The influence of the initial state of nutrition on the lifespan of patients with amyotrophic lateral sclerosis (ALS) during home enteral nutrition

Aim: Amyotrophic lateral sclerosis (ALS) is a chronic, neurodegenerative disease, which leads to development of malnutrition. The main purpose of this research was to analyze the impact of malnutrition on the course of the disease and long-term survival. Material and methods: A retrospective analysis has been performed on 48 patients (22 F [45,83%] and 26 M [54,17%], the average age of patients: 66,2 [43-83]) in 2008-2014.The analysis of the initial state of nutrition was measured by body mass index (BMI), nutritional status according to NRS 2002, SGA and concentration of albumin in blood serum. Patients were divided into two groups, depending on the state of nutrition: well-nourished and malnourished. The groups were created separately for each of these, which allowed an additional comparative analysis of techniques used for the assessment of nutritional status. Results: Proper state of nutrition was interrelated with longer survival (SGA: 456 vs. 679 days, NRS: 312 vs. 659 vs. 835 days, BMI: respectively, 411, 541, 631 days, results were statistically significant for NRS and BMI). Concentration of albumin was not a prognostic factor, but longer survival was observed when level of albumin was increased during nutritional therapy. Conclusions: The initial nutrition state and positive response to enteral feeding is associated with better survival among patients with ALS. For this reason, nutritional therapy should be introduced as soon as possible.

A Nine-Year-Old Girl With Left Ventricle Non-Compaction and Skin Lesions (Carvajal Syndrome)

Introduction: Arrhythmogenic right ventricular dysplasia (ARVD), a cardiomyopathy characterized by fibrofatty degeneration of the myocardium with progressive dysfunction, electrical instability, and sudden death, occurs in approximately 1 in 5000 people in the United States. Case Presentation: We present a nine-year-old girl complaining of dyspnea, easy fatigability and skin lesions. She had a history of an occasional epistaxis and weakness since 20 days before her admission, accompanied by the symptoms and signs of common cold, specially cough, during the last two days. Conclusions: This case does confirm that dilated cardiomyopathy’s spectrum is wider than ever known and that like what happened at the congress of Boston in 2006, a more comprehensive approach to its genetic types needs to be done.

Evidence for reductive genome evolution and lateral acquisition of virulence functions in two Corynebacterium pseudotuberculosis strains.

2011

Stairway Uniformity Measurement: What Lateral Location Should Be Measured?

BACKGROUND: This project addressed an unresolved issue involving measurement methods for determining step uniformity. Leading experts on stairway safety agree that lack of step uniformity within a flight contributes to risk of missteps. A relatively new method for precisely measuring step dimensions is the nosing-to-nosing method. An issue in applying the method is lack of agreement on the lateral location to make the measurements. That location depends on where stairway users ascend and descend relative to the width of the steps. A prior investigator examined people descending to determine the lateral distance between the handrail center and the mid-line of the person’s body. He found the median was 44 cm. AIM: The two objectives of the experiment were to: (1) determine if a different set of stairway users will have the same median lateral distance from the handrail as those described in the prior observational study, and (2) determine if the lateral distance of participants is affected by their direction of travel and use of a handrail. METHODS: The investigators established visible distance markers on one stairway in a campus building and videotaped volunteer students ascending and descending the flight of stairs. Each of the 16 participant ascended with and without using the handrail, and each descended with and without using the handrail. Images were printed and analyzed to determine their lateral distance between the inner edge of the handrail and a point midway between the participant’s knees. Results were analyzed statistically to test hypotheses corresponding to the two objectives. RESULTS: The previous study found a median lateral distance of 44 cm when measured from the center of the handrail. Using comparable data, the median found in this study was 25 cm. Results of this study indicated that lateral distance from the handrail is significantly affected by the direction of travel and by use of the handrail. The greatest lateral distance was for ascending with the handrail. The shortest lateral distance was for descending without the handrail. VALUE: Committees develop and periodically revise standards for stairways leading to fire exits and workplace facilities. The practical value of this project is providing empirical evidence that standard developing committees may consider when convening to update their requirements and guidelines on how to measure step uniformity.

Long-term strenuous endurance exercise and the right ventricle: is it a real matter of concern?

Letter to the editor about: La Gerche, A., & Claessen, G. (2015). Is exercise good for the right ventricle? Concepts for health and disease. Canadian Journal of Cardiology, 31(4), 502-508.

Impiego dell’ecografia ad alta risoluzione nella diagnosi di anomalie cerebrali fetali difficili. Focus sul complesso anteriore.

In questo lavoro di tesi vengono prese in esame le principali anomalie cerebrali fetali a carico del complesso anteriore, formato dal cavo del setto pellucido e dai corni frontali dei ventricoli laterali. Si è poi concentrata l’attenzione sull’oloprosencefalia e sull’obliterazione del cavo del setto pellucido, analizzando i casi che sono stati riferiti c/o la U.O. di Ostetricia e Medicina dell’Età Prenatale del Policlinico di S. Orsola – IRCCS. L’oloprosencefalia racchiude in sé uno spettro di anomalie cerebrali caratterizzate da un difetto di formazione della linea mediana con forme variabili di fusione degli emisferi cerebrali. Le forme alobari mostrano una distorsione della anatomia cerebrale, con un singolo ventricolo e sono spesso associate ad anomalie extracerebrali e del cariotipo. Nelle forme semilobari e lobari il setto pellucido è generalmente assente nei piani assiali, con corni frontali fusi ed ipoplasici, ma queste caratteristiche possono essere di difficile interpretazione ad un esame di screening. Le anomalie facciali sono invece più sfuggenti. L’obliterazione del cavo del setto consiste in un suo aspetto ecogeno, normalmente disteso da fluido; è ritenuta una variante della norma, ma queste conclusioni sono basate su casistiche limitate. Anche in questo caso abbiamo riportato l’eventuale presenza di anomalie associate ed abbiamo poi rivalutato questi bambini mediante una visita specialistica presso la U.O. di Neuropsichiatria Infantile. Nella nostra esperienza di 16 casi, la neurosonografia è stata in grado di definire la presenza o meno di anomalie cerebrali associate (1 caso di cisti interemisferiche e corpo calloso disgenetico) al pari della risonanza magnetica. Nei casi apparentemente isolati, in circa il 20% tale reperto è stato transitorio nel corso della gravidanza e non sono state riportate anomalie del cariotipo. Tutte le visite di follow up eseguite nel contesto dello studio (risultati parziali di 7/15 bambini) hanno dimostrato uno sviluppo nella norma per l’età del bambino.

Numerical prediction of the lateral displacement capacity of flexural reinforced concrete shear walls

Previous earthquakes showed that shear wall damage could lead to catastrophic failures of the reinforced concrete building. The lateral load capacity of shear walls needs to be estimated to minimize associated losses during catastrophic events; hence it is necessary to develop and validate reliable and stable numerical methods able to converge to reasonable estimations with minimum computational effort. The beam-column 1-D line element with fiber-type cross-section model is a practical option that yields results in agreement with experimental data. However, shortcomings of using this model to predict the local damage response may come from the fact that the model requires fine calibration of material properties to overcome regularization and size effects. To reduce the mesh-dependency of the numerical model, a regularization method based on the concept of post-yield energy is applied in this work to both the concrete and the steel material constitutive laws to predict the nonlinear cyclic response and failure mechanism of concrete shear walls. Different categories of wall specimens known to produce a different response under in plane cyclic loading for their varied geometric and detailing characteristics are considered in this study, namely: 1) scaled wall specimens designed according to the European seismic design code and 2) unique full-scale wall specimens detailed according to the U.S. design code to develop a ductile behavior under cyclic loading. To test the boundaries of application of the proposed method, two full-scale walls with a mixed shear-flexure response and different values of applied axial load are also considered. The results of this study show that the use of regularized constitutive models considerably enhances the response predictions capabilities of the model with regards to global force-drift response and failure mode. The simulations presented in this thesis demonstrate the proposed model to be a valuable tool for researchers and engineers.

Relationship Between Orofacial Function, Dentofacial Morphology, And Bite Force In Young Subjects.

The aim was to evaluate the relationship between orofacial function, dentofacial morphology, and bite force in young subjects. Three hundred and sixteen subjects were divided according to dentition stage (early, intermediate, and late mixed and permanent dentition). Orofacial function was screened using the Nordic Orofacial Test-Screening (NOT-S). Orthodontic treatment need, bite force, lateral and frontal craniofacial dimensions and presence of sleep bruxism were also assessed. The results were submitted to descriptive statistics, normality and correlation tests, analysis of variance, and multiple linear regression to test the relationship between NOT-S scores and the studied independent variables. The variance of NOT-S scores between groups was not significant. The evaluation of the variables that significantly contributed to NOT-S scores variation showed that age and presence of bruxism related to higher NOT-S total scores, while the increase in overbite measurement and presence of closed lip posture related to lower scores. Bite force did not show a significant relationship with scores of orofacial dysfunction. No significant correlations between craniofacial dimensions and NOT-S scores were observed. Age and sleep bruxism were related to higher NOT-S scores, while the increase in overbite measurement and closed lip posture contributed to lower scores of orofacial dysfunction.

The Inadequacy Of The Y-axis Of Growth (sngn) For The Vertical Pattern Assessment In Patients With Sagittal Discrepancies.

The aim of this cephalometric study was to evaluate the influence of the sagittal skeletal pattern on the 'Y-axis of growth' measurement in patients with different malocclusions. Lateral head films from 59 patients (mean age 16y 7m, ranging from 11 to 25 years) were selected after a subjective analysis of 1630 cases. Sample was grouped as follows: Group 1 - class I facial pattern; group 2 - class II facial pattern; and Group 3 - class III facial pattern. Two angular measurements, SNGoGn and SNGn, were taken in order to determine skeletal vertical facial pattern. A logistic regression with errors distributed according to a binomial distribution was used to test the influence of the sagittal relationship (Class I, II, III facial patterns) on vertical diagnostic measurement congruence (SNGoGn and SNGn). RESULTS show that the probability of congruence between the patterns SNGn and SNGoGn was relatively high (70%) for group 1, but for groups II (46%) and III (37%) this congruence was relatively low. The use of SNGn appears to be inappropriate to determine the vertical facial skeletal pattern of patients, due to Gn point shifting throughout sagittal discrepancies. Clinical Significance: Facial pattern determined by SNGn must be considered carefully, especially when severe sagittal discrepancies are present.

Influence Of Delivery Method On Neuroprotection By Bone Marrow Mononuclear Cell Therapy Following Ventral Root Reimplantation With Fibrin Sealant.

The present work compared the local injection of mononuclear cells to the spinal cord lateral funiculus with the alternative approach of local delivery with fibrin sealant after ventral root avulsion (VRA) and reimplantation. For that, female adult Lewis rats were divided into the following groups: avulsion only, reimplantation with fibrin sealant; root repair with fibrin sealant associated with mononuclear cells; and repair with fibrin sealant and injected mononuclear cells. Cell therapy resulted in greater survival of spinal motoneurons up to four weeks post-surgery, especially when mononuclear cells were added to the fibrin glue. Injection of mononuclear cells to the lateral funiculus yield similar results to the reimplantation alone. Additionally, mononuclear cells added to the fibrin glue increased neurotrophic factor gene transcript levels in the spinal cord ventral horn. Regarding the motor recovery, evaluated by the functional peroneal index, as well as the paw print pressure, cell treated rats performed equally well as compared to reimplanted only animals, and significantly better than the avulsion only subjects. The results herein demonstrate that mononuclear cells therapy is neuroprotective by increasing levels of brain derived neurotrophic factor (BDNF) and glial derived neurotrophic factor (GDNF). Moreover, the use of fibrin sealant mononuclear cells delivery approach gave the best and more long lasting results.

Toll-like Receptor 4 Activation Promotes Cardiac Arrhythmias By Decreasing The Transient Outward Potassium Current (ito) Through An Irf3-dependent And Myd88-independent Pathway.

Cardiac arrhythmias are one of the main causes of death worldwide. Several studies have shown that inflammation plays a key role in different cardiac diseases and Toll-like receptors (TLRs) seem to be involved in cardiac complications. In the present study, we investigated whether the activation of TLR4 induces cardiac electrical remodeling and arrhythmias, and the signaling pathway involved in these effects. Membrane potential was recorded in Wistar rat ventricle. Ca(2+) transients, as well as the L-type Ca(2+) current (ICaL) and the transient outward K(+) current (Ito), were recorded in isolated myocytes after 24 h exposure to the TLR4 agonist, lipopolysaccharide (LPS, 1 μg/ml). TLR4 stimulation in vitro promoted a cardiac electrical remodeling that leads to action potential prolongation associated with arrhythmic events, such as delayed afterdepolarization and triggered activity. After 24 h LPS incubation, Ito amplitude, as well as Kv4.3 and KChIP2 mRNA levels were reduced. The Ito decrease by LPS was prevented by inhibition of interferon regulatory factor 3 (IRF3), but not by inhibition of interleukin-1 receptor-associated kinase 4 (IRAK4) or nuclear factor kappa B (NF-κB). Extrasystolic activity was present in 25% of the cells, but apart from that, Ca(2+) transients and ICaL were not affected by LPS; however, Na(+)/Ca(2+) exchanger (NCX) activity was apparently increased. We conclude that TLR4 activation decreased Ito, which increased AP duration via a MyD88-independent, IRF3-dependent pathway. The longer action potential, associated with enhanced Ca(2+) efflux via NCX, could explain the presence of arrhythmias in the LPS group.

Brij Detergents Reveal New Aspects Of Membrane Microdomain In Erythrocytes.

Membrane microdomains enriched in cholesterol, sphingolipids (rafts), and specific proteins are involved in important physiological functions. However their structure, size and stability are still controversial. Given that detergent-resistant membranes (DRMs) are in the liquid-ordered state and are rich in raft-like components, they might correspond to rafts at least to some extent. Here we monitor the lateral order of biological membranes by characterizing DRMs from erythrocytes obtained with Brij-98, Brij-58, and TX-100 at 4 °C and 37 °C. All DRMs were enriched in cholesterol and contained the raft markers flotillin-2 and stomatin. However, sphingomyelin (SM) was only found to be enriched in TX-100-DRMs - a detergent that preferentially solubilizes the membrane inner leaflet - while Band 3 was present solely in Brij-DRMs. Electron paramagnetic resonance spectra showed that the acyl chain packing of Brij-DRMs was lower than TX-100-DRMs, providing evidence of their diverse lipid composition. Fatty acid analysis revealed that the SM fraction of the DRMs was enriched in lignoceric acid, which should specifically contribute to the resistance of SM to detergents. These results indicate that lipids from the outer leaflet, particularly SM, are essential for the formation of the liquid-ordered phase of DRMs. At last, the differential solubilization process induced by Brij-98 and TX-100 was monitored using giant unilamellar vesicles. This study suggests that Brij and TX-100-DRMs reflect different degrees of lateral order of the membrane microdomains. Additionally, Brij DRMs are composed by both inner and outer leaflet components, making them more physiologically relevant than TX-100-DRMs to the studies of membrane rafts.

Photoelastic And Finite Element Analyses Of Occlusal Loads In Mandibular Body.

This study proposed to evaluate the mandibular biomechanics in the posterior dentition based on experimental and computational analyses. The analyses were performed on a model of human mandible, which was modeled by epoxy resin for photoelastic analysis and by computer-aided design for finite element analysis. To standardize the evaluation, specific areas were determined at the lateral surface of mandibular body. The photoelastic analysis was configured through a vertical load on the first upper molar and fixed support at the ramus of mandible. The same configuration was used in the computer simulation. Force magnitudes of 50, 100, 150, and 200 N were applied to evaluate the bone stress. The stress results presented similar distribution in both analyses, with the more intense stress being at retromolar area and oblique line and alveolar process at molar level. This study presented the similarity of results in the experimental and computational analyses and, thus, showed the high importance of morphology biomechanical characterization at posterior dentition.