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Background The outcome measures (OMs) in clinical trials for Charcot-Marie-Tooth disease (CMT) still represent an issue. A recent study highlighted that three additional clinical OMs, the 10-Meter Walk Test (10MWT), the 9-Hole Peg Test, and foot dorsal flexion dynamometry, further improve discrimination between severely and mildly affected patients. Another study has recently assessed the validity and reliability of the 6-Minute Walk Test (6MWT). Aim The aim of this study was to identify the most useful scales in the clinical evaluation of CMT1A patients. Design Observational study of the baseline data collected in a multicenter, prospective, randomized, single blind, controlled study to evaluate the efficacy and safety of an innovative rehabilitation protocol based on treadmill training, stretching, respiratory, and proprioceptive exercises (TreSPE study) in CMT1A patients. Setting The outpatient service of the four Italian centers involved, which are specialized in hereditary neuropathies. Population Fifty-three subjects with a clinical and genetically confirmed diagnosis of CMT1A. Methods At baseline, in addition to the CMT Neuropathy Score, all subjects underwent walking evaluation (6MWT, 10MWT), balance assessment (Berg Balance Scale [BBS], Short Physical Performance Battery [SPPB]) and a subjective evaluation of quality of life (SF36) and walking ability (Walk12). Results Analyzing the baseline data, as expected, we found a strong correlation between walk and balance evaluation, proving the validity of these tests in investigating the functional impairment of CMT1A subjects. Particularly, we found that subjects with better balance control walk at higher speed and perceive less limitations in their physical activities or motor skills. This can be reconducted to the fact that ankle stability depends upon different factors such as anatomy integrity, muscle strength and proprioception. Conclusions We identify the 6MWT, 10MWT, and SPPB as the most useful scales, in addition to the CMTNS, to evaluate the functional impairment of CMT1A patients who retain their walking capability and we suggest the use of SPPB because of its rapidity to assess balance and gait disorders in clinical settings. Clinical rehabilitation impact In the clinical practice it is important to evaluate patients comprehensively but rapidly. These outcome measures can help us to correctly assess balance and walking ability in CMT1A patients.

The biodeterioration of historic-artistic manufacts is related to several biological systems, including fungi and bacteria, whose metabolic activities and vegetative development have a direct consequence on the conservation of cultural assets. Generally, different chemical compounds are utilized as biocides in order to control biodeteriogens growth, but recently the attention has been focused on potential risks of their use towards human health (operators, visitors) and the environment. In order to develop alternative methods, various natural products have been tested, particularly to control the colonization by fungi and bacteria. In this study, antimicrobial activity of three different plant products, Tea tree essential oil, Calamintha nepeta and Allium sativum L. extracts, has been evaluated against Bacillus subtilis, Micrococcus luteus, Penicillium chrysogenum and Aspergillus spp. (previously isolated from colonized artworks) through three different in vitro antimicrobial assays (micro-dilution in microtiter plates, well plates diffusion and agar disc diffusion method). The bioassays show a different microbial susceptibility to plant extracts, establishing for each bacteria and fungi the Minimum Inhibitory Concentration (MIC) and defining the diameter of the growth inhibition area. This result supports the data reported in literature and shows an important potential suggestion for the possible use in the control of biodeterioration of cultural heritage, safe both for human health and environment.

Atherosclerosis is enhanced in arterial segments exposed to disturbed flow. Perturbed shear stress increases the expression of oxidation-sensitive responsive genes (such as ELK-1 and p-JUN) in the endothelium. Evidence suggests that polyphenolic antioxidants contained in the juice derived from the pomegranate can contribute to the reduction of oxidative stress and atherogenesis. The aim of the present study was to evaluate the effects of intervention with pomegranate juice (PJ) on oxidation-sensitive genes and endothelial NO synthase (eNOS) expression induced by high shear stress in vitro and in vivo. Cultured human coronary artery endothelial cells (EC) exposed to high shear stress in vitro and hypercholesterolemic mice were used in this study. PJ concentrate reduced the activation of redox-sensitive genes (ELK-1 and p-JUN) and increased eNOS expression (which was decreased by perturbed shear stress) in cultured EC and in atherosclerosis-prone areas of hypercholesterolemic mice. Moreover, oral administration of PJ to hypercholesterolemic mice at various stages of disease reduced significantly the progression of atherosclerosis. This experimental study indicates that the proatherogenic effects induced by perturbed shear stress can be reversed by chronic administration of PJ. This approach may have implications for the prevention or treatment of atherosclerosis and its clinical manifestations.

OBJECTIVE: : We evaluated the sensitivity of various rehabilitation and lung function scales to detect differences between people with Charcot-Marie-Tooth (CMT) disease and healthy controls. We also studied whether these measurements are sensitive to disclose changes in patients with CMT disease after rehabilitative treatment. DESIGN: : Eight patients with different types of CMT participated in the study. Data were gathered at baseline; at the end of the treadmill training, stretching, respiratory, and proprioceptive exercise (TreSPE) treatment period; and after a washout period of 6 mos. The following instruments were used for data collection: Medical Research Council scale for lower limb strength; Tinetti Balance Scale; Physical Performance Battery; ankle angle, oxygen consumption, and lung function tests; peak treadmill velocity and slope; time to walk 6 m; and CMT Neuropathy Score. The participants underwent TreSPE treatment twice every week for 8 wks. RESULTS: : All rehabilitative measures were significantly worse in subjects with CMT disease than in healthy controls. Lung function was always normal except for the maximum expiratory pressure and maximum inspiratory pressure. No dropouts or worsening in any of the different outcome measures were observed after TreSPE. The ankle angle and the time to walk 6 m were the only measures that significantly improved after treatment. CONCLUSIONS: : The rehabilitative outcome measures used in this protocol are useful in detecting clinical impairment in people with CMT disease. Lung function tests were confirmed to be minimally abnormal in patients with CMT disease. The TreSPE treatment, besides being safe and well tolerated, induced some training effects in CMT neuropathy, in the absence of lung function amelioration and effort tolerance. Follow-up showed that CMT patients should be treated at least twice every year because a regression of all outcome measures to the baseline state was found after a 6-mo washout period.

It is widely accepted that nonspecific tissue reactivity is a distinct pathophysiological hallmark of allergic diseases, influenced by genetic and environmental factors different from those involved in causing sensitization and allergen response of target organs. This consensus document aims at reviewing procedures currently used for nonspecific provocation of the bronchi, nose and eye and for measuring their responsiveness to nonspecific stimuli.

Children's fear of pain in illness has been greatly underestimated. In cancer disease, pain characterizes most of the diagnostic and long-term therapeutic procedures. Children's psycho-emotional processing during illness has been recently recognized as characterized by loss of control, anger, and fear, to which pain adds fear of death jeopardizing the sense of survival. Illness and pain provoke a traumatic condition affecting psycho-emotional maturation. The aim of the pharmacologic and psychologic support is to help children better cope with pain and prevent the healed child having traumatic effects in his/her future personality structure and behavior. To assure the best pain control, support has to be offered right from the first intrusive procedure in order to avoid anticipatory anxiety. Respiration, relaxation, visualization, desensibilization through the "switch technique" and the "magic glove", distraction and involvement, muscular relaxation all have the common aim of focusing the child's mind and attention away from body perception of pain connected to the procedure. Different medical methods are utilized depending on the child's age and the level of consciousness required for the procedure. Local anaesthesia and conscious sedation are used. Children are particularly sensitive in this critical condition and need as serene and comfortable an environment as possible. Relief of pain and suffering in cancer treatments that cause further discomfort to the patient is a fundamental ethics in the treatment of clinical neoplasias.