrasonic surgical aspirator: technical description in a single institute experience.

Background: Actual challenge in surgical treatment of intra-axial gliomas involving eloquent areas is maximal safe resection. Mapping and monitoring of cortical and subcortical motor functions are important tools to avoid postoperative deficits. In the present study, we present our experience with a continuous dynamic motor mapping technique pairing a traditional monopolar stimulator with a Cavitron ultrasonic surgical aspirator (CUSA) to perform a continuous stimulation of the white matter avoiding removal interruption.

Mode of Neonatal Delivery Influences the Nutrient Composition of Human Milk: Results From a Multicenter European Cohort of Lactating Women.

Background: The effect of the mode of neonatal delivery (cesarean or vaginal) on the nutrient composition of human milk (HM) has rarely been studied. Given the increasing prevalence of cesarean section (C-section) globally, understanding the impact of C-section vs. vaginal delivery on the nutrient composition of HM is fundamental when HM is the preferred source of infant food during the first 4 postnatal months.

Are psychological debriefing groups after a potential traumatic event suitable to prevent the symptoms of PTSD?

Psychological debriefing is a psychotherapeutic approach developed for early intervention in civilians and professionals who have to cope with a potential traumatic event (PTE). Although initial works claimed that this approach may decrease symptoms of posttraumatic stress disorder (PTSD), some studies have concluded that the clinical benefits of psychological debriefing were limited. Several methodological variations could explain the discrepancies observed among studies.

Neuroanatomical correlates of reality monitoring in patients with schizophrenia and auditory hallucinations.

Background: Reality-monitoring process enables to discriminate memories of internally generated information from memories of externally derived information. Studies have reported impaired reality-monitoring abilities in schizophrenia patients with auditory hallucinations (AHs), specifically with an exacerbated externalization bias, as well as alterations in neural activity within frontotemporoparietal areas. In healthy subjects, impaired reality-monitoring abilities have been associated with reduction of the paracingulate sulcus (PCS).

Intracranial soft-tissue glomus tumor (glomangioma) in a young-woman. A case report and review of the literature.

Background: Glomus tumors, or glomangiomas, are benign vascular tumors typically seen at distal extremities. These tumors differ from paragangliomas and classically present in the female population between the 4th and 5th decade. Intracranial localizations have not been described in literature in the adult population.

Subpial technique in supratentorial glioma resection: state of the art and analysis of costs and effectiveness in a single institute experience.

Background: Many neurosurgeons advocate subpial technique as the best technique to remove supratentorial gliomas. However, few authors clearly defined advantages and features of this technique. The aim of our study is to describe microsurgical subpial technique related to glioma surgery, with regard to its safety and cost effectiveness.

Impaired self-recognition in individuals with no full-blown psychotic symptoms represented across the continuum of psychosis: a meta-analysis.

Background: Impairments in self-recognition (i.e. recognition of own thoughts and actions) have been repeatedly shown in individuals with schizophrenia.

Cognitive insight in individuals with an at-risk mental state for psychosis: A meta-analysis.

Aim: To compare cognitive insight abilities measured with the Beck Cognitive Insight Scale (BCIS) between individuals with an at-risk mental state (ARMS) and healthy controls.

Method: Review and meta-analysis based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.

Results: A search for articles investigating cognitive insight in ARMS in the MEDLINE and ScienceDirect databases revealed five studies including 303 ARMS and 376 controls.

Dissociable source-monitoring impairments in obsessive-compulsive disorder and schizophrenia.

Background: Based on the observed clinical overlap between obsessive-compulsive disorder (OCD) and schizophrenia (SCZ), both conditions may share, at least in part, common cognitive underpinnings. Among the cognitive deficits that could be involved, it has been hypothesized that patients share a failure in their abilities to monitor their own thoughts (source monitoring), leading to confusion between what they actually did or perceived and what they imagined. Although little is known regarding source-monitoring performances in patients with OCD, numerous studies in patients with SCZ have observed a relationship between delusions and/or hallucinations and deficits in both internal source- and reality-monitoring abilities.

Review of source-monitoring processes in obsessive-compulsive disorder.

Obsessive-compulsive disorder (OCD) is a severe mental illness characterized by persistent, intrusive and distressing obsessions and/or compulsions. Such symptoms have been conceptualized as resulting from a failure in source-monitoring processes, suggesting that patients with OCD fail to distinguish actions they perform from those they just imagine doing. In this study, we aimed to provide an updated and exhaustive review of the literature examining the relationship between source-monitoring and OCD.

Reality-monitoring deficits and visual hallucinations in schizophrenia.

The presence of visual hallucinations in addition to auditory hallucinations (V + AH) is associated with poor prognosis in patients with schizophrenia. However, little consideration has been given to these symptoms and their underlying cognitive bases remain unclear. Based on cognitive models of hallucinations, we hypothesized that V + AH are underpinned by an impairment in reality-monitoring processes.