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Borges T.,Centro Hospitalar Sao Joao
Reumatismo | Year: 2015

Systemic sclerosis (SSc) sine scleroderma (ssSSc) is characterized by the absence of skin involvement, despite other manifestations of systemic sclerosis are present. It is not known whether sSSc represents a forme fruste of limited cutaneous SSc or a distinct entity, but the 2013 American College of Rheumatology/European League Against Rheumatism criteria for the classification of SSc have considered SSc without skin involvement to be a distinct subset. The authors present the case of a 70-year old female that was referred for a consultation for Raynaud's phenomenon and a chronic anterior uveitis (CAU). She had a history of dysphagia, diffuse pulmonary emphysema and a biopsy-documented fibrosis of the upper lobes, and an idiopathic non-ischemic dilated cardiomyopathy with severe left ventricle systolic dysfunction and left bundle branch block. Anti-nuclear and anti-centromere antibodies were positive, while manometry revealed distal esophageal hypomotility. After establishing the diagnosis of ssSSc and starting immunosuppression, the ocular disease improved, while the lung and heart diseases remained stable. This case underlines that it is very important to suspect SSc when CAU is present and/or skin thickening is absent. To our knowledge, this is the first report of CAU in a patient with ssSSc.


Dias C.,Centro Hospitalar Sao Joao | Selmi C.,University of California at Davis | Selmi C.,University of Milan
Clinical Reviews in Allergy and Immunology | Year: 2014

Uncommon or orphan diseases are less frequently addressed in mainstream medical journals and, as a consequence, their understanding and clinical recognition may rely on case series or anecdotal data with limited guidelines and management directions. The study of selected underrepresented autoimmune and allergy conditions is the subject of the present issue of Clinical Reviews in Allergy and Immunology to provide peculiar perspectives on common and rare themes. First, allergy remains a major concern for physicians worldwide despite the limited developments over the past years, particularly for antigens such as mite or Alternaria alternata, and due to the increasing incidence of drug hypersensitivity. Second, the female predominance of autoimmune diseases such as systemic sclerosis is well recognized but enigmatic, and a unifying hypothesis remains elusive. Third, the management of conditions triggered by infectious agents as in Guillain-Barre syndrome or mixed cryoglobulinemia is challenging, and clinical guidelines are needed in the setting of infections and autoimmunity. Fourth, gamma-delta T cells represent major players in innate immunity and are the subject of extensive studies in autoimmune diseases to provide new therapeutic targets for disease prevention or modulation in the near future. Ultimately, we acknowledge the major developments in the broad fields of rheumatology and immunology and expect that microbiota definition, epigenetics studies, and microRNA analysis will provide new exciting avenues toward the understanding and treatment of chronic and acute inflammation. © 2014, Springer Science+Business Media New York.


Miranda J.O.,Evelina London Childrens Hospital | Miranda J.O.,Centro Hospitalar Sao Joao | Callaghan N.,Evelina London Childrens Hospital | Miller O.,Evelina London Childrens Hospital | 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 | 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.


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.


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.


Abreu T.,Centro Hospitalar Do Tamega e Sousa | Braganca M.,Centro Hospitalar Sao Joao
Journal of Affective Disorders | Year: 2015

Background Bipolar Disorder is characterized by episodes running the full mood spectrum, from mania to depression. Between mood episodes, residual symptoms remain, as sleep alterations, circadian cycle disturbances, emotional deregulation, cognitive impairment and increased risk for comorbidities. The present review intends to reflect about the most recent and relevant information concerning the biunivocal relation between bipolar disorder and circadian cycles. Methods It was conducted a literature search on PubMed database using the search terms "bipolar", "circadian", "melatonin", "cortisol", "body temperature", "Clock gene", "Bmal1 gene", "Per gene", "Cry gene", "GSK3β", "chronotype", "light therapy", "dark therapy", "sleep deprivation", "lithum" and "agomelatine". Search results were manually reviewed, and pertinent studies were selected for inclusion as appropriate. Results Several studies support the relationship between bipolar disorder and circadian cycles, discussing alterations in melatonin, body temperature and cortisol rhythms; disruption of sleep/wake cycle; variations of clock genes; and chronotype. Some therapeutics for bipolar disorder directed to the circadian cycles disturbances are also discussed, including lithium carbonate, agomelatine, light therapy, dark therapy, sleep deprivation and interpersonal and social rhythm therapy. Limitations This review provides a summary of an extensive research for the relevant literature on this theme, not a patient-wise meta-analysis. Conclusions In the future, it is essential to achieve a better understanding of the relation between bipolar disorder and the circadian system. It is required to establish new treatment protocols, combining psychotherapy, therapies targeting the circadian rhythms and the latest drugs, in order to reduce the risk of relapse and improve affective behaviour. © 2015 Elsevier B.V. Allrightsreserved.


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.


The physiopathology of symptoms and signs in multiple sclerosis (MS) is a less divulged topic albeit its importance in the patients' management. Objective: It was to summarize the main biophysical and biochemical mechanisms which produce the clinical manifestations in MS. Results: The mechanisms underpinning neurological deficits are described in the relapsing and in the progressive phases, stressing inflammatory and neurodegenerative components, especially demyelination, axonal damage and conduction impairment. Transient worsening based in Uhthoff's phenomenon, mechanisms producing positive symptoms, as paraesthesias and Lhermitte sign due to axonal hiperexcitability and ephaptic interactions, and development of cortical symptoms will also be addressed. The variety of processes leading to neural repair and functional recovery in the remitting phase is focused, as remyelination and adaptive changes due to neural plasticity. Conclusion: The awareness of mechanisms producing symptoms in MS emphasises the role of symptomatic and rehabilitation therapies in the improvement of patients' well-being.


Guimaraes J.,Centro Hospitalar Sao Joao | Guimaraes J.,University of Porto | Sa M.J.,Centro Hospitalar Sao Joao | Sa M.J.,Fernando Pessoa University
Frontiers in Neurology | Year: 2012

In Multiple Sclerosis (MS) prevalence studies of community and clinical samples, indicate that 45-60% of patients are cognitively impaired. These cognitive dysfunctions have been traditionally described as heterogeneous, but more recent studies suggest that there is a specific pattern of MS-related cognitive dysfunctions. With the advent of disease-modifying medications for MS and emphasis on early intervention and treatment, detection of cognitive impairment at its earliest stage becomes particularly important. In this review the authors address: the cognitive domains most commonly impaired in MS (memory, attention, executive functions, speed of information processing, and visual-spatial abilities); the pathophysiological mechanism implied in MS cognitive dysfunction and correlated brain MRI features; the importance of neuropsychological assessment of MS patients in different stages of the disease and the influence of its course on cognitive performance; the most used tests and batteries for neuropsychological assessment; therapeutic strategies to improve cognitive abilities. © 2012 Guimarães and Sá.

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