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Infectious, Rare and Idiopathic Pulmonary Diseases

Section edited by Sanjay Chotirmall and Giovanni Sotgiu

This section concerns all aspects of pulmonary infection, rare and idiopathic lung diseases including, but not limited to: prevention, diagnosis and management, as well as related molecular genetics, and pathophysiology.

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  1. Common variable immunodeficiency (CVID) is a group of heterogeneous primary immunodeficiencies characterised by a dysregulated and impaired immune response. In addition to an increased susceptibility to infect...

    Authors: Jake E. Cowen, James Stevenson, Madhusudan Paravasthu, James Darroch, Anu Jacob, Salaheddin Tueger, John R. Gosney, Anneliese Simons, Lisa G. Spencer and Eoin P. Judge

    Citation: BMC Pulmonary Medicine 2020 20:205

    Content type: Case report

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  2. Adiponectin is a biomarker closely related to heart failure. However, its role in pulmonary hypertension remains unclear. In this study, we investigated the association between adiponectin and hemodynamic abno...

    Authors: Dongling Luo, Pengyuan Chen, Ziyang Yang, Yongheng Fu, Yigao Huang, Hezhi Li, Jimei Chen, Jian Zhuang and Caojin Zhang

    Citation: BMC Pulmonary Medicine 2020 20:204

    Content type: Research article

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  3. COVID-19 is an infectious disease caused by a novel coronavirus (SARS-CoV-2). The immunopathogenesis of the infection is currently unknown. Healthcare workers (HCWs) are at highest risk of infection and disease.

    Authors: Giovanni Sotgiu, Alessandra Barassi, Monica Miozzo, Laura Saderi, Andrea Piana, Nicola Orfeo, Claudio Colosio, Giovanni Felisati, Matteo Davì, Alberto Giovanni Gerli and Stefano Centanni

    Citation: BMC Pulmonary Medicine 2020 20:203

    Content type: Research article

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  4. The clinical landscape of pulmonary arterial hypertension (PAH) has evolved in terms of disease definition and classification, trial designs, available therapies and treatment strategies as well as clinical gu...

    Authors: Max Schlueter, Amélie Beaudet, Evan Davies, Binu Gurung and Andreas Karabis

    Citation: BMC Pulmonary Medicine 2020 20:202

    Content type: Research article

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  5. To investigate the use of do-not-resuscitate (DNR) orders in patients hospitalized with community-acquired pneumonia (CAP) and the association with mortality.

    Authors: Gertrud Baunbæk Egelund, Andreas Vestergaard Jensen, Pelle Trier Petersen, Stine Bang Andersen, Bjarne Ørskov Lindhardt, Gernot Rohde, Pernille Ravn and Christian von Plessen

    Citation: BMC Pulmonary Medicine 2020 20:201

    Content type: Research article

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  6. Prednisone has been shown to reverse lung function declines in hypersensitivity pneumonitis patients without established fibrosis. Second line immunosuppressants like azathioprine and mycophenolate mofetil hav...

    Authors: Sungryong Noh, Ruchi Yadav, Manshi Li, Xiaofeng Wang, Debasis Sahoo, Daniel A. Culver and Aman Pande

    Citation: BMC Pulmonary Medicine 2020 20:199

    Content type: Research article

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  7. Dermatomyositis (DM) is a systemic autoimmune inflammatory disorder that affects primarily skin, muscle and lung, frequently associated with interstitial lung disease (ILD). The objective of this study is to i...

    Authors: Yueyan Lou, Yu Zheng, Bijun Fan, Liyan Zhang, Feng Zhu, Xiaodong Wang, Zhiwei Chen, Xiaoming Tan and Qing Wei

    Citation: BMC Pulmonary Medicine 2020 20:196

    Content type: Research article

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  8. Advanced lung disease in adult cystic fibrosis (CF) drives most clinical care requirements. The aim was to evaluate outcome (time to death while in the study) in a cohort of adult CF patients with severe lung ...

    Authors: Guilherme Figueiredo Silva, Nicholas J. Simmonds and Paulo de Tarso Roth Dalcin

    Citation: BMC Pulmonary Medicine 2020 20:194

    Content type: Research article

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  9. Bronchiolar adenoma(BA) is a recently recognized, rare tumor of the bronchioles. It can be divided into proximal and distal types according to the proportion of mucinous and ciliated cells on the luminal surfa...

    Authors: Yajing Sun, Min Liu, Zhongmin Jiang and Baojiang Li

    Citation: BMC Pulmonary Medicine 2020 20:192

    Content type: Case report

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  10. In patients with idiopathic pulmonary fibrosis (IPF) treated with pirfenidone (Esbriet®, Genentech USA, Inc. South San Francisco, CA.), effectively managing treatment-related adverse events (AEs) may improve a...

    Authors: Franck F. Rahaghi, Zeenat Safdar, Anne Whitney Brown, Joao A. de Andrade, Kevin R. Flaherty, Robert J. Kaner, Christopher S. King, Maria L. Padilla, Imre Noth, Mary Beth Scholand, Adrian Shifren and Steven D. Nathan

    Citation: BMC Pulmonary Medicine 2020 20:191

    Content type: Research article

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  11. Pirfenidone and nintedanib are antifibrotic therapies which slow disease progression in idiopathic pulmonary fibrosis (IPF), an irreversible, progressive lung disease with poor prognosis. We compared adherence...

    Authors: Mitra Corral, Kathryn DeYoung and Amanda M. Kong

    Citation: BMC Pulmonary Medicine 2020 20:188

    Content type: Research article

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  12. Pulmonary veno-occlusive disease (PVOD) and pulmonary arterial hypertension (PAH) share an overlapping disease phenotype. Hence it is necessary to distinguish them.

    Authors: Xiaofang Zeng, Fan Chen, Anandharajan Rathinasabapathy, Tangzhiming Li, Agila Adnan Ali Mohammed Mohammed and Zaixin Yu

    Citation: BMC Pulmonary Medicine 2020 20:186

    Content type: Case report

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  13. Accurately diagnosing pleural effusion is a frequent and significant problem in clinical practice. Combining pleural biomarkers with patients’ age may be a valuable method for diagnosing TPE. We sought to eval...

    Authors: Chun-Guo Jiang, Wen Wang, Qiong Zhou, Xiu-Zhi Wu, Xiao-Juan Wang, Zhen Wang, Kan Zhai and Huan-Zhong Shi

    Citation: BMC Pulmonary Medicine 2020 20:178

    Content type: Research article

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  14. Norwegian guideline recommendations on first-line empirical antibiotic prescribing in hospitalised patients with community-acquired pneumonia (CAP) are penicillin G/V in monotherapy, or penicillin G in combina...

    Authors: June Utnes Høgli, Beate Hennie Garcia, Kristian Svendsen, Vegard Skogen and Lars Småbrekke

    Citation: BMC Pulmonary Medicine 2020 20:169

    Content type: Research article

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  15. Pulmonary Cryptococcosis is a common fungal infection mainly caused by Cryptococcus neoformans/C.gattii species in immunocompromised patients. Cases of pulmonary cryptococcosis in patients with normal immune func...

    Authors: Kelin Yao, Xiaofang Qiu, Hongjie Hu, Yuxin Han, Wenming Zhang, Ruiming Xia, Liang Wang and Jieming Fang

    Citation: BMC Pulmonary Medicine 2020 20:161

    Content type: Case report

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  16. Ceftriaxone (CTRX) and ampicillin/sulbactam (ABPC/SBT) are recommended by various guidelines as the first-line antibiotics for community-acquired pneumonia (CAP). However, which of these antibiotics is more ef...

    Authors: Nobuyoshi Hamao, Isao Ito, Satoshi Konishi, Naoya Tanabe, Masahiro Shirata, Issei Oi, Mitsuhiro Tsukino, Hisako Matsumoto, Yoshiro Yasutomo, Seizo Kadowaki and Toyohiro Hirai

    Citation: BMC Pulmonary Medicine 2020 20:160

    Content type: Research article

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  17. Whereas Burkholderia infections are recognized to impair prognosis in cystic fibrosis (CF) patients, there is no recommendation to date for early eradication therapy. The aim of our study was to analyse the curre...

    Authors: Vianney Gruzelle, Hélène Guet-Revillet, Christine Segonds, Stéphanie Bui, Julie Macey, Raphaël Chiron, Marine Michelet, Marlène Murris-Espin and Marie Mittaine

    Citation: BMC Pulmonary Medicine 2020 20:159

    Content type: Research article

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  18. Sarcoidosis is a systemic granulomatous disease of unknown etiology. Clinical cohort studies of different populations are important to understand the high variability in clinical presentation and disease cours...

    Authors: Hok Sreng Te, David M. Perlman, Chetan Shenoy, Daniel J. Steinberger, Rebecca J. Cogswell, Henri Roukoz, Erik J. Peterson, Lin Zhang, Tadashi L. Allen and Maneesh Bhargava

    Citation: BMC Pulmonary Medicine 2020 20:155

    Content type: Research article

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  19. Variants in the gene encoding bone morphogenetic protein receptor type II (BMPR2) are the most common genetic cause of pulmonary arterial hypertension (PAH), whereas biallelic variants in the eukaryotic translati...

    Authors: Qixian Zeng, Hang Yang, Bingyang Liu, Yanyun Ma, Zhihong Liu, Qianlong Chen, Wenke Li, Qin Luo, Zhihui Zhao, Zhou Zhou and Changming Xiong

    Citation: BMC Pulmonary Medicine 2020 20:150

    Content type: Research article

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  20. Vascular Ehlers-Danlos syndrome (vEDS) is a rare autosomal dominant hereditary collagen disease caused by a defect or deficiency in the pro-α1 chain of type III procollagen encoded by the COL3A1 gene. Patients...

    Authors: Tingting Wan, Jinyan Ye, Peiliang Wu, Mengshi Cheng, Baihong Jiang, Hailong Wang, Jianmin Li, Jun Ma, Liangxing Wang and Xiaoying Huang

    Citation: BMC Pulmonary Medicine 2020 20:149

    Content type: Case report

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  21. We assessed the rate of and predictors for all-cause mortality in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) receiving plasma exchange (PLEX) and evaluated the surviva...

    Authors: Pil Gyu Park, Byung-Woo Yoo, Jason Jungsik Song, Yong-Beom Park and Sang-Won Lee

    Citation: BMC Pulmonary Medicine 2020 20:147

    Content type: Research article

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  22. Pulmonary tuberculosis is one of the most common infectious diseases worldwide. Patients with suspected pulmonary tuberculosis with negative smear are recommended to undergo further tests including sputum indu...

    Authors: Wen Luo, Yihua Lin, Zhibin Li, Wanyu Wang and Yonghong Shi

    Citation: BMC Pulmonary Medicine 2020 20:146

    Content type: Research article

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  23. Primary Ciliary Dyskinesia (PCD) is also known as immotile-cilia syndrome, an autosomal recessive disorder of ciliary function, leading to mucus retention in the respiratory system in childhood. Our knowledge ...

    Authors: Fuad Al Mutairi, Randa Alkhalaf, Abdullah Alkhorayyef, Fayhan Alroqi, Alyafee Yusra, Muhammad Umair, Fetaini Nouf, Amjad Khan, Alharbi Meshael, Aleidi Hamad, Alaujan Monira, Abdulaziz Asiri, Kheloud M. Alhamoudi and Majid Alfadhel

    Citation: BMC Pulmonary Medicine 2020 20:141

    Content type: Case report

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  24. Although typical and atypical CT image findings of COVID-19 are reported in current studies, the CT image features of COVID-19 overlap with those of viral pneumonia and other respiratory diseases. Hence, it is...

    Authors: Lin Luo, Zhendong Luo, Yizhen Jia, Cuiping Zhou, Jianlong He, Jianxun Lyu and Xinping Shen

    Citation: BMC Pulmonary Medicine 2020 20:129

    Content type: Research article

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  25. While antifibrotic drugs significantly decrease lung function decline in idiopathic pulmonary fibrosis (IPF), there is still an unmet need to halt disease progression. Antioxidative therapy with N-acetylcystei...

    Authors: Hanyu Shi, Dawei Yin, Francesco Bonella, Michael Kreuter, Ute Oltmanns, Xuren Li, Shouchun Peng and Luqing Wei

    Citation: BMC Pulmonary Medicine 2020 20:128

    Content type: Research article

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  26. Pulmonary tuberculosis (PTB) is frequently associated with chronic respiratory impairment despite microbiological cure. There are only a few clinical research studies that describe the course, type and severit...

    Authors: Celso Khosa, Nilesh Bhatt, Isabel Massango, Khalide Azam, Elmar Saathoff, Abhishek Bakuli, Friedrich Riess, Olena Ivanova, Michael Hoelscher and Andrea Rachow

    Citation: BMC Pulmonary Medicine 2020 20:127

    Content type: Research article

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  27. Improvement in health-related quality of life (HRQL) has been suggested as an alternative treatment goal of non-tuberculous mycobacterial pulmonary disease (NTM-PD). This study was performed to elucidate the l...

    Authors: Nakwon Kwak, Sung A Kim, Sun Mi Choi, Jinwoo Lee, Chang-Hoon Lee and Jae-Joon Yim

    Citation: BMC Pulmonary Medicine 2020 20:126

    Content type: Research article

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  28. Pirfenidone is an antifibrotic agent approved for the treatment of idiopathic pulmonary fibrosis (IPF). The drug is available for Polish patients with IPF since 2017. The PolExPIR study aimed to describe the r...

    Authors: Sebastian Majewski, Adam J. Białas, Małgorzata Buchczyk, Paweł Gomółka, Katarzyna Górska, Hanna Jagielska-Len, Agnieszka Jarzemska, Ewa Jassem, Dariusz Jastrzębski, Aleksander Kania, Marek Koprowski, Rafał Krenke, Jan Kuś, Katarzyna Lewandowska, Magdalena M. Martusewicz-Boros, Kazimierz Roszkowski-Śliż…

    Citation: BMC Pulmonary Medicine 2020 20:122

    Content type: Research article

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  29. An asymptomatic SCUBA (Self-contained underwater breathing apparatus) diver was discovered to have an intralobar bronchopulmonary sequestration during routine pre-course screening. This is the first reported c...

    Authors: Timothy Xin Zhong Tan, Andrew Yunkai Li, James Jie Sng, Mark Lim, Zhi Xiang Tan, Hope Xian’en Ang, Boon Hor Ho, David Zhiwei Law and Anne Ann Ling Hsu

    Citation: BMC Pulmonary Medicine 2020 20:121

    Content type: Case report

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  30. Pulmonary cryptococcosis is an uncommon infectious disease that can develop in both immunocompromised and immunocompetent patients. The severity of chronic kidney disease (CKD) was reported to be one of the ri...

    Authors: Hiroki Tashiro, Tetsuro Haraguchi, Koichiro Takahashi, Hironori Sadamatsu, Ryo Tajiri, Ayako Takamori, Shinya Kimura and Naoko Sueoka-Aragane

    Citation: BMC Pulmonary Medicine 2020 20:116

    Content type: Research article

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  31. Infection is the most common cause of mortality within the first year after lung transplantation (LTx). The management of perioperative antibiotic therapy is a major issue, but little is known about worldwide ...

    Authors: Benjamin Coiffard, Eloi Prud’Homme, Sami Hraiech, Nadim Cassir, Jérôme Le Pavec, Romain Kessler, Federica Meloni, Marc Leone, Pascal Alexandre Thomas, Martine Reynaud-Gaubert and Laurent Papazian

    Citation: BMC Pulmonary Medicine 2020 20:109

    Content type: Research article

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  32. Acute fibrinous and organizing pneumonitis (AFOP) is an uncommon variant of acute lung injury, characterized by intra-alveolar fibrin and organizing pneumonia. Proposed etiologies include connective tissue dis...

    Authors: Jiangnan Zhao, Yi Shi, Dongmei Yuan, Qunli Shi, Weiping Wang and Xin Su

    Citation: BMC Pulmonary Medicine 2020 20:98

    Content type: Case report

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  33. At present, serum Aspergillus IgG and IgM antibody detection is mainly used in the diagnosis of chronic pulmonary aspergillosis (CPA), but its value in the diagnosis of invasive pulmonary aspergillosis (IPA) in n...

    Authors: Qihong Yu, Jingdong He, Bin Xing, Xin Li, Hongyu Qian, Hong Zhang, Meilin Xu and Haiying Peng

    Citation: BMC Pulmonary Medicine 2020 20:89

    Content type: Research article

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  34. NTP42 is a novel antagonist of the thromboxane prostanoid receptor (TP), currently in development for the treatment of pulmonary arterial hypertension (PAH). PAH is a devastating disease with multiple pathophysio...

    Authors: Eamon P. Mulvaney, Helen M. Reid, Lucia Bialesova, Annie Bouchard, Dany Salvail and B. Therese Kinsella

    Citation: BMC Pulmonary Medicine 2020 20:85

    Content type: Research article

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  35. Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of surfactant proteins within the alveolar spaces. Autoimmune PAP (APAP) caused by elevated levels of GM-CSF autoantibodies (GM-Ab) is ...

    Authors: S. Sato, K. Akasaka, H. Ohta, Y. Tsukahara, G. Kida, E. Tsumiyama, K. Kusano, T. Oba, T. Nishizawa, R. Kawabe, H. Yamakawa, M. Amano, H. Matsushima and T. Takada

    Citation: BMC Pulmonary Medicine 2020 20:84

    Content type: Case report

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  36. Antipseudomonal antibiotics should be restricted to patients at risk of Pseudomonas aeruginosa infection. However, the indications in different guidelines on community-acquired pneumonia (CAP) are discordant. Our...

    Authors: Nuria Angrill, Miguel Gallego, Juli Font, Jordi Vallés, Anisi Morón, Eduard Monsó and Jordi Rello

    Citation: BMC Pulmonary Medicine 2020 20:83

    Content type: Research article

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  37. Eosinophilic pleural effusion (EPE) is attributed to several well-recognised causes. However, some patients remain idiopathic, even after thorough clinical work-up. The present study aimed to better characteri...

    Authors: Weizhan Luo, Yunxiang Zeng, Panxiao Shen, Jianxing He and Jinlin Wang

    Citation: BMC Pulmonary Medicine 2020 20:82

    Content type: Research article

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  38. Numerous publications suggest an association between herpes virus infection and idiopathic pulmonary fibrosis (IPF). These reports have employed immunohistochemistry, in situ hybridization and/or PCR, which ar...

    Authors: Qinyan Yin, Michael J. Strong, Yan Zhuang, Erik K. Flemington, Naftali Kaminski, Joao A. de Andrade and Joseph A. Lasky

    Citation: BMC Pulmonary Medicine 2020 20:81

    Content type: Research article

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  39. Sarcoidosis is a systemic granulomatous disease caused by CD4+ cell-dominant inflammation. Meanwhile, diffuse panbronchiolitis is a chronic inflammatory respiratory disease predominantly caused by CD8+ lymphoc...

    Authors: Tomohiro Akaba, Kiyoshi Takeyama, Mitsuko Kondo, Fumi Kobayashi, Asako Okabayashi, Tatsuo Sawada and Etsuko Tagaya

    Citation: BMC Pulmonary Medicine 2020 20:77

    Content type: Case report

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  40. Hypoxia affects myocardial oxygen supply resulting in subclinical cardiac dysfunction in obstructive sleep apnea (OSA) patients, with cardiovascular complications being associated with increased oxidative burs...

    Authors: Antonello D’Andrea, Angelo Canora, Simona Sperlongano, Domenico Galati, Serena Zanotta, Giorgio Emanuele Polistina, Carmine Nicoletta, Giacomo Ghinassi, Maurizio Galderisi, Alessandro Sanduzzi Zamparelli, Patrizio Lancellotti and Marialuisa Bocchino

    Citation: BMC Pulmonary Medicine 2020 20:76

    Content type: Research article

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  41. The concurrence of sarcoidosis and primary lung cancer is very rare. We report a very rare case with a delayed diagnosis of primary lung cancer due to its misdiagnosis as worsening of pulmonary sarcoidosis.

    Authors: Hong-Joon Shin, Min-Seok Kim, Bo Gun Kho, Ha Young Park, Tae-Ok Kim, Cheol-Kyu Park, In-Jae Oh, Yu-Il Kim, Young-Chul Kim, Yoo-Duk Choi and Sung-Chul Lim

    Citation: BMC Pulmonary Medicine 2020 20:71

    Content type: Case report

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  42. Interstitial lung abnormalities (ILA) are common in participants of lung cancer screening trials and broad population-based cohorts. They are associated with increased mortality, but less is known about diseas...

    Authors: Nils Hoyer, Laura H. Thomsen, Mathilde M. W. Wille, Torgny Wilcke, Asger Dirksen, Jesper H. Pedersen, Zaigham Saghir, Haseem Ashraf and Saher B. Shaker

    Citation: BMC Pulmonary Medicine 2020 20:67

    Content type: Research article

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  43. Chlamydia psittaci pneumonia is a zoonotic infectious disease caused by Chlamydia psittaci. Diagnostic tools, including culture, serologic test and PCR-based methods, are available but prone to false negative res...

    Authors: Lei Gu, Wei Liu, Meng Ru, Jing Lin, Guoqing Yu, Jia Ye, Zheng-an Zhu, Yuebin Liu, Jian Chen, Guoxiang Lai and Wen Wen

    Citation: BMC Pulmonary Medicine 2020 20:65

    Content type: Case report

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  44. Matrix metalloproteinases (MMPs) and tissue inhibitors of MMPs (TIMPs) play important roles in the turnover of extracellular matrix and in the pathogenesis of idiopathic pulmonary fibrosis (IPF). This study ai...

    Authors: Jamie L. Todd, Richard Vinisko, Yi Liu, Megan L. Neely, Robert Overton, Kevin R. Flaherty, Imre Noth, L. Kristin Newby, Joseph A. Lasky, Mitchell A. Olman, Christian Hesslinger, Thomas B. Leonard, Scott M. Palmer and John A. Belperio

    Citation: BMC Pulmonary Medicine 2020 20:64

    Content type: Research article

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  45. Many individuals with cystic fibrosis (CF) die from respiratory failure without referral for lung transplant. Physician practices that may expedite, delay, or preclude referral, are poorly understood.

    Authors: Bethany L. Bartley, Carolyn E. Schwartz, Roland B. Stark, Anna M. Georgiopoulos, Deborah Friedman, Christopher J. Richards, Henry L. Dorkin, T. Bernard Kinane, Isabel P. Neuringer and Lael M. Yonker

    Citation: BMC Pulmonary Medicine 2020 20:58

    Content type: Research article

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  46. Patients with fibrotic interstitial lung disease (FILD) often experience gas exchange abnormalities and ventilatory limitations, resulting in reduced exercise capacity. High-flow nasal cannula (HFNC) oxygen th...

    Authors: Atsushi Suzuki, Masahiko Ando, Tomoki Kimura, Kensuke Kataoka, Toshiki Yokoyama, Eiichi Shiroshita and Yasuhiro Kondoh

    Citation: BMC Pulmonary Medicine 2020 20:51

    Content type: Research article

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