Active clinical trials for "Syndrome"

Hypothesis #1: Factor analysis of the revised Sanfilippo Behavior Rating Scale (SBRS) will identify a group of externalizing behaviors and a group of Klüver-Bucy syndrome-like behaviors as two different factors that are at least partially independent. Hypothesis #2a: Children with MPS III will show more hyperlocomotion, fearlessness, asociality and noncompliance than children of similar cognitive ability with MPS I. Hypothesis #2b: These behaviors will become more frequent and/or intensify over time, consistent with the Cleary and Wraith (1993) model. Quantifying them will provide a more empirical framework for staging disease progression. Hypothesis #3: Brain volumetric analysis and diffusion-tensor imaging will reveal abnormalities of frontal and temporal lobe structures that will correlate with externalizing and Klüver-Bucy syndrome-like behaviors, respectively. Hypothesis #4. Loss of cognitive and language function as measures of neurologic decline will directly precede or co-vary with behavioral decline. The primary objective of this study is to identify the behavioral phenotype and its neural basis in MPS III (Sanfilippo syndrome). Is the behavioral phenotype similar to that of Klüver-Bucy syndrome, and is there evidence for amygdala abnormality? The secondary objective of this research study is to develop easily administered, sensitive and specific neurobehavioral and neuroimaging markers to characterize the behavioral phenotype(s) of MPS III; to track their progression; and to delineate their neural substrates. Such markers are critical for identifying the stage of disease for each patient, and to measure treatment outcome. Although we know that severe cognitive decline is one essential characteristic of MPS III, the other highly salient characteristic is a range of abnormal and disruptive behaviors that can include, but go well beyond, childhood noncompliance and oppositionality. These behaviors set Sanfilippo syndrome apart from the other MPS disorders. They cause major disruption in the child's familial, school, and community environments. Delineating these behavioral abnormalities will help in better understanding the neurological disease.

Objective: This study aims elucidate the pathophysiological link between the environment in the colon (mainly the microbiota), the local immune system and activation of the enteric nervous system in patients with post-infectious IBS (PI-IBS) and microscopic colitis (MC) with special emphasis on microbial-mucosa interactions and evaluation of the effect on the immune activation/response as well as how afferent gut-brain signalling leads to abdominal discomfort. Method: The project is based on data from three cohorts of patients, one with PI-IBS and one with MC as well as a gender- and age-matched cohort of healthy individuals. Measurement of perceived sensitivity in the gut will be evaluated by pain-response under mechanical stress using a barostat. The HIT (Human intestinal Tissue)-Chip array will be used to characterize the diversity, stability and functionality of the intestinal microbiota on mucosa level, giving a clue to the interactions with the host and insight to changes leading to the development of the two diseases. Immunohistochemistry and flowcytometry will be used to analyse the location, frequency and phenotype characteristics of lymphoid- and mast cells. Functional analysis of mucosal lymphocytes activated in vitro by products from the intestinal microbiota will be examined by cytokine production using the LuminexTM system. The Ussing chamber technique will allow investigation of the impact of the microbiota and its metabolites on intestinal barrier functions. In this method the sample has access to stressors under standard conditions.

Several lines of evidence indicate that a significant proportion of cardiovascular disease (CVD) events are attributable to the presence of a cluster of metabolic abnormalities and perturbations, defined as the metabolic syndrome. It has been estimated that approximately 25% of the North American adult population is living with the metabolic syndrome. Recent studies show that overaccumulation of atherogenic triglyceride-rich lipoproteins (TRL) seen in insulin-resistant patients is partly due to increased production rate of intestinally derived apolipoproteinB-48-containing lipoproteins. This is of interest because substantial evidence exists indicating that elevated levels of intestinal lipoproteins are associated with increased CVD risk. However, as indicated in the body of this grant proposal, the underlying mechanisms that lead to intestinal overproduction of lipoproteins in insulin-resistant states are poorly understood. The general objective of the proposed research is to investigate the mechanisms by which the metabolic syndrome affects apolipoproteinB-48 secretion in human. The primary hypothesis is that insulin resistance will be associated with higher levels of intestinal lipoproteins because of an increased secretion of these particles.

The purpose of this study is to compare the response to aspirin in the acute phase with the late phase of an acute coronary syndrome.

The software embedded in recent continuous positive pressure devices enables the analysis of the level of leakage that occurs during treatment around the mask or from the mouth. However, there is no clear threshold defining when it would be appropriate to implement means for correcting these leaks. In addition, the calculation methods used to detect/measure leaks and the terminology used vary according to the manufacturers of continuous pressure devices, which also complicates the clinical interpretation of the leakage reports provided by the various devices. In daily practice, when a patient complains of unintentional leakage, technicians adjust the mask, replace the nasal-mask by an oro-nasal mask or install a chinstrap. Nevertheless, those strategies are not always effective in reducing leakage or patient's complaints. A better understanding of the mechanisms beyond leak appearance for a given patient could lead to individualised leak-treatment solutions. Pressure level, mandibular behaviour, respiratory effort, sleep position or sleep stages may contribute to leak emergence. A recent exploratory study has proposed a genuine analysis method of determining factors of unintentional-leaks using polysomnographic recordings in OSA patients treated with automatic Continuous Positive Airway Pressure (auto-CPAP). Based on the same methodology, the NOMICS company (Liège, Belgium) has developed a polygraphic device (Brizzy®) recording unintentional leakage during auto-CPAP treatment and providing an etiological analysis of unintentional-leaks via a software named APIOS. Due to the innovative nature of the analysis, it appears necessary to validate it onto a population of OSA patients treated with auto-CPAP. Also, to our knowledge, there is no descriptive data of the distribution of the determinants of unintentional leaks in such a population. From this perspective, the investigators aim to retrospectively analyse some polygraphic recordings that have been performed with the Brizzy® on OSA patients treated with auto-CPAP. The primary objective of this study is to identify and describe the determinants of unintentional leaks for a population of patients with OSA and treated with an auto-CPAP device

This study is an observatory single center study investigating heart rate variability during on and off states of the spinal cord stimulator, in patients with failed back surgery syndrome.

Obstructive sleep apnea (OSA) is characterized by a repeated partial or complete obstruction of the airway that results in a intermittent hypoxia and sleep disturbance. A complex and reciprocal link between obesity and OSA exists. On the one hand, obesity is one of the main risk factors for OSA. On the other hand, OSA seems to promote obesity. The changes in sleep patterns that characterize OSA alter energy metabolism and promote weight gain. In particular, OSA is associated with metabolic disturbances, decreased physical activity and changes in energy expenditure resulting in weight gain. OSA is characterized by resistance to leptin which reduces the feeling of satiety and an increase in ghrelin levels which increases the feeling of hunger. There may be an increase in food intake, but very few studies have looked at this aspect. Our current knowledge is based on simple patient reports of the amount of food consumed per 24 hours. First-line treatment of OSA is based on continuous positive airway pressure (CPAP) but this approach is not curative and weight loss is encouraged. CAPP could facilitate weight loss by restoring sleep quality. Paradoxically, recent studies show weight gain proportional to the duration of use of CPP. Randomized controlled trials offering apneic patients a return to physical activity and hygiene-dietary rules have shown an improvement in OSA after weight loss. A better understanding of the influence of OSA and its treatment on the energy balance through food preferences and olfacto-gustatory sensoriality is an essential prerequisite for personalized nutritional management. In the face of unexpected weight gain under CPP, this type of intervention would be all the more beneficial as OSA and obesity are public health problems representing two independent risk factors for cardiovascular morbidity and mortality with increasing incidence. We hypothesize that the treatment of OSA with CPAP changes food preferences in favour of fatty and sugary high-calorie foods.

Carpal tunnel syndrome is the most common compression neuropathy. The compression of the median nerve occurs in the carpal tunnel and results in pain and other unpleasant sensations in hand and wrist. In more advanced stage, weakness and wasting of the thenar and other muscles innervated by median nerve occurs, which may lead to permanent impairment of manual performance. The Boston carpal tunnel questionnaire (BCTQ) is one of the most commonly used tools for monitoring of the disease progression and of the effect of therapy. Originally created in English, it has been translated into many languages and subsequently validated. Recently the Polish version has been created and the purpose of this study is to validate it.

Post-thrombotic syndrome (PTS) is a frequent and burdensome complication of deep-vein thrombosis (DVT). In the absence of curative treatment of established PTS, its management is based on the prevention of its occurrence thanks to anticoagulants and compression stockings. So far, predictors of disabling PTS are unknown precluding from optimally selecting patients for invasive (early thrombus removal) or innovative/expensive treatments. In addition, little is known on the incidence of PTS in the very long-term. Objectives: To assess, 12 years after a symptomatic venous thromboembolic (VTE) event, Primary objective: incidence and severity of PTS after a lower limb DVT. Main Secondary objectives: Incidence and severity of PTS according to VTE initial presentation (isolated distal DVT, isolated proximal DVT, PE + DVT). Incidence and risk factors of disabling PTS Methods: Very long-term follow-up (12 years) of patients recruited in the large, multicentre, prospective, observational OPTIMEV study for a suspicion of VTE confirmed or ruled out with objective tests (Clinical Trials NCT00670540). All patients with a DVT, an isolated PE and a random selection of controls (VTE - patients without any history of VTE after the 3 years of follow-up) will first benefit from a phone-PTS assessment. Those patients presenting at least a mild venous insufficiency and a selection of controls will undergo a clinical follow-up visit with clinical and Compleat Ultra Sound (CUS) assessment of PTS/venous insufficiency and an assessment of quality of life. Perspectives: Improving our knowledge of PTS' incidence and predictors and of the impact of usual treatment. Better selecting patients eligible for invasive/innovative/expensive preventative procedures.

Several epidemiologic studies reported that patients with renal insufficiency might have increased cardiovascular disease-related mortality rates after Percutaneous coronary intervention (PCI) . The increased risk in this population may be related to the less use of standard guideline-based treatment and the resulting inability to perform PCI effectively. Recently, with the technology improvement and the progress in clinical trials, Chinese guidelines have made new recommendations about the patients with acute coronary syndrome(ACS) in different states of renal function. However, scant epidemiologic information exists on the prognosis of those patients, especially in Henan. And there is still some uncertainty whether those patients are using the latest guideline recommended treatment. This multicenter, prospective, observational study is aimed to evaluate the long prognosis in patients with acute coronary syndrome complicated with renal insufficiency, and to analysis its related factors that influence the outcomes.