Enfisema pulmonar congênito: relato de caso / Congenital pulmonary enphysema: case report

Introdução: O enfisema pulmonar congênito (EPC) é uma doença rara, possuindo uma incidência de 1:20-30 mil nascimentos, é mais comum no sexo masculino do que no feminino, em uma razão de 3:1 e sua etiologia permanece desconhecida. Um terço dos casos são sintomáticos ao nascer e praticamente todos são diagnosticados nos primeiros seis meses de vida. Relato de Caso: Recém-nascido (RN) do sexo masculino, evoluiu com desconforto respiratório precoce, sendo encaminhado ao centro de terapia intensiva. Após uso de continuous positive airway pressure (CPAP) e cateter nasal de oxigênio de 12 horas, resultou em bom padrão respiratório e boa saturação. Após realização de tomografia computadorizada, foi diagnosticado o EPC. Conclusão: O EPC é uma patologia rara e deve ser suspeitado em RN com desconforto respiratório, atribuindo-se importância aos vários diagnósticos diferenciais possíveis. Apesar da etiologia incerta, é de fácil diagnóstico e possui opções de manejo clínico e cirúrgico.

Introduction: Congenital pulmonary emphysema (EPC) is a rare disease, with an incidence of 1:20-30 thousand births, it is more common in males than in females, in a ratio of 3:1 and its etiology remains unknown. One third of the cases are symptomatic at birth and practically all are diagnosed in the first six months of life. Case Report: Newborn (NB) male, developed early respiratory distress, being referred to the intensive care unit. After using continuous positive airway pressure (CPAP) and a 12-hour oxygen nasal catheter, it resulted in a good breathing pattern and good saturation. After performing computed tomography, EPC was diagnosed. Conclusion: EPC is a rare pathology and should be suspected in newborns with respiratory distress, with importance being given to the various possible differential diagnoses. Despite its uncertain etiology, it is easy to diagnose and has options for clinical and surgical management.

Risk factors associated with the detection of pulmonary emphysema in older asymptomatic respiratory subjects.

BACKGROUND: Several lung structural and functional abnormalities may occur associated with aging, including emphysema. In this study, we evaluated the frequency and risk factors associated with emphysema in respiratory asymptomatic individuals enrolled in our Lung Aging Program. From a cohort of 687 subjects, we found by high-resolution computed tomography (HRCT) 29 individuals (4%) with emphysematous changes that were compared with 87 controls (3:1) randomly selected from the same cohort. METHODS: This was a transversal, observational, case-control study where we examined demographics and functional characteristics, as well as telomere length and serum Klotho concentration, two conditions that have been associated with aging and some aging-associated diseases including emphysema. RESULTS: Individuals with subclinical pulmonary emphysema were older (72 ± 9 versus 67 ± 6 years), and primarily smoker males with low body mass index. Despite that they were asymptomatic, two of them exhibited a decrease of forced expiratory volume in 1 s (FEV1), with a lower FEV1/FVC suggesting airway obstruction. Cigarette smoking (OR = 5.43, CI95% 1.8-16.7), family history of lung disease (OR = 4.32, CI95% 1.0-19.0) and lower body mass index (OR 7.22, CI95% 1.2-3.5) were risk factors for the development of lung emphysematous changes. No association was found with telomere length and Klotho serum concentration. CONCLUSION: Our findings reveal that a small but important percentage of older people without respiratory symptoms, present pulmonary emphysema and indicate that smoking exposure and genetic background may contribute to etiological factors.

Quantitative computed tomography phenotypes, spirometric parameters, and episodes of exacerbation in heavy smokers: An analysis from South America.

OBJECTIVE: To evaluate the quantitative computed tomography (QCT) phenotypes, airflow limitations, and exacerbation-like episodes in heavy smokers in Southern Brazil. METHODS: We enrolled 172 smokers with a smoking history ≥30 pack-years who underwent pulmonary function tests (PFTs) and CT scan for lung cancer screening. Patients were classified regarding airflow limitation (FEV1/FVC <0.7 forced expiratory volume in 1 second/forced vital capacity) and the presence of emphysema on the QCT. The QCT were analyzed in specialized software and patients were classified in two disease-predominant phenotypes: emphysema-predominant (EP) and non-emphysema-predominant (NEP). EP was determined as ≥6% of percent low-attenuation areas (LAA%) with less than -950 Hounsfield units. NEP was defined as having a total LAA% of less than 6%. RESULTS: Most of our patients were classified in the EP phenotype. The EP group had significantly worse predicted FEV1 (60.6 ±22.9 vs. 89.7 ±15.9, p <0.001), higher rates of airflow limitation (85.7% vs. 15%; p <0.001), and had more exacerbation-like episodes (25.8% vs. 8.3%, p <0.001) compared to the NEP group. Smoking history, ethnicity, and BMI did not differ between the groups. The total LAA% was the QCT parameter with the strongest correlation to FEV1 (r = -0.669) and FEV1/FVC (r = -0.787). CONCLUSIONS: Heavy smokers with the EP phenotype on QCT were more likely to have airflow limitation, worse predicted FEV1, and a higher rate of exacerbation-like episodes than those with the NEP phenotype. Approximately 23% of patients with no airflow limitation on PFTs were classified in EP phenotype.

Geographic variations of the prevalence and distribution of COPD phenotypes in Spain: "the ESPIRAL-ES study".

PURPOSE: The purpose of this study was to assess the prevalence of COPD phenotypes at a national level and to determine their geographic distribution among different autonomous communities in Spain. PATIENTS AND METHODS: A total of 1,610 patients (82% men, median age 67 years) recruited in primary care centers and pneumology services participated in an observational, cross-sectional, and multicenter study. Phenotypes evaluated were the non-exacerbator phenotype, the asthma-COPD overlap syndrome (ACOS), the exacerbator phenotype with emphysema, and the exacerbator phenotype with chronic bronchitis. RESULTS: The non-exacerbator phenotype was the most common (46.7%) followed by exacerbator with chronic bronchitis (22.4%) and exacerbator with emphysema (16.4%). The ACOS phenotype accounted for the lowest rate (14.5%). For each phenotype, the highest prevalence rates were concentrated in two or three autonomous communities, with relatively similar rates for the remaining regions. Overall prevalence rates were higher for the non-exacerbator and the exacerbator with chronic bronchitis phenotypes than for ACOS and the exacerbator with chronic bronchitis phenotypes. Differences in the distribution of COPD phenotypes according to gender, age, physician specialty, smoking habit, number of comorbidities, quality of life assessed with the COPD Assessment Test, and BODEx index (body mass index, airflow obstruction, dyspnea, and exacerbations) were all statistically significant. CONCLUSION: Differences in the prevalence rates of COPD phenotypes among the Spanish autonomous communities have been documented. Mapping the distribution of COPD phenotypes is useful to highlight regional differences as starting point for comparisons across time. This geographic analysis provides health-care planners a valuable platform to assess changes in COPD burden at nationwide and regional levels.

Pulmonary interstitial emphysema in fatal asthma: case report and histopathological review.

BACKGROUND: Mortality related to asthma has decreased worldwide since the introduction of inhaled corticosteroid therapy in the past decades. However, there are still some asthma fatalities identified mainly in populations with less access to regular treatment. Pulmonary interstitial emphysema due to alveolar rupture has been rarely described as a complication of an acute severe asthma attack, and its identification in pathological analysis can be difficult. Previous studies reported the association of pulmonary interstitial emphysema and bronchial ductal gland ectasia in asthma. CASE PRESENTATION: We present the case of a 42-year- old man that died due to a fatal asthma attack. Postmortem computed tomography revealed the unusual finding of acute Pulmonary Interstitial Emphysema, confirmed by pathological analysis. We reviewed 28 cases of fatal asthma tissue and identified the presence of pulmonary interstitial emphysema in 10% of the cases. CONCLUSIONS: Postmortem computed tomography is a useful complimentary diagnostic tool for autopsies. Pulmonary Interstitial Emphysema in acute exacerbations of asthma seems to be more frequent than reported. Alveolar hyperdistension and bronchial duct gland ectasia causing tissue rupture are possible mechanisms involved in the formation of Pulmonary Interstitial Emphysema. The clinical impact of Pulmonary Interstitial Emphysema in asthma is unknown.

Mesenchymal stromal cell therapy in COPD: from bench to bedside.

COPD is the most frequent chronic respiratory disease and a leading cause of morbidity and mortality. The major risk factor for COPD development is cigarette smoke, and the most efficient treatment for COPD is smoking cessation. However, even after smoking cessation, inflammation, apoptosis, and oxidative stress may persist and continue contributing to disease progression. Although current therapies for COPD (primarily based on anti-inflammatory agents) contribute to the reduction of airway obstruction and minimize COPD exacerbations, none can avoid disease progression or reduce mortality. Within this context, recent advances in mesenchymal stromal cell (MSC) therapy have made this approach a strong candidate for clinical use in the treatment of several pulmonary diseases. MSCs can be readily harvested from diverse tissues and expanded with high efficiency, and have strong immunosuppressive properties. Preclinical studies have demonstrated encouraging outcomes of MSCs therapy for lung disorders, including emphysema. These findings instigated research groups to assess the impact of MSCs in human COPD/emphysema, but clinical results have fallen short of expectations. However, MSCs have demonstrated a good adjuvant role in the clinical scenario. Trials that used MSCs combined with another, primary treatment (eg, endobronchial valves) found that patients derived greater benefit in pulmonary function tests and/or quality of life reports, as well as reductions in systemic markers of inflammation. The present review summarizes and describes the more recent preclinical studies that have been published about MSC therapy for COPD/emphysema and discusses what has already been applied about MSCs treatment in COPD patients in the clinical setting.

Pulmonary placental transmogrification associated with adenocarcinoma of the lung: a case report with a comprehensive review of the literature

Pulmonary placental transmogrification (PT) is a rare entity with less than 40 cases reported in the literature. Most reported cases are associated with either bullous emphysema or with pulmonary fibrochondromatous hamartomas. We present only the second case of PT associated with adenocarcinoma of the lung. A 67-year-old female with multiple chronic medical ailments presented with shortness of breath and was found to have a 6-cm mass in the upper lobe of her right lung. A computed tomography (CT) guided core biopsy was performed that showed a well-differentiated adenocarcinoma. Interestingly the normal lung tissue showed placental villous architecture. A unique feature of our case is that the diagnosis was made on a needle core biopsy, unlike all the other cases in the literature. We also provide a comprehensive review of this rare entity.

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.

[Rheumatoid arthritis and combined pulmonary fibrosis and emphysema]. / Artritis reumatoidea y síndrome combinado de fibrosis pulmonar y enfisema.

The combination of pulmonary fibrosis and emphysema is a syndrome described in the last years, which has its own characteristics and it is not only the casual association between the two entities. The idiopathic pulmonary fibrosis is the most common type of pulmonary fibrosis. However other interstitial lung diseases could be part of this syndrome. Among them is the connective tissue disease-associated interstitial lung disease. We report a case of this syndrome associated with rheumatoid arthritis. It has the peculiarity that the connective disease became overt several years after the presentation of combined pulmonary fibrosis and emphysema syndrome, which is infrequently reported in the literature.

Artritis reumatoidea y síndrome combinado de fibrosis pulmonar y enfisema / Rheumatoid arthritis and combined pulmonary fibrosis and emphysema

La combinación de fibrosis pulmonar y enfisema es un síndrome descripto en los últimos años que tiene características propias y no es la casual asociación de dos entidades. El componente de fibrosis más común corresponde a la fibrosis pulmonar idiopática. Sin embargo, otras enfermedades intersticiales pueden formar parte de este síndrome, entre ellas las asociadas a enfermedades del tejido conectivo. Se presenta un caso de este síndrome asociado a artritis reumatoidea con la particularidad que la misma se hizo evidente varios años después del síndrome combinado fibrosis pulmonar y enfisema, hecho muy poco comunicado en la literatura.

The combination of pulmonary fibrosis and emphysema is a syndrome described in the last years, which has its own characteristics and it is not only the casual association between the two entities. The idiopathic pulmonary fibrosis is the most common type of pulmonary fibrosis. However other interstitial lung diseases could be part of this syndrome. Among them is the connective tissue disease-associated interstitial lung disease. We report a case of this syndrome associated with rheumatoid arthritis. It has the peculiarity that the connective disease became overt several years after the presentation of combined pulmonary fibrosis and emphysema syndrome, which is infrequently reported in the literature.

Knowledge about COPD among users of primary health care services.

BACKGROUND: Chronic obstructive pulmonary disease (COPD) is often underdiagnosed, which might be attributable to a lack of knowledge about the disease among the general population. The objective of this study was to evaluate COPD-related knowledge among primary care users in an urban area in Brazil. METHODS: This study was carried out at primary care clinics (PCCs), including 12 general PCCs and 26 family health PCCs, in the city of Goiânia, Brazil. Between May 2013 and February 2014, we interviewed 674 PCC users, applying a questionnaire designed to assess COPD-related knowledge. Satisfactory knowledge of COPD was defined as knowing at least two of its symptoms and that smoking is a risk factor for the disease. RESULTS: Of the 674 users interviewed, only 9.2% recognized the term "COPD", 75.1% recognized the term "emphysema", and 15.7% did not recognize either term. We found that recognizing either term was associated with a higher level of education (P<0.001). The prevalence of satisfactory knowledge of COPD was 16.2%, and having such knowledge was associated with being over 60 years of age. The COPD symptom known by the greatest proportion of users (70.6%) was dyspnea, and most (87.5%) knew that smoking is a risk factor, whereas only a few (4.9%) knew that exposure to wood smoke is also a risk factor. The most frequently cited sources of knowledge were the media (43.1%) and a relative with COPD (36.4%). CONCLUSION: Most of the PCC users evaluated did not know the term "COPD" but were familiar with the term "emphysema". The level of basic knowledge about the disease was low in this population. These results should alert health care administrators to the need for interventions aimed at increasing the diagnosis rate and thus promoting the early treatment of COPD.

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.

Tratamento do enfisema pulmonar avançado: Cirurgia redutora de volume pulmonar ou broncoscopia? / Treatment of advanced emphysema: Surgery or bronchoscopy?

A doença pulmonar obstrutiva crônica (DPOC) é uma grande causa de morbidade e mortalidade. O enfisema pulmonar, uma das formas clínicas da DPOC, se caracteriza pelo desenvolvimento progressivo de aprisionamento aéreo, alterações da arquitetura alveolar e hiperinsuflação pulmonar. Sua história natural é a evolução para a doença pulmonar terminal e necessidade de transplante pulmonar. Entretanto, sabe-se que uma redução da hiperinsuflação pulmonar é capaz de reestabelecer algumas das propriedades mecânicas do sistema respiratório e assim melhorar os volumes e a função pulmonar destes pacientes, com melhora da qualidade de vida e sobrevida. Há pelo menos duas formas de tratamento invasivo,baseadas nestes princípios fisiopatológicos, disponíveis na prática clínica: a cirurgia redutora do volume pulmonar (CRVP) e a redução volumétrica endoscópica (REV). Desta forma, este artigo abordará os aspectos relacionados ao emprego da CRVP e da RVE, de modo a discutir as evidências que sustentam o uso destas técnicas no tratamento do enfisema pulmonar avançado, bem como duas limitações.

Tratamento endoscópico do Enfisema: uma atualização / Bronchoscopic Treatment of Emphysema: an Update

O objetivo desta revisão foi apresentar uma atualização das alternativas endoscópicas utilizadas no tratamento do enfisema pulmonar. Os mecanismos bloqueadores de fluxo (válvulas) permanecem como os únicos dispositivos aprovados para uso clínico no tratamento do enfisema pulmonar na América do Sul. A válvula endobrônquica Zephyr® representa o método broncoscópico para o tratamento do enfisema mais bem estudado até o momento, e seu uso clínico está autorizado em diversos países da Europa, Ásia e América do Sul. A válvula IBV® obteve aprovação para uso no tratamento de fuga aérea prolongada nos EUA. Os critérios mais frequentemente analisados na indicação de tratamento endoscópico, além dos aspectos clínicos, incluem o aprisionamento dinâmico, a heterogeneidade e a ventilação colateral, em especial, a passagem de ar através das cissuras interlobares. Atualmente, há softwares que permitem a medida precisa da heterogeneidade e da integridade da cissura.Os resultados obtidos em diversas séries de casos e alguns ensaios randomizados têm trazido nova luz ao entendimento da fisiopatologia dessa doença. No entanto, ainda há necessidade de mais estudos randomizados utilizando o conhecimento adquirido até o momento

The objective of this review is to present an update on endoscopic alternatives for the treatment of emphysema.One-way endobronchial valves continue to be the only devices approved for clinical use in the treatment of emphysema inSouth America. The use of the Zephyr® endobronchial valve is currently the most widely studied bronchoscopic method fortreatment of emphysema and has been approved for clinical use in several countries in Europe, Asia, and South America. Another valve, the IBV® valve, has been approved for use in the treatment of persistent air leaks in the United States. In additionto clinical features, the criteria most often analyzed for indicating endoscopic treatment are dynamic hyperinflation, heterogeneity, and collateral ventilation, especially the passage of air through the interlobar fissures. Currently, there is softwarecapable of accurately measuring heterogeneity and fissure integrity.The results obtained in various case series and certain randomized trials have shed new light on the pathophysiology of emphysema. However, additional randomized trials using the knowledge gained thus far are warranted in order to furtherevaluate this procedure