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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

    Idiopathic Pulmonary Fibrosis (IPF) is an aggressive interstitial lung disease with an unpredictable course. Occupational dust exposure may contribute to IPF onset, but its impact on antifibrotic treatment and...

    Authors: Valeria Casillo, Stefania Cerri, Andrea Ciervo, Mariarita Stendardo, Lamberto Manzoli, Maria Elena Flacco, Maurizio Manno, Marialuisa Bocchino, Fabrizio Luppi and Piera Boschetto

    Citation: BMC Pulmonary Medicine 2019 19:170

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  2. Content type: Research article

    Pulmonary hypertension (PH) is traditionally defined as a resting mean pulmonary artery pressure (mPAP) of ≥25 mmHg, while mPAP in the range of 21 to 24 mmHg is recognized as “borderline PH.” Interstitial lung...

    Authors: Kenji Nemoto, Shuji Oh-ishi, Tatsuya Akiyama, Yuki Yabuuchi, Hitomi Goto, Mizu Nonaka, Yuika Sasatani, Hiroaki Tachi, Naoki Arai, Hiroaki Ishikawa, Kentaro Hyodo, Isano Hase, Yukiko Miura, Takio Takaku, Kenji Hayashihara and Takefumi Saito

    Citation: BMC Pulmonary Medicine 2019 19:167

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  3. Content type: Case report

    Myelolipoma is a rare benign tumor composed of mature adipose and hematopoietic tissues. Most myelolipomas are found in the adrenal glands, whereas intrathoracic myelolipoma is extremely rare. In particular, b...

    Authors: Hyun Sung Chung, Kyu Min Lee, Jung Seop Eom, Insu Kim, Seyeon Park, Jihyun Ahn, Ahrong Kim, Chang Hun Lee, Geewon Lee and Min Ki Lee

    Citation: BMC Pulmonary Medicine 2019 19:151

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  4. Content type: Research article

    Recent studies have highlighted the contribution of senescent mesenchymal and epithelial cells in Idiopathic Pulmonary Fibrosis (IPF), but little is known regarding the molecular mechanisms that regulate the a...

    Authors: David M. Habiel, Miriam S. Hohmann, Milena S. Espindola, Ana Lucia Coelho, Isabelle Jones, Heather Jones, Richard Carnibella, Isaac Pinar, Freda Werdiger and Cory M. Hogaboam

    Citation: BMC Pulmonary Medicine 2019 19:165

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  5. Content type: Case report

    Burkholderia contaminans is one of the 20 closely related bacterial of the Burkholderia cepacia complex, a group of bacteria that are ubiquitous in the environment and capable of infecting people with cystic fibr...

    Authors: Daniela Savi, Serena Quattrucci, Maria Trancassini, Claudia Dalmastri, Riccardo V. De Biase, Marta Maggisano, Paolo Palange and Annamaria Bevivino

    Citation: BMC Pulmonary Medicine 2019 19:164

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  7. Content type: Research article

    CTD-related pleural effusions are rare and challenging to diagnose. Our lung inflammation service (with expertise in rheumatology, interstitial lung disease and respiratory failure) works closely with the pleu...

    Authors: Hugh Ip, Parthipan Sivakumar, Eugene Ace McDermott, Sangita Agarwal, Boris Lams, Alex West and Liju Ahmed

    Citation: BMC Pulmonary Medicine 2019 19:161

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  8. Content type: Correspondence

    Lung ultrasound (LUS) is nowadays a fast-growing field of study since the technique has been widely acknowledged as a cost-effective, radiation free, and ready available alternative to standard X-ray imaging. ...

    Authors: Danilo Buonsenso, Anna Maria Musolino, Antonio Gatto, Ilaria Lazzareschi, Antonietta Curatola and Piero Valentini

    Citation: BMC Pulmonary Medicine 2019 19:159

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  9. Content type: Research article

    Nintedanib is an inhibitor of receptor tyrosine kinases, including vascular endothelial growth factor receptor, but its effects on pulmonary hypertension (PH) in idiopathic pulmonary fibrosis (IPF) patients wi...

    Authors: Masahiro Tahara, Keishi Oda, Kei Yamasaki, Takako Kawaguchi, Konomi Sennari, Shingo Noguchi, Noriho Sakamoto, Toshinori Kawanami, Hiroshi Mukae and Kazuhiro Yatera

    Citation: BMC Pulmonary Medicine 2019 19:157

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  10. Content type: Case report

    Nintedanib is a tyrosine kinase inhibitor that efficiently slows the progression of idiopathic pulmonary fibrosis (IPF) and has an acceptable tolerability profile. In contrast, immune checkpoint inhibitors (IC...

    Authors: Hideaki Yamakawa, Tomohiro Oba, Hiroki Ohta, Yuta Tsukahara, Gen Kida, Emiri Tsumiyama, Tomotaka Nishizawa, Rie Kawabe, Shintaro Sato, Keiichi Akasaka, Masako Amano, Kazuyoshi Kuwano and Hidekazu Matsushima

    Citation: BMC Pulmonary Medicine 2019 19:156

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  11. Content type: Case report

    Atypical manifestations, such as elevated serum immunoglobulin-G4 (IgG4) and extra-pulmonary IgG4 positive plasmacyte infiltration, have been described in patients with eosinophilic granulomatosis with polyang...

    Authors: Li Zhou, Fen Cao, Songqing Fan, Ping Chen, Shuizi Ding, Guiqian Liu and Ruoyun Ouyang

    Citation: BMC Pulmonary Medicine 2019 19:154

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  12. Content type: Research article

    Honeycombing on high-resolution computed tomography (HRCT) is a distinguishing feature of usual interstitial pneumonia and predictive of poor outcome in interstitial lung diseases (ILDs). Although fine crackle...

    Authors: Toshikazu Fukumitsu, Yasushi Obase, Yuji Ishimatsu, Shota Nakashima, Hiroshi Ishimoto, Noriho Sakamoto, Kosei Nishitsuji, Shunpei Shiwa, Tomoya Sakai, Sueharu Miyahara, Kazuto Ashizawa, Hiroshi Mukae and Ryo Kozu

    Citation: BMC Pulmonary Medicine 2019 19:153

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  13. Content type: Research article

    The phase III REMoxTB study prospectively enrolled HIV-positive (with CD4+ count > 250 cells, not on anti-retroviral therapy) and HIV-negative patients. We investigated the incidence of adverse events and cure...

    Authors: Conor D. Tweed, Angela M. Crook, Rodney Dawson, Andreas H. Diacon, Timothy D. McHugh, Carl M. Mendel, Sarah K. Meredith, Lerato Mohapi, Michael E. Murphy, Andrew J. Nunn, Patrick P. J. Phillips, Kasha P. Singh, Melvin Spigelman and Stephen H. Gillespie

    Citation: BMC Pulmonary Medicine 2019 19:152

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  14. Content type: Research article

    Clinical studies demonstrate that ivacaftor (IVA) improves health-related quality of life (HRQoL) in patients aged ≥6 years with cystic fibrosis (CF). The real-world impact of IVA and standard of care (SOC) in...

    Authors: Scott C. Bell, Jochen G. Mainz, Gordon MacGregor, Susan Madge, Julie Macey, Moshe Fridman, Ellison D. Suthoff, Siva Narayanan and Nils Kinnman

    Citation: BMC Pulmonary Medicine 2019 19:146

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  15. Content type: Study protocol

    A minority of patients presenting with lower respiratory tract infection (LRTI) to their general practitioner (GP) have community-acquired pneumonia (CAP) and require antibiotic therapy. Identifying them is ch...

    Authors: Loïc Lhopitallier, Andreas Kronenberg, Jean-Yves Meuwly, Isabella Locatelli, Julie Dubois, Joachim Marti, Yolanda Mueller, Nicolas Senn, Valérie D’Acremont and Noémie Boillat-Blanco

    Citation: BMC Pulmonary Medicine 2019 19:143

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  17. Content type: Research article

    Primary ciliary dyskinesia (PCD) is a rare genetic disorder. Although the genetic tests and new diagnostic algorithms have recently been recommended, clinical signs and electron microscope (EM) findings have h...

    Authors: Atsushi Inaba, Masanori Furuhata, Kozo Morimoto, Mahbubur Rahman, Osamu Takahashi, Minako Hijikata, Michael R. Knowles and Naoto Keicho

    Citation: BMC Pulmonary Medicine 2019 19:135

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  18. Content type: Case report

    Pulmonary capillary hemangiomatosis (PCH) is a progressive and refractory vascular disease in the lung. Pulmonary hypertension is frequently combined with PCH when capillary proliferation invades to nearby pul...

    Authors: Chang Dong Yeo, Deokjae Han, Jongmin Lee, Woo-Baek Chung, Jung Im Jung, Kyo-Young Lee, Tae-Jung Kim, Woori Jang, Myungshin Kim and Ji Young Kang

    Citation: BMC Pulmonary Medicine 2019 19:133

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  19. 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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  20. 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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  21. 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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  22. 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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  23. 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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  24. 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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  25. 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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  26. 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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  27. 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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  28. 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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  29. 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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  30. 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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  31. 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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  32. 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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  33. 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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  34. 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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  35. 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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  36. 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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  37. 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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  38. 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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  39. 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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  40. 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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  41. 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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  42. 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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  43. 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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  44. 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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  45. 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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  46. 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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  47. 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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  48. 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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  49. 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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  50. 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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