Motor function measure scale, steroid therapy and patients with Duchenne muscular dystrophy

Objective: To assess the evolution of motor function in patients with Duchenne muscular dystrophy (DMD) treated with steroids (prednisolone or deflazacort) through the Motor Function Measure (MFM), which evaluates three dimensions of motor performance (D1, D2, D3). Methods: Thirty-three patients with DMD (22 ambulant, 6 non-ambulant and 5 who lost the capacity to walk during the period of the study) were assessed using the MFM scale six times over a period of 18 months. Results: All the motor functions remained stable for 14 months in all patients, except D1 for those who lost their walking ability. In ambulant patients, D2 (axial and proximal motor capacities) motor functions improved during six months; an improvement in D3 (distal motor capacity) was noted during the total follow-up. D1 (standing posture and transfers) and total score were useful to predict the loss of the ability to walk. Conclusions: The use of the MFM in DMD patients confirms the benefits of the steroid treatment for slowing the progression of the disease.

Metabolic profile of dystrophic mdx mouse muscles analyzed with in vitro magnetic resonance spectroscopy (MRS)

Duchenne muscular dystrophy (DMD) is a recessive X-linked form of muscular dystrophy characterized by progressive and irreversible degeneration of the muscles. The mdx mouse is the classical animal model for DMD, showing similar molecular and protein defects. The mdx mouse, however, does not show significant muscle weakness, and the diaphragm muscle is significantly more degenerated than skeletal muscles. In this work, magnetic resonance spectroscopy (MRS) was used to study the metabolic profile of quadriceps and diaphragm muscles from mdx and control mice. Using principal components analysis (PCA), the animals were separated into groups according to age and lineages. The classification was compared to histopathological analysis. Among the 24 metabolites identified from the nuclear MR spectra, only 19 were used by the PCA program for classification purposes. These can be important key biomarkers associated with the progression of degeneration in mdx muscles and with natural aging in control mice. Glutamate, glutamine, succinate, isoleucine, acetate, alanine and glycerol were increased in mdx samples as compared to control mice, in contrast to carnosine, taurine, glycine, methionine and creatine that were decreased. These results suggest that MRS associated with pattern recognition analysis can be a reliable tool to assess the degree of pathological and metabolic alterations in the dystrophic tissue, thereby affording the possibility of evaluation of beneficial effects of putative therapies. (C) 2012 Elsevier Inc. All rights reserved.

NONSPECIFIC INFECTIOUS DISEASES IN PREHISTORIC SAN PEDRO DE ATACAMA, NORTHERN CHILE

The region of San Pedro de Atacama, Northern Chile, has undergone several cultural and social changes after humans settled in Atacama Desert around 500 BC. The Atacameno people experienced the highest degree of social and cultural changes between 400 and 900 AD when they were assimilated into the Tiwanaku trade and political web that influenced most of the Central-Southern Andes. Under the influence of Tiwanaku, San Pedro de Atacama experienced its greatest economic development. Prior analyses of local human skeletal remains have shown a significant increase in the stature of the local population during the same period. In this paper, we investigate the impact of the Tiwanaku influence on the local epidemiological profile using the incidence of periostitis and osteomyelitis as indicators of biological stress. Surprisingly, the best epidemiological condition occurred during the final phase of influence of Tiwanaku (910-960 AD), and not during the apex influence (480-920 AD), as expected by the archaeological context. We suggest that population growth and aggregation may have counteracted the benefits of improved nutrition during the peak Tiwanaku influence. A severe drought occurred between 1,100 and 1,400 AD in Northern Chile. This could also explain the marked increase of bone infections in the post-Tiwanaku period (920-1,240 AD).

Effects of strength and power training on neuromuscular adaptations and jumping movement pattern and performance

Effects of strength and power training on neuromuscular adaptations and jumping movement pattern and performance. J Strength Cond Res 26(12): 3335-3344, 2012-This study aimed at comparing the effects of strength and power training (ST and PT) regimens on neuromuscular adaptations and changes on vertical jump performance, kinetics, and kinematics parameters. Forty physically active men (178.2 +/- 7.0 cm; 75.1 +/- 8.6 kg; 23.6 +/- 3.5 years) with at least 2 years of ST experience were assigned to an ST (n = 14), a PT (n = 14), or a control group (C; n = 12). The training programs were performed during 8 weeks, 3 times per week. Dynamic and isometric maximum strength, cross-sectional area, and muscle activation were assessed before and after the experimental period. Squat jump (SJ) and countermovement jump (CMJ) performance, kinetics, and kinematics parameters were also assessed. Dynamic maximum strength increased similarly (p < 0.05) for the ST (22.8%) and PT (16.6%) groups. The maximum voluntary isometric contraction increased for the ST and PT groups (p < 0.05) in the posttraining assessments. There was a main time effect for muscle fiber cross-sectional area (p < 0.05), but there were no changes in muscle activation. The SJ height increased, after ST and PT, because of a faster concentric phase and a higher rate of force development (p < 0.05). The CMJ height increased only after PT (p < 0.05), but there were no significant changes in its kinetics and kinematics parameters. In conclusion, neuromuscular adaptations were similar between the training groups. The PT seemed more effective than the ST in increasing jumping performance, but neither the ST nor the PT was able to affect the SJ and the CMJ movement pattern (e.g., timing and sequencing of joint extension initiation).

Effects of strength and power training on neuromuscular variables in older adults

The purpose of this study was to compare the neuromuscular adaptations produced by strength-training (ST) and power-training (PT) regimens in older individuals. Participants were balanced by quadriceps cross-sectional area (CSA) and leg-press 1-repetition maximum and randomly assigned to an ST group (n = 14; 63.6 +/- 4.0 yr, 79.7 +/- 17.2 kg, and 163.9 +/- 9.8 cm), a PT group (n = 16; 64.9 +/- 3.9 yr. 63.9 +/- 11.9 kg, and 157.4 +/- 7.7 cm), or a control group (n = 13; 63.0 +/- 4.0 yr, 67.2 +/- 10.8 kg, and 159.8 +/- 6.8 cm). ST and PT were equally effective in increasing (a) maximum dynamic and isometric strength (p < .05), (b) increasing quadriceps muscle CSA (p < .05), and (c) decreasing electrical mechanical delay of the vastus lateralis muscle (p < .05). There were no significant changes in neuromuscular activation after training. The novel finding of the current study is that PT seems to be an attractive alternative to regular ST to maintain and improve muscle mass.

Increased IgE serum levels are unrelated to allergic and parasitic diseases in patients with juvenile systemic lupus erythematosus

OBJECTIVE: The aim of this study was to assess the IgE serum levels in juvenile systemic lupus erythematosus patients and to evaluate possible associations with clinical and laboratory features, disease activity and tissue damage. METHODS: The IgE serum concentrations in 69 consecutive juvenile systemic lupus erythematosus patients were determined by nephelometry. IgG, IgM and IgA concentrations were measured by immunoturbidimetry. All patients were negative for intestinal parasites. Statistical analysis methods included the Mann-Whitney, chi-square and Fisher's exact tests, as well as the Spearman rank correlation coefficient. RESULTS: Increased IgE concentrations above 100 IU/mL were observed in 31/69 (45%) juvenile systemic lupus erythematosus patients. The mean IgE concentration was 442.0 +/- 163.4 IU/ml (range 3.5- 9936.0 IU/ml). Fifteen of the 69 patients had atopic disease, nine patients had severe sepsis and 56 patients presented with nephritis. The mean IgE level in 54 juvenile systemic lupus erythematosus patients without atopic manifestations was 271.6 +/- 699.5 IU/ml, and only nine of the 31 (29%) patients with high IgE levels had atopic disease. The IgE levels did not statistically differ with respect to the presence of atopic disease, severe sepsis, nephritis, disease activity, or tissue damage. Interestingly, IgE concentrations were inversely correlated with C4 levels ( r = -0.25, p = 0.03) and with the SLICC/ACR-DI score (r = -0.34, p = 0.005). The IgE concentration was also found to be directly correlated with IgA levels (r = 0.52, p = 0.03). CONCLUSIONS: The present study demonstrated for the first time that juvenile systemic lupus erythematosus patients have increased IgE serum levels. This increase in IgE levels was not related to allergic or parasitic diseases. Our results are in line with the hypothesis that high IgE levels can be considered a marker of immune dysregulation.

Maximal strength, number of repetitions , and total volume are differently affected by static-ballistic, and proprioceptive neuromuscular facilitation stretching

Barroso, R, Tricoli, V, dos Santos Gil, S, Ugrinowitsch, C, and Roschel, H. Maximal strength, number of repetitions, and total volume are differently affected by static-, ballistic-, and proprioceptive neuromuscular facilitation stretching. J Strength Cond Res 26(9): 2432-2437, 2012-Stretching exercises have been traditionally incorporated into warm-up routines before training sessions and sport events. However, the effects of stretching on maximal strength and strength endurance performance seem to depend on the type of stretching employed. The objective of this study was to compare the effects of static stretching (SS), ballistic stretching (BS), and proprioceptive neuromuscular facilitation (PNF) stretching on maximal strength, number of repetitions at a submaximal load, and total volume (i.e., number of repetitions 3 external load) in a multiple-set resistance training bout. Twelve strength-trained men (20.4 +/- 4.5 years, 67.9 +/- 6.3 kg, 173.3 +/- 8.5 cm) volunteered to participate in this study. All of the subjects completed 8 experimental sessions. Four experimental sessions were designed to test maximal strength in the leg press (i.e., 1 repetition maximum [1RM]) after each stretching condition (SS, BS, PNF, or no-stretching [NS]). During the other 4 sessions, the number of repetitions performed at 80% 1RM was assessed after each stretching condition. All of the stretching protocols significantly improved the range of motion in the sit-and-reach test when compared with NS. Further, PNF induced greater changes in the sit-and-reach test than BS did (4.7 +/- 1.6, 2.9 +/- 1.5, and 1.9 +/- 1.4 cm for PNF, SS, and BS, respectively). Leg press 1RM values were decreased only after the PNF condition (5.5%, p < 0.001). All the stretching protocols significantly reduced the number of repetitions (SS: 20.8%, p < 0.001; BS: 17.8%, p = 0.01; PNF: 22.7%, p < 0.001) and total volume (SS: 20.4%, p < 0.001; BS: 17.9%, p = 0.01; PNF: 22.4%, p < 0.001) when compared with NS. The results from this study suggest that, to avoid a decrease in both the number of repetitions and total volume, stretching exercises should not be performed before a resistance training session. Additionally, strength-trained individuals may experience reduced maximal dynamic strength after PNF stretching.

Phosphene Thresholds Elicited by Transcorneal Electrical Stimulation in Healthy Subjects and Patients with Retinal Diseases

PURPOSE. To evaluate electrically evoked phosphene thresholds (EPTs) in healthy subjects and in patients with retinal disease and to assess repeatability and possible correlations with common ophthalmologic tests. METHODS. In all, 117 individuals participated: healthy subjects (n = 20) and patients with retinitis pigmentosa (RP, n = 30), Stargardt's disease (STG, n = 14), retinal artery occlusion (RAO, n = 20), nonarteritic anterior ischemic optic neuropathy (NAION, n = 16), and primary open-angle glaucoma (POAG, n = 17). EPTs were determined at 3, 6, 9, 20, 40, 60, and 80 Hz with 5+5-ms biphasic current pulses using DTL electrodes. Subjects were examined twice (test-retest range: 1-6 weeks). An empirical model was developed to describe the current-frequency relationship of EPTs. Visual acuity, visual field (kinetic + static), electrophysiology (RP, RAO, STG: Ganzfeld-electroretinography [ERG]/multifocal-ERG; POAG: pattern-ERG; NAION: VEP), slit-lamp biomicroscopy, fundus examination, and tonometry were assessed. RESULTS. EPTs varied between disease groups (20 Hz: healthy subjects: 0.062 +/- 0.038 mA; STG: 0.102 +/- 0.097 mA; POAG: 0.127 +/- 0.09 mA; NAION: 0.244 +/- 0.126 mA; RP: 0.371 +/- 0.223 mA; RAO: 0.988 +/- 1.142 mA). In all groups EPTs were lowest at 20 Hz. In patients with retinal diseases and across all frequencies EPTs were significantly higher than those in healthy subjects, except in STG at 20 Hz (P = 0.09) and 40 Hz (P = 0.17). Test-retest difference at 20 Hz was 0.006 mA in the healthy group and 0.003-0.04 mA in disease groups. CONCLUSIONS. Considering the fast, safe, and reliable practicability of EPT testing, this test might be used more often under clinical circumstances. Determination of EPTs could be potentially useful in elucidation of the progress of ophthalmologic diseases, either in addition to standard clinical assessment or under conditions in which these standard tests cannot be used meaningfully. (ClinicalTrials.gov number, NCT00804102.) (Invest Ophthalmol Vis Sci. 2012; 53: 7440-7448) DOI:10.1167/iovs.12-9612

Atmospheric pollution: influence on hospital admissions in paediatric rheumatic diseases

Objective: To investigate the lag structure effects from exposure to atmospheric pollution in acute outbursts in hospital admissions of paediatric rheumatic diseases (PRDs). Methods: Morbidity data were obtained from the Brazilian Hospital Information System in seven consecutive years, including admissions due to seven PRDs (juvenile idiopathic arthritis, systemic lupus erythematosus, dermatomyositis, Henoch-Schonlein purpura, polyarteritis nodosa, systemic sclerosis and ankylosing spondylitis). Cases with secondary diagnosis of respiratory diseases were excluded. Daily concentrations of inhaled particulate matter (PM10), sulphur dioxide (SO2) nitrogen dioxide (NO2), ozone (O-3) and carbon monoxide (CO) were evaluated. Generalized linear Poisson regression models controlling for short-term trend, seasonality, holidays, temperature and humidity were used. Lag structures and magnitude of air pollutants' effects were adopted to estimate restricted polynomial distributed lag models. Results: The total number of admissions due to acute outbursts PRD was 1,821. The SO2 interquartile range (7.79 mu g/m(3)) was associated with an increase of 1.98% (confidence interval 0.25-3.69) in the number of hospital admissions due to outcome studied after 14 days of exposure. This effect was maintained until day 17. Of note, the other pollutants, with the exception of O-3, showed an increase in the number of hospital admissions from the second week. Conclusion: This study is the first to demonstrate a delayed association between SO2 and PRD outburst, suggesting that oxidative stress reaction could trigger the inflammation of these diseases. Lupus (2012) 21, 526-533.

Prior history of sexually transmitted diseases in women living with AIDS in Sao Paulo, Brazil

Objectives: To describe the epidemiological profile, risk behaviors, and the prior history of sexually transmitted diseases (STDs) in women living with acquired immunodeficiency syndrome (AIDS). Methods: Cross-sectional study, performed at the Centro de Referencia e Treinamento em DST/AIDS of Sao Paulo. The social, demographic, behavioral, and clinical data such as age, schooling, marital status, age at first sexual intercourse, number of sexual partners, parity, use of drugs, time of HIV diagnosis, CD4 count, and viral load determination were abstracted from the medical records of women living with AIDS who had gynecological consultation scheduled in the period from June 2008 to May 2009. Results: Out of 710 women who were scheduled to a gynecological consultation during the period of the study, 598 were included. Previous STD was documented for 364 (60.9%; 95% CI: 56.9%-64.8%) women. The associated factors with previous STDs and their respective risks were: human development index (HDI) <0.50 (ORaj = 5.5; 95% CI: 2.8-11.0); non-white race (ORaj = 5.2; 95% CI: 2.5-11.0); first sexual intercourse at or before 15 years of age (ORaj = 4.4; 95% CI: 2.3-8.3); HIV infection follow-up time of nine years or more (ORaj = 4.2; 95% CI: 2.3-7.8)]; number of sexual partners during the entire life between three and five partners (ORaj = 2.2; 95% CI: 1.1-4.6), and six or more sexual partners (ORaj = 3.9; 95% CI: 1.9-8.0%); being a sex worker (ORaj = 1.9; 95% CI: 1.1-3.1). Conclusions: A high prevalence of a prior history of STDs in the studied population was found. It is essential to find better ways to access HIV infection prevention, so that effective interventions can be more widely implemented. (C) 2012 Elsevier Editora Ltda. All rights reserved.

Bioenergetics and neuromuscular determinants of the time to exhaustion at velocity corresponding to (V) over dotO(2)max in recreational long-distance runners

Bertuzzi, R, Bueno, S, Pasqua, LA, Acquesta, FM, Batista, MB, Roschel, H, Kiss, MAPDM, Serrao, JC, Tricoli, V, and Ugrinowitsch, C. Bioenergetics and neuromuscular determinants of the time to exhaustion at velocity corresponding to (V) over dotO(2)max in recreational long-distance runners. J Strength Cond Res 26(8): 2096-2102, 2012-The purpose of this study was to investigate the main bioenergetics and neuromuscular determinants of the time to exhaustion (T-lim) at the velocity corresponding to maximal oxygen uptake in recreational long-distance runners. Twenty runners performed the following tests on 5 different days: (a) maximal incremental treadmill test, (b) 2 submaximal tests to determine running economy and vertical stiffness, (c) exhaustive test to measured the T-lim, (d) maximum dynamic strength test, and (e) muscle power production test. Aerobic and anaerobic energy contributions during the T-lim test were also estimated. The stepwise multiple regression method selected 3 independent variables to explain T-lim variance. Total energy production explained 84.1% of the shared variance (p = 0.001), whereas peak oxygen uptake ((V) over dotO(2)peak) measured during T-lim and lower limb muscle power ability accounted for the additional 10% of the shared variance (p = 0.014). These data suggest that the total energy production, (V) over dotO(2)peak, and lower limb muscle power ability are the main physiological and neuromuscular determinants of T-lim in recreational long-distance runners.

JC virus-associated central nervous system diseases in HIV-infected patients in Brazil: clinical presentations, associated factors with mortality and outcome

Introduction: Several presentations of neurologic complications caused by JC virus (JCV) in human immunodeficiency virus (HIV)-infected patients have been described and need to be distinguished from the "classic" form of progressive multifocal leukoencephalopathy (PML). The objectives of this study were: 1) to describe the spectrum and frequency of presentations of JCV-associated central nervous system (CNS) diseases; 2) identify factors associated with in-hospital mortality of patients with JCV-associated CNS disease; and 3) to estimate the overall mortality of this population. Material and methods: This was a retrospective study of HIV-infected patients admitted consecutively for JCV-associated CNS diseases in a referral teaching center in Sao Paulo, Brazil, from 2002 to 2007. All patients with laboratory confirmed JCV-associated CNS diseases were included using the following criteria: compatible clinical and radiological features associated with the presence of JCV DNA in the cerebrospinal fluid. JCV-associated CNS diseases were classified as follows: 1) classic PML; 2) inflammatory PML; and 3) JC virus granule cell neuronopathy (GCN). Results: We included 47 cases. JCV-associated CNS diseases were classified as follows: 1) classic PML: 42 (89%); 2) inflammatory PML: three (6%); and 3) JC virus GCN: four (9%). Nosocomial pneumonia (p = 0.003), previous diagnosis of HIV infection (p = 0.03), and imaging showing cerebellar and/or brainstem involvement (p = 0.02) were associated with in-hospital mortality. Overall mortality during hospitalization was 34%. Conclusions: Novel presentations of JCV-associated CNS diseases were observed in our setting; nosocomial pneumonia, previous diagnosis of HIV infection, and cerebellar and/or brainstem involvement were associated with in-hospital mortality; and overall mortality was high. (C) 2012 Elsevier Editora Ltda. All rights reserved.

The effect of a Lucia jig for 30 minutes on neuromuscular re-programming, in normal subjects

The Lucia jig is a technique that promotes neuromuscular reprogramming of the masticatory system and allows the stabilization of the mandible without the interference of dental contacts, maintaining the mandible position in harmonic condition with the musculature in normal subjects or in patients with temporomandibular dysfunction (TMD). This study aimed to electromyographically analyze the activity (RMS) of the masseter and temporal muscles in normal subjects (control group) during the use of an anterior programming device, the Lucia jig, in place for 0, 5, 10, 20 and 30 minutes to demonstrate its effect on the stomatognathic system. Forty-two healthy dentate individuals (aged 21 to 40 years) with normal occlusion and without parafunctional habits or ternporomandibular dysfunction (RDC/TMD) were evaluated on the basis of the electromyographic activity of the masseter and temporal muscles before placement of a neuromuscular re-programming device, the Lucia jig, on the upper central incisors. There were no statistically significant differences (p < 0.05) in the electromyographic activity of the masticatory muscles in the different time periods. The Lucia jig changed the electromyographic activity by promoting a neuromuscular reprogramming. In most of the time periods, it decreased the activation of the masticatory muscles, showing that this device has wide applicability in dentistry. The use of a Lucia jig over 0, 5, 10, 15, 20 and 30 minutes did not promote any statistically significant increase in muscle activity despite differences in the data, thus showing that this intra-oral device can be used in dentistry.

Advancing the management of primary immunodeficiency diseases in Latin America: Latin American Society for Immunodeficiencies (LASID) Initiatives

Primary immunodeficiency diseases (PIDD) are associated with significant morbidity and mortality and result in a significant public health burden. This is in part due to the lack of appropriate diagnosis and treatment of these patients. It is critical that governments become aware of this problem and provide necessary resources to reduce this impact on health care systems. Leading physicians in their respective countries must be supported by their own governments in order to implement tools and provide education and thus improve the diagnosis and treatment of PIDD. The Latin American Society of Primary Immunodeficiencies (LASID) has initiated a large number of activities aimed at achieving these goals, including the establishment of a PIDD registry, development of educational programmes and guidelines, and the introduction of a PIDD fellowship programme. These initiatives are positively impacting the identification and appropriate treatment of patients with PIDD in Latin America. Nevertheless, much remains to be done to ensure that every person with PIDD receives proper therapy. (C) 2011 SEICAP. Published by Elsevier Espana, S.L. All rights reserved.

The therapeutic journey of families of children with respiratory diseases in the public health service

This study's purpose was to identify the therapeutic journey of families seeking health care for their children with respiratory diseases. This qualitative study had the participation of parents of children younger than five years old who were hospitalized with respiratory diseases. Path mapping was used as an instrument to collect data, which was analyzed through thematic analysis. The finding indicate that families sought the health services as soon as they perceived symptoms and had access to medical care, however such care was not decisive in resolving their health issues. Even though the families returned to the service at least another three times, the children had to be hospitalized. The attributes of primary health care were not observed in the public health services, while therapeutic encounters had no practical success.