Recurrent Pericarditis in Children and Adolescents

Recurrent pericarditis (RP) is a clinical syndrome characterized by recurrent attacks of acute pericardial inflammation. Prognosis quoad vitam is good, although morbidity might be significant, especially in children and adolescents. Multiple potential etiologies result in RP, in the vast majority of cases through autoimmune or autoinflammatory mechanisms. Idiopathic RP is one of the most frequent diagnoses, that requires the exclusion of all known etiologies. Therapeutic advances in the last decade have been significant with the recognition of the effectiveness of anti IL1 therapy, but a correct diagnostic and therapeutic algorithm is of key importance. Unfortunately, most of evidence comes from studies in adult patients. Here we review the etiopathogenesis, diagnosis and management of RP in pediatric patients

Effects of Enriched Environment (EE) on Depressive-Like Behavior and Hippocampal Structure in Rat Model of Chronic Stress

Chronic stress is associated with the development of depression. It can trigger structural and neurobehavioral changes in the brain and has been shown to induce depressivelike behavior in animals. An enriched environment can modulate the structure and function of the brain by altering the expression of various genes and proteins as well as affecting neurotransmitters’ activity. The hippocampus plays an important role incontrolling the networks for mood regulation and has been implicated in the course of depression. This study aimed to investigate the effect of an enriched environment on the depressive-like behavior and hippocampal structure in rats after unpredictable chronic mild stress (UCMS) exposure. Male Wistar rats (Rattus novergicus) were divided into three groups, each consisting of 6 rats including the control, UCMS and UCMS+EEgroup. Unpredictable chronic mild stress and EE were given for 21 days. Body weight gain, depressive-like behavior, and hippocampal structure were evaluated at the end of the experiment. Depressive-like behavior was assessed with Forced Swim Test (FST) and Sucrose Preference Test (SPT). Thickness of the pyramidal layer of CA1 and CA3 area were measured with histologic examination to see changes in the hippocampalstructure. Data were analyzed using One-Way ANOVA or Kruskal-Wallis followed by multiple comparison post hoc test. The enriched environment could significantly maintain body weight gain (p = 0.036) and rat’s preference to sucrose solution (p =0.001) in a stressful condition. Enriched environment reduced immobility time in FST but it was not statistically significant (p = 0.177). There was a significant difference in the thickness of CA1 and CA3 pyramidal layer of the hippocampus among groups (p=0.015 and p=0.019 respectively). Stress markedly decreased the thickness of CA1 and CA3 pyramidal layer (p=0.014 and 0.011 respectively). The enriched environment can ameliorate stress-induced depressive-like behavior and alteration in hippocampalstructure in rats. Keywords: Environmental enrichment, depression, stress, hippocampu

Endothelial Hyper-Permeability Induced by T1D Sera Can be Reversed by iNOS Inactivation

Type 1 Diabetes Mellitus (T1D) is associated with accelerated atherosclerosis that is responsible for high morbidity and mortality. Endothelial hyperpermeability, a feature of endothelial dysfunction, is an early step of atherogenesis since it favours intimal lipid uptake. Therefore, we tested endothelial leakage by loading the sera from T1D patients onto cultured human endothelial cells and found it increased by hyperglycaemic sera. These results were phenocopied in endothelial cells cultured in a medium containing high concentrations of glucose, which activates inducible nitric oxide synthase with a consequent increase of nitric oxide. Inhibition of the enzyme prevented high glucose-induced hyperpermeability, thus pointing to nitric oxide as the mediator involved in altering the endothelial barrier function. Since nitric oxide is much higher in sera from hyperglycaemic than normoglycaemic T1D patients, and the inhibition of inducible nitric oxide synthase prevents sera-dependent increased endothelial permeability, this enzyme might represent a promising biochemical marker to be monitored in T1D patients to predict alterations of the vascular wall, eventually promoting intimal lipid accumulation

POS1315 USEFULNESS OF SYNOVIAL BIOPSY IN THE DIFFERENTIAL DIAGNOSIS AND AS POSSIBLE PREDICTOR OF RESPONSE TO TREATMENT IN JUVENILE IDIOPATHIC ARTHRITIS

Background:While synovial biopsy is an invasive procedure and is not required for the diagnosis of juvenile idiopathic arthritis (JIA), it may be useful in doubtful cases.Objectives:Aims of the study were i.to verify the role of synovial biopsy in the differential diagnosis of JIA and ii. to review the pathology slides in order to evaluate possible associations of a histologic pattern with response to treatment.Methods:We reviewed data from medical records of patients under the age of 18 years who underwent a synovial biopsy requested by our Pediatric Rheumatology Unit over the last 10 years. We collected information on demographic, clinical, laboratory, radiological, histopathological characteristics, as well as treatment response (in particular, remission at the last visit and number of examination, number of biologic drugs used). Among variables in the histologic score, number of layers in the synovial lining and inflammatory infiltrate (0-5) were compared to clinical status at last visit. Potential differences in variables between responders and non responders were assessed by unpaired t-test or non-parametric Mann-Whitney test, as appropriate.Results:We identified 64 patients (40F, 24M) with a median age at disease onset of 9 years (range 1-15) and a median follow-up time of 161 months (range 8-1160). We recognized two groups of interest: patients with a known JIA diagnosis (28/64) and patients with unknown diagnosis (36/64) at the moment of synovial biopsy. In the group with known JIA, most underwent the procedure during orthopedic surgery, and in all cases the histology was consistent with JIA. Among the unknown diagnosis group, in 19 cases results were consistent with a chronic synovitis, while among the other 17 histology could lead to a diagnosis of other conditions in 6 cases (foreign body and villonodular synovitis n=2 each, sarcoidosis and osteochondromatosis n=1 each). In the remaining 11 the final diagnoses were varied (mostly genetic forms eg skeletal dysplasia, CACP, Thiemann disease).Between the two groups we identified 46 patients with a definite JIA diagnosis. At the last follow-up visit 29 of them were in clinical remission, albeit on medication. The remaining 17 had a severe course of disease, with persistent activity and use of at least two biologic drugs. In 26 cases we could evaluate the correlation between status at last visit and number of layers/inflammatory infiltrate, but no statistical significant correlation was found.Conclusion:Despite its limited use nowadays, synovial biopsy may still be a useful tool in patients whose diagnosis is unclear. In our study, while it confirmed the suspicion in most cases, in other instances it allowed the diagnosis of rare conditions that would have been otherwise missed. No association between disease course and histological features in a small JIA cohort was found. We are currently expanding the study with a larger series.Disclosure of Interests:None declare

The minimal N=4 no-scale model from generalized dimensional reduction

We consider the generalized dimensional reduction of pure ungauged N=4, D=5 supergravity, where supersymmetry is spontaneously broken to N=2 or N=0 with identically vanishing scalar potential. We explicitly construct the resulting gauged D=4 theory coupled to a single vector multiplet, which provides the minimal N=4 realization of a no-scale model. We discuss its relation with the standard classification of N=4 gaugings, extensions to non-compact twists and to higher dimensions, the N=2 theories obtained via consistent Z_2 orbifold projections and prospects for further generalizations.Comment: 1+28 pages, no figures, JHEP3 LaTeX, published versio

Role and Efficacy of Intraoperative Evaluation of Resection Adequacy in Conservative Breast Surgery

In the present study we considered the histology of 51 patients who have undergone breast conservative surgery and the related 54 re-excisions that were performed in the same surgical procedure or in delayed procedures, in order to evaluate the role of intraoperative re-excisions in completing tumor removal. In 13% of the cases the re excision obtained the resection of the target lesion. In this study, the occurrence of residual neoplastic lesions in intraoperative re-excisions (24%) is lower than in delayed re-excisions (62%; P = .03). The residual lesions that we could find with definitive histology of re excision specimens are related with lesions with ill defined profile. In 77% of the cases of re excision with tumoral residual the lesion was close to the new resection margin, thus the re-excisions couldn't achieve an adequate ablation of the neoplasm. Invasive or preinvasive nature of the main lesion resected for each case and the approach to the evaluation of the first resection specimen adequacy (surgical or radiological) don't affect the rate of tumoral residual in intraoperative re-excisions. In conclusion, our data are consistent with a low efficacy of intraoperative re excision in obtaining a complete removal of the tumor; intraoperative radiologic evaluation of the first resection specimen is however imperative in defining the effective removal of the target lesion