Enzyme replacement therapy in adult‐onset glycogenosis II: Is quantitative muscle MRI helpful?

Although it has been shown that muscle magnetic resonance imaging (MRI) improves the phenotypic characterization of patients with neuromuscular disorders and allows accurate quantification of muscle and adipose tissue distribution, to date quantitative MRI has not been used to assess the therapeutic response in clinical trials of neuromuscular diseases. We discuss quantitative MRI findings after a 6‐month course of enzyme replacement therapy administered to nine patients with adult‐onset glycogenosis II. Muscle Nerve 40: 122–125, 2009     

Tissue factor in human coronary atherosclerotic plaques

The rupture or fissuring of a coronary atherosclerotic plaque and subsequent thrombosis is considered the key event in the pathogenesis of unstable angina and myocardial infarction. Although plaque disruption frequently occurs during the evolution of atherosclerosis, only a minority of ruptured plaques develop thrombosis. The content and procoagulant activity of tissue factor in human coronary atherosclerotic plaques varies widely, and different studies confirm that it is higher in the plaques extracted from patients with unstable angina, myocardial infarction or histologic/angiographic evidence of coronary thrombosis than in those taken from patients with stable angina or uncomplicated coronary lesions. Variations in tissue factor content and activity may be responsible for the different thrombotic responses to human coronary atherosclerotic plaque rupture.     

Interleukin 6 causes growth impairment in transgenic mice through a decrease in insulin-like growth factor-I. A model for stunted growth in children with chronic inflammation.

Stunted growth is a major complication of chronic inflammation and recurrent infections in children. Systemic juvenile rheumatoid arthritis is a chronic inflammatory disorder characterized by markedly elevated circulating levels of IL-6 and stunted growth. In this study we found that NSE/hIL-6 transgenic mouse lines expressing high levels of circulating IL-6 since early after birth presented a reduced growth rate that led to mice 50-70% the size of nontransgenic littermates. Administration of a monoclonal antibody to the murine IL-6 receptor partially reverted the growth defect. In NSE/hIL-6 transgenic mice, circulating IGF-I levels were significantly lower than those of nontransgenic littermates; on the contrary, the distribution of growth hormone pituitary cells, as well as circulating growth hormone levels, were normal. Treatment of nontransgenic mice of the same strain with IL-6 resulted in a significant decrease in IGF-I levels. Moreover, in patients with systemic juvenile rheumatoid arthritis, circulating IL-6 levels were negatively correlated with IGF-I levels. Our findings suggest that IL-6-mediated decrease in IGF-I production represents a major mechanism by which chronic inflammation affects growth.     

AIDS and alternative treatments. A nursing point of view

The new therapeutics protocols used to cure for AIDS are efficient and important for the therapy but it is essential for the patient to know therapeutic modalities and use. These factors are important for the patient's compliance. But besides the official therapy, we can assist to the use of a diverse alternative therapies associated to the conventional. Sometime the alternative therapy helps the official medicine although, too often they are considered old lifestyles survival. The present work want to indicate by a survey, how positive and ill patients face their health reality, how they alleviate their problems and the nursing role. WHO has recommanded to nurses to pay attention to the therapeutic choices of the patients.     

Modulation of motor cortex neuronal networks by rTMS: comparison of local and remote effects of six different protocols of stimulation

Repetitive transcranial magnetic stimulation (rTMS) of human motor cortex can produce long-lasting changes in the excitability of excitatory and inhibitory neuronal networks. The effects of rTMS depend critically on stimulus frequency. The aim of our present study was to compare the effects of different rTMS protocols. We compared the aftereffects of 6 different rTMS protocols [paired associative stimulation at interstimulus intervals of 25 (PAS(25)) and 10 ms (PAS(10)); theta burst stimulation delivered as continuous (cTBS) or intermittent delivery pattern (iTBS); 1- and 5-Hz rTMS] on the excitability of stimulated and contralateral motor cortex in 10 healthy subjects. A pronounced increase of cortical excitability, evaluated by measuring the amplitude of motor evoked potentials (MEPs), was produced by iTBS (+56%) and PAS(25) (+45%). Five-hertz rTMS did not produce a significant increase of MEPs. A pronounced decrease of cortical excitability was produced by PAS(10) (-31%), cTBS (-29%), and 1-Hz rTMS (-20%). Short-interval intracortical inhibition was suppressed by PAS(10). Cortical silent period duration was increased by 1-Hz stimulation. No significant effect was observed in the contralateral hemisphere. Head-to-head comparison of the different protocols enabled us to identify the most effective paradigms for modulating the excitatory and inhibitory circuits activated by TMS.     

Intragastric Balloon Followed by Biliopancreatic Diversion in a Liver Transplant Recipient: A Case Report

Liver transplantation is a life-saving procedure for end-stage liver disease. In liver transplant recipients, morbid obesity influences post-operative survival and graft function. In 1996, our patient underwent a successful liver transplantation because of a HCV-related liver failure (body mass index (BMI) 31). Follow-up showed a functional graft and the development of severe obesity up to a BMI of 61 in January 2006. In January 2007, he was submitted to intragastric balloon therapy for 6 months, reaching a BMI of 54. In September 2007, he underwent a biliopancreatic diversion. During follow-up to March 2008, he reached a BMI of 42 with ameliorations of comorbidities. In May 2008, during a hospital admission, he suddenly died of a heart attack. Post mortem study revealed a myocardial infarction. This is the first world case report for this approach. According to our opinion, patient’s death was not related to bariatric surgery.