Abstract：
:Marfan syndrome is an autosomal dominant illness of the connective tissue, with typical skeletal, ocular and cardiovascular manifestations. Less frequently, pulmonary involvement occurs, namely spontaneous pneumothorax, which generally is recurrent and occurs in 4 -11% of cases. The authors describe a 14 -year -old boy, previously healthy, admitted with a unilateral spontaneous pneumothorax, and clinical phenotype of Marfan syndrome. The subsequent investigation led to the diagnosis of mitral valve prolapse and dilatation of the root of aorta. Pneumothorax resolved through active drainage and a computerised tomography scan excluded subpleural blebs. The patient was readmitted two months later with a new spontaneous pneumothorax, with an identical localization. Surgical resection of a newly identified enfisematous bleb and pleurodesis were performed. Two years later he is asymptomatic. We highlight the importance of an early diagnosis of and a multidisciplinary approach to these patients. Monitoring illness progression and prevention of serious complications, namely cardiovascular, are essential.
journal_name
Rev Port Pneumoljournal_title
Revista portuguesa de pneumologiaauthors
Rocha S,Pereira L,Barreto Cdoi
10.1016/s0873-2159(15)30279-8subject
Has Abstractpub_date
2008-09-01 00:00:00pages
699-704issue
5eissn
0873-2159issn
2172-6825pii
S0873-2159(15)30279-8journal_volume
14pub_type
杂志文章abstract：:Acinetobacter is involved in a variety of infectious diseases primarily associated with healthcare. Recently there has been increasing evidence of the important role these pathogens play in community acquired infections. We report on the case of a previously healthy child, aged 28 months, admitted for fever, cough and...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/j.rppneu.2011.07.006
更新日期：2012-03-01 00:00:00
abstract：UNLABELLED:Allergen-specific immunotherapy has been used for successful treatment of atopic diseases. They may act by modifying the patterns of cytokines produced by T cells. However, the precise mechanism by which it accomplishes these effects is still incompletely understood. OBJECTIVE:To evaluate the effect of one ...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：
更新日期：2006-03-01 00:00:00
abstract：INTRODUCTION:Tuberculosis is a leading cause of world -wide mortality and morbidity. A reduced rate of tuberculosis in people aged less than 15 years has been seen in Portugal over the last decade of the twentieth century. MATERIAL AND METHODS:Retrospective analysis of tuberculosis cases in children admitted to the Ce...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：
更新日期：2009-09-01 00:00:00
abstract：:The author proposes a comparison of tuberculids and extrapulmonary tuberculosis, highlighting the differences in definition, pathogenesis, clinical manifestations, diagnosis and treatment, proving that they are different expressions of Mycobacterium tuberculosis. The article further discusses updated information on tu...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章,评审
doi：
更新日期：2008-05-01 00:00:00
abstract：:The high activity antiretroviral treatment (HAART) induces the restoration of the number and function of CD4+ T lymphocytes and is changing the landscape of tuberculosis in patients HIV-infected. The objective of this study is to evaluate the clinical, radiographic features and evolution of these patients and compare ...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/s0873-2159(15)30578-x
更新日期：2004-05-01 00:00:00
abstract：INTRODUCTION:Congenital lobar emphysema (CLE) is a rare anomaly of lung development that presents in the neonatal period in about 50% of the cases. The authors report four clinical cases of congenital lobar emphysema in the newborn. CLINICAL CASES:Four term newborns, 3M/ 1F, were admitted for respiratory distress star...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/s0873-2159(15)30077-5
更新日期：2010-09-01 00:00:00
abstract：:Undernutrition is associated with worse clinical outcomes and so screening is recommended. Given the paucity of information on nutritional status and on the clinical impact of undernutrition in Pulmonology patients who have been hospitalized, it is of the utmost importance that it is studied. Through a longitudinal st...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/j.rppneu.2014.01.004
更新日期：2014-11-01 00:00:00
abstract：PURPOSE:Expression of ERCC1 has not been well described in fine-needle aspiration biopsies (FNABs) in patients with non-small cell lung cancer (NSCLC). We investigated the expression of ERCC1 in correlation with EGFR expression and clinicopathological factors in patients with NSCLC in order to determine if these play a...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/j.rppneu.2013.11.002
更新日期：2014-07-01 00:00:00
abstract：:The pulmonary sequestration corresponds to pulmonary tissue intra or extra-lobar with systemic arterial nutrition and collateral bronchial ramification. It is infrequent and the intra-lobar identification depends on the correct clinical signs and morphology. The correct study includes imagiologic identification of the...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/s0873-2159(15)30665-6
更新日期：2003-03-01 00:00:00
abstract：INTRODUCTION:Surgical lung biopsy is a technique that presents a morbi-mortality rate of considerable importance. We analyze our experience with surgical lung biopsies for the diagnosis of diffuse lung disease and the effect produced on the indications for surgical biopsy in these pathologies after the publication of t...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/j.rppneu.2012.11.003
更新日期：2013-03-01 00:00:00
abstract：OBJECTIVE:To analyze the effect of a Pulmonary Rehabilitation Program (PRP) on the levels of anxiety and depression and the quality of life of patients with chronic obstructive pulmonary disease. METHOD:Patients with chronic obstructive pulmonary disease (COPD) who completed the PRP of 3 weekly sessions of 60 min dura...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/j.rppneu.2014.03.007
更新日期：2014-11-01 00:00:00
abstract：INTRODUCTION:Tuberculosis (TB) is considered an occupational disease in health care workers (HCW) and its transmission in health care facilities is an important concern. Some hospital departments are at higher risk of infection. OBJECTIVE:To describe TB cases detected after TB screening in HCW from a hospital departme...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：
更新日期：2007-11-01 00:00:00
abstract：:Oxidative stress is a risk factor for bronchopulmonary dysplasia in the preterm newborn. Antioxidant defense is impaired in the preterm newborn. Oxidative stress is also involved in cell growth and development. The relationship between oxidative stress and cell growth needs to be understood before antioxidant therapy ...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章,评审
doi：10.1016/s0873-2159(15)30221-x
更新日期：2008-01-01 00:00:00
abstract：UNLABELLED:Asthma is a chronic illness of the airways that can reduce respiratory muscle strength due to the resulting hyperinflation or treatment with corticosteroids. One of the ways to evaluate this respiratory muscular weakness is the Maximal Inspiratory Pressure (PImax). METHODS:A systematic review of the databas...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章,评审
doi：
更新日期：2010-05-01 00:00:00
abstract：:We present four patients with non-small cell lung cancer treated in second and third line with Erlotinib. These are selected patients that obtained a good clinical response. Almost all presented cutaneous side effects. Rev Port Pneumol 2008; XIV (Supl 3): S65-S69. ...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/S0873-2159(15)30324-X
更新日期：2008-10-01 00:00:00
abstract：UNLABELLED:Amyotrophic lateral sclerosis (ALS) is a degenerative neurological disorder that affects motor neurons. Involvement of respiratory muscles causes the failure of the ventilator pump with more or less significant bulbar troubles. ALS course is highly variable but, in most cases, this disease entails a very sig...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章,多中心研究
doi：10.1016/j.rppneu.2014.03.001
更新日期：2014-07-01 00:00:00
abstract：:The differential diagnosis of Mycobacterium bovis is important in the control of transmission to the human population and also for treatment since M. bovis is naturally resistant to pyrazinamide. Eleven clinical isolates from the Fernando Fonseca Hospital with Spoligotypes indicative of M. bovis, through the absence o...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/s0873-2159(15)30528-6
更新日期：2005-11-01 00:00:00
abstract：:Well-differentiated fetal adenocarcinoma (WDFA) is a very uncommon malignant tumor originating in the lung. This report describes the case of a 38-year-old woman with a WDFA treated by surgery. The malignancy is low grade and associated with a good prognosis, and so it is important for clinicians to be aware of and to...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/j.rppneu.2011.06.003
更新日期：2012-01-01 00:00:00
abstract：:Benign metastasizing leiomyomas of the lung are rare smooth muscle cells tumours. We report the case of a 48 year-old female who was evaluated due to persistent cough, progressive dyspnoea and constitutional symptoms. Chest computed tomography revealed a left endobronchial mass, multiple parenchyma nodules and a pleur...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：
更新日期：2010-11-01 00:00:00
abstract：:Amyotrophic Lateral Sclerosis (ALS) is a fast progressing neuromuscular disease that affects all but the extrinsic muscles of the eye and sphincters. The main cause of morbidity and mortality are the respiratory complications that usually start in a late stage of the diseases natural history. In a small number of case...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/s0873-2159(15)30619-x
更新日期：2004-11-01 00:00:00
abstract：:Bronchiolitis obliterans (BO) is a rare disease in immunocompetent children that usually occurs after infection of the lower airways. While a diagnosis of BO was usually confirmed by lung biopsy, identification of prior lung lesion plus a typical clinical course and a suggestive chest X-ray and CT scan have replaced t...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：
更新日期：2007-07-01 00:00:00
abstract：:Legionella, as a cause of community-acquired pneumonia, is probably under-recognized because the diagnosis relies on the use of specific tests as well the existence of an in-numerous species and serogroups not easily identify by the tests available. In studies from Europe and North America, it ranged from 2 to 15 perc...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章,评审
doi：10.1016/s0873-2159(15)30499-2
更新日期：2005-03-01 00:00:00
abstract：:We conducted a review of asthma hospital admissions in mainland Portugal 2000-2007 to evaluate if one of the aims of the National Asthma Control Programme, a 20% reduction in hospital admissions in children and adolescents in 2007, had been achieved. Using the Health Services Central Administration data base we review...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/s2173-5115(09)70119-8
更新日期：2009-05-01 00:00:00
abstract：:Healthcare workers (HCWs) have an increased risk of tuberculosis (TB). Screening for latent tuberculosis infection and active TB is therefore essential in infection control programs. Tuberculin skin test (TST) and Interferon -gamma Release Assay (IGRA) were used simultaneously in 1686 HCWs between May 2007 and April 2...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：
更新日期：2010-03-01 00:00:00
abstract：:Spontaneous hemothorax is a rare and potentially lethal neurofibromatosis' complication. Several pathological mechanisms may explain the associated vasculopathy: a) direct vascular invasion from adjacent tumors such as Shwannoma, neurofibroma or neurofibrosarcoma; b) vascular dysplasia with stenotic or aneurysm format...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/s0873-2159(15)30188-4
更新日期：2009-09-01 00:00:00
abstract：:Pediatric management of complicated pleural effusion (CPE) remains controversial. Different approaches include antibiotics and chest tube drainage alone or the use of fibrinolitics, videothorascoscopy (VTC) and surgical decortication through thoracotomy. The aim of the present study was to review, evaluate and update ...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：
更新日期：2007-01-01 00:00:00
abstract：:Lung cancer is considered an environment-related disease that develops as a consequence of exposure to mutagenic agents, namely those present in tobacco. The CYP1A1 gene codifies the phase I enzyme aryl hydrocarbon hydroxilase (AHH) belonging to the cytochrome P450 system that plays a major role in the bio-activation ...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：
更新日期：2010-01-01 00:00:00
abstract：:The incidence of the syndrome may be greater than is suspected. Patients present with recurrent respiratory infections and bronchiectasis. The symptoms are non-specific and indistinguishable from those of other chronic respiratory diseases. The diagnosis is often made several years after the first clinical complaints....
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章,评审
doi：
更新日期：2007-09-01 00:00:00
abstract：:Lower lung lobe tuberculosis is less common than the classical radiological forms of pulmonary tuberculosis. However this radiological presentation has a particular relevance in patients with some associated pathological conditions, such as diabetes mellitus, HIV infection and chronic alcoholism. We make a review of t...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/s0873-2159(15)30112-4
更新日期：2009-01-01 00:00:00
abstract：:Clinical suspicion is the bedrock of a thorough clinical work-up, and a review of the clinical files with no definitive diagnosis is a must. Such a review can uncover rare diagnoses, such as Waldenstrom's macroglobulinaemia, a proliferative B-cell disease characterised by medular infiltration and monoclonocal IgM prod...
journal_title：Revista portuguesa de pneumologia
pub_type： 杂志文章
doi：10.1016/s0873-2159(15)30292-0
更新日期：2008-11-01 00:00:00
