Active lifestyles moderate clinical outcomes in autosomal dominant frontotemporal degeneration.

INTRODUCTION: Leisure activities impact brain aging and may be prevention targets. We characterized how physical and cognitive activities relate to brain health for the first time in autosomal dominant frontotemporal lobar degeneration (FTLD). METHODS: A total of 105 mutation carriers (C9orf72/MAPT/GRN) and 69 non-carriers reported current physical and cognitive activities at baseline, and completed longitudinal neurobehavioral assessments and brain magnetic resonance imaging (MRI) scans. RESULTS: Greater physical and cognitive activities were each associated with an estimated >55% slower clinical decline per year among dominant gene carriers. There was also an interaction between leisure activities and frontotemporal atrophy on cognition in mutation carriers. High-activity carriers with frontotemporal atrophy (-1 standard deviation/year) demonstrated >two-fold better cognitive performances per year compared to their less active peers with comparable atrophy rates. DISCUSSION: Active lifestyles were associated with less functional decline and moderated brain-to-behavior relationships longitudinally. More active carriers "outperformed" brain volume, commensurate with a cognitive reserve hypothesis. Lifestyle may confer clinical resilience, even in autosomal dominant FTLD.

Synthesis and characterization of zinc and calcium nanoferrites.

In this work, Zn(1-x)CaxFe(2)O(4) nanoparticles (x = 0, 0.5 and 1) have been synthesized by sol-gel method followed by heat treatment at a temperature within the range of 300-700 °C. The samples with appropriate saturation magnetization (Ms), low coercivity and remanence were Zn(0)Ca(1)Fe(2)O(4) treated at 300 °C (Ms ~ 25 emu/g), Zn(0)Ca(1)Fe(2)O(4) treated at 400 °C (Ms ~ 40 emu/g) and Zn(0.50)Ca(0.50)Fe(2)O(4) treated at 400 °C (Ms ~ 31 emu/g). These samples were analyzed by scanning electron microscopy, energy dispersive spectroscopy, X-ray diffraction and transmission electron microscopy. The heating ability of selected nanoparticles was evaluated under a magnetic field using a solid state induction heating equipment. The obtained nanoferrites showed a particle size within the range of 13-14 nm. The Zn(0)Ca(1)Fe(2)O(4) treated at 400 °C was able to heat the nanoferrite particles/water suspension (10 mg/2 ml) at a temperature of 44 °C under the selected magnetic field (10.2 kA/m and frequency 362 kHz). Additionally, in vitro bioactivity assessment was performed by immersing samples in a simulated body fluid for different periods of time at physiological conditions of pH and temperature. The samples showed an appropriate bioactivity. These nanoferrites are highly potential materials for hyperthermia treatment.

Study of the utilization of light egg-type males: a proposal for the sustainability of the egg industry.

This study evaluated the performance and viability of light egg-type males, usually euthanized at the hatcheries, from White and Brown Leghorn laying hen lines. One-day-old male chicks, half from each hen line, were raised in floor pens until they were 42 d of age. The birds were distributed into 48 floor pens, furnished with tube feeders and nipple drinkers, and submitted to 24 h of continuous light, 3 feeding phases (1-7, 8-21, and 22-42 d) and diets composed of corn and soybean meal as the main ingredients. A completely randomized design was used in a 4 × 2 factorial arrangement (phase levels of ME dietary treatments × strain) of 6 replicates of 40 birds each. The variables evaluated were bird performance, carcass quality, and yield at 42 d of age. Mortality and cannibalism were not observed during the entire experimental period, although the birds' beaks were not trimmed. The brown males line showed higher feed consumption and BW gain and better feed conversion compared with the white male line (P< 0.05). Metabolizable energy (kcal/kg) levels of 3,200 (1 to 7 d), 3,050 (8 to 21 d), and 3,200 (22 to 42 d) provided better performance (P< 0.05) in both lines. The carcass yields were similar (P> 0.05) between the 2 lines; males from the white line showed higher breast yield, and the brown line males showed higher yield of thighs and drumstick (P< 0.05). The treatments had no effect on meat quality (P > 0.05). Overall, the results suggest that there is great viability for the use of male chicks from laying hens of both leghorn lines as a high quality protein source for human consumption.

Large normal and reduced penetrance alleles in Huntington disease: instability in families and frequency at the laboratory, at the clinic and in the population.

Large normal ('intermediate') alleles may produce de novo expansions in Huntington disease; nevertheless, there is very little evidence about their population prevalence and impact in daily practice, and there are conflicting reports about the extent of their instability. We estimated the frequency of large normal alleles (27-35 CAGs) and of reduced penetrance alleles (36-39 CAGs), as well as the frequency of genotypes carrying them, in (i) a diagnostic laboratory, (ii) a genetic counselling clinic and (iii) the general population. Large normal alleles were present in 6% of a large control sample, 7% of consultands who took pre-symptomatic testing and 7% of samples in the laboratory. Reduced penetrance alleles were found in 1 of 1772 control chromosomes (0.1% of individuals), 5% of 146 pre-symptomatic testees and over 2% of 1214 diagnostic samples (350 families). All 16 alleles sized 27-32 CAGs seemed to be transmitted stably; alleles ≥ 36 repeats were unstable in five families. Seven small full penetrance alleles contracted into the reduced penetrance range, but none into the large normal range. Evidence showed that large normal alleles are relatively frequent and that those with reduced penetrance are not a rare event, either at the laboratory or the clinic. This reinforces the need to understand the genomic context of repeat instability in each family and population.

Resistance exercise training improves mucociliary clearance in subjects with COPD: A randomized clinical trial.

INTRODUCTION: Elastic tubing was recently investigated as an alternative to the conventional resistance training (RT) in chronic obstructive pulmonary disease (COPD). The effects of RT on the mucociliary system have not yet been reported in the literature. OBJECTIVE: The aim of this study was to evaluate the effects of two RT programs on mucociliary clearance in subjects with COPD. METHODS: Twentyeight subjects with COPD were randomly allocated by strata, according to individual strength of lower limbs, to defined groups: conventional resistance training (GCT) or resistance training using elastic tubing (GET). Nineteen subjects (GET: n=9; GCT: n=10) completed the study and were included in the analysis. The measurement of vital signs (blood pressure, heart rate and respiratory rate), lung function (spirometry) and the primary outcome mucociliary clearance analysis (saccharin transit time test (STT)) were performed before and after the 12 weeks of RT. RESULTS: In relation to the mucociliary transportability analysis, no differences were observed between the baseline evaluations of the training groups (p=0.05). There was a significant reduction in the STT values in both training groups, GET (10.64±5.06 to 6.01±4.91) and GCT (12.07±5.10 to 7.36±2.54) with p=0.03. However, no differences between groups were observed on the magnitude of SST changes after interventions (GET: -43.51%; GCT: -38.94%; p=0.97). CONCLUSION: The present study demonstrated that both RT with elastic tubing and conventional training with weights promoted similar gains in the mucociliary transportability of subjects with COPD.

Effects of exposure to biomass burning on pulmonary inflammatory markers and pulmonary function in individuals with COPD.

INTRODUCTION: Pulmonary rehabilitation (PR) has many benefits for individuals with COPD. However, it is not clear whether PR could prevent the hazards of air pollution exposure. This study aimed to analyze the effects of biomass burning exposure on pulmonary inflammatory markers and pulmonary function in individuals with COPD, participants and non-participants of PR. METHODS: 35 subjects were divided into three groups: individuals with COPD who received PR (G1, n=15), those who did not (G2, n=10), and a control group composed of healthy individuals without COPD (CG, n=10). Measurements of lung function and concentrations of IL-6, IL-10, and TNF-α in exhaled breath condensate samples were collected. The assessment and concentrations of particulate matter (PM10), nitrogen dioxide (NO2), ozone (O3), temperature (T), and relative air humidity (RAH) were recorded in biomass burning and non-burning periods. RESULTS: There was a significant increase in the concentrations of air pollutants in the biomass burning period. In this period, an increase in IL-6 (G1p=0.041, G2 p=.012), and a reduction in the FEV1/FVC ratio (G1p=0.021, G2 p=.007) were observed in individuals with COPD. In G1, the increase in IL-6 concentrations correlated positively with O3 (r=0.693; p=.006), and negatively with RAH (r=-0.773; p=.003) in the burning period. CONCLUSIONS: Individuals with COPD exposed to biomass burning demonstrated increased pulmonary inflammation and a reduction in the FEV1/FVC ratio, regardless of their engagement in PR.

Providing Access to Genomic Variant Knowledge in a Healthcare Setting: A Vision for the ClinGen Electronic Health Records Workgroup.

The Clinical Genome Resource (ClinGen) is a National Institutes of Health (NIH)-funded collaborative program that brings together a variety of projects designed to provide high-quality, curated information on clinically relevant genes and variants. ClinGen's EHR (Electronic Health Record) Workgroup aims to ensure that ClinGen is accessible to providers and patients through EHR and related systems. This article describes the current scope of these efforts and progress to date. The ClinGen public portal can be accessed at www.clinicalgenome.org.

Fatty acid profile and meat quality of young bulls fed ground soybean or ground cottonseed and vitamin E.

The objective of this study was to evaluate the fatty acid profile and qualitative characteristics of meat from feedlot young bulls fed ground soybean or ground cottonseed, with or without supplementation of vitamin E. A total of 40 Red Norte young bulls, with an initial average age of 20 months, and an initial average BW of 339±15 kg, were allotted in a completely randomized design using a 2×2 factorial arrangement, with two oilseeds, and daily supplementation or not of 2500 IU of vitamin E. The experimental period was for 84 days, which was preceded by an adaptation period of 28 days. The treatments were ground soybean (SB), ground soybean plus vitamin E (SBE), ground cottonseed (CS) and ground cottonseed plus vitamin E (CSE). The percentage of cottonseed and soybean in the diets (dry matter basis) was 24% and 20%, respectively. Diets were isonitrogenous (13% CP) and presented similar amount of ether extract (6.5%). The animals were slaughtered at average live weight of 464±15 kg, and samples were taken from the longissimus dorsi muscle for the measurement of fatty acid concentration and the evaluation of lipid oxidation and color of the beef. Before fatty acid extraction, muscle tissue and subcutaneous fat of the longissimus dorsi were separated to analyze fatty acid profile in both tissues. Supplementation of vitamin E did not affect fatty acid concentration, lipid oxidation and color (P>0.05). Subcutaneous fat from animals fed CS diet had greater C12:0, C16:0 and C18:0 contents (P<0.03). In addition, CS diets reduced the C18:1 and C18:2 cis-9, trans-11 contents in subcutaneous fat (P<0.05). The muscle from animals fed CS tended to higher C16:0 and C18:0 contents (P<0.11), and decreased C18:1, C18:2 cis-9, trans-11 and C18:3 contents (P<0.05) compared with SB. The Δ9-desaturase index was greater in muscle from animals fed SB (P<0.01). At 42 days of age, meat from cattle fed SB had a greater lipid oxidation rate (P<0.05). Meat from animals fed SB diets had less lightness and redness indices than meat from animals fed CS diets after 14 days of age. In conclusion, the addition of ground cottonseed in the finishing diets did increase the saturated fatty acid content of the longissimus dorsi. However, animals fed cottonseed exhibited greater lightness and redness of beef. In this study, the addition of vitamin E did not affect qualitative characteristics of meat.

Dual function of the hemagglutinin H5 fused to chicken CD154 in a potential strategy of DIVA against avian influenza disease: preliminary study.

In this study we demonstrated that the vaccine candidate against avian influenza virus H5N1 based on the hemagglutinin H5 (HA) fused to the chicken CD154 (HACD) can also be used for differentiating infected from vaccinated animals (DIVA). As the strategy of DIVA requires at least two proteins, we obtained a variant of the nucleoprotein (NP49-375) in E. coli. After its purification by IMAC, the competence of the proteins NP49-375 and HACD as coating antigens in indirect ELISA assays were tested by using the sera of chickens immunized with the proteins HA and HACD and the reference sera from several avian influenza subtypes. Together with these sera, the sera from different species of birds and the sera of chickens infected with other avian viral diseases were analyzed by competition ELISA assays coated with the proteins NP49-375 and HACD. The results showed that the segment CD154 in the chimeric protein HACD did not interfere with the recognition of the molecule HA by its specific antibodies. Also, we observed variable detection levels when the reference sera were analyzed in the ELISA plates coated with the protein NP49-375. Moreover, only the antibodies of the reference serum subtype H5 were detected in the ELISA plates coated with the protein HACD. The competition ELISA assays showed percentages of inhibition of 88-91% for the positives sera and less than 20% for the negative sera. We fixed the cut-off value of these assays at 25%. No antibody detection was observed in the sera from different species of birds or the sera of chickens infected with other avian viral diseases. This study supported the fact that the ELISA assays using the proteins NP49-375 and HACD could be valuable tools for avian influenza surveillance and as a strategy of DIVA for counteracting the highly pathogenic avian influenza virus H5N1 outbreaks.

Color evaluation of carbon monoxide treated porcine blood.

The stability of liquid porcine blood, treated with carbon monoxide (CO) at different pH values (7.40, 6.70, and 6.00) up to its complete saturation, was studied. Lowering the pH from 7.40 to 6.70 resulted in a decrease in the amount of CO necessary to obtain 100% carboxyhemoglobin. Further pH lowering to 6.00 did not result in additional reduction in the amount of gas. During 4 days of refrigerated storage CO treated liquid blood maintained, at every pH, a more stable and attractive red color than fresh blood, which was a result of an increase (P<0.05) of a(*) (redness) and b(*) (yellowness) values and no variation (P>0.05) on L(*) (lightness) value. Hue (h(*)) and chroma (C(*)) decreased in the untreated blood but not in the CO-treated blood. The results indicate that blood saturation with CO yields a product having greater potential for use in meat products without compromising its visual appearance.

Evaluation of mortadella formulated with carbon monoxide-treated porcine blood.

The proximate composition and color of mortadellas containing carbon monoxide-treated (COTB), untreated (UNTB), or CO-treated dried blood (CODB) were compared to that of control mortadella. Blood addition did not affect (P>0.05) the proximate composition and TBARS. The mortadella containing 10% UNTB were brown and those containing COTB or CODB were red. Residual nitrite level, L*, a*, b* and c* values of the mortadella decreased (P<0.05) with an increase in the amount of blood; TBARs did not vary (P>0.05). Increasing the amount of blood increased (P<0.05) the hue angle (h*) and browning index (BI) of the mortadella containing UNTB. Increasing blood addition decreased (P<0.05) h* and did not affect (P>0.05) BI. Increasing storage length decreased (P<0.05) residual nitrite, affected BI and color coordinates and did not affect TBARS (P>0.05). Addition of CO-treated blood allows the production of better-colored sausages having lower residual nitrite levels.

Fatty acid profile, color and lipid oxidation of meat from young bulls fed ground soybean or rumen protected fat with or without monensin.

The objective of the present study was to evaluate the meat quality and fatty acid (FA) profile of the muscle and subcutaneous fat of young bulls fed ground soybean grain (SB) or rumen protected fat (RPF) with (230 mg head(-1) day(-1)) or without monensin. Forty animals with an initial weight of 359 kg were allotted in a 2×2 factorial arrangement of treatments. The use of monensin increased the arachidonic and α-linolenic acids in the longissimus dorsi (LD) muscle and subcutaneous fat, respectively (P<0.05). The meat from the animals receiving RPF had greater C18:1 content (P<0.01). The CLA and C18:2 contents were greater in the LD muscle of the animals fed SB (P<0.01). However, α-C18:3 was greater in the LD muscle of animals fed RPF (P<0.01). In the subcutaneous fat, SB reduced C12:0 and C14:0 contents (P<0.01) and increased C18:0 (P<0.05). The inclusion of RPF increased the C18:1 and CLA contents (P<0.01) in the subcutaneous fat. Soybean elevated PUFA contents and increased susceptibility of muscle and subcutaneous fat to lipid oxidation.

Qualitative characteristics of meat from young bulls fed different levels of crude glycerin.

The objective was to evaluate the fatty acid profile and qualitative characteristics of meat from young bulls fed crude glycerin. Forty-four animals with an initial live weight of 368 ± 4 kg were used in a completely randomized design, with four treatments: no glycerin or addition of 6, 12 or 18% glycerin. The animals were slaughtered with 519.5 ± 14.9 kg of live weight. The meat characteristics assessed were chemical composition, shear force, fatty acid concentration, color and lipid oxidation. The addition of glycerin increased the content of ether extract (P<0.05) in the muscle. A linear increase was observed (P<0.05) in the oleic acid contents (C18:1 cis 9). The saturated fatty acid (SFA) contents linearly decreased in the muscle as a function of glycerin addition. The lightness (L*) and yellowness (b*) indices increased with the use of crude glycerin (P<0.05). The crude glycerin increased the intramuscular fat and oleic acid content in the longissimus dorsi muscle.

Mortadella sausage manufactured with Caiman yacare (Caiman crocodilus yacare) meat, pork backfat, and soybean oil.

Mortadellas manufactured using Caiman yacare meat (70%) and pork fat (30%) were reformulated by substituting pork fat with increasing amounts of soybean oil (25%, 50%, and 100%) and evaluated 7, 30, and 60 days post-manufacture. The substitutions resulted in an increase in fat content and in the percentage of polyunsaturated fatty acids (total PUFA; 18:2 n-6 and 18:3 n-3), and a reduction in saturated fatty acids (total SFA; 14:0 and 16:0). These alterations reduced (P<0.01) the indices of atherogenicity and thrombogenicity by 4- to 5-fold in the formulation with 100% soybean oil compared to the original formulation. The levels of TBARS obtained at day 7 were positively correlated (P<0.01) with 18:2 n-6 and 18:3 n-3 (r=0.83 and 0.84, respectively) and negatively correlated with 17:0 and 17:1 (r=-0.91 and -0.89, respectively). All formulations received favourable overall acceptability by the sensory panel.

Influence of the resistance training on heart rate variability, functional capacity and muscle strength in the chronic obstructive pulmonary disease.

BACKGROUND: The chronic obstructive pulmonary disease (COPD) is associated with the strength and resistance decreasing in addition to the dysfunction on autonomic nervous system (ANS). The aerobic training isolated or in association with the resistance training showed evidence of beneficial effects on an autonomic modulation of COPD; however, there are no studies addressing the effect of isolated resistance training. AIMS: This study aims at investigating the influence of resistance training on an autonomic modulation through heart rate variability (HRV), functional capacity and muscle strength in individuals with COPD. DESIGN: Clinical series study. SETTING: Outpatients. POPULATION: The study involved 13 individuals with COPD. METHODS: The experimental protocol was composed by an initial and final evaluation that consisted in autonomic evaluations (HRV), cardiopulmonary functional capacity evaluation (6-minute walk test) and strength evaluation (dynamometry) in addition by the resistance training performed by 24 sessions lasted 60 minutes each one and on a frequency of three times a week. The intensity was determined initially with 60% of one maximum repetition and was progressively increased in each five sessions until 80%. RESULTS: The HRV temporal and spectral indexes analysis demonstrates improvement of autonomic modulation, with significant statistical increases to sympathetic and parasympathetic components of ANS representing by SDNN, LF and HF. In addition, it was observed significant statistical increases to shoulder abduction and knee flexion strength and functional capacity. CONCLUSION: The exclusive resistance training performed was able to positively influence the autonomic modulation; in addition it promoted benefits on cardiorespiratory functional capacity and strength benefits in individuals with COPD. CLINICAL REHABILITATION IMPACT: This study could contribute to clinical and professionals researchers that act with COPD, even though the resistance component of pulmonary rehabilitation presents consensual benefits on several healthy indicators parameters. There is no evidence about the effects on HRV before. Moreover, this study showed, on clinical practice, the HRV uses as an ANS activity on sinus node evaluation and highlights further importance on scientific context.

Bioactividad y pruebas de hemólisis de compósitos de hidroxiapatita zirconia (HAp/ZrO2-8Y2O3) para implantes óseos / Bioactivity and hemolysis test of hydroxyapatite/zirconia composites (HAp/ZrO2-8Y2O3) for bone implants

Resumen Se presenta la síntesis de compósitos de hidroxiapatita/zirconia (HAp/ZrO2-8Y2O3) por el método de mezclado líquido en base al método de Pechini, cuya innovación radica en la obtención simultánea de ambas fases con distribución uniforme, aunque tiene la limitante de la interacción de los cationes, propiciando la formación de fases secundarias si no se controlan las variables. Los materiales fueron conformados en discos de 1 cm de diámetro y tratados a 1400 °C, para caracterizarse por espectrometría de infrarrojo (FTIR-ATR) y difracción de rayos X (DRX). Las pruebas de bioactividad fueron realizadas mediante el método de inmersión en fluidos fisiológicos simulados durante 21 días y caracterizadas por microscopia electrónica de barrido (MEB) y espectrometría de fotoelectrones emitidos por rayos X (XPS). Las pruebas de hemólisis se basaron en la norma ASTM F 756-00. Después de la inmersión, se observó la presencia de cristales de hidroxiapatita sobre la superficie del compósito, además los análisis de XPS muestran señales de energía para los elementos de calcio y fósforo. En cuanto a las pruebas de hemólisis se observaron grados de citotoxicidad por debajo del 3% con lo cual se infiere que son hemocompatibles, aunque se requieren más estudios de biocompatibilidad para su aplicación biomédica.

Abstract The synthesis of hydroxyapatite/zirconia composites (HAp/ZrO2-8Y2O3) is presented, using the liquid mixing method based on the Pechini method, whose innovation lies in the simultaneous synthesis of both phases with uniform distribution, although it has the limitation of the cations interactions, favoring the formation of secondary phases if the variables are not controlled. The obtained materials were formed into discs of 1 cm in diameter and treated at 1400 °C, and then characterized by infrared spectrometry (FTIR-ATR) and X-ray diffraction (XRD). The bioactivity tests were carried by the immersion method in simulated body fluid for 21 days and characterized by scanning electron microscopy (SEM) and X-ray photoelectron spectrometry (XPS). The hemolysis tests were based on the ASTM F 756-00 standard. After the immersion, the presence of hydroxyapatite crystals on the surface of the composite was observed; in addition, the XPS analyzes show energy signals for the elements of calcium and phosphorus. Regarding the hemolysis tests, degrees of cytotoxicity were observed below 3%, it is inferred that they are hemocompatible, although more biocompatibility studies are required for biomedical application.

Mucociliary clearance and its relation with the level of physical activity in daily life in healthy smokers and nonsmokers.

OBJECTIVES: To investigate the relationship between mucociliary transport and physical activity in daily life (PADL) in smokers and nonsmokers. METHODS: Fifty-two current smokers were submitted to assessment of mucociliary transport (Sacharin Transit Time, STT), carbon monoxide levels in the exhaled air, lung function and smoking history. In addition, subjects kept a pedometer worn at the waist for six days in order to determine their level of PADL (steps/day). The tests were also performed on 30 matched healthy nonsmokers who served as control group. RESULTS: Light smokers (≤15 cigarettes/day) had a STT of 9 (7-11) min (median [confidence interval]), which was similar to nonsmokers (8 [8-11]min; p=0.8). Both moderate (16-25 cigarettes/day) and heavy (>25 cigarettes/day) smokers had significantly higher STT (13 [11-17] min and 13 [10-21] min, respectively) than nonsmokers and light smokers (p<0.05 for all). There was no difference in the number of steps/day between any of the groups (p>0.05 for all). In the general group of smokers, STT was not significantly correlated with PADL, pack/years index, years of smoking or age (r<-0.23; p>0.09 for all). There was significant negative correlation between STT and PADL only in light smokers (r=-0.55; p=0.02) and nonsmokers (r=-0.42; p=0.02), but not in moderate and heavy smokers. CONCLUSION: In light smokers and non-smokers, better mucociliary function is associated to higher daily physical activity level, as opposed to the decreased mucociliary function observed in smokers, i.e., those with moderate and heavy cigarette consumption.

Is walking in daily life a moderate intensity activity in patients with chronic obstructive pulmonary disease?

BACKGROUND: So far there are no studies investigating whether the time spent walking in daily life by patients with COPD corresponds to an activity of at least moderate intensity according to the recommendations of the American College of Sports Medicine (ACSM). AIM: To quantify in patients with COPD the time spent/day in physical activity of at least moderate intensity according to the ACSM (TPA>moderate); and to compare their TPA>moderate with the total time spent walking/day in daily life (TW). DESIGN: Cross-sectional study. SETTING: Patients entering in a pulmonary rehabilitation program at a University Hospital in Brazil. POPULATION: Seventy-three patients with COPD (FEV1 40±15% pred; 65±9 years). METHODS: TW and TPA>moderate were respectively assessed with the DynaPort and SenseWear activity monitors for 12 hours/day during 2 days. RESULTS: There was significant difference between TW and TPA>moderate (53 [36-80] versus 12 [3-33] minutes/day, respectively; P<0.0001). Although 84% of patients reached more than 30 minutes/day of TW, only 29% reached more than 30 minutes/day of TPA>moderate. TPA>moderate correlated modestly with TW (r= 0.52; P<0.0001). CONCLUSION: The majority of patients with moderate-very severe COPD walk more than 30 minutes/day; however this does not mean that they are physically active, since less than 1/4 of their time spent walking can be considered as moderate-intensity activity. CLINICAL REHABILITATION IMPACT: Moderate-intensity physical activity in patients with COPD is more reduced than previously known. There is a clear need to increase their daily physical activity and lead a higher proportion of this population to comply with the minimum recommendations.

CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion.

OBJECTIVE: Age at onset of diagnostic motor manifestations in Huntington disease (HD) is strongly correlated with an expanded CAG trinucleotide repeat. The length of the normal CAG repeat allele has been reported also to influence age at onset, in interaction with the expanded allele. Due to profound implications for disease mechanism and modification, we tested whether the normal allele, interaction between the expanded and normal alleles, or presence of a second expanded allele affects age at onset of HD motor signs. METHODS: We modeled natural log-transformed age at onset as a function of CAG repeat lengths of expanded and normal alleles and their interaction by linear regression. RESULTS: An apparently significant effect of interaction on age at motor onset among 4,068 subjects was dependent on a single outlier data point. A rigorous statistical analysis with a well-behaved dataset that conformed to the fundamental assumptions of linear regression (e.g., constant variance and normally distributed error) revealed significance only for the expanded CAG repeat, with no effect of the normal CAG repeat. Ten subjects with 2 expanded alleles showed an age at motor onset consistent with the length of the larger expanded allele. CONCLUSIONS: Normal allele CAG length, interaction between expanded and normal alleles, and presence of a second expanded allele do not influence age at onset of motor manifestations, indicating that the rate of HD pathogenesis leading to motor diagnosis is determined by a completely dominant action of the longest expanded allele and as yet unidentified genetic or environmental factors.

[Immediate and short term effects of smoking on nasal mucociliary clearance in smokers]. / Efeito imediato e a curto prazo do cigarro sobre o transporte mucociliar nasal de fumadores.

BACKGROUND AND OBJECTIVES: The efficiency of mucociliary transport may vary in different conditions, such as in exposure to harmful particles of the cigarette smoke. The present study evaluated the acute and short term effects of smoking on nasal mucociliary clearance in current smokers by the quantification of the Saccharin Transit Time (STT), and to investigate its correlation with the history of tobacco consumption. METHODS: Nineteen current smokers (11 men, 51 ± 16 years; BMI 23 ± 9 kg/m(2), 27 ± 11 cigarettes per day, 44 ± 25 pack-years), entering a smoking cessation intervention program, responded to a questionnaire concerning smoking history and were submitted to lung function assessment (spirometry) and the STT test. STT was assessed immediately after smoking and 8 hours after smoking. The STT test was also performed in nineteen matched healthy non-smokers' who served as control group. RESULTS: When compared to STT in non-smokers' (10 ± 4 min; mean ± standard deviation), smokers presented similar STT immediately after smoking (11 ± 6 min; p=0.87) and slower STT 8 hours after smoking (16 ± 6 min; p=0.005 versus non-smokers' and p=0.003 versus immediately after smoking). STT 8 hours after smoking correlated positively with age (r=0.59; p=0.007), cigarettes per day (r=0.53; p=0.02) and pack-years index (r=0.74; p=0.0003). CONCLUSIONS: In smokers, although the mucociliary clearance immediately after smoking is similar to non-smokers', eight hours after smoking it is reduced, and this reduction is closely related to the smoking habits.