2,249 research outputs found

    QCALT: a tile calorimeter for KLOE-2 upgrade

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    The upgrade of the DAΦ\PhiNE machine layout requires a modification of the size and position of the inner focusing quadrupoles of KLOE-2 thus asking for the realization of two new calorimeters covering the quadrupoles area. To improve the reconstruction of KL→2π0K_L\to 2\pi^0 events with photons hitting the quadrupoles a calorimeter with high efficiency to low energy photons (20-300 MeV), time resolution of less than 1 ns and space resolution of few cm, is needed. To match these requirements, we are designing a tile calorimeter, QCALT, where each single tile is readout by mean of SiPM for a total granularity of 2400 channels. We show first tests of the different calorimeter components

    Expanding the neurological phenotype of ring chromosome 10 syndrome: A case report and review of the literature

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    Ring chromosome 10 [r(10)] syndrome is a rare genetic condition, currently described in the medical literature in a small number of case report studies. Typical clinical features include microcephaly, short stature, facial dysmorphisms, ophthalmologic abnormalities and genitourinary malformations. We report a novel case of r(10) syndrome and review the neurological and neuro-radiological phenotypes of the previously described cases. Our patient, a 3 year old Italian girl, represents the 20th case of r(10) syndrome described to date. Intellectual disability/developmental delay (ID/DD), microcephaly, strabismus, hypotonia, stereotyped/aggressive behaviors and elec-troencephalographic abnormalities were identified in our patient, and in a series of previous cases. A brain MRI disclosed a complex malformation involving both the vermis and cerebellar hemispheres; in the literature, posterior cranial fossa abnormalities were documented by CT scan in another case. Two genes deleted in our case (ZMYND11 in 10p and EBF3 in 10q) are involved in autosomal dominant neurodevelopmental disorders, characterized by different expressions of brain and posterior cranial fossa abnormalities, ID/DD, hypotonia and behavioral problems. Our case expands the neurological and neuroradiological phenotype of r(10) syndrome. Although r(10) syndrome represents an extremely rare condition, with a clinical characterization limited to case reports, the recurrence of specific neurological and neuroradiological features suggests the need for specific genotype-phenotype studies

    Neurological phenotype of mowat-wilson syndrome

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    Mowat-Wilson Syndrome (MWS) (OMIM # 235730) is a rare disorder due to ZEB2 gene defects (heterozygous mutation or deletion). The ZEB2 gene is a widely expressed regulatory gene, extremely important for the proper prenatal development. MWS is characterized by a specific facial gestalt and multiple musculoskeletal, cardiac, gastrointestinal, and urogenital anomalies. The nervous system involvement is extensive and constitutes one of the main features in MWS, heavily affecting prognosis and life quality of affected individuals. This review aims to comprehensively organize and discuss the neurological and neurodevelopmental phenotype of MWS. First, we will describe the role of ZEB2 in the formation and development of the nervous system by reviewing the preclinical studies in this regard. ZEB2 regulates the neural crest cell differentiation and migration, as well as in the modulation of GABAergic transmission. This leads to different degrees of structural and functional impairment that have been explored and deepened by various authors over the years. Subsequently, the different neurological aspects of MWS (head and brain malformations, epilepsy, sleep disorders, and enteric and peripheral nervous system involvement, as well as developmental, cognitive, and behavioral features) will be faced one at a time and extensively examined from both a clinical and etiopathogenetic point of view, linking them to the ZEB2 related pathways

    Post-Traumatic Headache in Children after Minor Head Trauma: Incidence, Phenotypes, and Risk Factors

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    Minor head trauma (MHT) is very frequent in children and post-traumatic headache (PTH) is one of its most common complications; however, its management is still a challenge. We aimed to assess the incidence and clinical characteristics of, and risk factors for, PTH among children referred to our pediatric emergency department (PED) for MHT. A total of 193 patients aged 3–14 years evaluated for MTH were enrolled and followed up for 6 months through phone calls and/or visits. PTH occurred in 25/193 patients (13%). PTH prevalence was significantly higher in school-aged (≥6 years) than in pre-school-aged children (21.6% vs. 4.9%, respectively, p < 0.009). Females were found to be more affected. The median time of onset was 4.6 days after MHT; resolution occurred in a median of 7 weeks. In 83.3% of patients, PTH subsided in <3 months, while in 16.7% it persisted longer. A total of 25% of children exhibited the migraine and 75% the tension-type variant. Our analysis indicates the presence of headache upon arrival in PED, isolated or associated with nausea and dizziness, as a factor predisposing the patient to the development of PTH. Our findings could be useful to identify children at risk for PTH for specific follow-up, family counseling, and treatment

    Energy spectra of quasiperiodic systems via information entropy

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    We study the relationship between the electronic spectrum structure and the configurational order of one-dimensional quasiperiodic systems. We take the Fibonacci case as an specific example, but the ideas outlined here may be useful to accurately describe the energy spectra of general quasiperiodic systems of technological interest. Our main result concerns the {\em minimization} of the information entropy as a characteristic feature associated to quasiperiodic arrangements. This feature is shown to be related to the ability of quasiperiodic systems to encode more information, in the Shannon sense, than periodic ones. In the conclusion we comment on interesting implications of these results on further developments on the issue of quasiperiodic order.Comment: REVTeX 3.0, 8 pages, 3 figures available on request from FD-A ([email protected]), Phys Rev E submitted, MA/UC3M/02/9

    Autism spectrum disorder and anorexia nervosa: an Italian prospective study

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    Background: Potential overlaps exist between psychopathological features of Anorexia Nervosa (AN) and Autism Spectrum Disorder (ASD). The impact of malnutrition on autistic traits in patients with AN should be considered. This study investigates possible associations among the psychopathology of Eating Disorders (EDs), ASD traits and BMI in a group of young patients with AN, using the EDI-3 (Eating Disorder Inventory-3) test and gold-standard measures for ASD. Methods: Prospective study involving 23 inpatients admitted to an Italian Centre for paediatric ED. ASD traits and ED psychopathology were assessed administering the ADOS-2 (Autism Diagnostic Observation Schedule-2), AQ (Autism Quotient) and EDI-3 tests. Both present and past autistic traits were investigated using different versions of AQ. Correlations were adjusted for BMI, Obsessive Compulsive Disorder (OCD) comorbidity and concurrent antipsychotic treatments. Results: An ASD diagnosis was possible in 22% of patients. Significant correlations were documented between ASD traits and ED psychopathology: AQ total-Interpersonal problems (IPC) (p = 0.041); AQ total-Global psychological maladjustment (GMPC) (p = 0.027); AQ social skills-Ineffectiveness (IC) (p = 0.018); AQ social skills-IPC (p = 0.019); AQ social skills-Affective problems (APC) (p = 0.025); AQ social skills-GMPC (p = 0.007); AQ attention switching-IPC (p = 0.020); ADOS-2 imagination-IC (p = 0.035). These correlations were independent of BMI, OCD and antipsychotic treatments. Conclusions: ASD traits presented high prevalence in a group of young inpatients with AN. These traits were significantly correlated to 4 specific EDI-3 subscales and independent of BMI. This is the first study to investigate the relationship between ASD traits as measured with gold-standard measures, EDI-3 scores, and BMI

    Energy and time resolution for a LYSO matrix prototype of the Mu2e experiment

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    We have measured the performances of a LYSO crystal matrix prototype tested with electron and photon beams in the energy range 60−-450 MeV. This study has been carried out to determine the achievable energy and time resolutions for the calorimeter of the Mu2e experiment.Comment: 2 pages, 3 figures, 13th Pisa Meeting on Advanced Detector

    The CDF Calorimetry Upgrade for Run IIb

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    The physics program at the Fermilab Tevatron Collider will continue to explore the high energy frontier of particle physics until the commissioning of the LHC at CERN. The luminosity increase provided by the Main Injector will require upgrades beyond those implemented for the first stage (Run IIa) of the Tevatron's Run II physics program. The upgrade of the CDF calorimetry includes: 1) the replacement of the slow gas detectors on the front face of the Central Calorimeter with a faster scintillator version which has a better segmentation, and 2) the addition of timing information to both the Central and EndPlug Electromagnetic Calorimeters to filter out cosmic ray and beam related backgrounds.Comment: Presented at `Frontier Detectors for Frontier Physics; 9th Pisa Meeting on Advanced Detectors', Biodola, Italy, 25-31 May 2003. 2 page

    Cannabidiol in the acute phase of febrile infection-related epilepsy syndrome (FIRES)

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    Febrile infection-related epilepsy syndrome (FIRES) is a prolonged refractory status epilepticus (SE) that develops among healthy individuals after a febrile infection. FIRES treatment is challenging due to its poor response to antiseizure medications (ASMs) and anesthetic drugs. The use of cannabidiol (CBD) as an adjunctive treatment has been suggested, albeit data about its role in the acute phase is lacking. This report describes the use of purified CBD in the acute phase of two pediatric cases of FIRES and their long-term outcome. Both children were treated with several ASMs, immunomodulators, anesthetics, and nonpharmacological treatment (ketogenic diet). CBD was administered, as an adjunctive treatment, through nasogastric tube about 30 days after onset. SE resolved within 3 days of reaching the target dose and both were seizure-free for 1 year after. Although it is difficult to define the extent to which each previous therapy contributed to recovery, in both cases CBD therapy was a turning point, reinforcing its potential role as add-on treatment in the acute phase of FIRES
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