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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. Content type: Research article

    Culturing of bronchoalveolar lavage (BAL) fluid is a commonly used method for pathogen detection in pneumonia. However, the sensitivity is low, especially in patients pre-treated with anti-infective agents. Th...

    Authors: Eva Tschiedel, Arkadius Goralski, Jörg Steinmann, Peter-Michael Rath, Margarete Olivier, Uwe Mellies, Tanja Kottmann and Florian Stehling

    Citation: BMC Pulmonary Medicine 2019 19:132

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  2. Content type: Research article

    The aim of this study was to analyze the relative frequency, clinical characteristics, disease onset and progression in f-IPF vs. sporadic IPF (s-IPF).

    Authors: Ekaterina Krauss, Godja Gehrken, Fotios Drakopanagiotakis, Silke Tello, Ruth C. Dartsch, Olga Maurer, Anita Windhorst, Daniel von der Beck, Matthias Griese, Werner Seeger and Andreas Guenther

    Citation: BMC Pulmonary Medicine 2019 19:130

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  3. Content type: Case report

    Granulomatosis with polyangiitis (GPA) is characterised by the main violation of the upper and lower respiratory tract and kidney. GPA is considered a systemic vasculitis of medium-sized and small blood vessel...

    Authors: Lei Pan, Jun-Hong Yan, Fu-Quan Gao, Hong Li, Sha-Sha Han, Guo-Hong Cao, Chang-Jun Lv and Xiao-Zhi Wang

    Citation: BMC Pulmonary Medicine 2019 19:122

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  4. Content type: Research article

    Patients with interstitial lung disease (ILD) who show features related to autoimmunity without meeting criteria for a defined connective tissue disease are categorized as interstitial pneumonia with autoimmun...

    Authors: Jeong Uk Lim, Bo Mi Gil, Hye Seon Kang, Jongyeol Oh, Yong Hyun Kim and Soon Seog Kwon

    Citation: BMC Pulmonary Medicine 2019 19:120

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  5. Content type: Case report

    There are few cases of multiple bronchial stenoses reported in the literature and none of the severity described here. The case is relevant due to its rareness, the pathophysiological insights derived, the suc...

    Authors: Thomas Crowhurst, Joshua Lightfoot, Aeneas Yeo, Benjamin Reddi, Phan Nguyen, Helen Whitford and Chien-Li Holmes-Liew

    Citation: BMC Pulmonary Medicine 2019 19:119

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  6. Content type: Research article

    Although infections caused by nontuberculous mycobacteria (NTM) are increasing in prevalence, there are few data about hemoptysis in patients with NTM lung disease. This study investigated the characteristics ...

    Authors: Su Hwan Lee, Jin Hwa Lee, Jung Hyun Chang, Soo Jung Kim, Hee-Young Yoon, Sung Shine Shim, Min Uk Kim, Sun Young Choi and Yon Ju Ryu

    Citation: BMC Pulmonary Medicine 2019 19:117

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  7. Content type: Research article

    Here, we present real-world data on the incidence and risk factors of acute exacerbation (AE) in patients with chronic fibrotic interstitial pneumonia (CFIP) treated with antifibrotic agents, which has been pr...

    Authors: Kodai Kawamura, Kazuya Ichikado, Hidenori Ichiyasu, Keisuke Anan, Yuko Yasuda, Moritaka Suga and Takuro Sakagami

    Citation: BMC Pulmonary Medicine 2019 19:113

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  8. Content type: Research article

    Interstitial lung disease (ILD) is a severe systemic manifestation of rheumatoid arthritis (RA). High-resolution computed tomography (HRCT) represents the gold standard for the diagnosis of ILD, but its routin...

    Authors: A. Manfredi, G. Cassone, S. Cerri, V. Venerito, A. L. Fedele, M. Trevisani, F. Furini, O. Addimanda, F. Pancaldi, G. Della Casa, R. D’Amico, R. Vicini, G. Sandri, P. Torricelli, I. Celentano, A. Bortoluzzi…

    Citation: BMC Pulmonary Medicine 2019 19:111

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  9. Content type: Research article

    Treatment of patients with Cystic Fibrosis homozygous for the Phe508del gene, with Lumacaftor /Ivacaftor (LUM/IVA) improves outcomes in patients with FEV1 > 40% predicted. We set out to observe the most sensitive...

    Authors: Peter A. B. Wark, Kim Cookson, Theeba Thiruchelvam, John Brannan and Douglas J. Dorahy

    Citation: BMC Pulmonary Medicine 2019 19:106

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  10. Content type: Research article

    Bronchial Dieulafoy’s disease (BDD) is a rare disease that is known to be a cause of hemorrhage. The characteristics of this disease are still unknown. The present study describes the disorder based on a revie...

    Authors: Xin Qian, Qiong Du, Na Wei, Meifang Wang, Hansheng Wang and Yijun Tang

    Citation: BMC Pulmonary Medicine 2019 19:104

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  11. Content type: Case report

    Pleural involvement by non-tuberculous mycobacteria (NTM) in patients without distinct pulmonary disease is extremely rare. Vertebral osteomyelitis (VO) with or without pulmonary disease is also a rare clinica...

    Authors: Naoki Kadota, Tsutomu Shinohara, Hiroyuki Hino, Yuichiro Goda, Yoshiro Murase, Satoshi Mitarai and Fumitaka Ogushi

    Citation: BMC Pulmonary Medicine 2019 19:100

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  12. Content type: Research article

    Pulmonary rehabilitation improves exercise capacity and reduces risk of future exacerbation in COPD when performed after an exacerbation. There have been no previous studies of post-exacerbation rehabilitation...

    Authors: James D. Chalmers, Megan L. Crichton, Gill Brady, Simon Finch, Mike Lonergan and Thomas C. Fardon

    Citation: BMC Pulmonary Medicine 2019 19:85

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  13. Content type: Research article

    Gastroesophageal reflux disease (GORD) is highly prevalent in idiopathic pulmonary fibrosis (IPF) and may play a role in its pathogenesis. Recent IPF treatment guidelines suggest that all patients with IPF be ...

    Authors: Helen E. Jo, Tamera J. Corte, Ian Glaspole, Christopher Grainge, Peter M. A. Hopkins, Yuben Moodley, Paul N. Reynolds, Sally Chapman, E. Haydn Walters, Christopher Zappala, Heather Allan, Gregory J. Keir, Wendy A. Cooper, Annabelle M. Mahar, Samantha Ellis, Sacha Macansh…

    Citation: BMC Pulmonary Medicine 2019 19:84

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  14. Content type: Case report

    Rosai-Dorfman disease (RDD) is a rare histiocytosis which involves principally lymph nodes. Thyroid involvement in RDD is a very rare situation, and lung involvement is even rarer.

    Authors: Pietro Gianella, Nicolas Dulguerov, Grégoire Arnoux, Marc Pusztaszeri and Jörg D. Seebach

    Citation: BMC Pulmonary Medicine 2019 19:83

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  15. Content type: Research article

    There is a paucity of data on long term clinical effects of high frequency chest wall oscillation (HFCWO) in the Bronchiectasis population. Other therapies such as nebulized mucolytics and long term antibiotic...

    Authors: Jordan Powner, Andrew Nesmith, Denay P. Kirkpatrick, Jessica K. Nichols, Brent Bermingham and George M. Solomon

    Citation: BMC Pulmonary Medicine 2019 19:82

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  16. Content type: Research article

    In many clinical disorders, there is a relationship between the ratio of the diameter of the main pulmonary artery (mPA) to that of the aorta (Ao) on chest computed tomography (CT). The aim of this study was t...

    Authors: Ji Soo Choi, Sang Hoon Lee, Ah. Young Leem, Joo Han Song, Kyung Soo Chung, Ji Ye Jung, Young Ae Kang, Moo Suk Park, Young Sam Kim, Joon Chang and Song Yee Kim

    Citation: BMC Pulmonary Medicine 2019 19:81

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  17. Content type: Case report

    Systemic artery to pulmonary artery fistulas (SA-PAFs), are extremely rare in people without congenital heart disease. In this group of patients pulmonary arterial hypertension was reported in the single case....

    Authors: Wojciech Jacheć, Andrzej Tomasik, Marcin Kurzyna, Radosław Pietura, Adam Torbicki, Jan Głowacki, Ewa Nowalany-Kozielska and Celina Wojciechowska

    Citation: BMC Pulmonary Medicine 2019 19:80

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  18. Content type: Research article

    In 2017, Australia experienced its highest levels of influenza virus activity since the 2009 pandemic. This allowed detailed comparison of the characteristics of patients with community and hospital-acquired i...

    Authors: Nikita Parkash, Wendy Beckingham, Patiyan Andersson, Paul Kelly, Sanjaya Senanayake and Nicholas Coatsworth

    Citation: BMC Pulmonary Medicine 2019 19:79

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  19. Content type: Research article

    Current clinical practice guidelines for idiopathic pulmonary fibrosis (IPF) conditionally recommend use of pirfenidone and nintedanib. However, an optimal treatment sequence has not been established, and the ...

    Authors: Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Takuma Katano, Erina Tabata, Ryota Shintani, Shinko Sadoyama, Hideaki Yamakawa, Tsuneyuki Oda, Ryo Okuda, Hideya Kitamura, Tae Iwasawa, Tamiko Takemura and Takashi Ogura

    Citation: BMC Pulmonary Medicine 2019 19:78

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  20. Content type: Research article

    Preventative medication reduces hospitalisations in people with cystic fibrosis (PWCF) but adherence is poor. We assessed the feasibility of a randomised controlled trial of a complex intervention, which combi...

    Authors: Daniel Hind, Sarah J. Drabble, Madelynne A. Arden, Laura Mandefield, Simon Waterhouse, Chin Maguire, Hannah Cantrill, Louisa Robinson, Daniel Beever, Alexander J. Scott, Sam Keating, Marlene Hutchings, Judy Bradley, Julia Nightingale, Mark I. Allenby, Jane Dewar…

    Citation: BMC Pulmonary Medicine 2019 19:77

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  21. Content type: Case report

    CFTR modulator therapy with ivacaftor is a treatment option for Cystic Fibrosis (CF) patients with at least one copy of a R117H-7T mutation in the CFTR gene. Desirable effects of this therapy are improvement o...

    Authors: Matthias Welsner, Svenja Straßburg, Christian Taube and Sivagurunathan Sutharsan

    Citation: BMC Pulmonary Medicine 2019 19:76

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  22. Content type: Study protocol

    Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease, with a median survival of 2–3 years and variable natural history, characterized by gradual and progressive deterioration. Acute exacerbation of idio...

    Authors: Jean-Marc Naccache, Melissa Montil, Jacques Cadranel, Marine Cachanado, Vincent Cottin, Bruno Crestani, Dominique Valeyre, Benoit Wallaert, Tabassome Simon and Hilario Nunes

    Citation: BMC Pulmonary Medicine 2019 19:75

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  23. Content type: Research article

    Pulmonary Hypertension Association UK (PHA-UK) is the only charity in the UK especially for people affected by pulmonary hypertension (PH). To better understand the impact of PH on patients and carers beyond c...

    Authors: Iain Armstrong, Catherine Billings, David G. Kiely, Janelle Yorke, Carl Harries, Shaun Clayton and Wendy Gin-Sing

    Citation: BMC Pulmonary Medicine 2019 19:67

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  24. Content type: Case report

    Fibrosing interstitial lung disease is the poor prognostic non-infectious lung disease by unknown etiology. Here, we present one case developing interstitial pneumonia with fibrosis after treatment of pneumocy...

    Authors: Tetsuya Suzuki, Yukiko Shimoda, Katsuji Teruya, Hiroyuki Gatanaga, Yoshimi Kikuchi, Shinichi Oka and Koji Watanabe

    Citation: BMC Pulmonary Medicine 2019 19:65

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  25. Content type: Case report

    Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of phospholipoproteinaceous material in the alveoli. The presentation is nonspecific but typically includes dyspnea; th...

    Authors: Dacia S. K. Boyce, John W. Lee, Phalgoon Shah, Judy H. Freeman, Matthew C. Aboudara and David C. Hostler

    Citation: BMC Pulmonary Medicine 2019 19:61

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  26. Content type: Research article

    Cambodia, a lower middle-income country of about 16 million individuals in southeast Asia, endures a high burden of both tuberculosis and other lower respiratory infections. Differentiating tuberculosis from o...

    Authors: Engi F. Attia, Yaty Pho, Somary Nhem, Chandara Sok, Borady By, Dariven Phann, Huy Nob, Sovanndeth Thann, Sinath Yin, Rachael Noce, Chamrouensann Kim, Joanne Letchford, Thomas Fassier, Sarin Chan and T. Eoin West

    Citation: BMC Pulmonary Medicine 2019 19:60

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  27. Content type: Research article

    The increasing prevalence and recognition of bronchiectasis in clinical practice necessitates a better understanding of the economic disease burden to improve the management and achieve better clinical and eco...

    Authors: Pieter C. Goeminne, Francisco Hernandez, Roland Diel, Anna Filonenko, Rowena Hughes, Fabian Juelich, George M. Solomon, Alex Upton, Kamonthip Wichmann, Weiwei Xu and James D. Chalmers

    Citation: BMC Pulmonary Medicine 2019 19:54

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  28. Content type: Research article

    Bronchoalveolar lavage (BAL) is utilized for diagnosing lung infiltrates in immunocompromised. There is heterogeneity in the data and reported diagnostic yields range from 26 to 69%. Therefore, selection crite...

    Authors: Randall Choo, Naser Salman Hamza Naser, Nivedita Vikas Nadkarni and Devanand Anantham

    Citation: BMC Pulmonary Medicine 2019 19:51

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  29. Content type: Case report

    The term hepatopulmonary syndrome typically applies to cyanosis that results from “intrapulmonary vascular dilatation” due to advanced liver disease. Similar findings may result from a congenital portosystemic sh...

    Authors: Mohammed T. Alsamri, Mohamed A. Hamdan, Mohamed Sulaiman, Hassib Narchi and Abdul-Kader Souid

    Citation: BMC Pulmonary Medicine 2019 19:49

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  30. Content type: Research article

    In patients with non-HIV Pneumocystis jirovecii pneumonia (PjP), computed tomography imaging reveals ground grass opacities (GGO). Previous reports show that some patients with non-HIV PjP exhibit GGO with crazy ...

    Authors: Shogo Kumagai, Machiko Arita, Takashi Koyama, Takao Kumazawa, Daiki Inoue, Atsushi Nakagawa, Yusuke Kaji, Kenjiro Furuta, Motonari Fukui, Keisuke Tomii, Yoshio Taguchi, Hiromi Tomioka and Tadashi Ishida

    Citation: BMC Pulmonary Medicine 2019 19:47

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  31. Content type: Case report

    Opportunistic infections, while well studied in the AIDS population, continue to have variable and surprising presentations. Here, we present a case of disseminated histoplasmosis with disseminated nontubercul...

    Authors: Julian Choi, Kia Nikoomanesh, Jusleen Uppal and Sharon Wang

    Citation: BMC Pulmonary Medicine 2019 19:46

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  32. Content type: Research article

    Diffuse lung diseases (DLD) are characterized by different immunophenotypes in the bronchoalveolar lavage fluid (BALF). We aimed to evaluate the diagnostic value of BALF NK and NKT cell counts of patients with...

    Authors: Oksana Sokhatska, Eva Padrão, Bernardo Sousa-Pinto, Marília Beltrão, Patrícia Caetano Mota, Natália Melo, Luís Delgado and António Morais

    Citation: BMC Pulmonary Medicine 2019 19:39

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  33. Content type: Research article

    Bronchoscopy plays a key role to diagnose the etiology, to localize the site, and to identify the sources of the bleeding in patients with hemoptysis, but the ideal timing of an endoscopic examination is still...

    Authors: Michele Mondoni, Paolo Carlucci, Giuseppe Cipolla, Alessandro Fois, Stefano Gasparini, Silvia Marani, Stefano Centanni and Giovanni Sotgiu

    Citation: BMC Pulmonary Medicine 2019 19:36

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  34. Content type: Case report

    Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disorder defined by high serum immunoglobulin E titers that is associated with recurrent respiratory infections, formation of pneumoatoc...

    Authors: Aaron Hakim, Isabel S. Bazan, Mamadou L. Sanogo, Edward P. Manning, Jeffrey S. Pollak and Geoffrey L. Chupp

    Citation: BMC Pulmonary Medicine 2019 19:34

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  35. Content type: Research article

    Tumour necrosis factor-related apoptosis-inducing ligand (TRAIL) has previously been demonstrated to play a pro-inflammatory role in allergic airways disease and COPD through the upregulation of the E3 ubiquit...

    Authors: Adam M. Collison, Junyao Li, Ana Pereira de Siqueira, Xuejiao Lv, Hamish D. Toop, Jonathan C. Morris, Malcolm R. Starkey, Philip M. Hansbro, Jie Zhang and Joerg Mattes

    Citation: BMC Pulmonary Medicine 2019 19:31

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  36. Content type: Research article

    Chronic cough is a difficult-to-treat comorbidity of idiopathic pulmonary fibrosis (IPF), and significantly impacts on the quality of life of patients with IPF. Transient receptor potential (TRP) channel prote...

    Authors: Yali Guo, Sun Ying, Xuehui Zhao, Jian Liu and Yuguang Wang

    Citation: BMC Pulmonary Medicine 2019 19:27

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  37. Content type: Case report

    Immunoglobulin G4-related disease (IgG4-RD) encompasses a group of immune-mediated disorders that are gaining increasing recognition. Pulmonary presentations are common, with four types of patterns been descri...

    Authors: Junping Fan, Ruie Feng, Xiaomeng Hou, Ji Li, Xuefeng Sun, Jinglan Wang, Juhong Shi, Mengzhao Wang and Yan Xu

    Citation: BMC Pulmonary Medicine 2019 19:26

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  38. Content type: Case report

    Any immunological mechanisms induced by influenza virus could cause severe secondary bacterial superinfection such as those by Streptococcus pyogenes [group A streptococcus (GAS)], Streptococcus pneumoniae or Sta...

    Authors: Nobuhiro Asai, Hiroyuki Suematsu, Daisuke Sakanashi, Hideo Kato, Mao Hagihara, Hiroki Watanabe, Arufumi Shiota, Yusuke Koizumi, Yuka Yamagishi and Hiroshige Mikamo

    Citation: BMC Pulmonary Medicine 2019 19:25

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  39. Content type: Study protocol

    Telehealth and telemonitoring is an emerging area of study in people with cystic fibrosis (CF), with the potential of increasing access to care, and minimising infection control risks to patients without compr...

    Authors: Ray Lei Lang, Christine Wilson, Kellie Stockton, Trevor Russell and Leanne Marie Johnston

    Citation: BMC Pulmonary Medicine 2019 19:21

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  40. Content type: Research article

    By comparing diagnoses made by pre-transplant surgical lung biopsy (SLB) and the final pathologic diagnosis of the explanted pathology (EP), we aimed to study the factors that could impact pathologic diagnoses...

    Authors: Tanmay S. Panchabhai, Andrea Valeria Arrossi, Kristin B. Highland, Debabrata Bandyopadhyay, Daniel A. Culver, Marie M. Budev and Carol F. Farver

    Citation: BMC Pulmonary Medicine 2019 19:20

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  41. Content type: Research article

    Standardization of trans-bronchial lung cryobiopsy in diffuse parenchymal lung diseases is imminent; however, the majority of published series on cryobiopsy include a limited number of patients and are charact...

    Authors: Claudia Ravaglia, Athol U. Wells, Sara Tomassetti, Carlo Gurioli, Christian Gurioli, Alessandra Dubini, Alberto Cavazza, Thomas V. Colby, Sara Piciucchi, Silvia Puglisi, Marcello Bosi and Venerino Poletti

    Citation: BMC Pulmonary Medicine 2019 19:16

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  42. Content type: Case report

    Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe condition with limited treatment strategies. Although respiratory infection is a major cause of AE-IPF, no reports have indicated pertus...

    Authors: Kuniaki Hirai, Tetsuya Homma, Fumihiro Yamaguchi, Munehiro Yamaguchi, Shintaro Suzuki, Akihiko Tanaka, Tsukasa Ohnishi and Hironori Sagara

    Citation: BMC Pulmonary Medicine 2019 19:15

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  43. Content type: Research article

    Relapse of cryptogenic organizing pneumonia (COP) may lead to poor long-term prognosis and necessitates multiple rounds of steroid treatment with potential adverse effects. The objective of this study is to id...

    Authors: Zenya Saito, Yugo Kaneko, Tsukasa Hasegawa, Masahiro Yoshida, Kyuto Odashima, Tsugumi Horikiri, Akira Kinoshita, Keisuke Saitoh and Kazuyoshi Kuwano

    Citation: BMC Pulmonary Medicine 2019 19:10

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  44. Content type: Research article

    A clinical heterogeneity was reported in patients with Cystic Fibrosis (CF) with the same CFTR genotype and between siblings with CF.

    Authors: Vito Terlizzi, Marco Lucarelli, Donatello Salvatore, Adriano Angioni, Arianna Bisogno, Cesare Braggion, Roberto Buzzetti, Vincenzo Carnovale, Rosaria Casciaro, Giuseppe Castaldo, Natalia Cirilli, Mirella Collura, Carla Colombo, Antonella Miriam Di Lullo, Ausilia Elce, Vincenzina Lucidi…

    Citation: BMC Pulmonary Medicine 2018 18:196

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  45. Content type: Research article

    The present study aimed to develop an automated computed tomography (CT) score based on the CT quantification of high-attenuating lung structures, in order to provide a quantitative assessment of lung structur...

    Authors: Trieu-Nghi Hoang-Thi, Marie-Pierre Revel, Pierre-Régis Burgel, Laurence Bassinet, Isabelle Honoré, Thong Hua-Huy, Charlotte Martin, Bernard Maitre and Guillaume Chassagnon

    Citation: BMC Pulmonary Medicine 2018 18:194

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  46. Content type: Research article

    Guidelines currently do not recommend the routine use of chest x-ray (CXR) in bronchiolitis. However, CXR is still performed in a high percentage of cases, mainly to diagnose or rule out pneumonia. The inappro...

    Authors: Carlotta Biagi, Luca Pierantoni, Michelangelo Baldazzi, Laura Greco, Ada Dormi, Arianna Dondi, Giacomo Faldella and Marcello Lanari

    Citation: BMC Pulmonary Medicine 2018 18:191

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  47. Content type: Case report

    Coexistence of aspergilloma and allergic bronchopulmonary aspergillosis (ABPA) has rarely been reported. Although the treatment for ABPA includes administration of corticosteroids and antifungal agents, little...

    Authors: Kohei Horiuchi, Takanori Asakura, Naoki Hasegawa and Fumitake Saito

    Citation: BMC Pulmonary Medicine 2018 18:185

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  48. Content type: Case report

    Granulomatosis with polyangiitis (GPA) is a systemic ANCA-associated vasculitis characterized by necrotizing granulomatous inflammation and a predilection for the upper and lower respiratory tract. Eosinophili...

    Authors: Michele V. Quan, Stephen K. Frankel, Mehrnaz Maleki-Fischbach and Laren D. Tan

    Citation: BMC Pulmonary Medicine 2018 18:181

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  49. Content type: Research article

    2′-5′-Oligoadenylate synthetase 1 (OAS1) plays an important role in inflammatory immune reactions. OAS1 polymorphisms have been associated with increased susceptibility to various diseases. We investigated the as...

    Authors: Shouquan Wu, Yu Wang, Guo Chen, Miaomiao Zhang, Minggui Wang and Jian-Qing He

    Citation: BMC Pulmonary Medicine 2018 18:180

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