Dilemas éticos en niños con enfermedades neuromusculares y dependencias tecnológicas: Ethical dilemmas in children with neuromuscular diseases and technological dependencies

En los últimos años, los cuidados respiratorios especializados, y en particular los cuidados respiratorios no invasivos y otros avances tecnológicos han contribuido a una mejor calidad de vida y sobrevida de los pacientes con enfermedades neuromusculares. La naturaleza de las opciones terapéuticas (como ser la ventilación invasiva versus ventilación no invasiva, las cargas psicológicas, sociales y financieras) tienen ramificaciones éticas. Por lo tanto resulta esencial que los médicos comprendan todas las opciones terapéuticas y los factores psicosociales al informar a sus pacientes, en lo que tiene que ver con sus ventajas y desventajas y sus costos asociados, de modo que los padres puedan tomar decisiones informadas. Los nuevos desarrollos incluyen exámenes prenatales y neonatales más precisos, nuevas terapias genéticas y soporte respiratorio no invasivo para evitar episodios de fallas respiratorias y vías aéreas invasivas. Al empoderar a los pacientes y sus familias para educar y capacitar servicios personales de cuidados para que no deban depender de las instituciones y servicios de enfermería constante, y los recientes avances tecnológicos facilitan su permanencia en el hogar y dejan de estar atados a una vida dependiendo de la institución y los servicios de enfermería. Estos últimos infantilizan a los pacientes en lugar de promover su autonomía. Las consideraciones financieras, las terapias genéticas, el screening prenatal y las distintas opciones terapéuticas que promueven la autonomía, todos tienen implicancias éticas. Algunos de los avances discutidos en este artículo requieren un cambio de paradigma en la forma en que los médicos ven y tratan a estos individuos.

Efectividad de la aplicación de la técnica de facilitación neuromuscular propioceptiva sub-acuática combinada con fisioterapia en pacientes con debilidad muscular en miembros superiores e inferiores en edades de 30 a 70 años de ambos sexos, atendidos en el área de terapia física del Hospital Militar regional de la ciudad de San Miguel; en el periodo comprendido de julio a septiembre de 2009.

La terapia subacuática se considera una alternativa innovadora para los pacientes con algún déficit en la realización de los movimientos que se ven afectados por algún padecimiento, razón por la cual es necesario de alguna manera aumentar su fuerza que es en si la causa de la debilidad muscular. Es por ello que los ejercicios dentro del agua proporcionan un mayor rendimiento debido a las propiedades físicas de ella, ya que se logra realizar los movimientos con un grado mínimo de dificultad. Es por esta razón que se considera importante la investigación mediante el cual se beneficiaron; los pacientes ya que tuvieron la oportunidad de lograr una máxima funcionabilidad con el aumento de la fuerza muscular y mejorar su calidad de vida. Así como la institución ya que estará aportando una nueva forma de tratamiento. A los profesionales en Fisioterapia y Terapia Ocupacional el estudio permitirá conocer y aplicar una nueva modalidad de tratamiento.

Studies of the gastro-oesophageal junction in normal and overweight healthy volunteers

Introduction: Oesophageal adenocarcinoma has increased dramatically in incidence over the past three decades with a particularly high burden of disease at the gastro-oesophageal junction. Many cases occur in individuals without known gastro-oesophageal reflux disease and in the absence of Barrett’s oesophagus suggesting that mechanisms other than traditional reflux may be important. Distal squamous mucosa may be prone to acid damage even in the absence of traditional reflux by the mechanism of distal opening of the lower oesophageal sphincter. This is splaying of the distal segment of lower oesophageal sphincter allowing acid ingress without traditional reflux. It has been suggested that the cardiac mucosa at the gastro-oesophageal junction, separating oesophageal squamous mucosa and acid secreting columnar mucosa of the stomach may be an abnormal mucosa arising as a consequence of acid damage. By this theory the cardiac mucosa is metaplastic and akin to ultra-short Barrett’s oesophagus. Obesity is a known risk factor for adenocarcinoma at the gastro-oesophageal junction and its rise has paralleled that of oesophageal cancer. Some of this excess risk undoubtedly operates through stress on the gastro-oesophageal junction and a predisposition to reflux. However we sought to explore the impact of obesity on the gastro-oesophageal junction in healthy volunteers without reflux and in particular to determine the characteristics of the cardiac mucosa and mechanisms of reflux in this group. Methods: 61 healthy volunteers with normal and increased waist circumference were recruited. 15 were found to have a hiatus hernia during the study protocol and were analysed separately. Volunteers had comprehensive pathological, physiological and anatomical assessments of the gastro-oesophageal junction including endoscopy with biopsies, MRI scanning before and after a standardised meal, prolonged recording of pH and manometry before and after a meal and screening by fluoroscopy to identify the squamo-columnar junction. In the course of the early manometric assessments a potential error associated with the manometry system recordings was identified. We therefore also sought to document and address this on the benchtop and in vivo. Key Findings: 1. In documenting the behaviour of the manoscan we described an immediate effect of temperature change on the pressure recorded by the sensors; ‘thermal effect’ and an ongoing drift of the recorded pressure with time; ‘baseline drift’. Thermal effect was well compensated within the standard operation of the system but baseline drift not addressed. Applying a linear correction to recorded data substantially reduced the error associated with baseline drift. 2. In asymptomatic healthy volunteers there was lengthening of the cardiac mucosa in association with central obesity and age. Furthermore, the cardiac mucosa in healthy volunteers demonstrated an almost identical immunophenotype to non-IM Barrett’s mucosa, which is considered to arise by metaplasia of oesophageal squamous mucosa. These findings support the hypothesis that the cardia is metaplastic in origin. 3. We have demonstrated a plausible mechanism of damage to distal squamous mucosa in association with obesity. In those with a large waist circumference we observed increased ingress of acid within but not across the lower oesophageal sphincter; ‘intrasphincteric reflux’ 4. The 15 healthy volunteers with a hiatus hernia were compared to 15 controls matched for age, gender and waist circumference. Those with a hiatus hernia had a longer cardiac mucosa and although they did not have excess traditional reflux they had excess distal acid exposure by short segment acid reflux and intrasphincteric acid reflux. Conclusions: These findings are likely to be relevant to adenocarcinoma of the gastro-oesophageal junction

Aplicación del Vendaje Neuromuscular en Logopedia

Este método de vendaje consiste en la colocación de un esparadrapo o cinta elástica adhesiva en la zona sobre la que se quiere actuar, favoreciendo así la función muscular y circulatoria, tanto sanguínea, como linfática ofreciendo una estimulación propioceptiva y actuando como analgésico además de actuar sobre la fascia externa con las consecuencia s beneficiosas que esto conlleva . Existen distintas técnicas de aplicación dependiendo de la zona y los efectos que queramos conseguir. Los beneficios que nos aporta el vendaje neuromuscular son muy amplios y pueden ser explicados desde la neurofisiología, la neuromecánica y la fisiología muscular: Efecto circulatorio : gracias a la elasticidad del vendaje y a la forma en que se aplica , con la zona a tratar en posición de estiramiento (por regla general ) pero sin estirar el vendaje. Debido a estas dos circunstancias, cuando la estructura a tratar vuelve a su posición inicial, la elasticidad del vendaje hace que se produzca una elevación de la piel formando pliegues cutáneos superficiales llamados circunvoluciones , que aumentan el espacio celular subcutáneo donde se encuentran capilares sanguíneos y perilinfáticos; de esta forma se consigue un aumento de la circulación de la zona en la que se aplica el kinesiotape Efecto analgésico : el aumento del espacio celular subcutáneo que provoca el vendaje , consigue que disminuya la presión de los mecanorreceptores ubicados en este espacio, y de esta forma se reducen las aferencias nociceptivas. Este aumento del espacio celular subcutáneo también mejora la circulación local, favoreciendo el drenaje de los detritos tisulares y de los mediadores inflamatorios acumulados en la zona lesionada Efecto neuromecánico: la elasticidad del vendaje hace que éste se retraiga hacia el primer punto al que se adhiere a la piel (llamado base del vendaje). Esta tracción sobre la piel y sobre la fascia superficial tensa las fibras de colágeno ubicadas perpendicular y diagonalmente entre esta última y la fascia profunda, desencadenando un reflejo protector para evitar el sobreestiramiento de estos tejidos ubicados en el tejido celular subcutáneo; dicho reflejo consiste en que la fascia profunda se desliza en el mismo sentido que la superficial haciendo que los tejidos comprendidos entre ambas vuelvan a la posición de reposo o silencio neurológico. Acompañando a la fascia profunda, por compartir inervación, irá también el músculo. Por tanto, en las aplicaciones musculares, en función del sentido en el que apliquemos el vendaje neuromuscular (de origen a inserción o de inserción a origen), el músculo tenderá hacia el acortamiento o hacia la elongación , es decir, se tonificará o se relajará Neurofisiológicamente, el kinesiotape aporta información exteroceptiva que es recogida por los mecano receptores ubicados en la piel y las fascias, y es transmitida en sentido aferente hacia el sistema nervioso central influyendo en la regulación del movimiento normal (fuerza, dirección, amplitud, coordinación, etc) . A esto hay que añadirle el soporte externo que supone para la articulación, favoreciendo la biomecánica articular y el funcionamiento muscular En el campo de la Logopedia su uso actualmente, no está muy extendido y sus beneficios no son demasiado conocidos. Sin embargo, usado como complemento, como ayuda en los tratamientos logopédicos, queda cada vez más demostrado En logopedia, son muchas las patologías que pueden beneficiarse de la utilización del vendaje neuromuscular: disfonía, disfagia, deglución atípica, hipotonía o hipertonía muscular, parálisis facial, reeducación respiratoria... También se puede ampliar a tratamientos en aquellos casos en los que es necesario actuar sobre determinados músculos para conseguir una función concreta: Para tonificar la musculatura facial en casos de debilidad muscular. Para conseguir un óptimo cierre labial Para reducir la sialorrea (babeo) Para la relajación de los músculos laríngeos y/ o faciales en casos de hipertonía o exceso de tono Para estabilizar y controlar la mandíbula Para aumentar la capacidad inspiratoria y el diámetro torácico Para conseguir un adecuado posicionamiento del cuello.

Neural Modulation of Leg Stiffness in Response to Neuromuscular Fatigue

The spring-mass model is able to accurately represent hopping spring-like behavior (leg and joint stiffness), and leg and joint stiffness changes can reveal overall motor control responses to neural and muscular contributors of neuromuscular fatigue. By understanding leg stiffness modulation, we can determine which variables the nervous system targets to maintain motor performance and stability. The purpose of this study was to determine how neuromuscular fatigue affects hopping behavior by examining leg and joint stiffness before and after a single-leg calf raise fatiguing protocol. Post-fatigue, leg stiffness decreased for the exercised leg, but not for the non-exercised leg. Ankle and knee joint stiffness did not significantly change for either leg. This indicates that leg stiffness decreases primarily from muscular fatigue, but was not explained by ankle and knee joint stiffness. The decrease in leg stiffness may be an attempt to soften landing impact, while at the same time maintaining performance.

Utilização do botox para a correção neuromuscular do sorriso gengival

Dissertação para obtenção do grau de Mestre no Instituto Superior de Ciências da Saúde Egas Moniz

Noncompaction in Healthy Subjects, Dilated and Hypertrophic Cardiomyopathy, and Neuromuscular Disorders Is the Same Entity

With interest we read the article by Khosroshahi et al. about a novel method for quantification of left ventricular hypertrabeculation/noncompaction (LVHT) using two-dimensional echocardiography in children (1). We appreciate their efforts to contribute to an improvement and unification of echocardiographic diagnostic criteria for LVHT, which is urgently needed. Concerning their proposed method, we have the following questions and concerns:

Noncompaction in Healthy Subjects, Dilated and Hypertrophic Cardiomyopathy, and Neuromuscular Disorders Is the Same Entity

With interest we read the article by Khosroshahi et al. about a novel method for quantification of left ventricular hypertrabeculation/noncompaction (LVHT) using two-dimensional echocardiography in children (1). We appreciate their efforts to contribute to an improvement and unification of echocardiographic diagnostic criteria for LVHT, which is urgently needed. Concerning their proposed method, we have the following questions and concerns:

Comparison Between Diuretic Urography (IVP) and Diuretic Renography for Diagnosis of Ureteropelvic Junction Obstruction in Children

Background: Ureteropelvic junction obstruction (UPJO) is one of the most common causes of urinary tract obstruction in children. Several methods are used to diagnose upper urinary tract obstruction including renal ultrasonography (US), intravenous pyelogram (IVP), diuretic renography (DR), magnetic resonance urography (MRU) and antegrade or retrograde pyelography. Nowadays it is suggested to use diuretic renography as the best method for diagnosing of UPJO. There is no comparative study between IVP and DR scan for diagnosis of UPJO in children. Objectives: The aim of the present study was to compare IVP with furosemide injection and diuretic renography in diagnosis of clinically significant UPJO. Patients and Methods: This was a cross sectional study performed in 153 UPJO suspected children (121 boys, 32 girls) based on US findings in cases presented with urinary tract infection (UTI), prenatal hydronephrosis, abdominal/flank pain, abdominal mass and hematuria. Renal ultrasound was used as an initial screening tool for detection of urinary tract abnormality. Vesicoureteral reflux (VUR) was ruled out by voiding cystourethrography (VCUG). Serum creatinin, blood urea nitrogen, urinalysis and urine culture was screened in all cases. IVP with furosemide and DR were performed as soon as possible after the mentioned workup. Results: During a five year period, 46 out of 153 patients were diagnosed as UPJO based on diuretic renography: the age ranged from 4 months to 13 years (mean: 3.1 ± 0.78 years). There was a significant higher (76%) proportion of UPJO in the boys and in the left side (78%). The sensitivity of IVP with furosemide injection in diagnosis of UPJO was 91.3% whereas DR was accepted as standard for diagnostic procedure in diagnosis of UPJO. Conclusions: Although DR is accepted as the best method for diagnosis of UPJO, we found a small sensitivity difference between IVP and DR in kidneys with normal or near normal function. In many settings such as small cities lacking facilities for advanced isotope imaging technology, use of IVP with diuretic maybe an acceptable procedure for diagnosis of UPJO.

Isolating the photovoltaic junction: atomic layer deposited TiO2-RuO2 alloy Schottky contacts for silicon photoanodes

We synthesized nanoscale TiO2-RuO2 alloys by atomic layer deposition (ALD) that possess a high work function and are highly conductive. As such, they function as good Schottky contacts to extract photogenerated holes from n-type silicon while simultaneously interfacing with water oxidation catalysts. The ratio of TiO2 to RuO2 can be precisely controlled by the number of ALD cycles for each precursor. Increasing the composition above 16% Ru sets the electronic conductivity and the metal work function. No significant Ohmic loss for hole transport is measured as film thickness increases from 3 to 45 nm for alloy compositions >= 16% Ru. Silicon photoanodes with a 2 nm SiO2 layer that are coated by these alloy Schottky contacts having compositions in the range of 13-46% Ru exhibit average photovoltages of 525 mV, with a maximum photovoltage of 570 mV achieved. Depositing TiO2-RuO2 alloys on nSi sets a high effective work function for the Schottky junction with the semiconductor substrate, thus generating a large photovoltage that is isolated from the properties of an overlying oxygen evolution catalyst or protection layer.

Synonymous co-variation across the E1/E2 gene junction of hepatitis C virus defines virion fitness

Hepatitis C virus is a positive-sense single-stranded RNA virus. The gene junction partitioning the viral glycoproteins E1 and E2 displays concurrent sequence evolution with the 3′-end of E1 highly conserved and the 5′-end of E2 highly heterogeneous. This gene junction is also believed to contain structured RNA elements, with a growing body of evidence suggesting that such structures can act as an additional level of viral replication and transcriptional control. We have previously used ultradeep pyrosequencing to analyze an amplicon library spanning the E1/E2 gene junction from a treatment naïve patient where samples were collected over 10 years of chronic HCV infection. During this timeframe maintenance of an in-frame insertion, recombination and humoral immune targeting of discrete virus sub-populations was reported. In the current study, we present evidence of epistatic evolution across the E1/E2 gene junction and observe the development of co-varying networks of codons set against a background of a complex virome with periodic shifts in population dominance. Overtime, the number of codons actively mutating decreases for all virus groupings. We identify strong synonymous co-variation between codon sites in a group of sequences harbouring a 3 bp in-frame insertion and propose that synonymous mutation acts to stabilize the RNA structural backbone.

Efeitos do suster-relaxar na cinemática tridimensional, função neuromuscular e sintomatologia álgica de um atleta de crossfit com dor lombar

Projeto de Graduação apresentado à Universidade Fernando Pessoa como parte dos requisitos para obtenção do grau de Licenciado em Fisioterapia