Nocardia spp. isolation in chronic lung diseases: Are there differences between patients with pulmonary nocardiosis and Nocardia colonization?

AIMS: Chronic lung diseases are a recognized risk factor for Nocardia spp. INFECTION: Nocardia spp. isolation does not inevitably imply disease, and thus colonization must be considered. Here, we aimed to analyse the differences between pulmonary nocardiosis (PN) and Nocardia spp. colonization in patients with chronic lung diseases. METHODS AND RESULTS: A retrospective study of patients with laboratory confirmation of isolation of Nocardia spp. in at least one respiratory sample was performed. Patients with PN and Nocardia spp. colonization were compared. There were 71 patients with Nocardia spp. identification, 64.8% were male, with a mean age of 67.7 ± 11.2 years. All patients had ≥1 pre-existing chronic lung disease, and 19.7% of patients were immunocompromised. PN and Nocardia spp. colonization were considered in 26.8% and 73.2% of patients, respectively. Symptoms and chest CT findings were significantly more frequent in patients with PN (p < 0.001). During follow-up time, 12 (16.9%) patients died, 6 in PN group. Immunosuppression, constitutional symptoms, haematological malignancy and PN diagnosis were associated with significantly shorter survival times, despite only immunosuppression (HR 3.399; 95% CI 1.052-10.989) and PN diagnosis (HR 3.568; 95% CI 1.078-11.910) remained associated with a higher death risk in multivariate analysis. CONCLUSIONS: PN was associated with clinical worsening, more chest CT findings and worse clinical outcomes. SIGNIFICANCE AND IMPACT OF STUDY: Nocardia spp. isolation in chronic lung disease patients is more common than expected and the differentiation between colonization and disease is crucial.

Lung Auscultation Using the Smartphone-Feasibility Study in Real-World Clinical Practice.

Conventional lung auscultation is essential in the management of respiratory diseases. However, detecting adventitious sounds outside medical facilities remains challenging. We assessed the feasibility of lung auscultation using the smartphone built-in microphone in real-world clinical practice. We recruited 134 patients (median[interquartile range] 16[11-22.25]y; 54% male; 31% cystic fibrosis, 29% other respiratory diseases, 28% asthma; 12% no respiratory diseases) at the Pediatrics and Pulmonology departments of a tertiary hospital. First, clinicians performed conventional auscultation with analog stethoscopes at 4 locations (trachea, right anterior chest, right and left lung bases), and documented any adventitious sounds. Then, smartphone auscultation was recorded twice in the same four locations. The recordings (n = 1060) were classified by two annotators. Seventy-three percent of recordings had quality (obtained in 92% of the participants), with the quality proportion being higher at the trachea (82%) and in the children's group (75%). Adventitious sounds were present in only 35% of the participants and 14% of the recordings, which may have contributed to the fair agreement between conventional and smartphone auscultation (85%; k = 0.35(95% CI 0.26-0.44)). Our results show that smartphone auscultation was feasible, but further investigation is required to improve its agreement with conventional auscultation.

The role of obstructive sleep apnea and nocturnal hypoxia as predictors of mortality in cancer patients.

BACKGROUND: Obstructive sleep apnea (OSA), due to its high prevalence, has been associated with a number of comorbidities, frequently impacting the overall course of these other diseases if left untreated. Recent studies highlight a potential association between OSA and cancer. This study investigates how OSA severity and hypoxia affect cancer prognosis, aiming to elucidate how they interplay. METHODS: Retrospective study including patients with a diagnosis of OSA after any cancer type followed up in a tertiary center during a 10-year period. OSA was mainly diagnosed after level III polysomnographic studies. RESULTS: Nocturnal hypoxia was significantly more prevalent in patients presenting lung cancer versus other malignancies and was associated with higher rates of oncologic disease progression. Overall survival was significantly lower in severe OSA patients and also in patients presenting nocturnal hypoxia. A composite hypoxia score considering both OSA severity and significant hypoxia was an independent predictor of mortality regardless of clinical cancer staging and treatment. Shorter time between cancer and OSA diagnosis was also associated with worse prognosis. CONCLUSION: This study suggests an association between OSA severity and nocturnal hypoxia and increased cancer mortality independently from possible confounding factors such as age, cancer clinical staging at diagnosis, treatment modality and also progression. Neoplastic patients with severe OSA and/or complex hypoxia seem to have lower overall survival rates than those with less severe OSA and nocturnal hypoxia.

Bone involvement in young adults with cystic fibrosis - a Portuguese cohort.

INTRODUCTION: Long-term survivors of cystic fibrosis (CF) have a dramatic increase in the risk of osteoporosis and incident fracture. The objective of this work is to characterize a CF related bone disease in a Portuguese cohort of CF patients. METHODS: We performed a cross-sectional, observational study on a cohort of CF adult patients. Clinical status, laboratory parametres, nutrition, lung function tests, genetics and bone mineral density (BMD) data were collected from a CF reference centre. RESULTS: Of 30 patients, 53.3% were males (n=16). Median age was 32.5 (27.0; 32,5) and median body mass index (BMI) was 22,04 (19,85; 24,55), with 4 patients (13.3%) being underweight (BMI<18.5 kg/m²). Four patients (13.3 %) were diagnosed with osteoporosis and 15 patients (50%) has low BMD. Among them, 2 (6.7%) had fragility fractures. A moderate correlation was found between the lumbar spine (LS) BMD and BMI and, as expected, femural neck BMD and LS BMD has moderate to strong correlations with BMD Z scores. CONCLUSION: Despite the young middle age we found a high prevalence of low BMD and osteoporosis in patients with CF. Early recognition and treatment are the most effective strategies for reducing the morbidity due to osteoporosis in these patients.

Chronic Bacterial Infection Prevalence, Risk Factors, and Characteristics: A Bronchiectasis Population-Based Prospective Study.

BACKGROUND: Few data are available on chronic bacterial infections (CBI) in bronchiectasis patients. Given that CBI seems to trigger longer hospital stays, worse outcomes, and morbimortality, this study was undertaken to assess CBI prevalence, characteristics, and risk factors in outpatients with bronchiectasis. METHODS: A total of 186 patients followed in a bronchiectasis tertiary referral centre in Portugal were included. Demographic data and information on aetiology, smoking history, mMRC score, Bronchiectasis Severity Index (BSI) score, sputum characteristics, lung function, exacerbations, and radiological involvement degree were collected. RESULTS: Patients included (mean age 54.7 ± 16.2 years; 60.8% females) were followed up for a period of 3.8 ± 1.7 years. The most common cause of bronchiectasis was infection (31.7%) followed by immune deficiencies (11.8%), whereas in 29% of cases, no cause was identified. Haemophilus influenzae (32.3%) and Pseudomonas aeruginosa (30.1%) were the most common CBI-associated possible pathogenic microorganisms. CBI patients presented a higher follow-up time than no-CBI patients (p = 0.003), worse lung function, BSI (p < 0.001), and radiological (p < 0.001) scores, and more prominent daily sputum production (p = 0.002), estimated mean volume (p < 0.001), and purulent sputum (p < 0.001). The number of exacerbations/year (p = 0.001), including those requiring hospital admission (p = 0.009), were also higher in the CBI group. Independent CBI predictors were BSI score (OR 3.577, 95% CI 1.233⁻10.378), sputum characteristics (OR 3.306, 95% CI 1.107⁻9.874), and radiological score (OR 1.052, 95% CI 1.004⁻1.102). CONCLUSION: According to the CBI status, two different sub-groups of patients were found on the basis of several clinical outcomes, emphasizing the importance of routine sputum microbiological monitoring. Further studies are needed to better characterize CBI profiles and to define the individual clinical impact of the most prevalent pathogenic microorganisms.

Health-Related Quality of Life in Patients With α1 Antitrypsin Deficency: A Cross Sectional Study. / Calidad de vida relacionada con la salud en pacientes con déficit de α1 antitripsina: estudio transversal.

BACKGROUND: Measures of health related quality of life (HRQoL) in patients with α1-antitrypsin deficiency (AATD) can help to determine the impact of the disease and provide an important insight into the intervention outcomes. There is few data regarding this issue in the literature. The aim of this study is to assess the relationship between HRQoL and gender, functional parameters and history of hospitalizations in patients with AATD. METHODS: This is a cross-sectional study of 26 patients with severe AATD recruited in the pulmonology outpatient clinic at a tertiary care medical center. Social-demographic, clinical and functional parameters were recorded and HRQoL was assessed with the Portuguese version of the medical outcome study short form-36 (SF-36) self-administered questionnaire. RESULTS: Older patients, females and patients with at least one hospitalization in the previous year due to respiratory disease had statistical lower scores in some dimensions of the SF-36 questionnaire. Superior FEV1 and higher distance mark in the 6-min walking test distance influenced positively several dimensions of the questionnaire. Higher scores in the mMRC scale influenced negatively the HRQoL. CONCLUSIONS: These data suggests that older and female patients with AATD have worse HRQoL. Hospitalizations and functional markers of respiratory disease progression influenced negatively the HRQoL, suggesting that the SF-36 questionnaire could be useful as an outcome for AATD patients with lung involvement.

Global impact of bronchiectasis and cystic fibrosis.

EDUCATIONAL AIMS: To recognise the clinical and radiological presentation of the spectrum of diseases associated with bronchiectasis.To understand variation in the aetiology, microbiology and burden of bronchiectasis and cystic fibrosis across different global healthcare systems.Bronchiectasis is the term used to refer to dilatation of the bronchi that is usually permanent and is associated with a clinical syndrome of cough, sputum production and recurrent respiratory infections. It can be caused by a range of inherited and acquired disorders, or may be idiopathic in nature. The most well recognised inherited disorder in Western countries is cystic fibrosis (CF), an autosomal recessive condition that leads to progressive bronchiectasis, bacterial infection and premature mortality. Both bronchiectasis due to CF and bronchiectasis due to other conditions are placing an increasing burden on healthcare systems internationally. Treatments for CF are becoming more effective leading to more adult patients with complex healthcare needs. Bronchiectasis not due to CF is becoming increasingly recognised, particularly in the elderly population. Recognition is important and can lead to identification of the underlying cause, appropriate treatment and improved quality of life. The disease is highly diverse in its presentation, requiring all respiratory physicians to have knowledge of the different "bronchiectasis syndromes". The most common aetiologies and presenting syndromes vary depending on geography, with nontuberculous mycobacterial disease predominating in some parts of North America, post-infectious and idiopathic disease predominating in Western Europe, and post-tuberculosis bronchiectasis dominating in South Asia and Eastern Europe. Ongoing global collaborative studies will greatly advance our understanding of the international impact of bronchiectasis and CF.

Pleuroparenchymal fibroelastosis: role of high-resolution computed tomography (HRCT) and CT-guided transthoracic core lung biopsy.

OBJECTIVES: Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia (IIP) with variable clinical and radiological features. Diagnosis is based on histology obtained by surgical lung biopsy, which is associated with significant mortality and morbidity. This study aims to briefly review PPFE and discuss the role of CT-guided transthoracic core lung biopsy (TTB) in its diagnosis. MATERIALS/METHODS: Four cases of PPFE diagnosed at our institution with TTB are reported and discussed. RESULTS: Clinical, radiological and histological features are in agreement with the previous literature cases. TTB provided the diagnosis in all cases. Iatrogenic pneumothorax was the main complication in all patients. Placement of a chest tube was needed in three patients. An overlap between PPFE and other interstitial lung diseases (ILD) was documented. CONCLUSION: PPFE is an underdiagnosed IIP, so radiologist awareness of it needs to be widespread in patients with fibrosis with apical-caudal distribution. Coexistence of different lung diseases strengthens the idea of a predisposing factor. TTB proved to be a good diagnostic tool and can be considered the first choice for invasive assessment of these patients. PFFE has a variable course with no established therapeutic options; therefore a multidisciplinary team is crucial in the approach to patients with ILD. MAIN MESSAGES/TEACHING POINTS: • PPFE should be considered in the differential diagnosis of fibrosis with apical-caudal distribution. • CT-guided TTB can be considered the first choice for invasive assessment of PPFE. • Site of biopsy has to be chosen carefully in order not to miss PPFE. • Coexistence of different lung diseases strengthens the idea of a predisposing factor. • A multidisciplinary team is crucial in the approach to patients with ILD.

Influence of emphysema distribution on pulmonary function parameters in COPD patients.

OBJECTIVE: To evaluate the impact that the distribution of emphysema has on clinical and functional severity in patients with COPD. METHODS: The distribution of the emphysema was analyzed in COPD patients, who were classified according to a 5-point visual classification system of lung CT findings. We assessed the influence of emphysema distribution type on the clinical and functional presentation of COPD. We also evaluated hypoxemia after the six-minute walk test (6MWT) and determined the six-minute walk distance (6MWD). RESULTS: Eighty-six patients were included. The mean age was 65.2 ± 12.2 years, 91.9% were male, and all but one were smokers (mean smoking history, 62.7 ± 38.4 pack-years). The emphysema distribution was categorized as obviously upper lung-predominant (type 1), in 36.0% of the patients; slightly upper lung-predominant (type 2), in 25.6%; homogeneous between the upper and lower lung (type 3), in 16.3%; and slightly lower lung-predominant (type 4), in 22.1%. Type 2 emphysema distribution was associated with lower FEV1, FVC, FEV1/FVC ratio, and DLCO. In comparison with the type 1 patients, the type 4 patients were more likely to have an FEV1 < 65% of the predicted value (OR = 6.91, 95% CI: 1.43-33.45; p = 0.016), a 6MWD < 350 m (OR = 6.36, 95% CI: 1.26-32.18; p = 0.025), and post-6MWT hypoxemia (OR = 32.66, 95% CI: 3.26-326.84; p = 0.003). The type 3 patients had a higher RV/TLC ratio, although the difference was not significant. CONCLUSIONS: The severity of COPD appears to be greater in type 4 patients, and type 3 patients tend to have greater hyperinflation. The distribution of emphysema could have a major impact on functional parameters and should be considered in the evaluation of COPD patients.

Bronchiectasis Guidelines-Recommendations Into Practice / Recomendaciones de las guías de bronquiectasias llevadas a la práctica clínica

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WITHDRAWN: Idiopathic pleuroparenchymal fibroelastosis: A rare but increasingly recognized entity.

This article has been withdrawn for editorial reasons because the journal will be published only in English. In order to avoid duplicated records, this article can be found at http://dx.doi.org/10.1016/j.rppnen.2014.04.008. The Publisher apologizes for any inconvenience this may cause. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.

Tuberculosis en los portugueses residentes en el extranjero / Tuberculosis Among Portuguese Living Abroad

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Calidad de vida relacionada con la salud en pacientes con déficit de alfa1 antitripsina: estudio transversal / Health-related quality of life in patients with alpha1 antitrypsin deficency: a cross sectional study

Introducción: Las medidas de calidad de vida relacionada con la salud (CVRS) pueden ayudar a determinar los efectos de la enfermedad de los pacientes con déficit de α1-antitripsina (DAAT) y proporcionar una perspectiva valiosa de los resultados de las intervenciones. Este tema se ha abordado poco en la literatura, y el objetivo de este estudio fue evaluar si existe alguna relación entre la CVRS y el sexo, los parámetros funcionales y los antecedentes de hospitalización de los pacientes con DAAT. Métodos: Para este estudio transversal se reclutaron 26 pacientes con DAAT grave que eran atendidos en las consultas externas de neumología de un hospital terciario. Se registraron parámetros sociodemográficos, clínicos y funcionales, y se evaluó la CVRS mediante la versión portuguesa del cuestionario de salud SF-36. Resultados: Los pacientes de mayor edad, de sexo femenino y los que habían sido hospitalizados por enfermedad respiratoria al menos una vez durante el año anterior mostraron puntuaciones más bajas en algunas dimensiones del cuestionario SF-36. Los valores más altos de FEV1 y distancias recorridas más largas en la prueba de la marcha de 6min tuvieron una influencia positiva sobre varias dimensiones del cuestionario, mientras que las puntuaciones más altas en la escala MRCm influyeron negativamente en la CVRS. Conclusiones: Los resultados muestran que la CVRS de los pacientes de mayor edad y las mujeres con DAAT es peor. Las hospitalizaciones y los marcadores funcionales de progresión de la enfermedad respiratoria tuvieron una influencia negativa sobre la CVRS, lo que indica que el cuestionario SF-36 podría ser de utilidad como medida de resultados de los pacientes con DAAT y afectación pulmonar

Background: Measures of health related quality of life (HRQoL) in patients with α1-antitrypsin deficiency (AATD) can help to determine the impact of the disease and provide an important insight into the intervention outcomes. There is few data regarding this issue in the literature. The aim of this study is to assess the relationship between HRQoL and gender, functional parameters and history of hospitalizations in patients with AATD. Methods: This is a cross-sectional study of 26 patients with severe AATD recruited in the pulmonology outpatient clinic at a tertiary care medical center. Social-demographic, clinical and functional parameters were recorded and HRQoL was assessed with the Portuguese version of the medical outcome study short form-36 (SF-36) self-administered questionnaire. Results: Older patients, females and patients with at least one hospitalization in the previous year due to respiratory disease had statistical lower scores in some dimensions of the SF-36 questionnaire. Superior FEV1 and higher distance mark in the 6-min walking test distance influenced positively several dimensions of the questionnaire. Higher scores in the mMRC scale influenced negatively the HRQoL. Conclusions: These data suggests that older and female patients with AATD have worse HRQoL. Hospitalizations and functional markers of respiratory disease progression influenced negatively the HRQoL, suggesting that the SF-36 questionnaire could be useful as an outcome for AATD patients with lung involvement

Cardiomegalia, ¿o no? / A big heart, or not?

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Influence of emphysema distribution on pulmonary function parameters in COPD patients / Influência da distribuição do enfisema nos parâmetros de função pulmonar em pacientes com DPOC

ABSTRACT OBJECTIVE: To evaluate the impact that the distribution of emphysema has on clinical and functional severity in patients with COPD. METHODS: The distribution of the emphysema was analyzed in COPD patients, who were classified according to a 5-point visual classification system of lung CT findings. We assessed the influence of emphysema distribution type on the clinical and functional presentation of COPD. We also evaluated hypoxemia after the six-minute walk test (6MWT) and determined the six-minute walk distance (6MWD). RESULTS: Eighty-six patients were included. The mean age was 65.2 ± 12.2 years, 91.9% were male, and all but one were smokers (mean smoking history, 62.7 ± 38.4 pack-years). The emphysema distribution was categorized as obviously upper lung-predominant (type 1), in 36.0% of the patients; slightly upper lung-predominant (type 2), in 25.6%; homogeneous between the upper and lower lung (type 3), in 16.3%; and slightly lower lung-predominant (type 4), in 22.1%. Type 2 emphysema distribution was associated with lower FEV1, FVC, FEV1/FVC ratio, and DLCO. In comparison with the type 1 patients, the type 4 patients were more likely to have an FEV1 < 65% of the predicted value (OR = 6.91, 95% CI: 1.43-33.45; p = 0.016), a 6MWD < 350 m (OR = 6.36, 95% CI: 1.26-32.18; p = 0.025), and post-6MWT hypoxemia (OR = 32.66, 95% CI: 3.26-326.84; p = 0.003). The type 3 patients had a higher RV/TLC ratio, although the difference was not significant. CONCLUSIONS: The severity of COPD appears to be greater in type 4 patients, and type 3 patients tend to have greater hyperinflation. The distribution of emphysema could have a major impact on functional parameters and should be considered in the evaluation of COPD patients.

RESUMO OBJETIVO: Avaliar o impacto que a distribuição do enfisema tem na gravidade clínica e funcional em pacientes com DPOC. MÉTODOS: A distribuição do enfisema foi analisada em pacientes com DPOC, que foram classificados de acordo com um sistema de classificação visual de cinco pontos a partir de achados de TC de tórax. Avaliou-se a influência do tipo de distribuição do enfisema na apresentação funcional e clínica da DPOC. Hipoxemia após o teste da caminhada de seis minutos (TC6) foi também avaliada e a distância percorrida (DTC6) foi determinada. RESULTADOS: Foram incluídos 86 pacientes. A média de idade foi de 65,2 ± 12,2 anos, 91,9% eram homens, e todos menos um eram fumantes (média de carga tabágica, 62,7 ± 38,4 anos-maço). A distribuição do enfisema foi categorizada como obviamente predominante no pulmão superior (tipo 1), em 36,0% dos pacientes; levemente predominante no pulmão superior (tipo 2), em 25,6%; homogêneo entre o pulmão superior e inferior (tipo 3), em 16,3%; e levemente predominante no pulmão inferior (tipo 4), em 22,1%. A distribuição do enfisema do tipo 2 foi associada a menores valores de VEF1, CVF, relação VEF1/CVF e DLCO. Em comparação com os pacientes do tipo 1, os do tipo 4 apresentaram maior probabilidade de ter VEF1 < 65% do previsto (OR = 6,91, IC95%: 1,43-33,45; p = 0,016), DTC6 < 350 m (OR = 6,36, IC95%: 1,26-32,18; p = 0,025),e hipoxemia após o TC6 (OR = 32,66, IC95%: 3,26-326,84; p = 0,003). Os pacientes do tipo 3 tiveram uma relação VR/CPT maior, embora sem diferença significativa. CONCLUSÕES: A gravidade da DPOC parece ser maior nos pacientes do tipo 4, e os do tipo 3 tendem a apresentar maior hiperinsuflação. A distribuição do enfisema pode ter um impacto importante nos parâmetros funcionais e deve ser considerada na avaliação de pacientes com DPOC.