Damage on DNA and hematological parameters of two bat species due to heavy metal exposure in a nickel-mining area in central Brazil.

Exposure to heavy metals in mining zones is a significant threat, which can affect ecosystem services and contribute to the decline of wild bat populations. The present study investigated the impacts caused by mining on two bat species in central Brazil, the nectarivorous Glossophaga soricina and the frugivorous Carollia perspicillata. The bats were collected from a nickel-mining zone (treatment) and a protected area (control). The leukocyte profile of each species was compiled and genotoxicity (comet assay) and mutagenicity (micronucleus test) were determined using the appropriate procedures. Glossophaga soricina presented significantly higher frequencies of eosinophils and monocytes in the mining zone in comparison with the protected area, whereas C. perspicillata presented higher frequencies of lymphocytes in the mining zone, but significantly lower frequencies of monocytes. Concomitantly, G. soricina also presented a higher frequency of DNA damage, although no variation was found in this parameter in C. perspicillata when comparing environments. We also found no significant differences between populations in terms of the frequency of micronuclei and other nuclear abnormalities. Overall, the results of the study indicate that bats are susceptible to immunological disorders and DNA damage in mining zones, with the nectarivorous G. soricina appearing to be relatively more susceptible and thus a potentially effective bioindicator of the impact of contamination in these environments.

The fate of spinal arteries after the stent-assisted balloon-induced intimal disruption and relamination in aortic dissection repair technique: a case series.

Objective: We evaluated the patency of the spinal arteries (intercostal and lumbar) after the STABILISE (stent-assisted balloon-induced intimal disruption and relamination in aortic dissection repair) technique. Methods: A retrospective analysis of all patients with aortic dissection treated with the STABILISE technique between April 2018 and July 2021 was performed. Imaging analysis of the spinal cord vascular supply was accomplished using multiplanar and maximum intensity projection reconstructed images of pre- and postoperative computed tomography angiograms at 1 month, 12 months, and annually thereafter. Results: Twelve patients were treated for complicated aortic dissection. Primary technical success was 100% and mid-term clinical success, at a mean follow-up of 27 ± 12 months, was 90%. No cases of spinal cord ischemia were identified. One patient died after 1 year (non-aortic related), and one patient was lost to follow-up. A significant decrease was found in the mean number of patent spinal arteries in the stent graft area at 1 month (P < .001), 1 year (P < .001), and 2 years (P = .004). However, no significant reduction was found in the number of spinal arteries in either the bare metal stented or nonstented aorta (P > .05). Conclusions: Use of the STABILISE technique decreased intercostal artery patency in the thoracic stent graft area, but spinal artery patency was not significantly affected by the bare metal stent nor its aggressive ballooning. These findings constitute a step toward a better understanding of the safety of this technique.

Current perspectives in acute type B aortic dissections: a literature review.

In this new millennial, endovascular strategies have revolutionized the treatment of acute type B aortic dissection (aTBAD). With reduced in-hospital mortality and good long-term outcomes TEVAR has become the gold standard for the treatment of complicated dissection and is gaining increasing support for its preventive applicability in some uncomplicated dissections. With this new paradigm came a shift of the treatment goal where just covering the entry tear is not enough and instead achieving long-term positive thoracoabdominal remodeling is needed. More extensive approaches with composite device designs (covered stent graft and bare metal stent) emerged to answer this aortic conundrum. At 5-year of follow-up, "Provisional ExTension To Induce COmplete Attachment technique" (PETTICOAT) and its evolution "Stent assisted balloon induced intimal disruption and relamination in aortic dissection repair" (STABILISE) seem to be safe techniques that can allow, when anatomically feasible, excellent aortic remodeling and, in some cases, even the healing of the dissection. Nevertheless, STABILISE results, although promising, are mostly based on small series and therefore need to be validated by analyzing medium-long-term results from the international registry. Given the plethora of new data and the disparity of expert opinions on the best treatment to adopt, in this review we aim to summarize the current knowledge on the results of these different strategies for acute TBAD.

The evolution of management of type B aortic dissection in a series of 100 consecutive cases in a tertiary center.

INTRODUCTION AND OBJECTIVES: Management of aortic dissection is rapidly evolving. The present study aims to assess paradigm shifts in type B aortic dissection (TBAD) treatment modalities and their outcomes according to clinical presentation and type of treatment. We also aim to assess the impact of endovascular technology in TBAD management in order to define organizational strategies to provide an integrated cardiovascular approach. METHODS: We performed a retrospective review with descriptive analysis of the last 100 consecutive patients with TBAD admitted to the Vascular Surgery Department of Centro Hospitalar Universitário Lisboa Norte over a 16-year period. Results were stratified according to treatment modality and stage of the disease. The study was further divided into two time periods, 2003-2010 and 2011-2019, respectively before and after the introduction of a dedicated endovascular program for aortic dissections. RESULTS: A total of 100 patients (83% male; mean age 60 years) were included, of whom 59 were admitted in the acute stage (50.8% with complicated dissections). The other 41 patients were admitted for chronic dissections, most of them for surgical treatment of aneurysmal degeneration. Temporal analysis demonstrated an increase in the number of patients operated for aortic dissection, mainly due to an increase in chronic patients (33.3% in 2003-2010 vs. 64.4% in 2011-2019) and a clear shift toward endovascular treatment from 2015 onward. Overall in-hospital mortality was 14% and was significantly higher in the chronic phase (acute 5.1% vs. chronic 26.8%; OR 5.30, 95% CI 1.71-16.39; p=0.003) and in patients with aneurysmal degeneration, regardless of the temporal phase. Only one death was recorded in the endovascular group. CONCLUSION: Management of TABD carried an overall mortality of 14% during a 16-year period, but the appropriate use of endovascular technology has substantially reduced in-hospital mortality.

The Impact of the Proctor Assistance for a Safe Learning Curve in the Development of a Complex Aortic Endovascular Program.

INTRODUCTION: Initiating an endovascular aortic program for treatment of complex aortic aneurysms with fenestrated and branched grafts (FB-EVAR) is challenging. Using a Proctor is one option for training and development of the team. However, this approach has not been formally analyzed. The aim of this study was to analyze the learning curve and the effect of the Proctor regarding safety and effectiveness in FB-EVAR. METHODS: A single-center retrospective cohort study was performed, including all consecutive elective patients submitted to FB-EVAR (including both thoraco-abdominal-TAAA and complex abdominal aortic aneurysms-C-AAA) from 2013 to 2021. Patients were divided into 2 groups, the first operated with the Proctor present and the second without. Primary outcomes were 30-day mortality (safety) and technical and procedure success (efficacy). Secondary outcomes included treatment performance (procedure time, blood loss, contrast, and radiation use), re-interventions, aneurysm shrinking, target vessel patency, 30-day mortality, aneurysm-related mortality, and overall mortality. RESULTS: Overall, 105 patients were included in the study, 35 operated with Proctor and 70 operated without. The first 20 patients were operated always with the Proctor, and the remaining were operated with the Proctor selectively. Mean age was 71.8 (±7.3) years and 95 patients were male (90.5%). Overall, 62 (65%) patients had C-AAA or extent IV TAAAs and 43 (35%) had extensive TAAAs. There were no significant differences regarding 30-day mortality (Log Rank=0.99), technical success (p=0.4), or procedure success (p=0.8). Mean surgical time was longer in the non-Proctor group (p=0.005), as well as significant intra-operative blood loss (p=0.042). Contrast use (p=0.5) and radiation (p=0.53) were non-significantly different between groups. There were no significant differences regarding length of stay (p=0.4), major adverse events (p=0.6), target vessel patency (Log Rank=0.97), early (p=0.7) and late endoleaks (0.7), aneurysm shrinking (p=0.6), re-interventions (p=0.2), and overall mortality (Log Rank=0.87). CONCLUSION: In our experience, the use of a Proctor to start and accompany our complex endovascular aortic program for FB-EVAR was both safe and effective and may serve as a template by other countries and centers that aim to developing their programs.

A real-world analysis of anti-atherosclerotic medical treatment and risk factor control in a cohort of vascular surgery patients.

INTRODUCTION: The aim of this study was to assess the pattern of anti-atherosclerosis medicines in patients admitted to a vascular surgery department, the effective control of the target values and its subsequent modification by the vascular surgery team. MATERIAL AND METHODS: A retrospective single-center cohort study of prospectively collected data was performed between May 2017 and May 2018 in a tertiary center. The STROBE guidelines were followed. All patients undergoing a primary elective surgery for carotid disease, aortic aneurysm and peripheral arterial disease were included. 'Best medical treatment' was defined as treatment with both anti-thrombotic and lipid-lowering treatment and, when appropriate, antihypertensive and anti-diabetic drugs. Both baseline and post-discharge best medical treatment were recorded. Blood work-up was performed at admission and 'adequately controlled patient' was defined if all blood test values were in agreement with the guidelines. RESULTS: A total of 279 patients (78% male; mean age 69 years-old) were included. Optimal medical therapy was registered in 58.8% upon admission but improved to 73.8% (95% CI, 2.197 - 7.781; p < 0.001) after discharge. At baseline, a total of 65.4% of patients were on lipid-lowering agents and of these, only 37% had LDL-C values within the targets. Likewise, only 34.6% of the 78 patients with diabetes had glycated hemoglobin within the normal range. Additionally, 8.5% of the remaining cohort had undiagnosed diabetes. CONCLUSION: In our current practice, only 75% of the patients receive best medical treatment. Although the admission in a Vascular Surgery department was an opportunity to optimize medical therapy, treatment remains suboptimal in one-quarter of patients. Further efforts should be carried out to alert vascular surgeons to this problem and to find future multidisciplinary solutions that can improve the cardiovascular risk profiles of these patients.

Introdução: Este estudo pretendeu avaliar o padrão de tratamento médico antiaterosclerótico em doentes internados num serviço de cirurgia vascular, o controlo efetivo dos valores-alvo e a sua posterior modificação pela equipa vascular. Material e Métodos: Foi realizado um estudo de coorte retrospetivo com dados coletados prospectivamente entre maio de 2017 e maio de 2018 num centro terciário. Foram seguidas as guidelines da STROBE e incluídos todos os doentes submetidos a cirurgias primárias eletivas para correção de doença carotídea, aneurisma de aorta e doença arterial periférica. Definiu-se como 'tratamento médico otimizado' o tratamento com fármacos anti-trombóticos e hipolipemiantes e, quando apropriado, com agentes anti-hipertensivos e antidiabéticos. Foi registado tratamento médico otimizado à entrada bem como após a alta. À admissão foi igualmente realizado um controlo analítico e os doentes foram classificados como 'adequadamente controlados' se todos os valores analíticos estivessem de acordo com as normas de orientação clínica. Resultados: Foram incluídos 279 pacientes (78% homens; idade média de 69 anos). O tratamento médico otimizado foi registado em 58,8% à data de admissão, tendo melhorado para 73,8% (IC 95%, 2,197 ­ 7,781; p < 0,001) após alta da enfermaria vascular. No início do estudo, 65,4% dos doentes estavam sob agentes hipolipemiantes e, destes, apenas 37% tinham valores de LDL-C dentro dos valores-alvo estabelecidos pelas normas de orientação clínica. Da forma semelhante, apenas 34,6% dos 78 doentes com diabetes tinham hemoglobina glicada dentro da normalidade. Da restante coorte, 8,5% tinha diabetes não diagnosticada. Conclusão: Na nossa prática atual apenas 75% dos pacientes seguem o tratamento médico otimizado. Apesar do internamento num serviço de Cirurgia Vascular ser uma oportunidade única para otimizar o tratamento médico, este permanece abaixo do ideal em cerca de um quarto dos doentes. Devem ser realizados esforços adicionais no sentido de alertar os cirurgiões vasculares para esse problema e encontrar soluções multidisciplinares futuras que permitam melhorar o perfil de risco cardiovascular destes doentes.

Incidence and Prevalence of Thoracic Aortic Aneurysms: A Systematic Review and Meta-analysis of Population-Based Studies.

Thoracic aortic aneurysms (TAA) may grow asymptomatically until they rupture, with a mortality over 90%. The true incidence and prevalence of this condition is uncertain and epidemiologic data is scarce, understudied and dispersed. Therefore, we aimed to conduct a systematic review and meta-analysis of the incidence and prevalence of TAAs in population-based studies. We searched MEDLINE, EMBASE and CENTRAL from inception to October 2020 for all population-based studies reporting on incidence and/or prevalence of TAAs. Data were pooled using a random effects model. The main outcome was the overall available worldwide incidence and prevalence of TAAs. The secondary outcomes were to evaluate the incidence of ruptured TAAs, differences in the location of these aneurysms (either ascending, arch or descending aorta) and differences in prevalence/incidence across different study designs. Twenty-two studies were included in the review and meta-analysis. The pooled incidence and prevalence of TAAs was 5.3 per 100,000 individuals/year (95% confidence interval [CI]: 3.0; 8.3) and 0.16% (95% CI: 0.12; 0.20), respectively. The pooled incidence of ruptured aneurysms was 1.6 per 100,000 individuals/year (95% CI: 1.3; 2.1). We found a significant difference of the prevalence in autopsy-only studies, which was 0.76% (95% CI: 0.47; 1.13) and the prevalence of TAAs dropped down to 0.07% (95% CI: 0.05;0.11) when these studies were excluded from the overall analysis. The current epidemiologic information provided serve as a base for future public-health decisions. The lack of well-design population-base studies and the limitations encountered serve as calling for future research in this field.

A systematic review and meta-analysis of the incidence of acute aortic dissections in population-based studies.

OBJECTIVE: To perform a systematic review and meta-analysis of all population-based studies reporting on incidence of acute aortic dissections (AADs). METHODS: We searched the MEDLINE, EMBASE, CENTRAL, and Open Grey databases from inception to August 2020 for population-based studies reporting on the incidence of AAD. A systematic review was conducted following the PRISMA guidelines using a registered protocol (CRD42020204007). Data were pooled using a random effects model of proportions using Freeman-Tukey double arcsine transformation. The main outcome was the incidence of AAD. Secondary outcomes were incidence type A aortic dissections (TAAD) and type B aortic dissections (TBAD), the incidence of aortic dissection repair and medical management, and the incidence of in-hospital mortality. In addition, we estimated the proportion of aortic dissection repair and mortality (in hospital, overall and specific mortality according to subtype) among patients with AAD. RESULTS: Thirty-three studies were included. The pooled incidence of AADs was 4.8 per 100,000 individuals/year (95% confidence interval [CI], 3.6-6.1). The incidence of TAAD was 3.0 per 100,000/year (95% CI, 1.8-4.4) and the incidence of TBAD was 1.6 per 100,000/year (95% CI, 1.1-2.2). The incidence of AAD needing repair was 1.4 per 100,000/year (95% CI, 1.0-2.0) (or 1.4 [95% CI, 1.2-1.7] for TAAD and 0.4 [95% CI, 0.2-0.7] for TBAD). The incidence of medically managed AAD was 3.4 per 100,000/year (95% CI, 2.4-4.5). The incidence of in-hospital death owing to AAD was 1.3 per 100,000 individuals/year (95% CI, 0.9-1.9), 1.0 (95% CI, 0.6-1.4; I2 = 97%) for TAAD, and 0.3 for TBAD (95% CI, 0.2-0.4; I2 = 96%). CONCLUSIONS: A global estimate regarding the incidence rate of AADs was achieved. The incidence of AAD varied significantly between study designs and geographical regions. More accurate information on AAD epidemiology is crucial for public health decisions, clinical understanding, and healthcare management.

Lessons Learned from the Impact of the COVID-19 Pandemic in a Vascular Surgery Department and Preparation for Future Outbreaks.

OBJECTIVES: This study aims to report the changes and adaptations of a vascular tertiary center during a global pandemic and the impact on its activity and patients. METHODS: We conducted a retrospective cohort study within the Vascular Surgery ward in Centro Hospitalar Universitário Lisboa Norte, Portugal. All data from surgical, inpatient and outpatient activity were collected from February to June 2020 and compared to the same 5-month period in 2018 and 2019. We ran a descriptive analysis of all data and performed statistical tests for the variation of procedures and admissions between February and June 2018 and the same time period in 2020. RESULTS: During the outbreak, our staff had to be readapted. Six nurses were transferred to COVID-19 units (out of a total of 33 nurses) while 1 of the 7 residents was transferred to an intensive care unit and 1 senior surgeon was put on prophylactic leave. In the outpatient clinic, there was an increase in the number of telemedicine consultations with a greater focus on first-time referrals and urgent cases. There was a significant increase in the total number of elective admissions whereas there were significantly less admissions from an emergency setting (+57% and -54%, respectively, P < 0.001). The vascular surgery team performed a total number of 584 procedures between February and June 2020 (-17.8% compared to 2018 and 2019), with a significant increase in the number of endovascular procedures (P < 0.001) and in the use of local and regional anesthesia (P < 0.001), especially in the Angio Suite (+600%, P < 0.001). Comparing with 2018 and 2019, the surgical team performed less outpatient procedures in early 2020. We reported a significant increase in the total number of procedures for patients with a chronic limb-threatening ischemia (CLTI) diagnosis (+21%, P < 0.001). We did not report significant changes in the proportion of other vascular conditions. Regarding mortality, we observed a 16% decrease in the intraoperative mortality (P 0.67). CONCLUSIONS: In this study, we assessed the impact of the COVID-19 outbreak in daily activity during the contingency period. During the outbreak, there was an overall decline in outpatient clinics and inpatient admissions. Nevertheless, and despite the restrictions imposed by the pandemic and health authorities, we managed to maintain most procedures for most vascular diseases, particularly for CLTI urgent cases, without a significant increase in the mortality rate. Stringent protective measures for patient and staff or higher use of endovascular techniques and local anesthesia are some of the successful changes implemented in the department. These learned lessons are to be pursued as the pandemic evolves with future outbreaks of COVID-19, such as the current second outbreak currently spreading through Europe.

Psychosocial risk factors for impaired health-related quality of life in living kidney donors: results from the ELIPSY prospective study.

Living kidney donors' follow-up is usually focused on the assessment of the surgical and medical outcomes. Whilst the psychosocial follow-up is advocated in literature. It is still not entirely clear which exact psychosocial factors are related to a poor psychosocial outcome of donors. The aim of our study is to prospectively assess the donors' psychosocial risks factors to impaired health-related quality of life at 1-year post-donation and link their psychosocial profile before donation with their respective outcomes. The influence of the recipient's medical outcomes on their donor's psychosocial outcome was also examined. Sixty donors completed a battery of standardized psychometric instruments (quality of life, mental health, coping strategies, personality, socio-economic status), and ad hoc items regarding the donation process (e.g., motivations for donation, decision-making, risk assessment, and donor-recipient relationship). Donors' 1-year psychosocial follow-up was favorable and comparable with the general population. So far, cluster-analysis identified a subgroup of donors (28%) with a post-donation reduction of their health-related quality of life. This subgroup expressed comparatively to the rest, the need for more pre-donation information regarding surgery risks, and elevated fear of losing the recipient and commitment to stop their suffering.

A single-center experience in the eversion femoral endarterectomy.

OBJECTIVES: Endarterectomy is the treatment of choice for arterial occlusive disease of the femoral bifurcation. Longitudinal arteriotomy and prosthetic patch angioplasty is the standard technique but, due to the increasing concerns with prosthetic-related infections and multidrug-resistant pathogens our group adopted an alternative approach. We present our experience with eversion femoral endarterectomy. METHODS: All patients submitted to eversion femoral endarterectomy in a single institution during 2016-2019 were retrospectively analyzed. Patient demographics, surgical data, and complications were captured from medical records. RESULTS: Nineteen patients, 84.2% male and a median age of 67 years (IQR 62-78) were submitted to eversion femoral endarterectomy with a median follow-up of 180 days (IQR 71-395). Seventeen (89.4%) patients were treated for chronic limb ischemia and the other two were submitted to femoral endarterectomy during endovascular aortic aneurysm repair. Most of the patients had smoking history (84.2%), followed by hypertension (68.4%), dyslipidemia (63.2%), coronary heart disease (29.4%), and diabetes (26.3%). Only 3 patients (15.8%) were submitted exclusively to endarterectomy, 13 (68.4%) were submitted to endarterectomy as an adjuvant for peripheral endovascular treatment, 2 (10.5%) as a concomitant procedure to endovascular repair of aortic aneurysm, and 1 (5.3%) was complemented with thrombectomy of the femoro-popliteal sector. Primary patency rates were 100% and 87.5% (CI (38.7-98.1)) at 6 and 12 months, respectively. Primary-assisted and secondary patency rates were 100%. The 30-day mortality rate was 5.3% (n = 1) and complication rate 10.5% (n = 2). One patient complicated with acute renal disease related to rhabdomyolysis. Another patient developed a wound-related hematoma treated with surgical drainage, but died three days after consequent to ischemia-reperfusion injury. CONCLUSIONS: Eversion femoral endarterectomy is a safe and feasible technique, with good patency results and respecting the concept of leaving nothing behind. A careful control of the proximal and distal endpoints is essential for the success of the technique.

Surgical Treatment of Post-Carotid Endarterectomy Carotid Pseudoaneurysm.

Post-endarterectomy pseudoaneurysms (PEPA) are a rare complication of carotid endarterectomy (CEA), but are associated with high morbidity risk. Therefore, once they are diagnosed, treatment is urgent to prevent possible complications such as rupture, embolisation, thrombosis, or airway and cranial nerve compression. In this video, the surgical procedure is shown in a case of PEPA 10 years after CEA with patch angioplasty, which was successfully managed by surgical excision and interposition of great saphenous vein graft. Follow up duplex examination at six months was normal, with patency of the vein graft. This case reiterates the importance of open surgery as the treatment of choice for this difficult clinical setting.

An Original Bailout Solution for Renal Artery Dissection after Fenestrated/Branched EVAR.

BACKGROUND: Renal artery (RA) dissection may occur during endovascular treatment of thoracoabdominal aneurysms. The aim of this paper is to report the use of kissing coronary stents in the renal bifurcation as a bailout solution for dissection after fenestraded/branched endovascular aortic repair (F/B-EVAR). METHODS/RESULTS: A 73-year-old male with an asymptomatic Crawford type 4 thoracoabdominal aneurysm and a concomitant right common iliac artery aneurysm was proposed for endovascular repair, consisting of thoracic endovascular aortic repair plus custom-made device F/B-EVAR, followed by staged bifurcated EVAR plus right-sided IBD. In the control angiogram of the first procedure, a distal occlusion of the left renal artery was observed and attributed to iatrogenic dissection. The 6F sheath was reintroduced and the two main branches of the RA were catheterized with 0.014 wires. Then, two coronary drug-eluting stents were used for a kissing stenting technique with good angiographic and clinical results. As planned, one week later the patient underwent an uneventful second stage procedure. Follow-up CTA at 1 year showed normal patency of the renal stents as well as aneurysm shrinking and no signs of endoleak. CONCLUSIONS: In the reported case, the use of coronary stents was a safe and long-lasting solution to rescue an iatrogenic renal artery dissection during F/B-EVAR.

Family dynamics in transthyretin-related familial amyloid polyneuropathy Val30Met: Does genetic risk affect family functioning?

Adult-onset, chronic, genetic diseases like transthyretin-related familial amyloid polyneuropathy Val30Met (TTR-FAP Val30Met), have a major psychosocial impact not only on patients, but also on families. Genetic risk may therefore be an increased factor in psychosocial impact of the disease on these families' functioning. To evaluate impact of genetic risk, a study was conducted to perceive the impact of the illness on families' functioning. Groups of TTR-FAP Val30Met patients, pre-symptomatic carriers, partners and patients with multiple sclerosis (MS), a non-hereditary disease, were studied. Sample included 190 adults: 87 patients and 28 pre-symptomatic carriers for TTR-FAP Val30Met, 41 partners and 34 patients with MS. Family Adaptability and Cohesion Scale IV (FACES IV) and a social-demographic questionnaire were applied. No significant differences were observed between patients and pre-symptomatic carriers and both these and their partners regarding cohesion and flexibility. MS patients scored significantly higher in median scores for balanced scales. Satisfaction and communication levels were also lower in patients with TTR-FAP Val30Met than with MS. Family functioning was perceived as balanced by most TTR-FAP Val30Met patients and pre-symptomatic carriers. These families may be considered as mostly healthy. Difficulties in family communication should be taken into account when caring for these families.

Psychopathological dimensions in subjects with hereditary ATTR V30M amyloidosis and their relation with life events due to the disease.

BACKGROUND: Chronic physical illness has been associated with emotional distress. Chronic diseases may change usual family patterns with economic, social and family losses. Hereditary ATTR V30M amyloidosis is a rare, fatal inherited systemic amyloidosis, with chronic evolution and beginning in adulthood. AIMS AND METHODS: To evaluate psychopathological dimensions and how they correlated with disease-related life events, 209 symptomatic and asymptomatic carriers, participated in the study. Sociodemographic and Family and Personal History Disease questionnaires and brief symptom inventory (BSI) were applied. RESULTS: BSI indices, global severity index (GSI), positive symptom index (PSI) and positive symptom total (PST) scored higher than general population. Independent predictors for GSI >0.83 were female sex (OR = 3.46, p = .005) and being symptomatic carriers (OR = 3.03, p = .039). Independent predictors of a PST >26.99 were female sex (OR = 3.74, p = .012) symptomatic carrier (OR = 5.32, p = .025), age between 15 and 24 years at affected parent's death (OR = 5.26, p = .04). Independent predictors of a PSI >1.56 were being asymptomatic carrier (OR = 6.3, p = .036); to have children (OR = 3.19, p = .043) and have ≤14 years at parent's disease onset (OR = 6.39, p = .05). CONCLUSIONS: Results point to an important vulnerability of this population for psychological distress and psychiatric disease. Early life events related to disease, being sick and sex are associated with psychopathological distress.

Life paths of patients with transthyretin-related familial amyloid polyneuropathy Val30Met: a descriptive study.

Transthyretin-related familial amyloid polyneuropathy Val30Met is a fatal progressive disease. It is a rare hereditary amyloidosis, manifesting as a sensorimotor neuropathy and autonomic dysfunction. It begins during adulthood and is a disabling disease, posing a great psychological burden to patients and their families. Our aim was to describe and characterize life events related to the disease and discuss its psychosocial implications. Social and demographic data and a questionnaire on history of family and personal disease, and biographic events, were applied to 209 subjects attending an outpatient specialized clinic. Descriptive and statistical analyses were performed. They were 84 men and 127 women belonging to three groups: pre-symptomatic carriers, patients, and subjects with no established diagnosis. Most subjects were married/lived with a partner and had children (mean of 4). Most (96.3%) had contact with the disease before having a diagnosis; the affected or at-risk parent was the mother in 53.8% and the father in 43.3%; 71.8% of these had deceased. At their parent's death, many subjects were aged under 10 (9.9%), 10-14 (15.5%), or 15-24 years (31.7%). Most were under age 14 (44.9%) at their parent's disease onset; 37.2% referred this brought life changes with psychological and familial impact; most had been parent's caregivers; 7.5% had not been raised by the parents. Some (8.4%) declined to know their genetic tests results for over 1 year. Parent's disease and death are very common early in these patient's lives. During childhood or youth, many subjects became caregivers, implying changes in family roles. This disease and its life implications pose a significant psychosocial burden since childhood. TTR-FAP patients and their relatives are highly vulnerable to emotional stress and psychopathology during their lifetime. Psychological and psychiatric support, implying a multidisciplinary group, must thus be available for all of them.

Psychopathological Dimensions in Portuguese Subjects with Transthyretin Familial Amyloid Polyneuropathy.

BACKGROUND: Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a fatal, chronic, progressive disease. It is a rare hereditary amyloidosis, which manifests as a sensorimotor neuropathy and autonomic dysfunction. It begins during adulthood. AIMS AND METHODS: Our aim is to evaluate psychopathological dimensions in a population attending a consultation center for TTR-FAP. Two hundred and nine subjects (symptomatic and asymptomatic carriers), 84 men and 127, women participated in the study. Most subjects were married (67.1%) and most of them were still working; 33% were retired from work or on a sick leave. A sociodemographic questionnaire and The Brief Symptom Inventory (BSI) were applied. Statistical analysis was performed (descriptive analysis, Mann-Whitney, Wilcoxon, and Spearman tests). RESULTS: The Global Symptom Index (GSI) was significantly higher in patients (p = 0.001). Considering GSI, 32.7% of total subjects were above the median for general population. When subgroups were evaluated, 25.6% of symptomatic carriers, 26.3% of subjects without established diagnosis, and 39.1% of patients were above median. GSI was significantly higher in patients (p = 0.001). Some BSI dimensions were also significantly higher in the patient group (somatization, depression, anxiety, and psychoticism) when compared with carriers. Women scored higher than men. Sick women scored higher for all dimensions except somatization. Asymptomatic carriers scored statistically higher for phobic anxiety (p = 0.01), interpersonal sensitivity, anxiety, and depression. In patients, most dimensions and GSI (rho = 0.33, p = 0.002) had positive correlations with years of disease. CONCLUSIONS: TTR-FAP patients and carriers are a very vulnerable group for psychological distress and psychopathological problems. Women and patients are at higher risk.

A performatividade nas políticas de currículo: o caso do ENEM / The performativity in the curriculum policy: the case of ENEM

Este artigo articula os efeitos da globalização com as políticas de currículo e de avaliação. Defendemos, com base em Stephen Ball, a compreensão das políticas de currículo como produção de sentidos e significados para as decisões curriculares em um ciclo contínuo de produção de políticas. Entendemos que uma das dimensões dessas políticas de currículo é a produção de uma cultura da performatividade, expressa, entre outras ações, pelo Exame Nacional do Ensino Médio (ENEM). Pela investigação de documentos relativos ao ENEM, identificamos que o foco desse exame é a formação do indivíduo onicompetente para a eficiência social do sistema, porém, diferentemente de outras épocas, centrado na autorregulação de suas performances. Além disso, consideramos que a cultura da performatividade influencia dimensões locais da avaliação, com diferentes extensões e modos de avaliar, não necessariamente associados às mesmas finalidades dos exames centralizados.

This paper articulates globalization effects with curriculum policy and evaluation. We argue, based on Stephen Ball, that curriculum policy is a production of meanings to curricular decisions in a continuous policy cycle. We also argue that one of the dimensions of this curriculum policy is the production of the performance-oriented tivity culture. This culture is expressed, for example, in the National Exam of to High School (Exame Nacional do Ensino Médio - ENEM). We have identified in the ENEM's documents that the focus of this exam is the development formation of an omnicompetent individual to the system's social efficiency. However, this focus is differs ent at from other times, as because it is centered in the performances self-regulation. Furthermore, the improvement of a performance-oriented tivity culture influences local dimensions of evaluation, with different extensions and ways of evaluation. These local dimensions of evaluation are not necessarily associated to the same centralized exams´ aims.

Efeitos da mobilização neural em terra e na água: um estudo comparativo / Effects of neural mobilization on land and water: a comparative study

Durante a realização de testes neurodinâmicos existe uma atividade muscular protetora, que possui como objetivo evitar o alongamento neural excessivo. na imersão em águas aquecidas ocorre um relaxamento de tecidos moles, permitindo assim um aumento do arco de movimento. O objetivo do estudo foi verificar o ganho de amplitude de movimento (ADM) de cotovelo após a realização da técnica de mobilização neural em terra e na água, através do teste neurodinâmico "Upper Limb Tension Test" (ULTT1) para o nervo mediano, comparando os resultados obtidos. A amostra foi composta de 20 sujeitos. Estes realizaram a técnica de mobilização neural mantida por 3 minutos uma vez na água e outra em terra. Assim foi obtido o ganho da ADM de cotovelo pré e pós técnica em cada meio. A amostra apresentou uma melhora em relação à ADM de cotovelo tanto em terra quanto na água. 16 sujeitos apresentaram melhores na água e apenas 4 em terra. O ganho mediano da ADM de cotovelo na água foi de 23º enquanto em terra foi de 7º. Embora em ambos os meios ocorra uma melhora significativa da excursão neural (p<0,01), a técnica de mobilização neural possui uma efetividade maior quando realizada na água (p<0,01). Este estudo apresentou uma maior efetividade da técnica de mobilização neural realizada na água comparada com a realização em terra.

It is know that while neurodynamic tests are being realized, there is a muscle protector activity which avoids the exceeding neural prolongament. A relaxing on the soft tissues occurs with an immersion in warm water, permitting an increase of the movement arch. To verify the elbow movement amplitude gain after the realization of the neural mobilization technique in the earth and in the water, through the neurodynamic test for the median nerve "Upper Limb Tension Test" (ULTT1). The sample was formed of 20 people. The neural mobilizationa technique for 3 minutes once in the water and then in the earth. Thus it was obtained, in each environment, the elbow's amplitude gain before and after the technique, using a universal goniometer. The sample showed an improvement in elbow's amplitude as much in the earth as in the water. 16 people presented better results in the water and only 4 in the earth. The medium elbow's amplitude gain in the water was of 23º while in the earth it was of 7º. Although in both enviroments there are significant improvements of elbow's range of motion (P<0,01), the neural mobilization technique has a better effectiveness when realized in the water (P<0,01). This research showed better effectivenes of the neural mobilization technique realized in the water in comparison with the one realized in the earth.