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Abstract Objective: Adult patients with the genetic disease neurofibromatosis type 1 (NF1) frequently report social difficulties. To date, however, only two studies have explored whether these difficulties are caused by social cognition deficits, and these yielded contradictory data. The aim of the present study was to exhaustively assess social cognition abilities (emotion, theory of mind, moral reasoning, and social information processing) in adults with NF1, compared with a control group, and to explore links between social cognition and disease characteristics (mode of inheritance, severity, and visibility). Method: We administered a social cognition battery to 20 adults with NF1 (mean age = 26.5 years, SD = 7.4) and 20 healthy adults matched for sociodemographic variables. Results: Patients scored significantly lower than controls on emotion, theory of mind, moral reasoning, and social information processing tasks. No effects of disease characteristics were found. Conclusions: These results appear to confirm that adults with NF1 have a social cognition weaknesses that could explain, at least in part, their social difficulties, although social abilities are not all impaired to the same extent. Regarding the impact of the disease characteristics, the patient sample seemed slightly insufficient for the power analyses performed. Thus, this exploratory study should form the basis of further research, with the objective of replicating these results with larger and more appropriately matched samples.

The atrial flow regulator is a new self-expandable double-disc fenestrated device providing a calibrated inter-atrial communication. Paediatric reports are scarce. We herein describe a case of complete atrioventricular block complicating the exemption use of an atrial flow regulator in a 5-kg infant with transposition of the great arteries, ventricular septal defect, and right ventricular outflow tract obstruction.

Geophagia is a feeding behavior involving the regular intake of soil, including clay-like kaolin. Frequent in Africa, kaolin consumption is associated with heavy metal intoxication, iron and other micronutrient deficiencies, geohelminth infection and inactivation of concomitantly taken drugs. It is expected that this practice would be imported into an asylum country during the immigration process. To confirm this hypothesis, a single center, cross-sectional study was conducted at the University Hospital of Nantes, France, whose main objective was to assess whether the prevalence of kaolin consumers was high in a migrant population living in a large French metropolitan area (the city of Nantes). Each woman consulting for the first time at the Medical and Psychosocial Gynecology Obstetric Unit during the inclusion period ranging from January 1, 2017, to July 1, 2017, was asked for consent to be included in the study. The main outcome was the proportion of positive answers regarding consumption of kaolin within the last twelve months, with its 95% confidence interval (CI). A logistic regression was performed to identify drivers of consumption, and a clustering approach was conducted to identify profiles of consumers. A total of 284 women were included in the study, of whom 110 (38.7%) were pregnant. Our main finding was a 14.1% (95% CI: 10.5-18.6) prevalence of clay consumers. Second, the characteristic most strongly associated with consumption was Central or West Africa origin (adjusted Odds Ratio (aOR) = 52.7; 95% CI: 13.7-202.2). Finally, 60% of consumers showed signs of addictive-like phenomena, and three profiles were identified, depicting a continuum of patients in regard to their control over their kaolin consumption. Our results suggest that kaolin consumption is frequent in particular subpopulations of migrants. This warrants further study of the clinical consequences of kaolin consumption and its associated addictive-like symptoms.

BackgroundAfter idiopathic epiretinal membrane (iERM) removal, it is unclear whether the internal limiting membrane (ILM) should be removed. The objective was to assess if active ILM peeling after iERM removal could induce microscotomas.MethodsThe PEELING study is a national randomised clinical trial. When no spontaneous ILM peeling occurred, patients were randomised either to the ILM peeling or no ILM peeling group. Groups were compared at the month 1 (M1), M6 and M12 visits in terms of microperimetry, best-corrected visual acuity (BCVA) and optical coherence tomography findings. The primary outcome was the difference in microscotoma number between baseline and M6.Results213 patients were included, 101 experienced spontaneous ILM peeling and 100 were randomised to the ILM peeling (n=51) or no ILM peeling group (n=49). The difference in microscotoma number between both groups was significant at M1 (3.9 more microscotomas in ILM peeling group, (0.8;7.0) p=0.0155) but not at M6 (2.1 more microscotomas in ILM peeling group (−0.5;4.7) p=0.1155). Only in the no ILM peeling group, the number of microscotomas significantly decreased and the mean retinal sensitivity significantly improved. The ERM recurred in nine patients in the no ILM peeling group (19.6%) versus zero in the ILM peeling group (p=0.0008): two of them underwent revision surgery. There was no difference in mean BCVA and microperimetry between patients experiencing or not a recurrence at M12.ConclusionSpontaneous ILM peeling is very common. Active ILM peeling prevents anatomical ERM recurrence but may induce retinal impairments and delay visual recovery.Trial Registration NCT02146144.

Pulmonary atresia with ventricular septal defect, non-confluent pulmonary arteries, and bilateral arterial duct is a rare and complex CHD. Physiologic ductal closure may lead to life-threatening hypoxia. We present a case of successful bilateral ductal stenting as a bridge to further lower-risk surgical repair.

Escherichia coli is one of the first causes of Gram-negative orthopedic implant infections (OII), but little is known about the pathogenicity of this species in such infections that are increasing due to the ageing of the population. We report how this pathogen interacts with human osteoblastic MG-63 cells in vitro, by comparing 20 OII E. coli strains to two Staphylococcus aureus and two Pseudomonas aeruginosa strains. LDH release assay revealed that 6/20 (30%) OII E. coli induced MG-63 cell lysis whereas none of the four control strains was cytotoxic after 4 h of coculture. This high cytotoxicity was associated with hemolytic properties and linked to hlyA gene expression. We further showed by gentamicin protection assay and confocal microscopy that the non-cytotoxic E. coli were not able to invade MG-63 cells unlike S. aureus strains (internalization rate <0.01% for the non-cytotoxic E. coli versus 8.88 ± 2.31% and 4.60 ± 0.42% for both S. aureus). The non-cytotoxic E. coli also demonstrated low adherence rates (<7%), the most adherent E. coli eliciting higher IL-6 and TNF-α mRNA expression in the osteoblastic cells. Either highly cytotoxic or slightly invasive OII E. coli do not show the same infection strategies as S. aureus towards osteoblasts. This study describes the interaction of Escherichia coli clinical strains with an osteoblastic cell line, showing no internalization unlike Staphylococcus aureus, but a strong cytotoxicity of the Hly-A producing strains.

Background Preterm birth is associated with higher risks of suboptimal neurodevelopment and cardiometabolic disease later in life. Altered maternal-fetal lipid supply could play a role in such risks. Our hypothesis was that very preterm infants born with very low birth weight (VLBW) have altered lipidome and apolipoprotein profiles, compared with term infants. Methods Seven mothers of VLBW infants born at <32 GA and 8 full-term mother-infant dyads were included. Cholesterol and triglycerides in lipoproteins were determined in maternal plasma and in the two blood vessels of the umbilical cord (vein (UV) and artery (UA)) following FPLC isolation. Apolipoprotein concentrations in lipoproteins and plasma lipidomic analysis were performed by LC-MS/MS. Results We found higher cholesterol and VLDL-cholesterol in UV and UA and lower apolipoprotein A-I in HDL2 in UV in preterm neonates. Phosphatidylcholine (PC) containing saturated and monounsaturated fatty acids and specific sphingomyelin species were increased in UV and UA, whereas PC containing docosahexaenoic acid (DHA) was reduced in UV of VLBW neonates. Conclusions Lower DHA-PC suggests a lower DHA bioavailability and may contribute to the impaired neurodevelopment. Altered HDL-2, VLDL, and sphingomyelin profile reflect an atherogenic risk and increased metabolic risk at adulthood in infants born prematurely. Impact Lower ApoA-I in HDL2, and increased specific sphingomyelin and phosphatidylcholine containing saturated and monounsaturated fatty acid could explain the accumulation of cholesterol in umbilical vein in VLBW preterm neonates. Decreased phosphatidylcholine containing DHA suggest a reduced DHA availability for brain development in VLBW preterm infants. Characterization of alterations in fetal lipid plasma and lipoprotein profiles may help to explain at least in part the causes of the elevated cardiovascular risk known in people born prematurely and may suggest that a targeted nutritional strategy based on the composition of fatty acids carried by phosphatidylcholine may be promising in infants born very early.

While the effects of growth from birth to expected term on the subsequent development of preterm children has attracted plentiful attention, less is known about the effects of post-term growth. We aimed to delineate distinct patterns of post-term growth and to determine their association with the cognitive development of preterm children. Data from a prospective population-based cohort of 3,850 surviving infants born at less than 35 weeks of gestational age were used. Growth was assessed as the Body Mass Index (BMI) Z-scores at 3, 9, 18, 24, 36, 48, and 60 months. Cognitive development at five years of age was evaluated by the Global School Adaptation score (GSA). Latent class analysis was implemented to identify distinct growth patterns and logistic regressions based on propensity matching were used to evaluate the relationship between identified growth trajectories and cognitive development. Four patterns of post-term growth were identified: a normal group with a Z-score consistently around zero during childhood (n = 2,469; 64%); a group with an early rapid rise in the BMI Z-score, but only up to 2 years of age (n = 195; 5%); a group with a slow yet steady rise in the BMI Z-score during childhood (n = 510; 13%); and a group with a negative Z-score growth until 3 years of age (n = 676; 18%). The group with a slow yet steady rise in the BMI Z-score was significantly associated with low GSA scores. Our findings indicate heterogeneous post-term growth of preterm children, with potential for association with their cognitive development.

Breastfeeding provides the ideal nutrition for infants. Human milk contains a plethora of functional ingredients which foster the development of the immune system. The human milk microbiota predominantly contributes to this protective effect. This is mediated by various mechanisms, such as an antimicrobial effect, pathogen exclusion and barrier integrity, beneficial effects on the gastrointestinal microbiota, vitamin synthesis, immunity enhancement, secreted probiotic factors, and postbiotic mechanisms. Therefore, human milk is a good source for isolating probiotics for infants who cannot be exclusively breastfed. One such probiotic which was isolated from human milk is Limosilactobacillus fermentum CECT5716. In this review, we give an overview of available interventional studies using Limosilactobacillus fermentum CECT5716 and summarise preclinical trials in several animal models of different pathologies, which have given first insights into its mechanisms of action. We present several randomised clinical studies, which have been conducted to investigate the clinical efficacy of the Limosilactobacillus fermentum CECT5716 strain in supporting the host’s health.

Background Effective physician-patient communication is crucial to compassionate healthcare, particularly when conveying life-altering diagnoses such as those associated with congenital heart diseases. Despite its importance, medical practitioners often face challenges in communicating effectively. Because of these gaps, we aim to introduce a simulation-based training protocol to improve pediatric cardiology trainee’s communication skills. This study will be conducted in collaboration with associations supporting caregivers of children with congenital heart disease. It strives to demonstrate how specific training programs can efficiently foster humanistic, patient-centered care in standard medical practice. Methods This multicenter, open-label randomized controlled trial will be conducted in pediatric cardiac units and simulation centers of across 10 universities in France. The study population comprises pediatric cardiologists in training (including pediatric cardiac fellows or specialist assistants). The SIMUL-CHD intervention will consist of simulation-based training with standardized patients, focusing on improving communication skills for pediatric cardiology trainees during diagnostic counselling. Patients and caregivers have been recruited from a National Patient Association named “Petit Cœur de Beurre”. The primary outcome is the quality of physicians’ communication skills. The evaluation committee, which will review video recordings of the sessions, will be blinded to which participants received simulation-based training (group of interest) and which received theory-based training (control group). Secondary outcomes are the effect of SIMUL-CHD on empathy and anxiety levels in young pediatric cardiologists. Baseline scores pre and post-intervention will be compared, and skill improvement resulting from the intervention measured. Discussion Simulation-based training has proven efficacy in teaching technical skills in various scenarios however its application to communication skills in pediatric cardiology remains unexplored. The involvement of experienced parents provides a unique perspective, incorporating their profound understanding of the emotional challenges and specific hurdles faced by families dealing with congenital heart disease. Trial registration This trial is registered with the OSF registry (registered https://osf.io/ed78q ).