Development and Preliminary Validation of an Electromyography-Scoring Protocol for the Assessment and Grading of Muscle Involvement in Patients With Juvenile Idiopathic Inflammatory Myopathies.

Abstract Introduction We performed a pilot study in order to investigate the feasibility of an electromyography (EMG)-scoring protocol for the assessment of disease activity in juvenile idiopathic inflammatory myopathies (JIIM). Methods Children with JIIM followed up in a tertiary-level care center underwent standardized clinical, laboratory, and EMG assessment. An EMG-scoring protocol was devised by a consensus panel including a pediatric neurophysiologist and two pediatric rheumatologists, based on a combined score obtained as the sum of (1) the presence of denervation signs (fibrillation potentials) and (2) motor unit remodeling (mixed pattern of short- and long-duration motor unit action potentials). The EMG-scoring protocol was then validated following the Outcome Measures in Rheumatoid Arthritis Clinical Trials filter for outcome measures in rheumatology and the consensus-based standards for the selection of health measurement instruments methodology. Results Thirteen children (77% females) were included in the study, with a median age of 10 years (interquartile range: 7-17 years) and median disease duration of 11.8 months (interquartile range: 2.1-44.5). A total of 39 EMG examinations were evaluated. A strong positive association between a standardized tool for muscle strength assessment and the combined score was observed. No significant associations were found with both creatine kinase and erythrocyte sedimentation rate levels. Discussion Our EMG-scoring protocol is the first standardized and reproducible tool for the neurophysiologic evaluation and grading of muscle involvement in patients with JIIM and could provide relevant additional information in the assessment and follow-up of these rare conditions

Evaluación del comportamiento agronómico de distintos ecotipos de orégano en 25 de Mayo, La Pampa

Oregano is cultivated in semiarid regions with a negative water balance, therefore is necessary to apply irrigation according water needs. The area of 25 de mayo, La Pampa, which is fed by the Río Colorado, has an irrigation system that makes it suitable for this kind of farming. The goal of this paper was characterize productivity of four oregano ecotypes (Criollo, Mendocino, Cordobés and Compacto) in the irrigated area of 25 de Mayo. Dry matterin flowers and leaves of each ecotype was determined. The results showed that there were no significant differences in yield (p=0.05). However, it should be highlighted that important differences could be observed in the field where, Criollo and Cordobés ecotypes had highest productivity. The yield obtained was highest than in traditional areas, such as Mendoza and Córdoba, which makes the region under study a promising option for oregano production. DOI: http://dx.doi.org/10.19137/semiarida.2016(01).35-42El cultivo de orégano se desarrolla en regiones de clima semiárido en donde existe un balance hídrico negativo, siendo necesario contar con un sistema de irrigación que permita cubrir las necesidades de agua. La zona de 25 de Mayo, La Pampa, alimentada por el Río Colorado, posee un sistema de riego que la convierte en un área con alto potencial para dicha producción. El objetivo de este trabajo fue caracterizar el comportamiento productivo de cuatro ecotipos de orégano (Criollo, Mendocino, Cordobés y Compacto) en la zona bajo riego de 25 de Mayo. Para ello, se determinó el porcentaje de materia seca de flor y hoja, peso fresco y seco de parte aérea de las muestras de cada ecotipo. Los resultados mostraron que no existen diferencias significativas en relación al rendimiento (p=0.05). Sin embargo, productivamente se observan diferencias de importancia, siendo los ecotipos Criollo y Cordobés los de mejor comportamiento. Los rendimientos obtenidos fueron mayores al promedio de las regiones productoras por excelencia, tales como Mendoza y Córdoba, por lo que el área en estudio se presenta como una opción prometedora para la producción de orégano. DOI: http://dx.doi.org/10.19137/semiarida.2016(01).35-4

Aberrant Crosstalk between Insulin Signaling and mTOR in Young Down Syndrome Individuals Revealed by Neuronal-Derived Extracellular Vesicles

INTRODUCTION: Intellectual disability, accelerated aging, and early-onset Alzheimer-like neurodegeneration are key brain pathological features of Down syndrome (DS). Although growing research aims at the identification of molecular pathways underlying the aging trajectory of DS population, data on infants and adolescents with DS are missing. METHODS: Neuronal-derived extracellular vesicles (nEVs) were isolated form healthy donors (HDs, n = 17) and DS children (n = 18) from 2 to 17 years of age and nEV content was interrogated for markers of insulin/mTOR pathways. RESULTS: nEVs isolated from DS children were characterized by a significant increase in pIRS1Ser636, a marker of insulin resistance, and the hyperactivation of the Akt/mTOR/p70S6K axis downstream from IRS1, likely driven by the higher inhibition of Phosphatase and tensin homolog (PTEN). High levels of pGSK3βSer9 were also found. CONCLUSIONS: The alteration of the insulin-signaling/mTOR pathways represents an early event in DS brain and likely contributes to the cerebral dysfunction and intellectual disability observed in this unique population

Diagnosis, treatment and prevention of pediatric obesity: consensus position statement of the Italian Society for Pediatric Endocrinology and Diabetology and the Italian Society of Pediatrics

The Italian Consensus Position Statement on Diagnosis, Treatment and Prevention of Obesity in Children and Adolescents integrates and updates the previous guidelines to deliver an evidence based approach to the disease. The following areas were reviewed: (1) obesity definition and causes of secondary obesity; (2) physical and psychosocial comorbidities; (3) treatment and care settings; (4) prevention.The main novelties deriving from the Italian experience lie in the definition, screening of the cardiometabolic and hepatic risk factors and the endorsement of a staged approach to treatment. The evidence based efficacy of behavioral intervention versus pharmacological or surgical treatments is reported. Lastly, the prevention by promoting healthful diet, physical activity, sleep pattern, and environment is strongly recommended since the intrauterine phase

Epstein-Barr Virus and immune status imprint the immunogenomics of non-Hodgkin lymphomas occurring in immune-suppressed environments

Non-Hodgkin lymphomas (NHL) commonly occur in immune-deficient (ID) patients, both HIV-infected and transplanted, and are often EBV-driven with cerebral localization, raising the question of tumor immunogenicity, a critical issue for treatment responses. We investigated the immunogenomics of 68 lymphoproliferative disorders from 51 ID (34 posttransplant, 17 HIV+) and 17 immunocompetent patients. Overall, 72% were Large B Cells Lymphoma (LBCL) and 25% were primary central-nervous-system lymphoma (PCNSL) while 40% were EBV-positive. Tumor whole-exome and RNA sequencing, along with a bioinformatics pipeline allowed analysis of tumor mutational burden (TMB), tumor landscape and microenvironment (TME) and prediction of tumor neoepitopes. Both TMB (2.2 vs 3.4/Mb, p=0.001) and neoepitopes numbers (40 vs 200, p=0.00019) were lower in EBVpositive than in EBV-negative NHL, regardless of the immune status. In contrast both EBV and the immune status influenced the tumor mutational profile, with HNRNPF and STAT3 mutations exclusively observed in EBV-positive and ID NHL, respectively. Peripheral blood T-cell responses against tumor neoepitopes were detected in all EBV-negative cases but in only half EBV-positive ones, including responses against IgH-derived MHC-class-II restricted neoepitopes. The TME analysis showed higher CD8 T cell infiltrates in EBVpositive vs EBV-negative NHL, together with a more tolerogenic profile composed of Tregs, type-M2 macrophages and an increased expression of negative immune-regulators. Our results highlight that the immunogenomics of NHL in patients with immunodeficiency primarily relies on the tumor EBV status, while T cell recognition of tumor- and IgH-specific neoepitopes is conserved in EBV-negative patients, offering potential opportunities for future T cell-based immune therapies

Innovating Strategies and Tailored Approaches in Neuro-Oncology

Diffuse gliomas, the most frequent and aggressive primary central nervous system neoplasms, currently lack effective curative treatments, particularly for cases lacking the favorable prognostic marker IDH mutation. Nonetheless, advances in molecular biology allowed to identify several druggable alterations in a subset of IDH wild-type gliomas, such as NTRK and FGFR-TACC fusions, and BRAF hotspot mutations. Multi-tyrosine kinase inhibitors, such as regorafenib, also showed efficacy in the setting of recurrent glioblastoma. IDH inhibitors are currently in the advanced phase of clinical evaluation for patients with IDH-mutant gliomas. Several immunotherapeutic approaches, such as tumor vaccines or checkpoint inhibitors, failed to improve patients’ outcomes. Even so, they may be still beneficial in a subset of them. New methods, such as using pulsed ultrasound to disrupt the blood–brain barrier, gene therapy, and oncolytic virotherapy, are well tolerated and may be included in the therapeutic armamentarium soon

Current therapeutic approaches to diffuse grade II and III gliomas

The 2016 WHO classification of Tumors of the Central Nervous System brought major conceptual and practical changes in the classification of diffuse gliomas, by combining molecular features and histology into ‘integrated’ diagnoses. In diffuse gliomas, molecular profiling has thus become essential for nosological purposes, as well as to plan adequate treatment strategies and identify patients susceptible of target therapy. WHO grade II (low grade) and grade III (anaplastic) diffuse gliomas form a heterogeneous group of neoplasms, also known as ‘lower-grade gliomas’, characterized by a wide range of malignant potential. Molecular profile accounts for this biological diversity, and provides an accurate prognostic stratification of tumors in this group. Treatment strategies in lower-grade gliomas are ultimately based on molecular profile and WHO grade, as well as on patient characteristics such as age and Karnofsky performance status. The purpose of this review is to summarize recent advances in the classification of grade II and III gliomas, synthesize current treatment schemes according to molecular profile and describe ongoing research and future perspectives for the use of target therapies

Induced-field enhancement of band-structure effects in photoelectron spectra from Al surfaces by ultrashort laser pulses

The electron emission produced by the grazing incidence of ultrashort laser pulses on two faces of aluminum, Al(100) and Al(111), is investigated using the band-structure-based-Volkov (BSB-V) approximation. The present version of the BSB-V approach includes not only a realistic description of the surface interaction, accounting for band-structure effects, but also effects due to the induced potential that originates from the collective response of valence electrons to the external electromagnetic field. For both crystallographic orientations we found that the induced potential contributes to the emergence of band-structure signatures in the near-threshold region of photoelectron spectra. This result opens a window to scrutinize band-structure effects in metal surfaces via ultrashort-laser interactions.Fil: Rios Rubiano, Carlos Alberto. Comisión Nacional de Energí­a Atómica. Gerencia del Area Investigación y Aplicaciones No Nucleares. Gerencia de Física (Centro Atómico Balseiro). División Colisiones Atómicas; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Patagonia Norte; ArgentinaFil: Della Picca, Renata. Comisión Nacional de Energí­a Atómica. Gerencia del Area Investigación y Aplicaciones No Nucleares. Gerencia de Física (Centro Atómico Balseiro). División Colisiones Atómicas; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Patagonia Norte; ArgentinaFil: Mitnik, Dario Marcelo. Consejo Nacional de Investigaciónes Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Astronomía y Física del Espacio. - Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Astronomía y Física del Espacio; ArgentinaFil: Silkin, Slava M.. Donostia International Physics Center; EspañaFil: Gravielle, Maria Silvia. Consejo Nacional de Investigaciónes Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Astronomía y Física del Espacio. - Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Astronomía y Física del Espacio; Argentin

In situ conservation of the wild relative of the common bean (Phaseolus vulgaris var. aborigineus) at the south of its neotropical distribution: environmental characterization of a population in central Argentina

The common bean (Phaseolus vulgaris L.) is a food of worldwide vital importance. Like any crop, the conservation of its wild relatives is essential to maintain the genetic variability needed for plant breeding. In situ conservation of such population has the additional benefit of generating new variations through the evolution of its natural environment. In 2004, wild populations of P. vulgaris var. aborigineus were found in the province of Córdoba (Argentina), being the southernmost specimen of its current distribution. In order to study the state and characterize the environment of such populations, vegetation censuses were conducted along a stretch of the Tanti stream-arroyo Tanti-(Punilla, Córdoba). Results showed that the wild bean has an intermediate to high constancy throughout the watercourse and its presence decreases upstream and away from the stream. Most cover took place in plots with scarce slope, high incidence of light and intense magnitude of floods. Vegetation develops in an environment surrounded by forests, meadows and intermediate physiognomy. Besides adventitious species, the studied area still has native species of high conservation value. Based on the analysis of this work, and considering the biogeographical significance of this population of wild bean, we conclude that this stretch of the studied stream is a priority area for the in situ conservation of P. vulgaris var. aborigineus.Fil: Sirolli, Horacio. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Departamento de Ecología, Genética y Evolución; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Drewes, Susana Ines. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Departamento de Biodiversidad y Biología Experimental; ArgentinaFil: Picca, Pablo Ignacio. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Departamento de Biodiversidad y Biología Experimental; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Kalesnik, Fabio Alberto. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Departamento de Ecología, Genética y Evolución; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentin