Lymphangioleiomyomatosis (LAM) is a rare diffuse cystic lung disease. Knowledge on LAM-related pulmonary hypertension (PH) is limited. This study aimed to analyze the clinical characteristics of LAM with elevated pulm...Lymphangioleiomyomatosis (LAM) is a rare diffuse cystic lung disease. Knowledge on LAM-related pulmonary hypertension (PH) is limited. This study aimed to analyze the clinical characteristics of LAM with elevated pulmonary artery pressure (PAP) and evaluate the potential efficacy of sirolimus. The study involved 50 LAM patients who underwent echocardiography. According to the tricuspid regurgitation velocity (TRV), these patients were divided into the TRV≤2.8 m/s group and TRV>2.8 m/s group. Both groups comprised 25 females with an average age of 38.6±8.1 and 41.5±8.9 years. In the TRV>2.8 m/s group, the estimated systolic PAP (SPAP) was significantly elevated (52.08±12.45 mmHg vs. 30.24±5.25 mmHg, P<0.01). Linear analysis showed that SPAP was correlated with forced expiratory volume in 1 s (FEV1), diffusing capacity of the lungs for carbon monoxide, alveolar arterial oxygen gradient (PA-aO2), and 6 min walking distance (r =-0.392,-0.351, 0.450, and -0.591, respectively;P<0.05), in which PA-aO2 was a risk factor for SPAP elevation (β= 0.064, OR= 1.066, P<0.05). Moreover, in 10 patients who received sirolimus therapy, SPAP decreased from 57.0±12.6 mmHg to 35.2±11.1 mmHg. The study showed that LAM patients with PH exhibit poor pulmonary function and hypoxemia and may benefit from sirolimus treatment.展开更多
An unexpected observation among the COVID-19 pandemic is that smokers constituted only 1.4%–18.5%of hospitalized adults,calling for an urgent investigation to determine the role of smoking in SARS-CoV-2 infection.Her...An unexpected observation among the COVID-19 pandemic is that smokers constituted only 1.4%–18.5%of hospitalized adults,calling for an urgent investigation to determine the role of smoking in SARS-CoV-2 infection.Here,we show that cigarette smoke extract(CSE)and carcinogen benzo(a)pyrene(BaP)increase ACE2 mRNA but trigger ACE2 protein catabolism.BaP induces an aryl hydrocarbon receptor(AhR)-dependent upregulation of the ubiquitin E3 ligase Skp2 for ACE2 ubiquitination.ACE2 in lung tissues of non-smokers is higher than in smokers,consistent with the findings that tobacco carcinogens downregulate ACE2 in mice.Tobacco carcinogens inhibit SARS-CoV-2 spike protein pseudovirions infection of the cells.Given that tobacco smoke accounts for 8 million deaths including 2.1 million cancer deaths annually and Skp2 is an oncoprotein,tobacco use should not be recommended and cessation plan should be prepared for smokers in COVID-19 pandemic.展开更多
Diffuse cystic lung diseases are uncommon but can present a diagnostic challenge because increasing number of diseases have been associated with this presentation.Cyst in the lung is defined as a round parenchymal luc...Diffuse cystic lung diseases are uncommon but can present a diagnostic challenge because increasing number of diseases have been associated with this presentation.Cyst in the lung is defined as a round parenchymal lucency with a well-defined thin wall(<2 mm thickness).Focal or multifocal cystic lesions include blebs,bullae,pneumatoceles,congenital cystic lesions,traumatic lesions,and several infectious processes such as coccidioidomycosis,Pneumocystis jiroveci pneumonia,and hydatid disease.“Diffuse”distribution in the lung implies involvement of all lobes.Diffuse lung involvement with cystic lesions can be seen in pulmonary lymphangioleiomyomatosis,pulmonary Langerhans’cell histiocytosis,lymphoid interstitial pneumonia,Birt-Hogg-Dubésyndrome,amyloidosis,light chain deposition disease,honeycomb lung associated with advanced fibrosis,and several other rare causes including metastatic disease.High-resolution computed tomography of the chest helps define morphologic features of the lung lesions as well as their distribution and associated features such as intrathoracic lymphadenopathy.Correlating the tempo of the disease process and clinical context with chest imaging findings serve as important clues to defining the underlying nature of the cystic lung disease and guide diagnostic evaluation as well as management.展开更多
文摘Lymphangioleiomyomatosis (LAM) is a rare diffuse cystic lung disease. Knowledge on LAM-related pulmonary hypertension (PH) is limited. This study aimed to analyze the clinical characteristics of LAM with elevated pulmonary artery pressure (PAP) and evaluate the potential efficacy of sirolimus. The study involved 50 LAM patients who underwent echocardiography. According to the tricuspid regurgitation velocity (TRV), these patients were divided into the TRV≤2.8 m/s group and TRV>2.8 m/s group. Both groups comprised 25 females with an average age of 38.6±8.1 and 41.5±8.9 years. In the TRV>2.8 m/s group, the estimated systolic PAP (SPAP) was significantly elevated (52.08±12.45 mmHg vs. 30.24±5.25 mmHg, P<0.01). Linear analysis showed that SPAP was correlated with forced expiratory volume in 1 s (FEV1), diffusing capacity of the lungs for carbon monoxide, alveolar arterial oxygen gradient (PA-aO2), and 6 min walking distance (r =-0.392,-0.351, 0.450, and -0.591, respectively;P<0.05), in which PA-aO2 was a risk factor for SPAP elevation (β= 0.064, OR= 1.066, P<0.05). Moreover, in 10 patients who received sirolimus therapy, SPAP decreased from 57.0±12.6 mmHg to 35.2±11.1 mmHg. The study showed that LAM patients with PH exhibit poor pulmonary function and hypoxemia and may benefit from sirolimus treatment.
基金the National Key Research and Development Program of China(Nos.2020YFA0803300 and 2018YFC1313600)the National Natural Science Funds for Distinguished Young Scholar(No.81425025)+2 种基金the Key Project of the National Natural Science Foundation of China(No.81830093)the CAMS Innovation Fund for Medical Sciences(CIFMS,No.2019-I2M-1-003)the National Natural Science Foundation of China(Nos.81672765 and 81802796).
文摘An unexpected observation among the COVID-19 pandemic is that smokers constituted only 1.4%–18.5%of hospitalized adults,calling for an urgent investigation to determine the role of smoking in SARS-CoV-2 infection.Here,we show that cigarette smoke extract(CSE)and carcinogen benzo(a)pyrene(BaP)increase ACE2 mRNA but trigger ACE2 protein catabolism.BaP induces an aryl hydrocarbon receptor(AhR)-dependent upregulation of the ubiquitin E3 ligase Skp2 for ACE2 ubiquitination.ACE2 in lung tissues of non-smokers is higher than in smokers,consistent with the findings that tobacco carcinogens downregulate ACE2 in mice.Tobacco carcinogens inhibit SARS-CoV-2 spike protein pseudovirions infection of the cells.Given that tobacco smoke accounts for 8 million deaths including 2.1 million cancer deaths annually and Skp2 is an oncoprotein,tobacco use should not be recommended and cessation plan should be prepared for smokers in COVID-19 pandemic.
文摘Diffuse cystic lung diseases are uncommon but can present a diagnostic challenge because increasing number of diseases have been associated with this presentation.Cyst in the lung is defined as a round parenchymal lucency with a well-defined thin wall(<2 mm thickness).Focal or multifocal cystic lesions include blebs,bullae,pneumatoceles,congenital cystic lesions,traumatic lesions,and several infectious processes such as coccidioidomycosis,Pneumocystis jiroveci pneumonia,and hydatid disease.“Diffuse”distribution in the lung implies involvement of all lobes.Diffuse lung involvement with cystic lesions can be seen in pulmonary lymphangioleiomyomatosis,pulmonary Langerhans’cell histiocytosis,lymphoid interstitial pneumonia,Birt-Hogg-Dubésyndrome,amyloidosis,light chain deposition disease,honeycomb lung associated with advanced fibrosis,and several other rare causes including metastatic disease.High-resolution computed tomography of the chest helps define morphologic features of the lung lesions as well as their distribution and associated features such as intrathoracic lymphadenopathy.Correlating the tempo of the disease process and clinical context with chest imaging findings serve as important clues to defining the underlying nature of the cystic lung disease and guide diagnostic evaluation as well as management.
