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Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a devastating disease, that despite being increasingly diagnosed, there are no consensus guidelines for the optimal management. A previously healthy 3-year-old-boy brought to the emergency department due to seizures. Neurological examination was normal, and electroencephalogram (EEG) suggested focal epilepsy. Anticonvulsive medication was initiated. He progressively lost age-appropriate language skills, presented behavioural changes and psychiatric symptoms. Neurological examination at that time revealed symmetric gross motor weakness of the lower limbs. Brain and spinal cord MRI and cerebrospinal fluid were normal. Repeated EEG showed global lentification. Steroid therapy was initiated for the suspicion of autoimmune encephalitis, later confirmed as NMDAR encephalitis. He became clinically improved after 10 days of treatment but only returned to his baseline after 3 months of disease onset. The authors emphasised the variable course of the disease and possible late response to treatment., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Portugal is one of the countries with the highest tuberculosis (TB) burden in the European Union. Patients who are receiving antitumor necrosis factor (TNF) treatment should be screened and treated for latent tuberculosis infection (LTBI). This study aimed to evaluate the prevalence of LTBI and the number of active TB cases in patients with inflammatory bowel disease (IBD) treated with anti-TNF agents., Patients and Methods: This was a retrospective study from a Portuguese center that included patients with IBD who started anti-TNF treatment between 2013 and 2017. LTBI screening was considered positive in case of positive tuberculin skin test, positive/indeterminate interferon gamma release test, or history of exposure to TB irrespective of the results of the screening., Results: One hundred and seventeen patients were included, 56% female, mean age 40 years; 91% had Crohn's disease; infliximab was initiated in 79%, adalimumab in 21%, and golimumab in 1%. Of these, 37 (32%) presented LTBI - tuberculin skin test was positive in 18 (49%) patients; interferon gamma release test was positive in 14 (38%) patients and undetermined in seven (19%); and there was a history of exposure in 12 (32%) patients. All patients screened with LTBI were on isoniazid for 9 months. During follow-up (mean 21.6 months), one patient under infliximab developed pleural TB 5 years after receiving treatment with isoniazid. None of the patients with negative LTBI screening developed active TB., Conclusion: In this sample of patients with IBD, the prevalence of LTBI before starting anti-TNF treatment was significant (32%), but only one patient had active TB after LTBI treatment.

Objective: Varicose vein (VV) surgery is frequently performed by surgeons without formal vascular training. We aimed to compare the outcomes of the procedure based on the background of the surgeon., Methods: All patients registered with VV surgery between 2004 and 2016 in Portuguese public hospitals were included in the study. Intrahospital outcomes were assessed from this administrative database. A random multicenter sample of 315 patients submitted to saphenous high ligation and stripping (175 patients from six vascular surgery departments and 140 patients from five general surgery divisions) were further queried over the phone, whereby additional nonregistered outcomes were evaluated: preoperative venous ultrasound, impact on quality of life by the 14-item Chronic Venous Insufficiency Quality of Life Questionnaire, visual analogue scale evaluation (score of 1 to 5) of the aesthetic results and general satisfaction, work absence days, and time to return to physical activities., Results: In 13 years, there were 153,382 patients submitted to VV surgery. Of these, 49% were operated on by general surgeons and 40% by vascular surgeons; in 11%, it was not possible to identify the specialty performing the operation. Twenty-three deaths were registered (no differences between groups). In the general surgery group, 14% of patients were hospitalized for more than one night compared with 3% in the vascular group (P < .001). Reintervention rate during the period analyzed was significantly higher in the general surgery group (13.5% vs 8.2%; P < .001). Rate of outpatient surgery was higher in the vascular surgery group (60% vs 36%; P < .001). Phone query revealed similar overall satisfaction and improvement in quality of life in both groups (4.2 vs 4.0 [P = .275] and 35% vs 36% [P = .745], respectively). However, patients operated on by general surgeons reported worse surgical scars (2.8 vs 2.1; P = .007), higher number of residual VVs (2.4 vs 1.7; P = .006), and higher number of days absent from work (40 vs 27 days; P = .005) and took longer to resume physical activities (60 vs 41 days; P = .001)., Conclusions: Despite that the majority of VV surgery in Portugal is executed by general surgeons, this study highlights important advantages when it is performed by surgeons with vascular training., (Copyright © 2019 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Competing Interests: The authors received no financial support and have no competing interests.

Competing Interests: The authors have no financial support or competing interests to disclose.

In up to one-third of cases of sudden death, the medico-legal autopsy finding is inconclusive, and the option to perform a molecular autopsy is covered in international guidelines. The importance of postmortem genetic testing lies in its ability to identify hereditary diseases, often those with an autosomal dominant transmission pattern, and, through consultations and screening of relatives, to identify family members with a pathogenic mutation, who are often asymptomatic, providing an opportunity to change the course of their lives. The authors present three clinical cases that highlight the importance of postmortem genetic studies and family studies, as well as the integration of the data obtained in a cardiology consultation, which may be for arrhythmology, coronary disease or cardiomyopathy, depending on the specific condition. This could modify the course of the disease in many relatives., (Copyright © 2019. Publicado por Elsevier España, S.L.U.)

Background: Prosthetic joint infection (PJI) is a serious complication after total joint arthroplasty, and prevention is of great importance. The genitourinary tract is a potential source of bacterial seeding and one that can be easily managed. Despite little supportive evidence, routine urine screening and subsequent treatment before elective surgery in patients without symptoms has found its way into clinical practice in many countries. This systematic review and meta-analysis aims to ascertain whether asymptomatic bacteriuria (ASB) is a risk factor for PJI and if so, whether preoperative antibiotic treatment is effective in reducing its risk., Methods: PubMed, Ovid Medline, and Cochrane databases were searched using a systematic strategy. Selection of papers was exclusive to include only those which offered information about PJI rate specifically in patients with or without asymptomatic abnormal urinalysis or bacteriuria and/or information on whether ASB patients were preoperatively treated with antibiotics or not to be included in the analysis., Results: Six-hundred sixty-three papers were screened, and 10 papers were ultimately included (28,588 patients). Results show an increased risk of developing PJI among ASB patients (odds ratio = 3.64, 95% confidence interval = 1.40-9.42). However, most PJI microorganisms are unrelated to those previously found in the urine and preoperative antibiotic therapy does not influence PJI risk (odds ratio = 0.98, 95% confidence interval = 0.39-2.44)., Conclusion: Routine urinary screening prior to elective total joint arthroplasty and treatment of asymptomatic patients is not recommended., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Superior patient and graft survival rates have been attributed to living donor kidney transplant (LDKT) when compared to deceased donor transplantation. The aim of this study was to assess graft survival in a population of LDKT in the last 14 years and the potential impact of some clinical features., Methods: A retrospective observational study was conducted, reviewing the records of all patients undergoing LDKT in one center from January 1, 2004, to December 31, 2017. Survival data were evaluated by Kaplan-Meier, log rank test, and Cox regression., Results: Two hundred seventy-seven LDKT were performed. The median follow-up time was 4 (0-13) years. Graft loss was observed in 9% of patients; 4 patients died. The overall survival was 97% at year 1, 94% at year 5, and 83% at years 10 and 13. We found a significantly worse graft survival in patients with early vascular complications that required surgical intervention (P = .00) ≥3 HLA MM (P = .01), ≥1 HLA-DR MM (P = .04) and female recipients (P = .01). The negative impact of ≥1 HLA-B MM on survival was borderline (P = .05). After excluding early graft losses secondary to vascular events, ≥1 HLA-A MM and rejection have also implicated a negative impact on survival (P = .04 and .01, respectively). In the multivariate analysis, these variables were still related to inferior survival., Conclusions: We observed a good overall graft survival (>80% after 13 years). Possible factors related to poor outcomes suggested by this study were early vascular complications; HLA mismatches; rejection; and, with less certainty, female recipients., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this paper is to assess the applicability of direct computed tomography venography (DCTV) in assessing dominant inflow vein in the femoral confluence in extensive chronic iliofemoral venous obstruction, using venography as the gold standard., Methods: All DCTVs performed in symptomatic patients with previous iliofemoral deep vein thrombosis subsequently submitted to venography in the period from January 2014 to August 2018 were retrospectively reviewed. Two groups were defined depending on whether the femoral vein (FV) or the deep femoral vein (DFV) was the dominant inflow on venography in order to identify predictors of DFV as dominant inflow in DCTV. Statistical analysis was performed with SPSS V25., Results: A total of 30 DCTVs and subsequent venographies were reviewed. Venography identified the FV as the dominant inflow in 18 (60%) and the DFV in 12 (40%) patients. Predictors for DFV as dominant inflow were identified as follows: larger DFV diameter 50 mm and 250 mm below lesser trochanter (8.73 ± 4.34 mm vs. 11.9 ± 3.52 mm; p = 0.043 and 5.4 ± 3.90 mm vs. 8.90 ± 2.70 mm; p = 0.011); lower FV/DFV ratio 150 mm below lesser trochanter (11.39 ± 20.01 mm vs. 1.05 ± 0.47 mm; p = 0.043); and presence of FV scarring/synechiae, collaterals and abnormal wall thickness (p = 0.003, p = 0.003 and p < 0.0001)., Conclusion: In cases of extensive chronic iliofemoral venous obstruction, especially when stent deployment into the DFV is entertained, the key to success is thorough pre-procedure planning focusing on choosing the access site. This study suggests DCTV is valuable in defining the dominant iliac vein inflow, but additional findings are necessary to validate these preliminary data.

Introduction: Six-minute walk test (6MWT) is used for evaluating functional exercise capacity. To the best of our knowledge, there are no reference equations to predict six-minute walk distance (6MWD) for the Portuguese population. The aims of the present study were to measure anthropometric data and 6MWD in a sample of healthy Portuguese population, to establish reference equations to predict 6MWD and to compare our equations with those obtained by previously published studies., Methods: We conducted an observational prospective study. We consecutively recruited 158 healthy 18-70 years old subjects from Porto district, who performed two 6MWTs using a standardized protocol. The best 6MWD was used for further analysis., Results: The mean 6MWD was 627.8m (SD=73.3m). The variables that were significantly associated with the 6MWD were age, sex, BMI and ΔHR (Heart Rate at the end of the test -HR at rest) . We found three explanatory models for 6MWD, the best with an explanatory power of 38%: 6MWD=721.7-1.6×Age-4.0×BMI+0.9×ΔHR+58.4×Sex. We verified that 6MWD decreased 1.6m per year of age, and 4.0m per unit of BMI and increased 0.892m per beat per minute. Moreover, on average, males walk 58.4m more than females (p<0.001). Applying equations from other studies to our population resulted in an overestimation or underestimation of the 6MWD., Conclusion: The present study was the first to describe the 6MWD in healthy Portuguese people aged 18-70 years old and to propose predictive equations. These can contribute to improving the evaluation of Caucasian Mediterranean patients with diseases that affect their functional capacity., (Copyright © 2018. Published by Elsevier España, S.L.U.)

Objective: Acute carotid stent thrombosis (ACST) occurring in the first hours after the procedure is an exceedingly rare complication of carotid artery stenting, but it is potentially devastating. This review aimed to evaluate current literature, identifying all reported cases during the last two decades, with the final purpose of reporting predictive factors and early management., Methods: A systematic review and meta-analysis was conducted according to the recommendations of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement., Results: A total of 464 potentially relevant articles were selected. After review of records at title and abstract level, 29 articles with 60 patients were included. Twelve studies reported on ACST incidence rate in their cohorts, ranging from 0.36% to as high as 33%. In considering etiology, antiplatelet noncompliance or resistance is the most frequently reported risk factor. Emergency procedures seemed to be associated with greater risk for ACST, reaching 5.6% to 33% incidence. Dual-layer stents were also associated with greater risk (45% vs 3.7%; P = .0001; odds ratio, 21.3). Use of an overlapping stent as a bailout procedure because of dissection, malposition, or long lesions was correlated with increased risk (7.3% vs 0.002%), as were long stenotic lesions (22.9 ± 6.83 mm vs 14.2 ± 6.42 mm; P = .0034) and stent length (3.8 ± 0.4 cm vs 2.8 ± 0.86 cm; P = .0055). ACST was associated with neurologic status deterioration in 56.7% of cases. Time to symptoms or ACST diagnosis had a median of 1.5 hours, with 30% occurring intraprocedurally. In asymptomatic ACST, conservative management was unanimous. Endovascular treatment was the most common approach to intraprocedural ACST. Surgical options included carotid endarterectomy with stent explantation (n = 9), which was also a bailout after failed endovascular treatment in two cases., Conclusions: ACST incidence is higher in emergent, neurologically unstable patients. Antiplatelet noncompliance, antiplatelet resistance, long stenotic lesions, use of more than one stent, and dual-layer stents are also associated with increased risk. The decision as to the best approach depends on whether ACST occurs intraprocedurally or afterward, the development of neurologic status deterioration, and the center's experience. However, additional studies must be undertaken to better define optimal management., (Copyright © 2018 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Background and Aims: The guidelines by the American Society for Gastrointestinal Endoscopy (ASGE) suggest that in patients with gallbladder in situ, endoscopic retrograde cholangiopancreatography (ERCP) should be performed in the presence of high-risk criteria for choledocholithiasis, after biochemical tests and abdominal ultrasound. There are no specific recommendations for cholecystectomized patients. The aim of this study was to evaluate the applicability of ASGE criteria for ERCP in cholecystectomized patients with suspected choledocholithiasis., Methods: We conducted a retrospective study that included patients with high-risk ASGE criteria for choledocholithiasis who underwent ERCP from 2013-2016., Results: We included 327 patients in our analysis - 258 with gallbladder in situ (79%) and 69 with cholecystectomy (21%). We showed that the ASGE criteria true positive rate was similar between patients with and without cholecystectomy - the prevalence of choledocholithiasis on ERCP was 71% in cholecystectomized and 70% in non-cholecystectomized. In addition, both the presence and the diameter of the CBD stone were predictors of positive ERCP in both groups. In contrast, the diameter of the CBD was predictor of positive ERCP only in non-cholecystectomized patients., Conclusions: Although dilatation of the CBD was not a predictive factor for choledocholithiasis in cholecystectomized patients, the ASGE criteria true positive rate was similar to that of non-cholecystectomized patients., (Copyright © 2018 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Introduction: Asthma and obesity have a considerable impact on public health and their prevalence is increasing. Obesity is a known risk factor for asthma and can make it more difficult to control. Omalizumab is recommended in patients with severe allergic persistent asthma. The aim of this study was to assess the impact of omalizumab treatment in obese asthmatic patients with poorly controlled severe persistent asthma., Methodology: A non-interventional, prospective study was conducted, in an outpatient asthma clinic. All patients with severe asthma who started treatment with omalizumab were included and followed over 12 months. The study population was divided into two groups (obese and non-obese) for statistical analysis (descriptive and comparative analysis)., Results: Thirty-two patients (19 obese) were followed. After 12 months of omalizumab treatment, there was a statistically significant improvement in body mass index, number of exacerbations in the previous year, rescue medication, disease control and lung function, in the whole population. At the end of the study obese patients had a significantly better lung function (FEV 1 ) than non-obese., Discussion: As described in the literature, there was a significant reduction in the number of exacerbations in the previous year, rescue medication and better disease control, in the whole population. In relation to lung function, about which published data are inconsistent, treatment with omalizumab significantly improved it in obese patients., Conclusion: Our study showed that omalizumab significantly improved asthma control, reduced rescue medication and asthma exacerbations in all the population; and for the first time showed that obese patients achieved significantly improved lung function., (Copyright © 2018 Sociedade Portuguesa de Pneumologia. Published by Elsevier España, S.L.U. All rights reserved.)

Background: Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) is an autosomal recessive disorder characterized by 3 overlapping phenotypes: salt-wasting (SW), simple virilizing (SV), and non-classic (NC). We aimed at conducting a nationwide genotype description of the CAH pediatric patients and to establish their genotype-phenotype correlation., Methods: CAH patients were recruited from Portuguese pediatric endocrinology centers and classified as SW, SV, or NC. Genetic analysis was performed by polymerase chain reaction (sequence specific primer, restriction fragment length polymorphism) or direct Sanger sequencing. Genotypes were categorized into 4 groups (0, A, B, and C), according to their predicted enzymatic activity. In each group, the expected phenotype was compared to the observed phenotype to assess the genotype-phenotype correlation., Results: Our cohort comprises 212 unrelated pediatric CAH patients (29% SW, 11% SV, 60% NC). The most common pathogenic variant was p.(Val282Leu; 41.3% of the 424 alleles analyzed). The p.(Val282Leu) variant, together with c.293-13A/C>G, p.(Ile173Asn), p.(Leu308Thr), p.(Gln319*), and large deletions/conversions were responsible for 86.4% of the mutated alleles. Patients' stratification by disease subtype revealed that the most frequent pathogenic variants were c.293-13A/C>G in SW (31.1%), p.(Ile173Asn) in SV (46.9%), and p.(Val282Leu) in NC (69.5%). The most common genotype was homozygosity for p.(Val282Leu; 33.0%). Moreover, we found 2 novel variants: p.(Ile161Thr) and p.(Trp202Arg), in exons 4 and 5, respectively. The global genotype-phenotype correlation was 92.4%. Group B (associated with the SV form) showed the lowest genotype-phenotype correlation (80%)., Conclusion: Our cohort has one of the largest NC CAH pediatric populations described. We emphasize the high frequency of the p.(Val282Leu) variant and the very high genotype-phenotype correlation observed., (© 2019 S. Karger AG, Basel.)

Introduction and aims: Nonalcoholic fatty liver disease (NAFLD) has become highly prevalent, paralleling the pandemic of obesity and diabetes, and represents an important burden. Nutrition knowledge is fundamental, in prevention, evolution and treatment of NAFLD. Association of low serum levels of vitamin D (VD) with several diseases, including NAFLD, has been emphasized in the last decade. We evaluated how serum levels of VD correlate with the presence of hepatic steatosis, and VD intake, in a random sample of the Portuguese adult population. Methods: Participants underwent a dietary intake inquiry, using a semi-quantitative food frequency questionnaire representative of the usual intake over the previous year. Anthropometric measures, blood tests and ultrasound were done. Hepatic steatosis was quantified according to Hamaguchi's ultrasonographic score (steatosis defined by a score ≥ 2). Results: We recruited 789 adult individuals, 416 males (52.7%), mean age of 49.9 ± 17.0 years (18-79). Prevalence of hepatic steatosis was 35.5%, and after exclusion of excessive alcohol consumption, 28.0%. Mean VD serum levels were 26.0 ± 9.8 ng/ml and 68.4% participants had serum VD levels below 30 ng/ml. Mean serum levels of VD were not significantly different between participants with steatosis vs . no steatosis: 25.2±8.7 vs . 26.4±10.3 ng/ml, respectively (p=0.071). There was no correlation between VD serum levels and VD intake, measured by the FFQ, r=0.075 (p= 0.383). Conclusions: In spite of a high prevalence rate, there was no evidence that decreased VD serum levels were associated with hepatic steatosis. No significant correlation was found between VD dietary ingestion and VD serum levels., Competing Interests: Competing Interests: A Carvalho, received fees on consultancy, from Intercept and H Cortez-Pinto received fees on consultancy from Genfit, Intercept and Gilead. The other authors declare that they have no conflict of interest.

Introduction: Along with increased life expectancy, the proportion of elderly patients with choledocholithiasis will increase and with this, the need for endoscopic cholangiopancreatography (ERCP). Current recommendations suggest laparoscopic cholecystectomy in all patients with choledocholithiasis to prevent biliary events. However, adherence to these recommendations is low, especially in older patients., Methods: Retrospective study that included non-cholecystectomized patients aged > =75 years who underwent ERCP for choledocholithiasis from 2013-2016 (n = 131). A new biliary event was defined as the need for a new ERCP, cholecystitis, cholangitis or gallstone pancreatitis., Aim: The aim of this study was to compare the outcomes of new biliary events and mortality in cholecystectomized vs non-cholecystectomized patients after ERCP., Results: Cholecystectomy was performed in 22% of the patients (92% laparoscopic). The post-cholecystectomy complication rate was 13% and the mortality rate was 7%. During the follow-up period (669 ± 487 days) a new biliary event occurred in 20% of patients - 10% new ERCP, 9% cholecystitis, 9% cholangitis and 2% pancreatitis. Cholecystectomized patients had fewer events (7% vs 24%, p = .048) and longer time to event (p = .016). There was no statistically significant difference in all-cause mortality (14% vs 27%, p = .13), mortality related to lithiasis (0% vs 9%, p = .11) or time to mortality from all causes (p = .07) and related to biliary events (p = .07)., Conclusions: In this group of elderly patients, cholecystectomy after ERCP prevented the occurrence of new biliary events but resulted in a non-statistically significant difference in mortality.

Introduction: Neuromuscular diseases (NMD) are a group of rare heterogeneous disorders that may be accompanied by respiratory muscle weakness. The simplest measurements of respiratory muscle strength are maximum inspiratory pressure (P Imax ) and maximum expiratory pressure (P Emax ) of the mouth. Inspiratory muscle weakness can also be evaluated by the sniff test (sniff nasal inspiratory pressure method). This study tested the agreements in P Imax and P Emax (measured by using a plethysmograph and portable equipment) as well as the correlations of P Imax and P Emax by using the sniff nasal inspiratory pressure method, lung function, and arterial blood gas parameters in subjects with NMD., Methods: This prospective, noninterventional study measured respiratory parameters in all the subjects with NMD who underwent measurement of maximum respiratory pressures., Results: A total of 55 subjects with NMD were included. There were no statistically significant differences in P Imax and P Emax measured by using a plethysmograph and portable equipment. Moreover, P Imax showed a good correlation with the sniff nasal inspiratory pressure method., Conclusions: Measurements of P Imax and P Emax by using portable equipment were equivalent to those performed by using the accepted standard, plethysmography, in the subjects with NMD. Noninvasive evaluation of the sniff test with the portable equipment correlates with P Imax , which makes this approach a good method for measuring the maximum strength of inspiratory muscles in patients with NMD., Competing Interests: The authors have disclosed no conflicts of interest., (Copyright © 2018 by Daedalus Enterprises.)

Background: Major pelvic ilio-iliac arteriovenous fistula (AVF) is an exceedingly rare diagnosis with only a few described cases in the literature, most of them related to congenital defects or trauma. In this case report, we aim to present a case of an ilio-iliac AVF with an atypical clinical presentation., Methods: Relevant medical data were collected from hospital database., Results: The patient is a 77-year-old woman, with a relevant medical history of a temporally remote hysterectomy. She developed an exuberant unilateral right leg edema and was diagnosed with a femoro-iliac deep vein thrombosis (DVT) and started on anticoagulation and daily use of elastic compression stockings. No improvement in leg edema was evident, and she reported painful complaints refractory to medication. She also progressively developed right foot numbness and foot drop. A computed tomography angiography (CTA) was performed to exclude any compressive or paraneoplastic syndrome, with no remarkable findings other than common iliac vein (CIV) occlusion. As the patient's symptoms continued to worsen, a new CTA was performed 5 months later, which revealed an ilio-iliac AVF that was confirmed by angiography. After 2 ineffective attempts to embolize AVF afferents, we chose to completely embolize the arterial component of the AVF with Helix EV3 coils and Onyx glue (Covidien, Irvine, CA, USA). CIV recanalization and deployment of a Venovo stent (Bard Inc, Tempe, AZ, USA) was also performed. The final angiograms showed exclusion of the AVF and rapid venous flow through the stent. There was progressive improvement of edema and pain but little improvement of foot drop., Conclusion: AVF etiology and mechanism of neurologic deficits are controversial, with multiple possible explanations. Endovascular treatment modalities are promising a safer and more efficient approach when compared with open surgery. Our experience in this case was encouraging, but long-term results are currently lacking., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction and Objectives: Left atrial dysfunction in aortic stenosis may precede atrial enlargement and predict the occurrence of atrial fibrillation (AF). To test this hypothesis, we assessed left atrial function and determined its impact on the incidence of AF after aortic valve replacement., Methods: A total of 149 severe aortic stenosis patients (74±8.6 years, 51% men) with no prior AF were assessed using speckle-tracking echocardiography. Left atrial function was evaluated using peak atrial longitudinal strain (PALS), peak atrial contraction strain (PACS), and phasic left atrial volumes. The occurrence of AF was monitored in 114 patients from surgery until hospital discharge., Results: In multiple linear regression, PALS and PACS were inversely correlated with left atrial dilation, left ventricular hypertrophy, and diastolic function. Atrial fibrillation occurred in 36 patients within a median time of 3 days [interquartile range, 1-4] after aortic valve replacement. In multiple Cox regression, PALS and PACS were independently associated with the incidence of AF (HR, 0.946; 95%CI, 0.910-0.983; P=.005 and HR, 0.932; 95%CI, 0.883-0.984; P=.011, respectively), even after further adjustment for left atrial dimensions. Both reduced PALS and PACS were associated with the incidence of AF in patients with nondilated left atria (P value for the interaction of PALS with left atrial dimensions=.013)., Conclusions: In severe aortic stenosis, left atrial dysfunction predicted the incidence of postoperative AF independently of left atrial dilation, suggesting that speckle-tracking echocardiography before surgery may help in risk stratification, particularly in patients with nondilated left atria., (Copyright © 2017 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.)

Purpose: Retinal vein occlusion (RVO) is an important cause of visual disability in the modern world. We aim to evaluate the real-world outcomes of patients with RVO treated with anti-vascular endothelial growth factor (VEGF) in Portugal., Methods: We performed a retrospective, observational, multicenter study including 8 centers across Portugal and 200 patients treated with either ranibizumab or bevacizumab. Data were collected at 3 time points: time of diagnosis (0 time point) and 6 and 12 months after initiating treatment. Demographic and clinical data were collected., Results: Median visual acuity (VA) and central macular thickness (CMT) improved in the branch RVO (BRVO), central RVO (CRVO), bevacizumab, and ranibizumab groups at 6 and 12 months compared to baseline, with CMT improving further only in the CRVO and ranibizumab groups between 6 and 12 months (p = 0.002 and p = 0.001, respectively). The CMT was lower in the ranibizumab group compared to the bevacizumab group both at 6 and 12 months (p<0.02). Median CMT improved in both the good and poor baseline VA groups at 6 and 12 months compared to baseline (p<0.001). Median VA only improved for the group with poor baseline VA at 6 and 12 months of follow-up (p<0.001). Regression analysis identified several baseline variables as predictors of visual outcomes at 6 and 12 months, with different results depending on the analyzed group., Conclusions: Both treatments were effective, although less effective than results reported in clinical trials. The morphologic response was better with ranibizumab compared to bevacizumab, although functionally there were no differences.

Background: Previous studies showed that metabolic syndrome is associated with aortic valve calcification (AVC) and poor outcomes in aortic stenosis (AS). However, if these associations change and how body fat impacts the prognosis of patients in late stage of the disease have been not yet explored., Aims: To determine the association of body mass index (BMI) and visceral fat with AVC and mortality after transcatheter aortic valve replacement (TAVR)., Methods: This was a prospective cohort of 170 severe AS patients referred to TAVR. We quantified AVC mass score and fat depots including epicardial adipose tissue, intrathoracic fat, and abdominal visceral (VAF) and subcutaneous fats by computed tomography. Fat depots were indexed to body surface area. All-cause and cardiovascular-related deaths after TAVR were recorded over a median follow-up of 1.2 years., Results: Higher AVC mass was independently associated with low BMI and low VAF. All-cause mortality risk increased with the decrease of BMI and increment of VAF. A stratified analysis by obesity showed that in non-obese, VAF was inversely associated with mortality, whereas in obese, high VAF was associated with higher mortality (p value for interaction < 0.05). At long-term, hazard ratio [HR] with non-obese/low VAF was 2.3 (95% confidence interval [CI] 1.1-4.9; p = 0.021) and HR with obese/high VAF was 2.5 (95% CI 1.1-5.8; p = 0.031) compared with obese/low VAF patients., Conclusions: In AS patients submitted to TAVR, BMI and VAF were inversely associated with AVC. Pre-intervention assessment of VAF by computed tomography may provide a better discrimination of mortality than BMI alone.

Objective: To compare healthcare in acute myocardial infarction (AMI) treatment between contrasting health systems using comparable representative data from Europe and USA., Design: Repeated cross-sectional retrospective cohort study., Setting: Acute care hospitals in Portugal and USA during 2000-2010., Participants: Adults discharged with AMI., Interventions: Coronary revascularizations procedures (percutaneous coronary intervention (PCI), coronary artery bypass graft (CABG) surgery)., Main Outcome Measures: In-hospital mortality and length of stay., Results: We identified 1 566 601 AMI hospitalizations. Relative to the USA, more hospitalizations in Portugal presented with elevated ST-segment, and fewer had documented comorbidities. Age-sex-adjusted AMI hospitalization rates decreased in USA but increased in Portugal. Crude procedure rates were generally lower in Portugal (PCI: 44% vs. 47%; CABG: 2% vs. 9%, 2010) but only CABG rates differed significantly after standardization. PCI use increased annually in both countries but CABG decreased only in the USA (USA: 0.95 [0.94, 0.95], Portugal: 1.04 [1.02, 1.07], odds ratios). Both countries observed annual decreases in risk-adjusted mortality (USA: 0.97 [0.965, 0.969]; Portugal: 0.99 [0.979, 0.991], hazard ratios). While between-hospital variability in procedure use was larger in USA, the risk of dying in a high relative to a low mortality hospital (hospitals in percentiles 95 and 5) was 2.65 in Portugal when in USA was only 1.03., Conclusions: Although in-hospital mortality due to an AMI improved in both countries, patient management in USA seems more effective and alarming disparities in quality of care across hospitals are more likely to exist in Portugal., (© The Author 2017. Published by Oxford University Press in association with the International Society for Quality in Health Care. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com)

Background and Aims: According to the Baveno VI consensus, patients with liver stiffness < 20 kPa and a platelet count > 150,000 ul have very low risk of clinically significant varices and do not need a screening endoscopy. The aim of this study was to evaluate non-invasive methods as predictors of esophageal varices according to the Baveno VI recommendations, in real life clinical practice., Methods: Retrospective evaluation of patients with chronic liver disease who underwent transient elastography between January 2013 and December 2015., Results: One hundred and four patients were included in the study, the median age was 56.8 years and 69.2% were male. The etiology of liver disease was hepatitis C in 80% of patients (including 20% with HIV co-infection), alcohol in 12%, hepatitis B in 4% and other causes in 5%. Varices were present in 25% of patients. A liver stiffness < 20 kPa had a sensitivity of 92.3% and a specificity of 84.6%. When considering high risk varices (small with red wales or large varices), a liver stiffness < 20 kPa had 100% sensitivity. A platelet count > 150,000/l had a sensitivity of 84.6% and a specificity of 64.1%. Four patients with a platelet count (PLT) > 150,000/l had esophageal varices. When both criteria were applied to the patient cohort, according to the Baveno VI consensus, the sensitivity was 100% and the specificity, 61.5%., Conclusion: In this study, the Baveno VI criteria had 100% sensitivity but a relatively low specificity for the non-invasive diagnosis of esophageal varices. In clinical practice, all patients with varices are identified but many patients have a subsequent negative endoscopy.

Azathioprine (AZA) is commonly used in inflammatory bowel disease and many other medical diseases. The most common adverse events of AZA include gastrointestinal effects, hepatotoxicity, myelotoxicity and pancreatitis.

Introduction and Objectives: We aimed to compare access to new health technologies to treat coronary heart disease (CHD) in the health systems of Portugal and the US, characterizing the needs of the populations and the resources available., Methods: We reviewed data for 2000 and 2010 on epidemiologic profiles of CHD and on health care available to patients. Thirty health technologies (16 medical devices and 14 drugs) introduced during the period 1980-2015 were identified by interventional cardiologists. Approval and marketing dates were compared between countries., Results: Relative to the US, Portugal has lower risk profiles and less than half the hospitalizations per capita, but fewer centers per capita provide catheterization and cardiothoracic surgery services. More than 70% of drugs were available sooner in the US, whereas 12 out of 16 medical devices were approved earlier in Portugal. Nevertheless, at least five of these devices were adopted first or diffused faster in the US. Mortality due to CHD and myocardial infarction (MI) was lower in Portugal (CHD: 72.8 vs. 168 and MI: 48.7 vs. 54.1 in Portugal and the US, respectively; age- and gender-adjusted deaths per 100000 population, 2010); but only CHD deaths exhibited a statistically significant difference between the countries., Conclusions: Differences in regulatory mechanisms and price regulations have a significant impact on the types of health technologies available in the two countries. However, other factors may influence their adoption and diffusion, and this appears to have a greater impact on mortality, due to acute conditions., (Copyright © 2017 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Obstructive sleep apnea syndrome (OSAS) is associated with cardiovascular/metabolic complications. Some analytical parameters (homocysteine, glycemic and lipidic profiles) are recognized markers of these consequences. Limited data is available on the association of these markers and OSAS's severity/response to positive airway pressure therapy (PAP)., Material and Methods: In this prospective study we analyzed polysomnographic and analytical data of male patients admitted to sleep laboratory. The aim was to evaluate metabolic/cardiovascular markers in snorers and OSAS patients, to relate with sleep parameters and PAP response. One-hundred and three patients were included, and 73 (71%) were OSAS patients. OSAS patients were similar to snorers except for higher body mass index (BMI) and dyslipidemia. Severe OSAS patients showed higher glycemia, HbA1c, insulin, and insulin resistance, and lower HDL cholesterol in comparison to mild-moderate (p<0.05, p<0.05, p<0.001, p<0.001, p<0.05, respectively). Glycemic profile and triglycerides were slightly correlated with OSAS severity. 46 OSAS patients were submitted to 6 months of PAP, with a statistical decrease in mean values of homocysteine, glycemia, total and LDL cholesterol (p<0.05, p<0.05, p<0.05, respectively), and in glycemia and LDL cholesterol in severe group only (p<0.05, p<0.05, respectively)., Results: This study demonstrated an association between glucose metabolism parameters and triglycerides with OSAS severity underlying the complexity of the process leading to cardiovascular/metabolic complications in this disorder. Moreover, homocysteine, glycemic and lipidic profiles changed significantly after 6 months of PAP therapy in OSAS, supporting its cardiovascular and metabolic protective effect., Conclusion: Our study has reinforced the importance of analytical cardiovascular/metabolic evaluation as complementary tool of diagnosis/treatment response in OSAS., (Copyright © 2017 Sociedade Portuguesa de Pneumologia. Published by Elsevier España, S.L.U. All rights reserved.)

We present a case report of a meningoradiculopathy associated with human herpesvirus 7, with long-term motor neurologic sequelae. It is important to consider human herpesvirus 7 as a potential pathogen of severe neurologic disease and sequelae in immunocompetent children, especially in older patients presenting neurologic signs.

Introduction: Lung cancer staging has recently evolved to include endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) for nodal assessment., Aim: Evaluate the performance and safety of EBUS-TBNA as a key component of a staging algorithm for non-small cell lung carcinoma (NSCLC) and as a single investigation technique for diagnosis and staging of NSCLC., Methods: Patients undergoing EBUS-TBNA for NSCLC staging at our institution between April 1, 2010 and December 31, 2014 were consecutively included with prospective data collection. EBUS-TBNA was performed under general anesthesia through a rigid scope., Results: A total of 122 patients, 84.4% males, mean age 64.2 years. Histological type: 78 (63.9%) adenocarcinoma, 33 (27.0%) squamous cell carcinoma, 11 (8.9%) undifferentiated/other NSCLC. A total of 435 lymph node stations were punctured. Median number of nodes per patient was 4. EBUS-TBNA nodal staging: 63 (51.6%) N0; 8 (6.5%) N1; 34 (27.9%) N2, and 17 (13.9%) N3. EBUS-TBNA was the primary diagnostic procedure in 27 (22.1%) patients. EBUS-TBNA NSCLC staging had a sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy rate of 83.3, 100, 100, 86.1, and 91.8%, respectively. No complications were attributable to the procedure., Conclusion: A comprehensive lung cancer staging strategy that includes EBUS-TBNA seems to be safe and effective. Our EBUS-TBNA performance and safety in this particular setting was in line with previously published reports. Additionally, our study showed that, in selected patients, lung cancer diagnosis and staging are achievable with a single endoscopic technique., (Copyright © 2017 Sociedade Portuguesa de Pneumologia. Published by Elsevier España, S.L.U. All rights reserved.)

Background: Omalizumab is indicated to treat chronic spontaneous urticaria (CSU) refractory to antihistamines. We aim to describe the experience of our department in the treatment of CSU with omalizumab., Materials and Methods: Retrospective review of the clinical records of patients., Results: Six patients (5 females, median age 33 years) treated with omalizumab for a median of 17.5 months were evaluated. All patients had improvement of symptoms after the first dose. In one case, the treatment was suspended after 7 months, but in 9 weeks there was recurrence of symptoms. The main side effect was headache in the drug administration's day. Currently, all patients maintain therapy with omalizumab and are clinically stable., Conclusion: Omalizumab proved to be an effective and safe drug for the treatment of patients with refractory CSU.

Introduction: In this study we estimated the prevalence, incidence, and mortality of myasthenia gravis (MG) in northern Portugal and characterized the clinical features of the patients identified., Methods: We used 2 data sources: clinical records from the hospitals and pyridostigmine prescription registers., Results: On December 31, 2013, we estimated a point prevalence of 111.7 patients per million population. The highest prevalence was observed in the group >65 years of age, especially in men (288.1 per million). During 2013, we estimated an incidence rate of 6.3 per million per year. Among women, the incidence rate was highest in the 15-49-year age group; in men, incidence increased with age up to 22.1 per million in those >65 years old. The MG-related mortality rate was 0.5 per million., Conclusions: These figures are in keeping with similar studies and emphasize the importance of diagnosis and management of MG in elderly populations. Muscle Nerve 54: 413-421, 2016., (© 2016 Wiley Periodicals, Inc.)

The symptoms of chronic renal disease-related mineral and bone disease improve significantly in patients after successful simultaneous pancreas-kidney transplantation (SPKT); however, bone pathology is still present even after many post-transplant years. The aim of this study was to analyze the bone densitometry in different periods after SPKT. Three-point densitometry was performed with the dual-energy X-ray absorptiometry (DXA) technique. Serum levels of alkaline phosphatase (ALP), total serum calcium, phosphate and parathyroid hormone were analyzed as markers of mineral metabolism. The study population consisted of 48 patients (28 females, 20 males) with a mean age of 35 ± 6 years and mean 24 ± 6 years of prior diabetes. Mean period of maintenance dialysis was 36 ± 26 months. The median time from SPKT and DXA measurement was 0.53, 26.2 and 41.9 months, respectively. Based on the DXA technique, 35.4 % of patients were categorized as having osteoporosis at the lumbar spine and 39.6 % at the femoral neck. Patients with diagnosed osteoporosis had significantly higher levels of ALP (OR = 1.5; 95 % CI = 1.1-2.2; p < 0.05 at the lumbar spine; OR = 1.4; 95 % CI = 1.0-1.9; p < 0.05 at the femoral neck). In addition, subjects with lumbar osteoporosis were characterized by a significantly lower body mass index (BMI) (OR = 0.5; 95 % CI = 0.3-0.9; p < 0.05). In the long-term follow-up, BMD increased significantly at the lumbar spine (T-score -1.86 ± 1.07 to -1.08 ± 0.89) and femoral neck (T-score -2.12 ± 0.78 to -1.63 ± 0.65). A multivariate linear model identified a BMI increase as a significant factor associated with improvement in BMD. Results of our study led us to conclude that, according to three-point densitometry, BMD among patients with functioning kidney and pancreas grafts improved. Increased serum levels of ALP were significantly associated with a decrease in BMD, suggesting a higher risk of osteoporosis. BMI gain was predictive of BMD improvement.

Background: Bronchiectasis is defined as an abnormal and irreversible dilation and distortion of the bronchi, which has numerous causes. Surgical treatment of this disease is usually reserved for focal disease and when the medical treatment is no longer effective. We report our center experience and outcomes in bronchiectasis surgery during the last 20 years., Methods: Between 1994 and 2014, sixty-nine patients underwent surgical resection for bronchiectasis. Patient demographics, presenting symptoms, indications for surgical treatment, type of lung resection, morbidity and mortality, as well as clinical follow-up and outcomes were analyzed., Results: From the 69 patients included, 31 (44.9%) were male and 38 (55.1%) were female. Surgery was indicated because of unsuccessful medical therapy in 33 patients (47.8%), haemoptysis in 22 patients (31.9%), nondiagnostic lung mass in 9 patients (13.0%) and lung abscess in 5 patients (7.3%). The surgical procedures were lobectomy in 45 (65.2%) patients, pneumonectomy in 10 (14.5%) patients, bilobectomy in 8 (11.6%) patients, lobectomy plus segmentectomy in 3 (4.3%) patients and only segmentectomy in 3 (4.3%) patients. Morbidity rate was 14.5% and there was no perioperative mortality. The follow-up was possible in 60 patients, with an outcome reported as excellent in 44 (73.3%) patients, as improved in 11 (18.3%) and as unchanged in 5 (8.3%)., Conclusion: Although the number of patients with bronchiectasis referred for surgical treatment has decreased, pulmonary resection still plays a significant role. Surgical resection of localized bronchiectasis is a safe procedure with proven improvement of quality of life for the majority of patients., (Copyright © 2015 Sociedade Portuguesa de Pneumologia. Published by Elsevier España, S.L.U. All rights reserved.)