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Miranda J.O.,Fetal Cardiology Unit | Miranda J.O.,Centro Hospitalar Sao Joao | Callaghan N.,Fetal Cardiology Unit | Miller O.,Fetal Cardiology Unit | And 2 more authors.
Heart | Year: 2014

Objective To analyse the main characteristics, associated conditions and outcome of right aortic arch (RAA) detected in fetal life, and to assess if further investigation is required in cases of isolated RAA. Methods Retrospective observational study of all fetuses diagnosed with a RAA between 2004 and 2012 at a tertiary centre for fetal cardiology. Results A RAA was identified in 98 fetuses: 27 had normal intracardiac anatomy and 71 were associated with other congenital heart disease (CHD); conotruncal anomalies being the most common. An aberrant left subclavian artery was diagnosed in 18.4% of cases, a double aortic arch in 6.1%, and 12.2% had a vascular ring confirmed after birth. Overall, an extracardiac anomaly was present in 31.6% of the patients and a chromosomal anomaly in 15.3%, with half of the latter cases being 22q11.2 microdeletion. Extracardiac and chromosomal anomalies were more commonly associated with RAA with structural CHD (39.4% and 19.7%, respectively), compared to cases of RAA with normal intracardiac anatomy (11.1% and 3.7%, respectively) ( p<0.05). First year mortality was 10.3%, with all deaths being in cases with associated structural CHD. Conclusions Detailed fetal extracardiac examination should be undertaken in all cases of RAA. Isolated RAA has a good prognosis, and in the majority of the patients it is an asymptomatic vascular variant with a relatively low risk for chromosomal anomaly. The prognosis of RAA with CHD depends on the complexity of the CHD and/or the associated extracardiac anomalies. In these cases, there is a higher risk for chromosomal anomaly, particularly 22q11.2 microdeletion.


Sa M.J.,Centro Hospitalar Sao Joao
Journal of neurology | Year: 2014

Multiple sclerosis (MS) is an incurable disease, and despite current pharmacologic treatment being effective in reducing relapse rates and lesion burden, there is little evidence that these treatments work as effectively in preventing disability progression. In such cases, non-pharmacologic techniques such as exercise therapy with rehabilitation purposes may play an important role. This systematic review of randomised controlled trials (RCTs) aims at investigating the effects of exercise therapy in MS patients. The electronic database PubMed was searched for studies indexed between February 2004 and June 2012. Studies eligibility criteria included: clinical diagnosis of MS free of exacerbation; and intervention with exercise therapy, measured as activities of daily living (ADL). Two reviewers independently screened the titles and abstracts of the references retrieved. The methodological quality of the RCTs was assessed using the Physiotherapy Evidence Database scale (PEDro scale). The PubMed search resulted in a total of 72 articles, 11 of which were included in this review. The analysis included 591 participants, of which 358 (60.6 %) were women. Patients had a mean age between 37.1 and 54.6 years. Duration of MS since diagnosis was reported in nine of the 11 studies and varied between 5.2 and 15.9 years. According to PEDro scale, nine of the 11 included studies were considered to be of high methodological quality, with scores ranging from 7 to 10. In eight of the 11 included studies, the effectiveness of exercise therapy was compared to standard care, in two it was compared to those on a waiting list, and in one, to control treatment. The results of this review suggest that exercise therapy may have a beneficial effect in patients with MS, and therefore may be recommended for the rehabilitation of these patients.


Sa M.J.,Centro Hospitalar Sao Joao | Sa M.J.,Fernando Pessoa University
Journal of Neurology | Year: 2013

Multiple sclerosis (MS) is an incurable disease, and despite current pharmacologic treatment being effective in reducing relapse rates and lesion burden, there is little evidence that these treatments work as effectively in preventing disability progression. In such cases, non-pharmacologic techniques such as exercise therapy with rehabilitation purposes may play an important role. This systematic review of randomised controlled trials (RCTs) aims at investigating the effects of exercise therapy in MS patients. The electronic database PubMed was searched for studies indexed between February 2004 and June 2012. Studies eligibility criteria included: clinical diagnosis of MS free of exacerbation; and intervention with exercise therapy, measured as activities of daily living (ADL). Two reviewers independently screened the titles and abstracts of the references retrieved. The methodological quality of the RCTs was assessed using the Physiotherapy Evidence Database scale (PEDro scale). The PubMed search resulted in a total of 72 articles, 11 of which were included in this review. The analysis included 591 participants, of which 358 (60.6 %) were women. Patients had a mean age between 37.1 and 54.6 years. Duration of MS since diagnosis was reported in nine of the 11 studies and varied between 5.2 and 15.9 years. According to PEDro scale, nine of the 11 included studies were considered to be of high methodological quality, with scores ranging from 7 to 10. In eight of the 11 included studies, the effectiveness of exercise therapy was compared to standard care, in two it was compared to those on a waiting list, and in one, to control treatment. The results of this review suggest that exercise therapy may have a beneficial effect in patients with MS, and therefore may be recommended for the rehabilitation of these patients. © 2013 Springer-Verlag Berlin Heidelberg.


Chorao P.,University of Porto | Pereira A.M.,University of Porto | Pereira A.M.,Centro Hospitalar Sao Joao | Fonseca J.A.,University of Porto
Respiratory Medicine | Year: 2014

Background: Incorrect use of inhaler devices remains an obstacle for respiratory diseases management. We aimed to evaluate the frequency of inhaler technique errors; to determine the devices perceived as the easiest and favourite to use; to study the association of device type, demographics and patient preferences with inhaler technique (IT). Methods: Cross-sectional assessment of 301 adults, with asthma (194) or chronic pulmonary obstructive disease, undergoing treatment with Aerolizer®, Autohaler®, Breezehaler®, Diskus®, Handihaler®, MDI without spacer, Miat-haler®, Novolizer®, Respimat® and/or Turbohaler®. Patients completed self-assessment questionnaires and face-to-face interview, with demonstration of inhaler technique. The rate of wrong steps (number of wrong steps ÷ number of total steps; RWS) was the primary outcome. Adjusted odds ratio (aOR) (95% confidence intervals [CI]) for presenting ≥1 IT errors were computed. Results: From the 464 inhaler technique performances, the median RWS was 18%. Turbohaler® (21%) and Diskus® (19%) were chosen as easiest and Novolizer® (18%), Diskus® (18%), Turbohaler® (17%) as favourite for daily use. Females (aOR 2.68 [95% CI 1.55-4.65]; vs. males], patients with >64 yr (aOR 2.73 [95% CI 1.15-6.48]; vs <45 yr) and patients using Aerolizer® or Handihaler® (aOR 3.24 [95% CI 1.13-9.32] and aOR 3.71 [95% CI 1.38-10.2], respectively) were more likely to perform IT errors; otherwise, no association was found, including with using the favourite device (aOR 1.43 [95% CI 0.84-2.42]). Conclusion: The frequency of inhaler technique errors was high and no device was clearly preferred over the others. Using the preferred inhaler device was not associated with less errors. © 2014 Elsevier Ltd. All rights reserved.


Liu P.,Pfizer | Ruhnke M.,Charite University Hospital | Meersseman W.,University Hospital Leuven | Paiva J.A.,Centro Hospitalar Sao Joao | And 2 more authors.
Antimicrobial Agents and Chemotherapy | Year: 2013

The pharmacokinetics of intravenous anidulafungin in adult intensive care unit (ICU) patients were assessed in this study and compared with historical data from a general patient population and healthy subjects. Intensive plasma sampling was performed over a dosing interval at steady state from 21 ICU patients with candidemia/invasive candidiasis. All patients received the recommended dosing regimen (a 200-mg loading dose on day 1, followed by a daily 100-mg maintenance dose), except for a 54-yearold 240-kg female patient (who received a daily 150-mg maintenance dose instead). Plasma samples were assayed for anidulafungin using a validated liquid chromatography-tandem mass spectrometry method. Pharmacokinetic parameters in ICU patients were calculated by a noncompartmental method. With the exclusion of the 240-kg patient, the median (minimum, maximum) age, weight, and body mass index (BMI) of 20 ICU patients were 57 (39, 78) years, 65 (48, 106) kg, and 23.3 (16.2, 33.8) kg/m2, respectively. The average anidulafungin area under the curve over the 24-hour dosing interval (AUC0-24), maximum concentration (Cmax), and clearance (CL) in 20 ICU patients were 92.7 mg · h/liter, 7.7 mg/liter, and 1.3 liters/h, respectively. The exposure in the 240-kg patient at a daily 150-mg dose was within the range observed in ICU patients overall. The average AUC0-24 and Cmax in the general patient population and healthy subjects were 110.3 and 105.9 mg · h/liter and 7.2 and 7.0 mg/liter, respectively. The pharmacokinetics of anidulafungin in ICU patients appeared to be comparable to those in the general patient population and healthy subjects at the same dosing regimen. Copyright © 2013, American Society for Microbiology. All Rights Reserved.

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