Revista Portuguesa de Pneumologia

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Pompe disease is a rare autosomal recessive neuromuscular disorder caused by acid α-glucosidase enzyme (GAA) deficiency and divided into two distinct variants, infantile- and late-onset. The late-onset variant is characterized by a spectrum of phenotypic variation that may range from asymptomatic, to reduced muscle strength and/or diaphragmatic paralysis. Since muscle strength loss is characteristic of several different conditions, which may also cause diaphragmatic paralysis, a protocol was created to search for the diagnosis of Pompe disease and exclude other possible causes...

OBJECTIVE: Quality of life (QOL), sexual satisfaction (SS) and physical performance have been assessed in the management of numerous chronic diseases. METHODS: In this study, the following tests and surveys were applied: (i) QOL questionnaire [Cystic Fibrosis Questionnaire (CFQ)]; (ii) SS questionnaire (SSQ) [female sexual quotient (FSQ) and male sexual quotient (MSQ)]; (iii) 6-minute walk test (6MWT). Spearman's correlation was used for comparison between the data; the Mann-Whitney test was applied to analyze the difference between genders...

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Second-hand tobacco smoke (SHS) exposure is a major and entirely avoidable health risk for children's health, well-being and development. The main objective of the current study was to investigate the association between parents' educational level and children's SHS home exposure. A self-administered questionnaire was conducted within a sample of 949 students in 4th grade (mean age 9.56±0.75, 53.4% male). The sample was randomly selected from all schools located at Lisbon District, Portugal. The current study confirmed that Portuguese children are exposed to unacceptable high levels of SHS at home, mainly by their parents' smoke...

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BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive, fatal disease. Long-term outcomes data are scarce in Portugal. We aimed to estimate survival of newly diagnosed PAH at a Portuguese referral center in the modern management era. METHODS: Between January 2009 and November 2015 all incident PAH cases were consecutively enrolled in a prospective cohort study. Sixty-five patients were followed up for a median of 3.1 [interquartile range 1.7-5.4] years...

BACKGROUND: An outbreak of Legionella pneumophila serogroup 1, with 403 cases was identified on the 7th November 2014 in Vila Franca de Xira, Portugal. Outbreak source was the wet cooling system of a local factory. Hospital Pulido Valente was one of the hospitals receiving patients with Legionnaires' disease (LD). METHODS: We describe the clinical findings and diagnostic methods used among the 43 confirmed or probable cases admitted to our department. RESULTS: 60...

BACKGROUND: Despite the importance of traditional pulmonary function tests (PFTs) in managing systemic sclerosis (SSc), many patients with pulmonary disease diagnosed by computed tomography (CT) present with normal PFTs. OBJECTIVE: To evaluate the efficacy of the nitrogen single-breath washout (N2 SBW) test in diagnosing SSc and to correlate N2 SBW parameters with the PFT indexes used in the follow-up of these patients, clinical data, and CT findings. METHODS: Cross-sectional study in which 52 consecutive SSc patients were subjected to spirometry, body plethysmography, analysis of the diffusing capacity for carbon monoxide (DLCO), analysis of respiratory muscle strength, N2 SBW testing, and CT analysis...