Multi-disciplinary insights from the First European Forum on Visceral Myopathy 2022 Meeting

Visceral myopathy is a rare, life-threatening disease linked to identified genetic mutations in 60% of cases. Mostly due to the dearth of knowledge regarding its pathogenesis, effective treatments are lacking. The disease is most commonly diagnosed in children with recurrent or persistent disabling episodes of functional intestinal obstruction, which can be life threatening, often requiring long-term parenteral or specialized enteral nutritional support. Although these interventions are undisputedly life-saving as they allow affected individuals to avoid malnutrition and related complications, they also seriously compromise their quality of life and can carry the risk of sepsis and thrombosis. Animal models for visceral myopathy, which could be crucial for advancing the scientific knowledge of this condition, are scarce. Clearly, a collaborative network is needed to develop research plans to clarify genotype–phenotype correlations and unravel molecular mechanisms to provide targeted therapeutic strategies. This paper represents a summary report of the first ‘European Forum on Visceral Myopathy’. This forum was attended by an international interdisciplinary working group that met to better understand visceral myopathy and foster interaction among scientists actively involved in the field and clinicians who specialize in care of people with visceral myopathy

Increased DHEAS and Decreased Total Testosterone Serum Levels in a Subset of Men with Early-Onset Androgenetic Alopecia: Does a Male PCOS-Equivalent Exist?

Background. Increased dehydroepiandrosterone sulfate (DHEAS) levels have been reported in men with early-onset (25 kg/m2, insulin resistance (IR), and/or SHBG 25 kg/m2, IR, and SHBG <25 nmol/l have increased DHEAS levels and a worse gonadal steroidogenesis. They might have a greater risk to develop gonadal dysfunction later in life. These criteria may be used to define male PCOS-equivalent

Children accessing accident and emergency department for non-urgent consultations: A cross-sectional study about parents’ use of primary care services

Introduction: Parents often take their children to the Paediatric Accident and Emergency Department (A&amp;E) for non-urgent consultations rather than using community-based primary care services. This study describes the use of primary care services in parents taking their children to the A&amp;E for non-urgent consultations. Methods: A cross-sectional study was conducted from July 2018 to June 2019, in a second-level Italian paediatric A&amp;E of a tertiary-level children's academic research and hub hospital. Parents of children aged between 3 months and 6 years assigned with a white code at the triage were asked to complete a paper-and-pencil 40-item questionnaire after accessing the A&amp;E for a non-urgent consultation. Results: The questionnaire was completed by the parents of 237 children (males 58 %; median age = 2.3 years). Overall, 48.1 % (n = 114) of the parents reported consulting 'often/always' the primary care paediatrician, mainly when their child was sick and for check-ups (n = 182, 76.8 %). However, only 7.2 % (n = 17) of the parents 'often/always' used any other health service in the community. Most of them (n = 191, 82 %) did not even know where the community health centre was located. Conclusion: Parents accessing the A&amp;E for non-urgent consultations should be better informed/educated on how to use community health services

Topical prebiotics/postbiotics and PRURISCORE validation in atopic dermatitis. International study of 396 patients

Aim: To investigate the efficacy and tolerability of a cream (Rilastil Xerolact PB) containing a mixture of prebiotics and postbiotics, and to validate the PRURISCORE itch scale in the management of atopic dermatitis. Methods: The study is based on 396 subjects of both sexes in three age groups (i.e., infants, children, adults) suffering from mild/moderate Atopic Dermatitis, recruited from 8 European countries and followed for 3 months. Results: The product demonstrated good efficacy combined with good/very good tolerability in all age groups. In particular, SCORAD, PRURISCORE and IGA scores decreased significantly over the course of the study. The PRURISCORE was preferred to VAS by the vast majority of patients. Conclusion: Even though the role of prebiotics and postbiotics was not formally demonstrated since these substances were part of a complex formulation, it can be reasonably stated that prebiotics and postbiotics have safety and standardization features that probiotics do not have. In addition they are authorized by regulatory authorities, whereas topical probiotics are not