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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. 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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  2. 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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  3. 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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  4. 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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  5. 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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  6. 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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  7. 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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  8. 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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  9. 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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  10. 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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  11. 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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  12. 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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  13. 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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  14. 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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  15. 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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  16. 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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  17. 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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  18. 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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  19. 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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  20. 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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  21. 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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  22. 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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  23. 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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  24. 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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  25. 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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  26. 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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  27. 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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  28. 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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  29. Lung transplantation (LTx) is still limited by the shortage of suitable donor lungs. Developing flexible surgical procedures can help to increase the chances of LTx by unfolding recipient-to-donor matching opt...

    Authors: Haruchika Yamamoto, Kentaroh Miyoshi, Shinji Otani, Takeshi Kurosaki, Seiichiro Sugimoto, Masaomi Yamane, Shinichi Toyooka, Motomu Kobayashi and Takahiro Oto

    Citation: BMC Pulmonary Medicine 2020 20:46

    Content type: Case report

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  30. Descending necrotizing mediastinitis is a severe infection of the mediastinum. This syndrome manifests as fever and chest pain following cough and sputum production. A 49-year-old woman presented with fever an...

    Authors: Rui-hai Ye, Jun-chao Yang, Hui-hua Hong, Yu-ping Mao, Yuan-hong Zhu, Yu Cao and Zhen Wang

    Citation: BMC Pulmonary Medicine 2020 20:43

    Content type: Case report

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  31. Massive hemoptysis is a serious complication in Cystic Fibrosis (CF), occurring commonly in older patients. Bronchial artery embolization (BAE) can be performed to stop the bleeding. BAE is generally safe and ...

    Authors: V. Terlizzi, M. Botti, G. Gabbani, F. Fanelli, M. De Martino and G. Taccetti

    Citation: BMC Pulmonary Medicine 2020 20:38

    Content type: Case report

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  32. Pulmonary alveolar proteinosis (PAP) is a very rare lung disease and its prevalence and incidence remain unclear. The prevalence and incidence of PAP were investigated by using nationwide claims data from the ...

    Authors: Hee-young Yoon, Ji Hyeon Kim, Ye-Jee Kim and Jin Woo Song

    Citation: BMC Pulmonary Medicine 2020 20:34

    Content type: Research article

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  33. Early detection of pulmonary contamination in children with cystic fibrosis (CF) is essential since these children are vulnerable to Pseudomonas aeruginosa (P. aeruginosa) colonization. In Iran, home nebulization...

    Authors: Seyed Ahmad Tabatabaii, Ghamartaj Khanbabaee, Saeed Sadr, Nazanin Farahbakhsh, Maryam Kazemi Aghdam, Saran Lotfollahzadeh, Amirhossein Hosseini, Naghi Dara, Mohammad Nanbakhsh and Fatemeh Abdollah Gorji

    Citation: BMC Pulmonary Medicine 2020 20:33

    Content type: Research article

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  34. Idiopathic pulmonary fibrosis (IPF) is a progressive and fibrosing lung disease with poor prognosis. Pirfenidone and nintedanib are anti-fibrotic drugs used for patients with IPF. These drugs reduce the rate o...

    Authors: Takumi Yoshikawa, Mitsuo Otsuka, Hirofumi Chiba, Kimiyuki Ikeda, Yuki Mori, Yasuaki Umeda, Hirotaka Nishikiori, Koji Kuronuma and Hiroki Takahashi

    Citation: BMC Pulmonary Medicine 2020 20:27

    Content type: Research article

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    The Correction to this article has been published in BMC Pulmonary Medicine 2020 20:131

  35. Interstitial lung disease (ILD) is the most common and important pulmonary manifestation of rheumatoid arthritis (RA). A radiological honeycomb pattern has been described in diverse forms of ILD that can impac...

    Authors: Hideaki Yamakawa, Shintaro Sato, Tomotaka Nishizawa, Rie Kawabe, Tomohiro Oba, Akari Kato, Masanobu Horikoshi, Keiichi Akasaka, Masako Amano, Hiroki Sasaki, Kazuyoshi Kuwano and Hidekazu Matsushima

    Citation: BMC Pulmonary Medicine 2020 20:25

    Content type: Research article

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  36. We investigated the association between a combination of two markers, peripheral (PEC) and bronchoalveolar lavage (BAL) eosinophil percentage (BEP), and oxygen requirements in patients with acute eosinophilic ...

    Authors: Joon Young Choi, Jeong Uk Lim, Ho Jung Jeong, Ji Eun Lee and Chin Kook Rhee

    Citation: BMC Pulmonary Medicine 2020 20:22

    Content type: Research article

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  37. The 2013 update of the Infection Prevention and Control (IP&C) Guideline outlined recommendations to prevent the spread of CF respiratory pathogens. We aimed to investigate the current infection control practi...

    Authors: Rebecca Elizabeth Stockwell, Michelle ELizabeth Wood, Emma Ballard, Vanessa Moore, Claire Elizabeth Wainwright and Scott Cameron Bell

    Citation: BMC Pulmonary Medicine 2020 20:16

    Content type: Research article

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  38. The understanding of the epidemiology of bronchiectasis is still affected by major limitations with very few data published worldwide. The aim of this study was to estimate the epidemiological burden of bronch...

    Authors: Stefano Aliberti, Giovanni Sotgiu, Francesco Lapi, Andrea Gramegna, Claudio Cricelli and Francesco Blasi

    Citation: BMC Pulmonary Medicine 2020 20:15

    Content type: Research article

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  39. Scleromyxedema is a progressive, systemic connective tissue disorder characterized by fibro-mucous skin lesions and increased serum monoclonal immunoglobulin levels. Pulmonary involvement occurs in a subset of...

    Authors: Mazen Kreidy, Ali Al-Hilli, Ralph Yachoui and Jeffrey Resnick

    Citation: BMC Pulmonary Medicine 2020 20:8

    Content type: Case report

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  40. Simultaneous infection in tuberculosis (TB) is rare. The mixed infection between Streptococcus anginosus group (SAG) and M. tuberculosis (MTB) has not been reported in children. The aim of this report was to desc...

    Authors: Napoleon González Saldaña, José Iván Castillo Bejarano, Marte Hernández Porras, Eduardo Arias de la Garza, Sofia Fortes Gutiérrez, Jose Luis Copado Gutiérrez and Hugo Juarez Olguin

    Citation: BMC Pulmonary Medicine 2020 20:5

    Content type: Case report

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  41. The two 52-week INPULSIS trials investigated nintedanib versus placebo in patients with IPF, FVC ≥50% predicted and DLco 30–79% predicted. The 24-week INSTAGE trial investigated nintedanib plus sildenafil vers...

    Authors: Luca Richeldi, Martin Kolb, Stéphane Jouneau, Wim A. Wuyts, Birgit Schinzel, Susanne Stowasser, Manuel Quaresma and Ganesh Raghu

    Citation: BMC Pulmonary Medicine 2020 20:3

    Content type: Research article

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  42. Skeletal muscle function dysfunction has been reported in patients with cystic fibrosis (CF). Studies so far showed inconclusive data whether reduced exercise capacity is related to intrinsic muscle dysfunctio...

    Authors: Katharina Ruf, Meinrad Beer, Herbert Köstler, Andreas Max Weng, Henning Neubauer, Alexander Klein, Kathleen Platek, Kristina Roth, Ralph Beneke and Helge Hebestreit

    Citation: BMC Pulmonary Medicine 2019 19:269

    Content type: Research article

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  43. Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is life-threatening. Several serum biomarkers, such as Krebs von den Lungen-6 (KL-6) and surfactant protein D (SP-D), are clinically used for evalua...

    Authors: Tetsuya Hanaka, Takashi Kido, Shingo Noguchi, Sohsuke Yamada, Hirotsugu Noguchi, Xin Guo, Aya Nawata, Ke-Yong Wang, Keishi Oda, Tsutomu Takaki, Hiroto Izumi, Hiroshi Ishimoto, Kazuhiro Yatera and Hiroshi Mukae

    Citation: BMC Pulmonary Medicine 2019 19:265

    Content type: Research article

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  44. There is little information about vitamin D (Vit D) deficiency in patients with pulmonary hypertension (PH). The objective of this study was: 1) compare Vit D levels between patients with PH, left ventricular ...

    Authors: Andrés N. Atamañuk, Diego F. Litewka, Sergio J. Baratta, Ignacio M. Seropian, Graciela Perez Prados, Miguel O. Payaslian, Juan P. Ortiz Fragola and Pilar Escribano Subias

    Citation: BMC Pulmonary Medicine 2019 19:258

    Content type: Research article

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  45. Pulmonary veno-occlusive disease (PVOD) is a rare condition with poor prognosis, and lung transplantation is recommended as the only curative therapy. The role of pulmonary arterial hypertension targeted thera...

    Authors: Qin Luo, Qi Jin, Zhihui Zhao, Qing Zhao, Xue Yu, Lu Yan, Yi Zhang, Changming Xiong and Zhihong Liu

    Citation: BMC Pulmonary Medicine 2019 19:257

    Content type: Research article

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  46. Health-related quality of life (HRQL) is impaired in patients with idiopathic pulmonary fibrosis (IPF). The King’s Brief Interstitial Lung Disease questionnaire (K-BILD) is a validated measure of HRQL, but no ...

    Authors: Thomas Skovhus Prior, Ole Hilberg, Saher Burhan Shaker, Jesper Rømhild Davidsen, Nils Hoyer, Surinder S. Birring and Elisabeth Bendstrup

    Citation: BMC Pulmonary Medicine 2019 19:255

    Content type: Research article

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  47. Regular participation in physical activity by people with cystic fibrosis (CF) promotes positive clinical and health outcomes including reduced rate of decline in lung function, fewer hospitalizations and grea...

    Authors: Narelle S. Cox, Beverley Eldridge, Sarah Rawlings, Julianna Dreger, Jennifer Corda, Jennifer Hauser, Brenda M. Button, Jennifer Bishop, Amanda Nichols, Anna Middleton, Nathan Ward, Tiffany Dwyer, Owen W. Tomlinson, Sarah Denford, Alan R. Barker, Craig A. Williams…

    Citation: BMC Pulmonary Medicine 2019 19:253

    Content type: Study protocol

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  48. Mycoplasma pneumoniae is one of the most common pathogens causing community acquired pneumonia in children. Although the rate of macrolide-refractory Mycoplasma pneumoniae (MRMP) has increased, systemic glucocort...

    Authors: Hwan Soo Kim, In Suk Sol, Donghe Li, Miyoung Choi, Yun Jung Choi, Kyung Suk Lee, Ju Hee Seo, Yong Ju Lee, Hyeon-Jong Yang and Hyun Hee Kim

    Citation: BMC Pulmonary Medicine 2019 19:251

    Content type: Research article

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