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Cutaneous horn is a relatively uncommon lesion consisting of keratotic material resembling that of an animal horn. This kind of lesion is more common in Caucasians and in older age groups. The primary lesion underlying the horny material may be benign, premalignant or malignant. The treatment of choice is an excisional biopsy with a narrow margin, because of the possibility of malignancy. The material must be submitted for histopathological evaluation. The first reported case of cutaneous horn arising on the vermillion border of the lower lip in a black child is presented. [ABSTRACT FROM AUTHOR]

OBJETIVO: Analisar o resultado cirúrgico em portadores de transposição das grandes artérias com septo interventricular intacto, operados após o período neonatal. MÉTODOS: Entre janeiro de 1998 e março de 2004 foram atendidas 121 crianças com transposição das grandes artérias com septo interventricular intacto, sendo 29 (24%) após o período neonatal. A seleção para tratamento cirúrgico foi baseada na avaliação ecocardiográfica pelo cálculo da massa do ventrículo esquerdo e da configuração do septo interventricular. Das 29 crianças, 12 foram selecionadas para correção anatômica primária, 12 para correção em dois estágios, após preparo cirúrgico do ventrículo esquerdo e 5 submetidas a correção atrial. RESULTADOS: No grupo submetido à correção anatômica primária houve 1 (8,3%) óbito hospitalar por sepsis. No grupo de correção em dois estágios, 5 pacientes foram submetidos ao preparo lento, com correção 3-6 meses após o 1º estágio, ocorrendo 4 óbitos após o 1º estágio. Este fato determinou mudança no nosso protocolo, adotando-se a técnica de preparo rápido nos outros 7 pacientes, tendo todos atingido o 2º estágio. Das 8 crianças submetidas ao 2º estágio houve 1 óbito hospitalar e outro óbito tardio. A evolução clínica tardia das crianças de ambos os grupos é excelente. CONCLUSÃO: A seleção ecocardiográfica adequada da transposição de grandes artérias com septo interventricular intacto quando abordada após o período neonatal, permitiu uma orientação segura da escolha da melhor abordagem cirúrgica nestes pacientes.

Anthracyclines have been intensely studied worldwide due to their importance as anticancer agents. In this work investigation was made on the production of ciclamycin, an antitumour complex of the anthracycline class, produced by Streptomyces capoamus. The fermentation medium consisted of glucose, as the main carbon source, and soymilk, as the sole nitrogen source. Experiments were performed in a 5-liter batch bioreactor under nitrogen and carbon-limiting conditions. Increasing antibiotic activity was observed both intra and extracellularly during growth under the two conditions used. Progressive loss of activity occurred in both cases after glucose was depleted from the medium. The results obtained showed that harvest of ciclamycin in a batch process should be controlled by the level of glucose in the reactor and that nitrogen should preferably be the limiting substrate. Alternatively, results indicate that extended production might be achieved in a fed-batch process where nitrogen was the limiting substrate.

The homogenous sulfonation of polystyrene was developed in a pilot plant scale producing polymers with different sulfonation degrees (18 to 22 mole % of sulfonated styrene units). The reaction yield depends chiefly on the concentration ratio of acetyl sulfate and polystyrene. The morphological and thermal properties of the sulfonated polystyrene obtained by homogeneous sulfonation were studied by means of scanning electron microscopy, differential scanning calorimetry and thermogravimetry. The glass transition temperature of sulfonated polystyrene increases in relation to pure polystyrene and DCp was evaluated in order to confirm the strong interactions among the ~SO3H groups.

A reconstrução biventricuiar de uma série de cardiopatias congênitas requer a interposição de condutos valvados ventrículo-arteriais. Com o objetivo de analisar a evolução a longo prazo de um conduto de pericárdio bovino valvulado com bíoprótese porcina sem suporte tratado com glutaraldeído, foram revistos os prontuários de 33 pacientes operados de novembro de 1985 a outubro de 1995. A idade variou de 15 dias a 18 anos (média 5,7 ± 4,3 anos). A atresia pulmonar com comunicação interventricular (CIV) foi a lesão mais freqüente (16 casos), seguida da síndrome da valva pulmonar ausente (5), truncus arteriosus (4), transposição das grandes artérias com CIV e estenose pulmonar (3) e outras (5). A mortalidade imediata foi de 18,2%, diretamente relacionada à condição pré-operatória. Vinte e três (70%) pacientes foram acompanhados por períodos que variaram de 3 meses a 10 anos (média 4,8 ± 3,0 anos). A complicação mais freqüentemente observada no seguimento tardio foi a estenose da anastomose distai do conduto, presente em 17,4% (4/23) dos pacientes. Foram reoperados 3 (13%) pacientes, sendo que 2 deles por estenose distal (p=0,02) e 1 por endocardite tardia do conduto. A mortalidade tardia foi de 17,4% (4/23), em 1 caso devido a estenose distai. A causa da estenose distal parece ser devida a retração tecidual na área de transição entre o pericárdio bovino e o tronco pulmonar. Em até 10 anos de seguimento não ocorreu calcificação significativa que prejudicasse a função tanto da valva quanto do conduto. Em conclusão, os condutos de pericárdio bovino apresentaram uma performance satisfatória como substitutos vasculares, não tendo ocorrido calcificação significativa da valva porcina ou das paredes do conduto no seguimento tardio. A incidência de estenose na anastomose distal parece estar mais relacionada a um fenômeno de retração tecidual do que a problemas técnicos.The biventricular repair of congenital heart diseases with an inadequate or absent ventricle to pulmonary artery connection sometimes requires the use of extracardiac conduits. In order to study the longterm outcome of a glutaraldehyde-preserved bovine pericardial conduit valved with a stentless porcine aortic valve, we reviewed the data of 33 patients operated between November 1985 and October 1995. Patients ages ranged from 15 days to 18 years (mean 5.7 ± 4.3 years). Pulmonary atresia with ventricular septal defect (VSD) was the most frequent disease (16 cases), followed by tetralogy of Fallot with absent pulmonary valve (5), truncus arteriosus (4), transposition of great arteries with VSD and pulmonary stenosis (3) and other miscellaneous (5). The overall hospital mortality was 18.2% and was related to the preoperative clinical condition; 23 patients (70%) were followed by a mean of 4.8 ± 3.0 years (ranging from 3 months to 10 years). The most common late complication observed was stenosis of the anastomosis between the conduit and the pulmonary artery present in 17.4% (4/23) of the patients and was the cause of 2 later reoperations (p=0,02); another patient was reoperated for late conduit endocarditis. This complication was also the cause of 1 of the 4 late deaths (17.4%). The last patient is waiting for surgery. The development of the anastomotic stenosis was problably due to a retraction of the pericardial tissue along the transition with the thin wall of the pulmonary artery. Up to 10 years, gross calcification impairing the function of the valve or the conduit itself could not be detected. In conclusion, the bovine pericardial conduits have shown a good performance as a vascular substitute. Calcification has not been a major drawback. The righ incidence of distal stenosis appears to be more related to a shrinking of the pericardial tissue than to technical reasons.

ABSTARCTThe antibiotic amoxicillin (AMX) is a semisynthetic aminopenicillin, classified as an β-lactam antibiotic. This work aims to evaluate the AMX degradation (190 mg L -1 ), in aqueous medium, applying photo-Fenton ([TOC] 0  = 100 mgC L -1 ; F H2O2  = 3.27 mmol min -1 ; [Fe 2+ ] = 0.27 mmol L -1 ; pH = 3.0; T = 40°C) and acid hydrolysis processes. Along the experiments, samples were withdrawn and analyzed by a total organic carbon (TOC) analyzer and a liquid chromatography system coupled to diode array (HPLC-DAD) and mass spectrometry (HPLC-MS) detectors. The hydrolysis process proved to be less efficient, because AMX removals greater than 80% were observed only after 24 hours of reaction (pH 2). Conversely, the photo-Fenton process removed completely AMX in just 20 minutes, reaching 85% of TOC removal in 2 hours. Finally, the AMX aqueous solutions treated by the studied processes was also evaluated in respect of its toxicity to some microorganisms, applying two antimicrobial susceptibility tests: disk-diffusion and broth microdilution methods. It was observed that the AMX aqueous solutions, pretreated by the photo-Fenton process, for just 7.5 min of reaction time, did not inhibit the microorganisms growth. The obtained results show that the photo-Fenton process was able to degrade AMX, in a relatively short time, and that the generated degradation products did not inhibit the microorganisms growth, when compared to acid hydrolysis process. Thus, it was verified the potential application of the photo-Fenton system as a pretreatment step to conventional biological oxidation processes for the treatment of industrial wastewaters.

Study Design: Cross-sectional, international survey., Objectives: To evaluate the knowledge of spine surgeons regarding the use of electromyography (EMG) and nerve conduction studies (NCS) for degenerative cervical spine conditions (DCC)., Methods: All members of AO Spine International were emailed an anonymous survey to evaluate their clinical knowledge about the use of EMG and nerve conduction studies for DCC. Descriptive statistics were used to analyze the results, as well as to compare the answers among different groups of surgeons and assess demographic characteristics., Results: A total of 402 participants answered the survey, 91.79% were men from the 5 continents. There were 221 orthopedic surgeons (55.39%) and 171 neurosurgeons (42.86%), more than a half of them with a complete spinal fellowship (56.44%). The most common reasons that surgeons obtain the test is to differentiate a radiculopathy from a peripheral nerve compression (88.06%). As a group, the responding surgeons' knowledge regarding EMG-NCS was poor. Only 53.46% of surgeons correctly answered that EMG-NCS is unable to differentiate a C5 from a C6 radiculopathy. Only 23.47% of the surgeons knew that EMG-NCS are not able to diagnose a pre vs a post-fixed brachial plexus. Only 25% of the surgeons correctly answered a question regarding the test's ability to diagnose other neurological diseases., Conclusions: We found that our respondents' knowledge regarding EMG-NCS for DCC was poor. Identifying the weak points of knowledge about EMG-NCS may help to educate surgeons on the indications for the test and the proper way to interpret the results.

The iconic caged shape of fullerenes gives rise to a series of unique chemical and physical properties; hence a deeper understanding of the attractive and repulsive forces between two buckyballs can bring detrimental information about the structural stability of such complexes, providing significant data applicable for several studies. The potential energy curves for the interaction of multiple van der Waals buckyball complexes with increasing mass were theoretically obtained within the DFT framework at ωB97xD/6-31G(d) compound model. These potential energy curves were employed to estimate the spectroscopic constants and the lifetime of the fullerene complexes with the Discrete Variable Representation and with the Dunham approaches. It was revealed that both methods are compatible in determining the rovibrational structure of the dimers and that they are genuinely stable, i.e., long-lived complexes. To further inquire into the nature of such interaction, Bader's QTAIM approach was applied. QTAIM descriptors indicate that the interactions of these closed-shell systems are dominated by weak van der Waals forces. This non-covalent interaction character was confirmed by the RDG analysis scheme. Indirectly, QTAIM also allowed us to confirm the stability of the non-covalent bonded fullerene dimers. Our lifetime calculations have shown that the studied dimers are stable for more than 1 ps, which increases accordingly with the number of carbon atoms.

(1) Background: The adverse effects of continuous use of antiretroviral therapy can generate dissatisfaction with body image in children and adolescents diagnosed with Human Immunodeficiency Virus infection (HIV) infection. The aim of this study was to verify the prevalence and factors associated with body image dissatisfaction (BI) in children and adolescents diagnosed with HIV infection; (2) This is a cross-sectional study with 60 children and adolescents (32 females; 28 males) aged 8-15 years diagnosed with HIV, carried out in Florianópolis, Brazil. Information on BI was collected through a silhouette scale, as well as sociodemographic information, biological maturation, lifestyle, self-esteem, adherence to antiretroviral treatment and aspects of HIV infection; (3) Results: The results indicated that 53.13% of female patients and 53.57% of male patients were dissatisfied with their BI. Lower subscapular skinfold and higher calf skinfold values were associated with BI dissatisfaction in females. Pre-pubertal maturation stage, higher economic level, lower concentrations of CD4+ lymphocytes, lower viral load, lower level of physical activity and longer time in front of the computer and/or video game were associated with BI dissatisfaction in males; (4) Conclusions: BI dissatisfaction was observed in more than half of patients and morphological, economic, behavioral and HIV infection aspects were associated with BI dissatisfaction.

Purpose: verify whether there is difference in body fat values assessed by different methods according to the body image perception of HIV-infected children and adolescents., Design and Methods: This is a cross-sectional study with 65 HIV-infected children and adolescents (aged 8-15 years). Total fat mass, trunk fat mass, arm fat mass and leg fat mass were obtained through dual-energy X-ray absorptiometry (DXA). Anthropometric variables were measured according to international standardization. Body image was assessed using a scale of body silhouettes. Bone age covariates were assessed using carpal wrist X-rays and physical activity by accelerometers. Information regarding viral load and use of combined antiretroviral therapy was obtained from medical records. In males, no significant difference in body fat values and body image categories was observed., Results: In the model with covariates, girls who desired to reduce body weight had higher BMI (18.96 kg / m2 ± 2.47, R 2 adj: 0.613), total fat mass (14.25 kg ± 1.37, R 2 adj: 0.589), trunk fat mass (6.50 kg ± 0.66, R 2 adj: 0.611) and leg fat mass values (6.01Kg ± 0.57, R 2 adj: 0.503) in comparison to girls who were satisfied and to those who desired to increase body weight., Conclusions: HIV + girls who desired to reduce body weight had greater amount of BMI, total fat mass, trunk fat mass and leg fat mass compared to those who were satisfied and those who desired to increase body weight., Practice Implications: These data can be used to assist in the process of coping and accepting body image., Competing Interests: Declaration of Competing Interest None., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Postpartum depression (PPD) affects a high number of women, often the first manifestation of a mood disorder that will occur later in life, bringing serious consequences for the patient and her offspring. Depression today is the leading cause of disability worldwide. The aim of this study was to evaluate the effectiveness of a preventive cognitive behavioral therapy (CBT) for PPD., Methods: Pre-post therapy study, as part of a population-based cohort study. Pregnant women without a diagnosis of depression participated, who were divided into two groups: risk of depression (CBT) and a control group (without therapy). The preventive therapy consisted of six sessions of CBT, administered weekly. The Outcome Questionnaire (OQ-45) was used in all sessions. The Mini International Neuropsychiatric Interview and Beck Depression Inventory-II were used on three occasions. The final statistical analyses were performed by Poisson regression., Results: The prevalence of PPD in the risk group was 5.5% and in the control group 2.2%, with no difference between the groups (PR 1.66 95% CI 0.44-6.18). The OQ-45 averages gradually reduced during the therapy sessions, indicating therapeutic progress. Schooling was an associated factor, both with the manifestation of PPD and with the greater effectiveness of the therapy., Limitations: Rate of 40.5% refusal to preventive treatment and absence of a group with similar characteristics in another therapy model., Conclusions: Brief cognitive behavioral therapy applied by mental health professionals with basic training was effective in preventing the manifestation of PPD., (Copyright © 2021 The Author(s). Published by Elsevier B.V. All rights reserved.)

Background: Hereditary spastic paraplegia presents spasticity as the main clinical manifestation, reducing gait quality and producing incapacity. Management with botulinum toxin type A (BoNT-A) is not well elucidated. The objective of the current study was to evaluate the efficacy and safety of BoNT-A in patients with hereditary spastic paraplegias., Methods: This was a double-blind, randomized, placebo-controlled crossover trial. Each participant was randomly assigned to receive 1 injection session of either BoNT-A (100 IU/2 mL of Prosigne in each adductor magnus and each triceps surae) or saline 0.9% (2 mL). The primary outcome measure was change from baseline in maximal gait velocity, and secondary outcome measures included changes in gait at self-selected velocity, spasticity, muscle strength, Spastic Paraplegia Rating Scale, pain, fatigue, and subjective perception of improvement. We also looked at adverse events reported by the patients., Results: We enrolled 55 patients, 36 of whom were men and 41 with the pure phenotype. Mean age was 43 ± 13.4 years (range, 19-72 years), mean age of onset waws 27 ± 13.1 years (range, <1 to 55 yars), and mean disease duration was 17 ± 12.7 years (range, 1-62 years). Compared with baseline, we did not find significant differences between groups in primary and secondary outcomes, except for reduction in adductor tone (P = 0.01). The adverse events were transient and tolerable, and their incidence did not significantly differ between treatments (P = 0.17)., Conclusions: BoNT-A was safe in patients with hereditary spastic paraplegias and reduced the adductor tone, but it was not able to produce functional improvement considering the doses, injection protocol, measures, and instruments used. © 2021 International Parkinson and Movement Disorder Society., (© 2021 International Parkinson and Movement Disorder Society.)

Blackberry is a species which has high potential to grow in an organic production system. However, there is scarce information on the behavior of cultivars and selections of this species conducted in an ecologically based system, a fact that makes it difficult to recommend cultivars for this production system. This study aimed to evaluate and characterize the phenology and productive aspects of six blackberry genotypes grown in an organic system, in Pelotas, Rio Grande do Sul (RS) state, Brazil. Evaluation of the following variables was carried out in the three first production cycles (2015-2016, 2016-2017 and 2017-2018): phenology, number of fruits.pl-¹, mean fruit yield.pl-¹, yield (kg.ha-¹), mean fruit mass (g) and soluble solid content (° Brix). Regarding phenology, genotype Black 112 was later than the others whereas Black 178 was the most precocious one. In the last year under evaluation, genotypes yielded around 10 ton.ha-¹, except Black 128 selection, which yielded approximately 6,767 ton.ha-¹. Genotypes Black 178, Black 112 and Black 145 and both cultivars BRS Xingu and Tupy exhibited potential to grow in an organic production system.

Pecan trees require adequate soil moisture conditions to produce nuts with good yield and quality. Irrigation should be an important considered practice in orchards management plan, mainly in regions with water deficit periods. The objective of this research was to evaluate irrigation on pecan nuts growth and yield. This study was conducted in Uruguay, during the fast development phase until the pecan nuts harvest. Three irrigation treatments were used: 0 L (control), 70 L and 140 L plant-1 of water every two days, applied via drip irrigation in seven-year-old pecan trees of the cultivar Success. The number of nuts per cluster and nuts' diameter and length were evaluated during nut growth. After harvesting, peeled nuts' width and length were measured, and after peeling, the kernel length, width, height and filling and the kernel and peel percentage were evaluated. According to the results, it was possible to identify that periods of water deficit during pecan nuts development affected the kernel filling stageand size. Irrigation in pecan plants provides greater dimensions and nuts mass. With the use of 140 L plant-1, there was an increase in the nut mass of more than 100% in relation to the nuts from plants without irrigation.

The aim of this study was to analyze, in vitro, the reduction of Enterococcus faecalis in root canals of primary teeth after final irrigation and photodynamic therapy (PDT) use. Twenty primary molars were contaminated with Enterococcus faecalis. The teeth were randomly distributed into four groups according to the irrigation solutions and PDT use: G1 (Saline solution and no PDT use), G2 (17 % EDTA and no PDT use), G3 (Saline solution and PDT use) and G4 (17 % EDTA and PDT use). For PDT, 0.005 % toluidine blue was chosen as the photosensitizer, which was inserted in the canals with sterile paper cones. Bacterial counts were performed with a BHI test in blood agar plate, where bacteria were collected inside the canal for 30 s using sterile paper cones. The collection took place before and after the irrigation and PDT protocols. The samples were diluted, spread onto a blood agar plate and then incubated at 37 °C for 24 h. There was a reduction of the microbiota from the irrigation solutions before and after the final irrigation for all groups. It was observed a statistically significant reduction (p < 0,05) when PDT was used (97.6 % at the saline solution and 89.8 % at the 17 % EDTA) when compared to the groups with no PDT use. Our data demonstrated that PDT, according to the parameters used, increased the disinfection performance of the solutions tested in the root canals of primary teeth., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Objective: To investigate whether tri-ponderal mass index and body mass index Z scores are equivalent for screening children and adolescents with insulin resistance., Methods: Cross-sectional study with 296 children and adolescents enrolled at public schools of Vitória, Espírito Santo, Brazil, aged eight to 14 years. The tri-ponderal mass index was calculated as the ratio between weight and height cubed. The body mass index was calculated as the ratio between weight and height squared. Insulin resistance was defined with the homeostatic model assessment (HOMA-IR)., Results: The HOMA-IR was higher in the 4th quartile of body mass index Z scores and tri-ponderal mass index compared to 1st and 2nd quartiles for both girls and boys. The areas under the age-adjusted receiver operating characteristic curves were similar between the indices for girls (body mass index Z scores=0.756; tri-ponderal mass index=0.763) and boys (body mass index Z scores=0.831; tri-ponderal mass index=0.843). In addition, according to the simple linear regression analyses estimations, both body mass index Z scores and tri-ponderal mass index explained a significant fraction of the homeostatic model assessment variability for girls (body mass index Z scores: R2=0.269; tri-ponderal mass index: R2=0.289; p<0.001) and boys (body mass index Z scores: R2=0.175; tri-ponderal mass index: R2=0.210; p<0.001)., Conclusions: The tri-ponderal mass index and body mass index Z scores were similar to discriminate children and adolescents with insulin resistance. It is noteworthy that the use of tri-ponderal mass index is clearly advantageous, because it can be calculated with no concerns on adjustments for the age, a fact that makes it very applicable in the clinical practice.

Cathepsin S (Cat S) is a protein expressed in some epithelial cells, which appears to be associated with cancer metastasis and recurrence. The abnormal expression of Cat S has been reported to be associated with the progression of certain types of gastrointestinal neoplasms, including gastric cancer (GC). There is a need to identify novel biomarkers and therapeutic targets associated with the growth, invasion and migration of GC cells, in order to develop non-invasive diagnostic and prognostic procedures and design new therapeutic approaches. The aim of the present study was to assess the association between Cat S and oncogenic processes implicated in the development of GC, focusing on the diagnostic and therapeutic potential of this molecule in GC. A search was conducted through the PubMed and Cochrane Central Register of Controlled Trials electronic databases for relevant literature published between 2003 and 2018, using the mesh terms 'cathepsin S' and 'cancer' and 'gastric cancer'., (Copyright: © da Costa et al.)

Cerebellar ataxias are a clinically heterogeneous group of neurological disorders. Besides the cerebellum, several forms of hereditary ataxias or non-genetic ataxias also affect other areas of the brain. Some forms of cerebellar ataxias may have cochlear and vestibular involvement and may present with deafness and symptoms or signs of vestibulopathy (dizziness, nystagmus and diplopia). Recognizing otoneurological symptoms in patients with cerebellar ataxias is mandatory, since these signs may guide a specific diagnosis, and clinicians may provide a suitable therapeutic approach. In this review, we describe and discuss the most common forms of cerebellar ataxias associated with deafness and vestibulopathy.

Sensory neuronopathies (SN) result from dorsal root ganglia damage and manifest with a combination of sensory deficits and proprioceptive ataxia. Characterization of the natural history and development of therapeutic trials are hampered by the lack of clinical scales that capture the whole spectrum of SN-related manifestations. We propose and validate a rating instrument for SN. Three experienced neuromuscular specialists developed items to rate SN. The resultant instrument was later validated by the assessment of the intra-class correlation coefficient, for inter-rater validity in 48 SN patients, and later in a smaller subset of 16 patients to assess its intra-rater validity. Standardized Crombach's alpha and Oblimin rotation analysis were performed to verify internal consistency and items' relationship, respectively. Evaluation of Sensory Ataxia Rating Scale (SEARS)'s external validity was performed by comparison to: scale for the assessment and rating of ataxia (SARA), Beck balance scale (BBS), and INCAT sensory sum score (ISS). A 10-item scale with an intra-class correlation coefficient >0.95 for intra- and inter-rating measurements with a good internal consistency (standardized Cronbach's alpha of 0.83) were observed. There was a normal distribution of the scores without a floor or ceiling effect. A moderate to good correlation between SEARS and SARA, BBS, and ISS was observed. SEARS is a reliable, easy-to-perform and consistent instrument to rate SN. Larger cohorts and multicenter studies are needed to validate its usefulness towards possible treatment trials., (© 2019 Peripheral Nerve Society.)

Methods: Sensory neuronopathies (SN) are a group of peripheral nerve disorders characterized by multifocal non-length-dependent sensory deficits and sensory ataxia. Its recognition is essential not only for proper management but also to guide the etiological investigation. The uncommon SN clinical picture and its rarity set the conditions for the misdiagnosis and the diagnostic delay, especially in non-paraneoplastic SN. Therefore, our objectives were to characterize the diagnostic odyssey for non-paraneoplastic SN patients, as well as to identify possible associated factors. We consecutively enrolled 48 non-paraneoplastic SN patients followed in a tertiary neuromuscular clinic at the University of Campinas (Brazil). All patients were instructed to retrieve their previous medical records, and we collected the data regarding demographics, disease onset, previous incorrect diagnoses made and the recommended treatments., Results: There were 34 women, with a mean age at the diagnosis of 45.9 ± 12.2 years, and 28/48 (58%) of the patients were idiopathic. Negative sensory symptoms were the heralding symptoms in 25/48 (52%); these were asymmetric in 36/48 (75%) and followed a chronic course in 35/48 (73%). On average, it took 5.4 ± 5.3 years for SN to be diagnosed; patients had an average of 3.4 ± 1.5 incorrect diagnoses. A disease onset before the age of 40 was associated to shorter diagnosis delay (3.7 ± 3.4 vs. 7.8 ± 6.7 years, p = 0.01)., Conclusions: These results suggest that diagnostic delay and misdiagnosis are frequent in non-paraneoplastic SN patients. As in other rare conditions, increased awareness in all the healthcare system levels is paramount to ensure accurate diagnosis and to improve care of these patients.

Objective: Given the importance of incorporating simple and low-cost tools into the pediatric clinical setting to provide screening for insulin resistance, the present study sought to investigate whether waist-to-height ratio is comparable to biochemical markers for the discrimination of insulin resistance in children and adolescents., Methods: This cross-sectional study involved students from nine public schools. In total, 296 children and adolescents of both sexes, aged 8-14 years, composed the sample. Waist-to-height ratio, triglycerides/glucose index, and triglycerides-to-HDL-C ratio were determined according to standard protocols. Insulin resistance was defined as homeostatic model assessment for insulin resistance with cut-off point ≥ 3.16., Results: Age, body mass index, frequency of overweight, waist circumference, waist-to-height ratio, insulin, glucose, homeostatic model assessment for insulin resistance, triglycerides, triglycerides/glucose index, and triglycerides-to-HDL-C were higher among insulin resistant boys and girls. Moderate correlation of all indicators (waist-to-height ratio, triglycerides/glucose index, and triglycerides-to-HDL-C ratio) with homeostatic model assessment for insulin resistance was observed for both sexes. The areas under the receiver operational characteristic curves ware similar between waist-to-height ratio and biochemical markers., Conclusion: The indicators provided similar discriminatory power for insulin resistance. However, taking into account the cost-benefit ratio, we suggest that waist-to-height ratio may be a useful tool to provide screening for insulin resistance in pediatric populations., (Copyright © 2018 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Background: We aimed to compare the accuracy of the ponderal index (PI) vs. BMI-for-age z-scores transformed (BMIz) in estimating body fat levels and classifying obesity in children and adolescents from a Brazilian urban population., Methods: This is a cross-sectional study with 1149 participants (53.2% male), aged 6 to 18 years. Body fat percent (BFP) was obtained by multi-frequency bioelectrical impedance. Non-linear regression analysis provided the accuracy of both BMIz and PI in estimating BFP. False positive rate was obtained from the proportion of individuals placed at or above the 95 th percentile for BMIz or PI, whereas their BFP was discordantly below the 95 th percentile., Results: PI and BMIz appeared with similar stability from childhood to adolescence for both boys and girls. The portion of the variability in BFP explained by BMIz (R 2  = 0.74 and R 2  = 0.75) was close to the variability in BFP explained by PI (R 2  = 0.73 and R 2  = 0.75) for boys and girls, respectively. False positive rate was higher for BMIz compared with PI among boys (21.8% vs. 3.9%) and girls (28.5% vs. 17.5%)., Conclusions: PI is a promising index for replacing BMIz in children and adolescents due to its potential to reduce false diagnosis of obesity.

Background: Sedentary behavior (SB) is related to unhealthy outcomes and is performed in many contexts, including school. The aim of this study was to identify sociodemographic, biological, and psychosocial correlates of SB performed at school in a sample of adolescents., Methods: Adolescents provided information regarding sex, age, socioeconomic status, and psychosocial variables related to physical activity (self-efficacy, attitudes, perception of school environment, peer and parental support). Body fat was estimated using skinfolds, and maximum aerobic capacity with a 20 m shuttle run. Adolescents wore accelerometers and SB performed during class time, physical education (PE) classes, and recesses was estimated., Results: Adolescents (N = 567, 53% female, 12.9 ± 5.3 years old) spent 67% of class time, 41% of PE classes, and 41% of recesses in SB. Girls spent more time in SB, and age was negatively associated with SB. Body fat was positively associated with SB in class time and PE classes. Peer support for physical activity was negatively associated with SB in class time, whereas attitudes related to physical activity were negatively associated with SB during PE classes and recesses., Conclusion: Adolescents spend long periods in SB at school, even during opportunities to be active like PE classes and recesses., (© 2019, American School Health Association.)

Objective: Our study evaluated the performance of different two-chambered microbial fuel cell (MFC) prototypes, operated with variable distance between electrodes and Nafion membrane and specific inoculum concentration, applied for vinasse treatment., Results: The performance of the developed MFC resulted in a maximum current density of 1200 mA m -2 and power density of 800 mW m -2 in a period of 61 days. MFC performed a chemical oxygen demand removal at a rate ranging from 51 to 60%., Conclusions: Taking our preliminary results into consideration, we concluded that the MFC technology presents itself as highly promising for the treatment of vinasse.

Microfabrication technologies have been widely explored to produce microgels that can be assembled in functional constructs for tissue engineering and regenerative medicine applications. Here, we propose microfluidics coupled to a source of UV light to produce multifunctional methacrylated laminarin microparticles with narrow distribution of sizes using photopolymerization. The multifunctional microparticles were loaded with platelet lysates and further conjugated with an adhesive peptide. The adhesive peptides dictated cell adhesiveness to the laminarin microparticles, the incorporation of platelet lysates have resulted in improved cell expansion compared to clear microparticles. Overall, our findings demonstrate that multifunctional methacrylated laminarin microparticles provide an effective support for cell attachment and expansion. Moreover, expanded cells provide the link for microparticles aggregation resulting in robust 3D structures. This suggest the potential for using the methacrylated laminarin microplatforms capable to be assembled by the action of cells to rapidly produce large tissue engineered constructs., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background: Molecular imaging has proven to be a powerful tool to elucidate degenerated paths in a wide variety of neurological diseases and has not been systematically studied in hereditary spastic paraplegias., Objectives: To investigate dopaminergic degeneration in a cohort of 22 patients with hereditary spastic paraplegia attributed to SPG11 mutations and evaluate treatment response to l-dopa., Methods: Patients and controls underwent single-photon emission computed tomography imaging utilizing 99m Tc-TRODAT-1 tracer. A single-blind trial with 600 mg of l-dopa was performed comparing UPDRS scores., Results: Reduced dopamine transporter density was universal among patients. Nigral degeneration was symmetrical and correlated with disease duration and motor and cognitive handicap. No statistically significant benefit could be demonstrated with l-dopa intake during the trial., Conclusion: Disruption of presynaptic dopaminergic pathways is a widespread phenomenon in patients with SPG11 mutations, even in the absence of parkinsonism. Unresponsiveness to treatment could be related to postsynaptic damage that needs to be further investigated., (© 2018 International Parkinson and Movement Disorder Society.)

To identify the predictors of dropout in the 'Mexa-se' intervention according to the body mass index (BMI) category. This was a controlled, non-randomized study. The intervention included: (i) increase in the intensity of physical activities (PA) in physical education (PE) classes; (ii) active recess; (iii) educational sessions on PA, nutrition and body image; and (iv) educational materials. Dropout was considered when students dropped out of intervention, or did not reach 75% attendance in PE classes. The independent variables were gender, age, study period, socioeconomic status, BMI, PA, screen time, food consumption, health perception, attitudes toward PA, self-efficacy for PA, perception of the school environment, body image and self-esteem. Binary logistic regression analysis was used. The dropout rate was 26.8%. In the total sample and among students with an adequate BMI, there was a greater probability of dropout with an increase in age. For overweight students, increased age and socioeconomic status, and studying in the afternoon period were predictors of dropout from the intervention. Socio-demographic factors were predictors of dropout from the 'Mexa-se' intervention; the associated factors differed based on the BMI category.

SPG11 mutations are the major cause of autosomal recessive Hereditary Spastic Paraplegia. The disease has a wide phenotypic variability indicating many regions of the nervous system besides the corticospinal tract are affected. Despite this, anatomical and phenotypic characterization is restricted. In the present study, we investigate the anatomical abnormalities related to SPG11 mutations and how they relate to clinical and cognitive measures. Moreover, we aim to depict how the disease course influences the regions affected, unraveling different susceptibility of specific neuronal populations. We performed clinical and paraclinical studies encompassing neuropsychological, neuroimaging, and neurophysiological tools in a cohort of twenty-five patients and age matched controls. We assessed cortical thickness (FreeSurfer software), deep grey matter volumes (T1-MultiAtlas tool), white matter microstructural damage (DTI-MultiAtlas) and spinal cord morphometry (Spineseg software) on a 3 T MRI scan. Mean age and disease duration were 29 and 13.2 years respectively. Sixty-four percent of the patients were wheelchair bound while 84% were demented. We were able to unfold a diffuse pattern of white matter integrity loss as well as basal ganglia and spinal cord atrophy. Such findings contrasted with a restricted pattern of cortical thinning (motor, limbic and parietal cortices). Electromyography revealed motor neuronopathy affecting 96% of the probands. Correlations with disease duration pointed towards a progressive degeneration of multiple grey matter structures and spinal cord, but not of the white matter. SPG11 -related hereditary spastic paraplegia is characterized by selective neuronal vulnerability, in which a precocious and widespread white matter involvement is later followed by a restricted but clearly progressive grey matter degeneration.

Neurological phenotypes of human T cell lymphotropic virus type I (HTLV-1) are numerous and rarely may not manifest the classic HTLV-associated myelopathy/tropical spastic paraparesis (HAM/TSP). We report a case of HTLV-1-related sensory neuronopathy heralding the classic HAM/TSP.

The cranial nerves IX and X emerge from medulla oblongata and have motor, sensory, and parasympathetic functions. Some of these are amenable to neurophysiological assessment. It is often hard to separate the individual contribution of each nerve; in fact, some of the techniques are indeed a composite functional measure of both nerves. The main methods are the evaluation of the swallowing function (combined IX and X), laryngeal electromyogram (predominant motor vagal function), and heart rate variability (predominant parasympathetic vagal function). This review describes, therefore, the techniques that best evaluate the major symptoms presented in IX and X cranial nerve disturbance: dysphagia, dysphonia, and autonomic parasympathetic dysfunction.

Background and Objectives: Colonic diverticulosis (CD) is related to advanced age and a lack of dietary fiber. Recently, several studies have shown that metabolic syndrome (MS) is also implicated in the etiopathogenesis of CD. This study aimed to assess the association between MS, obesity and CD., Methods: This was a prospective study of a one-year duration. The MS was defined according to the National Cholesterol Education Program-Adult Treatment Panel III (NCEP-ATP III). Demographic data, risk factors for MS and endoscopic findings of patients who underwent a total colonoscopy in the department were collected. Obesity was defined as a body mass index ≥ 30 kg/m2. Informed consent was obtained. The local Ethics Committee and National Data Protection Committee approved the study. Statistical analysis was performed with SPSS 21 and statistical significance was defined as p < 0.05., Results: The study included 203 patients, 95 males with a mean age of 65.5 years. CD was diagnosed in 30.5% of patients. Univariate analysis showed that age, hypertension, increased waist circumference and hyperlipidemia were associated with colonic diverticulosis. There was no association with gender, obesity or type 2 diabetes mellitus. Multivariate analysis showed that age and a greater waist circumference increased the risk of diverticulosis. Age-adjusted analysis showed that MS was associated with diverticulosis. The prevalence of adenoma in patients with CD was similar to that in patients without CD., Conclusion: In this series, MS was significantly associated with CD. The identification of risk groups is important since diverticulosis can have serious and potentially fatal complications. To our knowledge, this is the first Southern European prospective study evaluating the association between MS and CD.

Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant disorder caused by a CAG repeat expansion, characterized by progressive cerebellar ataxia and pyramidal signs. Non-motor and extracerebellar symptoms may occur. MRI-based studies in SCA1 focused in the cerebellum and connections, but there are no data about cord damage in the disease and its clinical relevance. To evaluate in vivo spinal cord damage in SCA1, a group of 31 patients with SCA1 and 31 age- and gender-matched healthy controls underwent MRI on a 3T scanner. We used T1-weighted 3D images to estimate the cervical spinal cord area (CA) and eccentricity (CE) at three C2/C3 levels based on a semi-automatic image segmentation protocol. The scale for assessment and rating of ataxia (SARA) was used to quantify disease severity. The groups were significantly different regarding CA (47.26 ± 7.4 vs. 68.8 ± 5.7 mm2, p < 0.001) and CE values (0.803 ± 0.044 vs. 0.774 ± 0.043, p < 0.05). Furthermore, in the patient group, CA presented significant correlation with SARA scores (R = -0.633, p < 0.001) and CAGn expansion (R = -0.658, p < 0.001). CE was not associated with SARA scores (p = 0.431). In the multiple variable regression, CA was strongly associated with disease duration (coefficient -0.360, p < 0.05) and CAGn expansion (coefficient -1.124, p < 0.001). SCA1 is characterized by cervical cord atrophy and anteroposterior flattening. Morphometric analyses of the spinal cord MRI might be a useful biomarker in the disease.

Primary intestinal lymphangiectasia or Waldmann's disease is an uncommon cause of protein losing enteropathy with an unknown etiology and is usually diagnosed during childhood. It is characterized by dilation and leakage of intestinal lymph vessels leading to hypoalbuminemia, hypogammaglobulinemia and lymphopenia. Differential diagnosis should include erosive and non-erosive gastrointestinal disorders, conditions involving mesenteric lymphatic obstruction and cardiovascular disorders that increase central venous pressure. Since there are no accurate serological or radiological available tests, enteroscopy with histopathological examination based on intestinal biopsy specimens is currently the gold standard diagnostic modality of intestinal lymphangiectasia. We report a rare case of a primary intestinal lymphangiectasia in a 60-year-old Caucasian female who presented with asymptomatic hypoalbuminemia and hypogammaglobulinemia. After the diagnosis of a protein losing enteropathy, the patient underwent an enteroscopy and biopsies were taken, whose histological examination confirmed dilated intestinal lymphatics with broadened villi of the small bowel. Secondary causes of intestinal lymphangiectasia were excluded and the diagnosis of Waldmann's disease was recorded. The patient was put on a high-protein and low-fat diet with medium-chain triglyceride supplementation with improvement.

Inflammatory fibroid polyps (Vanek's tumor) are rare, accounting for 0.1% of all gastric polyps. This letter describes an unusual case of an intermittent gastric outlet obstruction by a large Vanek's tumor.

The authors present an historical review about the main contributions of Professor Derek Denny-Brown to neurology. Some of his achievements include the first description of sensory neuronopathies, and some of the essential textbooks on the function and anatomy of the basal ganglia. In 2016, on the 35th anniversary of his death, modern neurologists are still strongly influenced by his legacy.

Friedreich's ataxia (FDRA) is the most common inherited ataxia worldwide, caused by homozygous GAA expansions in the FXN gene. Patients usually have early onset ataxia, areflexia, Babinski sign, scoliosis and pes cavus, but at least 25 % of cases have atypical phenotypes. Disease begins after the age of 25 in occasional patients (late-onset Friedreich ataxia (LOFA)). Little is known about the frequency and clinical profile of LOFA patients. One hundred six patients with molecular confirmation of FDRA and followed in three Brazilian outpatient centers were enrolled. General demographics, GAA expansion size, age at onset, cardiac, endocrine, and skeletal manifestations were evaluated and compared between LOFA and classic FDRA (cFDRA) groups. We used Mann-Whitney and Fisher tests to compare means and proportions between groups; p values <0.05 were considered significant. LOFA accounted for 17 % (18/106) and cFDRA for 83 % (88/106) of the patients. There were 13 and 48 women in each group, respectively. LOFA patients were significantly older and had smaller GAA expansions. Clinically, LOFA group had a tendency toward lower frequency of diabetes/impaired glucose tolerance (5.8 vs. 17 %, p = 0.29) and cardiomyopathy (16.6 vs. 28.4 %, p = 0.38). Skeletal abnormalities were significantly less frequent in LOFA (scoliosis 22 vs. 61 %, p = 0.003, and pes cavus 22 vs.75 %, p < 0.001) as were spasticity and sustained reflexes, found in 22 % of LOFA patients but in none of the cFDRA patients (p = 0.001). LOFA accounts for 17 % of Brazilian FDRA patients evaluated herein. Clinically, orthopedic features and spasticity with retained reflexes are helpful tips to differentiate LOFA from cFDRA patients.

Purpose of Review: This article provides a description on clinical features and pathophysiology of the main sleep disorders observed in Machado-Joseph disease (MJD)., Recent Findings: Pathological studies have clearly demonstrated that degenerative process in MJD is widespread in the nervous system, and not restricted to the cerebellum. Nonmotor manifestations are frequent and may include pain, cramps, dysautonomia, cognitive deficits, psychiatric manifestations, olfactory deficits, fatigue, nutritional issues, and sleep disorders., Summary: Sleep disorders are frequent in MJD, and include restless legs syndrome, rapid eye movement sleep behavior disorder, excessive daytime sleepiness, insomnia, sleep apnea, periodic limb movements during sleep, parasomnia, and others. Pathophysiological mechanisms related to sleep disorders in Machado-Joseph are complex and poorly understood. Considering that sleep complaints are a treatable condition, recognizing sleep disorders in MJD is relevant.

Background: The outcome of Chagas disease patients after receiving implantable cardioverter defibrillator (ICD) is still controversial., Objective: To compare clinical outcomes after ICD implantation in patients with chronic Chagas cardiomyopathy (CCC) and ischemic heart disease (IHD)., Methods: Prospective study of a population of 153 patients receiving ICD (65 with CCC and 88 with IHD). The devices were implanted between 2003 and 2011. Survival rates and event-free survival were compared., Results: The groups were similar regarding sex, functional class and ejection fraction. Ischemic patients were, on average, 10 years older than CCC patients (p < 0.05). Patients with CCC had lower schooling and monthly income than IHD patients (p < 0.05). The number of appropriate therapies was 2.07 higher in CCC patients, who had a greater incidence of appropriate shock (p < 0.05). Annual mortality rate and electrical storm incidence were similar in both groups. There was no sudden death in CCC patients, and only one in IHD patients. Neither survival time (p = 0.720) nor event-free survival (p = 0.143) significantly differed between the groups., Conclusion: CCC doubles the risk of receiving appropriate therapies as compared to IHD, showing the greater complexity of arrhythmias in Chagas patients., Competing Interests: Potential Conflict of Interest No potential conflict of interest relevant to this article was reported.

Objectives: To analyze and correlate the clinical and radiographic results from patients with distal radius fractures who underwent surgical treatment with a fixed-angle volar locked plate., Methods: Sixty-four patients with distal radius fractures were evaluated. They all underwent surgical treatment with a volar locked plate for the distal radius, with a minimum of six months of postoperative follow-up. They underwent a physical examination that measured range of motion and grip strength, answered the Disabilities of the Arm, Shoulder, and Hand (DASH) questionnaire and underwent radiographic examination., Results: In the physical examination on the patients, all the range-of-motion measurements were reduced. Grip strength measured in kgf was on average 85.8% of the strength on the unaffected side. The mean DASH score was 15.99. A significant relationship was found between lower DASH scores and losses of extension and grip strength. On the radiographs, the mean values in relation to the unfractured side were 84.0% for radial inclination, 85.4% for radial length and 86.8% for volar deviation of the radius. Loss of radial length was correlated with losses of extension and grip strength.

Background: Cardiac resynchronization therapy (CRT) is the recommended treatment by leading global guidelines. However, 30%-40% of selected patients are non-responders., Objective: To develop an echocardiographic model to predict cardiac death or transplantation (Tx) 1 year after CRT., Method: Observational, prospective study, with the inclusion of 116 patients, aged 64.89 ± 11.18 years, 69.8% male, 68,1% in NYHA FC III and 31,9% in FC IV, 71.55% with left bundle-branch block, and median ejection fraction (EF) of 29%. Evaluations were made in the pre‑implantation period and 6-12 months after that, and correlated with cardiac mortality/Tx at the end of follow-up. Cox and logistic regression analyses were performed with ROC and Kaplan-Meier curves. The model was internally validated by bootstrapping., Results: There were 29 (25%) deaths/Tx during follow-up of 34.09 ± 17.9 months. Cardiac mortality/Tx was 16.3%. In the multivariate Cox model, EF < 30%, grade III/IV diastolic dysfunction and grade III mitral regurgitation at 6‑12 months were independently related to increased cardiac mortality or Tx, with hazard ratios of 3.1, 4.63 and 7.11, respectively. The area under the ROC curve was 0.78., Conclusion: EF lower than 30%, severe diastolic dysfunction and severe mitral regurgitation indicate poor prognosis 1 year after CRT. The combination of two of those variables indicate the need for other treatment options.

Background: 30-40% of cardiac resynchronization therapy cases do not achieve favorable outcomes., Objective: This study aimed to develop predictive models for the combined endpoint of cardiac death and transplantation (Tx) at different stages of cardiac resynchronization therapy (CRT)., Methods: Prospective observational study of 116 patients aged 64.8 ± 11.1 years, 68.1% of whom had functional class (FC) III and 31.9% had ambulatory class IV. Clinical, electrocardiographic and echocardiographic variables were assessed by using Cox regression and Kaplan-Meier curves., Results: The cardiac mortality/Tx rate was 16.3% during the follow-up period of 34.0 ± 17.9 months. Prior to implantation, right ventricular dysfunction (RVD), ejection fraction < 25% and use of high doses of diuretics (HDD) increased the risk of cardiac death and Tx by 3.9-, 4.8-, and 5.9-fold, respectively. In the first year after CRT, RVD, HDD and hospitalization due to congestive heart failure increased the risk of death at hazard ratios of 3.5, 5.3, and 12.5, respectively. In the second year after CRT, RVD and FC III/IV were significant risk factors of mortality in the multivariate Cox model. The accuracy rates of the models were 84.6% at preimplantation, 93% in the first year after CRT, and 90.5% in the second year after CRT. The models were validated by bootstrapping., Conclusion: We developed predictive models of cardiac death and Tx at different stages of CRT based on the analysis of simple and easily obtainable clinical and echocardiographic variables. The models showed good accuracy and adjustment, were validated internally, and are useful in the selection, monitoring and counseling of patients indicated for CRT.