Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies

Background. Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. Methods. The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. Results. Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). Conclusions. Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies represent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity and stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent

Risk of Guillain-Barré syndrome after 2010–2011 influenza vaccination

Influenza vaccination has been implicated in Guillain Barré Syndrome (GBS) although the evidence for this link is controversial. A case–control study was conducted between October 2010 and May 2011 in seven Italian Regions to explore the relation between influenza vaccination and GBS. The study included 176 GBS incident cases aged ≥18 years from 86 neurological centers. Controls were selected among patients admitted for acute conditions to the Emergency Department of the same hospital as cases. Each control was matched to a case by sex, age, Region and admission date. Two different analyses were conducted: a matched case–control analysis and a self-controlled case series analysis (SCCS). Case–control analysis included 140 cases matched to 308 controls. The adjusted matched odds ratio (OR) for GBS occurrence within 6 weeks after influenza vaccination was 3.8 (95 % CI: 1.3, 10.5). A much stronger association with gastrointestinal infections (OR = 23.8; 95 % CI 7.3, 77.6) and influenza-like illness or upper respiratory tract infections (OR = 11.5; 95 % CI 5.6, 23.5) was highlighted. The SCCS analysis included all 176 GBS cases. Influenza vaccination was associated with GBS, with a relative risk of 2.1 (95 % CI 1.1, 3.9). According to these results the attributable risk in adults ranges from two to five GBS cases per 1,000,000 vaccinations

Conceptual design of the enhanced coolant purification systems for the European HCLL and HCPB test blanket modules

The Coolant Purification Systems (CPSs) is one of the most relevant ancillary systems of European Helium Cooled Lead Lithium (HCLL) and Helium Cooled Pebble Bed (HCPB) Test Blanket Modules (TBMs) which are currently in the preliminary design phase in view of their installation and operation in ITER. The CPS implements mainly two functions: the extraction and concentration of the tritium permeated from the TBM modules into the primary cooling circuit and the chemistry control of helium primary coolant. During the HCLL and HCPB-TBSs (Test Blanket Systems) Conceptual Design Review (CDR) in 2015 it was recognized the need of reducing the tritium permeation into the Port Cell #16 of ITER. To achieve this and, then, to lower the tritium partial pressure in the Helium Cooling Systems in normal operation, the helium flow-rate treated by each CPS has been increased of almost one order of magnitude. In 2017, to satisfy the CDR outcomes and the new design requirements requested by Fusion for Energy (F4E, the European Domestic Agency for ITER), ENEA performed a preliminary design of the “enhanced” CPSs. This paper presents the current design of the “enhanced” CPSs, focusing on design requirements, assumptions, selection of technologies and preliminary components sizing

Analytical Model of Thermo-electrical Behaviour in Superconducting Resistive Core Cables

High field superconducting Nb$_{3}$Sn accelerators magnets above 14 T, for future High Energy Physics applications, call for improvements in the design of the protection system against resistive transitions. The longitudinal quench propagation velocity (vq) is one of the parameters defining the requirements of the protection. Up to now vq has been always considered as a physical parameter defined by the operating conditions (the bath temperature, cooling conditions, the magnetic field and the over all current density) and the type of superconductor and stabilizer used. It is possible to enhance the quench propagation velocity by segregating a percent of the stabilizer into the core, although keeping the total amount constant and tuning the contact resistance between the superconducting strands and the core. Analytical model and computer simulations are presented to explain the phenomenon. The consequences with respect to minimum quench energy are evidenced and the strategy to optimize the cable designed is discussed

Investigation of the prevalence of antibodies against neurotropic polyomaviruses BK, JC and SV40 in sera from patients affected by multiple sclerosis.

Viral agents seem to be linked to multiple sclerosis (MS). This association is based on evidence of (1) early exposure to viruses and MS onset; (2) increased prevalence of MS disease in specific geographic regions; (3) likelihood of developing MS being more prevalent in high-risk areas; (4) altered immune responses to different viruses. In this study, sera from patients affected by MS and controls, represented by sera from patients with other neurologic diseases, both inflammatory and non-inflammatory, and from healthy donors, were investigated for the presence of antibodies against neurotropic polyomaviruses BKV, JCV and SV40 in their sera. Our study has indicated that the prevalence of BKV antibodies in sera from MS patients is higher than that detected in normal individuals, while levels of antibodies against BKV and JCV are lower in MS patients compared to those of normal subjects

REsiDENT 1 (Re-assessment of Appendicitis Evaluation during laparoscopic appendectomy: Do we End a Non-standardized Treatment approach and habit?): Peritoneal irrigation during laparoscopic appendectomy - Does the grade of contamination matter? A prospective multicenter resident-based evaluation of a new classification system

Background: Laparoscopic appendectomy has progressively gained acceptance as the standard of care for acute appendicitis. Focusing on the incidence of postoperative intra-abdominal abscess after a laparoscopic appendectomy, discordant data have been reported ranging from 1.5 to 20%. Besides, evidence advocating advantages from peritoneal irrigation over suction only are lacking. Most studies are burdened by a high level of heterogeneity regarding the severity of the appendicitis and modalities of peritoneal irrigation. One of the main drawbacks is the lack of an accepted classification for different degrees of appendicitis and peritoneal contamination. The aim of the study is to introduce a classification to clarify the relationship between grade of appendicitis, contamination, and postoperative incidence of IAA considering the surgeon's attitude toward irrigation or suction alone. Preoperative, intra-operative, and postoperative predictive factors for infectious complication will also be assessed. This study is meant to be the first Italian multicenter resident-based observational study. Methods: Patients suffering from acute appendicitis will be enrolled during a 1-year period, according to inclusion and exclusion criteria. Participants will fill an online form reporting all clinical and intra-operative data of each patient undergoing a laparoscopic appendectomy. General surgery residents will be responsible for data collection. Our proposal of classification is based on the histological grade of appendicitis and intra-operative degree of peritoneal contamination. For each grade, a progressively increasing score is assigned. Discussion: The observational nature of this study is mandatory to examine surgeons' attitude toward peritoneal contamination during laparoscopic appendectomy for appendicitis. Identification of different severity grades of acute appendicitis and their relationship with the development of postoperative abscesses is necessary. The resulting classification and score, even considering peritoneal lavage or suction alone, will define risk classes of peri-appendiceal contamination each one related to a specific incidence rate of postoperative IAA. Nowadays, maximum effort should be made to reach the best procedural standardization and surgical decision-making should be supported by solid evidence, especially in an emergency surgery setting