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Although less extensively studied compared to pulmonary obstructive diseases, restrictive lung disease (RLD) is highly prevalent and frequently disabling in the adult and, more, the elderly population. The underlying conditions may be either primarily pulmonary diseases, such as idiopathic pulmonary fibrosis, or non respiratory conditions secondarily affecting the lung, e. g. congestive heart failure, or else conditions affecting the lung expansion, e. g. obesity or rib cage deformity. The diagnosis is frequently based on the measurement of surrogate indexes such as the forced vital capacity (FVC) used as a proxy for total lung capacity (TLC). As a consequence, diagnosis of RLD is often characterized by poor specificity. In the elderly, worsening in the quality of life and poor prognosis are variably, but significantly, associated to RLD, being the underlying condition an important source of variability. Several causes of RLD are preventable and treatable conditions. A prompt identification of these conditions may allow to slow the decline of respiratory reserve and, thus, to preserve both personal independence and resistance to acute respiratory infections. This review gives an update on the latest evidence available on the prevalence and the prognosis of RLD in the elderly. Studies were identified through systematic searches of the electronic database MEDLINE. Reference list of eligible papers were also manually searched. 相似文献
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Paula Navas Tejedor Julián Palomino Doza Jair Antonio Tenorio Castaño Ana Belén Enguita Valls José Julián Rodríguez Reguero Amaya Martínez Meñaca Ignacio Hernández González Héctor Bueno Zamora Pablo Daniel Lapunzina Badía Pilar Escribano Subías 《Revista espa?ola de cardiología》2018,71(2):86-94
Introduction and objectives
Hereditary pulmonary veno-occlusive disease (PVOD) has been associated with biallelic mutations in EIF2AK4 with the recent discovery of a founder mutation in Iberian Romani patients with familial PVOD. The aims of this study were phenotypical characterization and survival analysis of Iberian Romani patients with familial PVOD carrying the founder p.Pro1115Leu mutation in EIF2AK4, according to their tolerance to pulmonary vasodilators (PVD). Familial genetic screening was conducted, as well as assessment of sociocultural determinants with a potential influence on disease course.Methods
Observational study of Romani patients with familial PVOD included in the Spanish Registry of Pulmonary Arterial Hypertension. Genetic screening of EIF2AK4 was performed in index cases and relatives between November 2011 and July 2016 and histological pulmonary examination was carried out in patients who received a lung transplant or died. The patients were divided into 2 groups depending on their tolerance to PVD, with comparison of baseline characteristics and survival free of death or lung transplant.Results
Eighteen Romani patients were included: 9 index cases and 9 relatives. The biallelic founder mutation in EIF2AK4 was found in all affected cases and 2 unaffected relatives. Family screening showed 34.2% of healthy heterozygotes, high consanguinity, young age at childbirth, and frequent multiparity. Prognosis was bleak, with significant differences depending on tolerance to PVD.Conclusions
We describe 2 phenotypes of hereditary PVOD depending on tolerance to PVD, with prognostic impact and familial distribution. Consanguinity may have a negative impact on the transmission of PVOD, with familial genetic screening showing high effectiveness.Full English text available from: www.revespcardiol.org/en 相似文献4.
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Atsushi Miyamoto Atsuko Kurosaki Shuhei Moriguchi Yui Takahashi Kazumasa Ogawa Kyoko Murase Shigeo Hanada Hironori Uruga Hisashi Takaya Nasa Morokawa Takeshi Fujii Junichi Hoshino Kazuma Kishi 《Respiratory investigation》2019,57(2):140-149
Background
This study aimed to determine the radiologic predictors and clarify the clinical features related to survival in patients with combined pulmonary fibrosis and emphysema (CPFE) and lung cancer.Methods
We retrospectively reviewed the medical chart data and high-resolution computed tomography (HRCT) findings for 81 consecutive patients with CPFE and 92 primary lung cancers (70 men, 11 women; mean age, 70.9 years). We selected 8 axial HRCT images per patient, and visually determined the normal lung, modified Goddard, and fibrosis scores. Multivariate analysis was performed using the Cox proportional hazards regression model.Results
The major clinical features were a high smoking index of 54.8 pack-years and idiopathic pulmonary fibrosis (n = 44). The major lung cancer profile was a peripherally located squamous cell carcinoma (n = 40) or adenocarcinoma (n = 31) adjacent to emphysema in the upper/middle lobe (n = 27) or fibrosis in the lower lobe (n = 26). The median total normal lung, modified Goddard, and fibrosis scores were 10, 8, and 8, respectively. TNM Classification of malignant tumors (TNM) stage I, II, III, and IV was noted in 37, 7, 26, and 22 patients, respectively. Acute exacerbation occurred in 20 patients. Multivariate analysis showed that a higher normal lung score and TNM stage were independent radiologic and clinical predictors of poor survival at the time of diagnosis of lung cancer.Conclusions
A markedly reduced area of normal lung on HRCT was a relevant radiologic predictor of survival. 相似文献7.
《Archivos de bronconeumología》2023,59(7):418-426
IntroductionThis study aimed to evaluate a potential relationship between the diffusing capacity of the lung for carbon monoxide (DLCO) and the aggressiveness of lung adenocarcinoma (ADC).MethodsPatients who underwent radical surgery for lung ADC between 2001 and 2018 were retrospectively reviewed. DLCO values were dichotomized into DLCOlow (<80% of predicted) and DLCOnormal (≥80%). Relationships between DLCO and ADC histopathological features, clinical features, as well as with overall survival (OS), were evaluated.ResultsFour-hundred and sixty patients were enrolled, of which 193 (42%) were included in the DLCOlow group. DLCOlow was associated with smoking status, low FEV1, micropapillary and solid ADC, tumour grade 3, high tumour lymphoid infiltrate and presence of tumour desmoplasia. In addition, DLCO values were higher in low-grade ADC and progressively decreased in intermediate and high-grade ADC (p = 0.024). After adjusting for clinical variables, at multivariable logistic regression analysis, DLCOlow still showed a significant correlation with high lymphoid infiltrate (p = 0.017), presence of desmoplasia (p = 0.065), tumour grade 3 (p = 0.062), micropapillary and solid ADC subtypes (p = 0.008). To exclude the association between non-smokers and well-differentiated ADC, the relationship between DLCO and histopathological ADC patterns was confirmed in the subset of 377 former and current smokers (p = 0.021). At univariate analysis, gender, DLCO, FEV1, ADC histotype, tumour grade, stage, pleural invasion, tumour necrosis, tumour desmoplasia, lymphatic and blood invasion were significantly related with OS. At multivariate analysis, only gender (p < 0.001), tumour stage (p < 0.001) and DLCO (p = 0.050) were significantly related with the OS.ConclusionsWe found a relationship between DLCO and ADC patterns as well as with tumour grade, tumour lymphoid infiltrate and desmoplasia, suggesting that lung damage may be associated with tumour aggressiveness. 相似文献
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Kuziemski K Pieńkowska J Słomiński W Specjalski K Dziadziuszko K Jassem E Studniarek M Kalicka R Słomiński JM 《Diabetes research and clinical practice》2011,91(1):80-86
Aims
Aim of the study was to determine the role of perfusion chest computed tomography (pCT) in evaluation of pulmonary diabetic angiopathy.Methods
18 never-smoking patients (10 diabetic patients and 8 healthy controls) underwent chest high resolution CT (HRCT) and then pCT scanning. In both groups, blood tests, biochemical analysis, fibrinogen, HbA1c, spirometry, diffusion capacity for carbon monoxide (DLCO) and body pletysmography were performed.Following parameters of pulmonary perfusion have been analysed: blood volume (BV), blood flow (BF), mean transit time (MTT), time to peak (TTP) and permeability surface (PS).Results
There were no statistically significant differences between groups in terms of age, sex, BMI, forced expiratory volume in one second (FEV1), DLCO. Chest HRCT revealed no pathologies. Significantly higher values of chest pCT for BF (p = 0.05), BV (p = 0.05) and PS (p = 0.01) have been found in diabetics in comparison to controls. No differences were found in MTT.Conclusions
Significant increase of perfusion parameters in diabetes seems to confirm pulmonary microangiopathy. The results indicate that further studies on application of pCT in diabetic patients may be beneficial for better understanding of lung microangiopathy, its diagnosing and monitoring. 相似文献9.
Yuhei Ito Motoaki Tanigawa Keisuke Iwamoto Akina Nigi Hidetoshi Itani Shigeto Kondo Toshiya Tokui Eiji Usui Shigehisa Tamaki 《Respiratory investigation》2019,57(5):506-509
Dasatinib has increasingly been used to treat chronic myeloid leukemia (CML), although interstitial pneumonitis has been found as a complication in large clinical trials. In the present study, 23 patients received dasatinib for CML between 2012 and 2017 at our institution, of whom 2 developed symptomatic interstitial pneumonitis. Notably, the first patient developed interstitial pneumonitis five years after initiating dasatinib. Interstitial pneumonitis should be considered as a complication in patients receiving dasatinib for CML, which may even occur after a long period of uncomplicated administration. 相似文献
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Ricard Ramos Ernest Nadal Inma Peiró Cristina Masuet-Aumatell Ivan Macia Francisco Rivas Gabriela Rosado Pau Rodriguez Anna Ureña Susana Padrones Samantha Aso Carlos Deniz Arturo Navarro Ignacio Escobar 《European journal of surgical oncology》2018,44(9):1419-1424