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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. 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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  2. 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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  3. 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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  4. 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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  5. 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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  6. 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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  7. 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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  8. 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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  9. 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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  10. 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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  11. 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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  12. 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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  13. 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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  14. 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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  15. 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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  16. 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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  17. 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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  18. 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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  19. 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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  20. 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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  21. 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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  22. The intensity and frequency of cough remain unclear in interstitial lung disease (ILD). The aim of this study was to evaluate the intensity and frequency of cough in idiopathic interstitial pneumonias (IIPs), ...

    Authors: Ryuhei Sato, Tomohiro Handa, Hisako Matsumoto, Takeshi Kubo and Toyohiro Hirai

    Citation: BMC Pulmonary Medicine 2019 19:247

    Content type: Research article

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  23. During cardiopulmonary exercise testing (CPET), Idiopathic Pulmonary Fibrosis (IPF) patients do not reach their direct maximum voluntary ventilation (MVV) and have deranged gas exchange. Their exercise limitat...

    Authors: Benjamin D. Fox, Yael Shostak, Barak Pertzov, Baruch Vainshelboim, Shimon Itzhakian, Irit Terner and Mordechai R. Kramer

    Citation: BMC Pulmonary Medicine 2019 19:244

    Content type: Research article

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  24. Exogenous lipoid pneumonia is a rare disease resulting from intra-alveolar accumulation of lipids of mineral, vegetal, or animal origin, that induce a foreign body type of inflammatory reaction in the lungs. G...

    Authors: Jacques BOUTROS, Marine MUZZONE, Jonathan BENZAQUEN, Michael LEVRAUT, Charles-Hugo MARQUETTE, Fanny ROCHER, Yann DIASCORN, Bernard PADOVANI, Véronique HOFMAN and Sylvie LEROY

    Citation: BMC Pulmonary Medicine 2019 19:234

    Content type: Case report

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  25. Systemic sclerosis (SSc) is a severe rheumatic disease of the interstitial tissue, in which heart and lung involvement can lead to disease-specific mortality. Our study tests the hypothesis that in addition to...

    Authors: Ralf Ewert, Till Ittermann, Dirk Habedank, Matthias Held, Tobias J. Lange, Michael Halank, Jörg Winkler, Sven Gläser, Horst Olschewski and Gabor Kovacs

    Citation: BMC Pulmonary Medicine 2019 19:230

    Content type: Research article

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  26. Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial pneumonia with an unpredictable course. The aims of this study were to retrospectively re-evaluate a cohort of patients with IPF according to t...

    Authors: Miia Kärkkäinen, Hannu-Pekka Kettunen, Hanna Nurmi, Tuomas Selander, Minna Purokivi and Riitta Kaarteenaho

    Citation: BMC Pulmonary Medicine 2019 19:228

    Content type: Research article

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  27. The CAnadian REgistry for Pulmonary Fibrosis (CARE-PF) is a multi-center, prospective registry designed to study the natural history of fibrotic interstitial lung disease (ILD) in adults. The aim of this cross...

    Authors: J. H. Fisher, M. Kolb, M. Algamdi, J. Morisset, K. A. Johannson, S. Shapera, P. Wilcox, T. To, M. Sadatsafavi, H. Manganas, N. Khalil, N. Hambly, A. J. Halayko, A. S. Gershon, C. D. Fell, G. Cox…

    Citation: BMC Pulmonary Medicine 2019 19:223

    Content type: Research article

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  28. Idiopathic pulmonary fibrosis (IPF) is a disease with poor prognosis mainly affecting males. Differences in clinical presentation between genders may be important both for the diagnostic work-up and for follow...

    Authors: Dimitrios Kalafatis, Jing Gao, Ida Pesonen, Lisa Carlson, C. Magnus Sköld and Giovanni Ferrara

    Citation: BMC Pulmonary Medicine 2019 19:222

    Content type: Research article

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  29. Eosinophilic lung diseases represent a heterogeneous group of disorders with prominent infiltrate of eosinophils in lung interstitium and alveolar spaces. Peripheral blood eosinophilia is often present. Infect...

    Authors: Magda Zanelli, Maxwell Smith, Maurizio Zizzo, Angelo Carloni, Riccardo Valli, Loredana De Marco, Moira Foroni, Andrea Palicelli, Giovanni Martino and Stefano Ascani

    Citation: BMC Pulmonary Medicine 2019 19:216

    Content type: Case report

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  30. Acute exacerbation (AE) is the major cause of morbidity and mortality in patients with idiopathic pulmonary fibrosis (IPF). AEs also occur in other forms of fibrosing interstitial lung disease (fILD). The clin...

    Authors: Mengshu Cao, Jian Sheng, Xiaohua Qiu, Dandan Wang, Dongmei Wang, Yang Wang, Yonglong Xiao and Hourong Cai

    Citation: BMC Pulmonary Medicine 2019 19:215

    Content type: Research article

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  31. Fibrosing, non-idiopathic pulmonary fibrosis (non-IPF) interstitial lung diseases (fILDs) are a heterogeneous group of diseases characterized by a different amount of inflammation and fibrosis. Therapy is curr...

    Authors: Sebastiano Emanuele Torrisi, Nicolas Kahn, Julia Wälscher, Nilab Sarmand, Markus Polke, Kehler Lars, Monika Eichinger, Claus Peter Heussel, Stefano Palmucci, Francesca Maria Sambataro, Gianluca Sambataro, Domenico Sambataro, Carlo Vancheri and Michael Kreuter

    Citation: BMC Pulmonary Medicine 2019 19:213

    Content type: Research article

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  32. A three-drug regimen (macrolide, ethambutol, and rifampicin) is recommended for the treatment of Mycobacterium avium complex pulmonary disease (MAC-PD). Although macrolide has proven efficacy, the role of ethambu...

    Authors: Hyung-Jun Kim, Jong Sik Lee, Nakwon Kwak, Jaeyoung Cho, Chang-Hoon Lee, Sung Koo Han and Jae-Joon Yim

    Citation: BMC Pulmonary Medicine 2019 19:212

    Content type: Research article

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  33. Delay in diagnosis and treatment of pulmonary tuberculosis (PTB) leads to severe disease, adverse outcomes and increased transmission. Assessing the extent of delay and its effect on disease progression in TB ...

    Authors: Fentabil Getnet, Meaza Demissie, Alemayehu Worku, Tesfaye Gobena, Rea Tschopp, Michael Girmachew, Gebeyehu Assefa and Berhanu Seyoum

    Citation: BMC Pulmonary Medicine 2019 19:201

    Content type: Research article

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  34. Myeloperoxidase anti-neutrophil cytoplasmic antibody-related nephritis (MPO-ANCA nephritis) is occasionally accompanied by lung abnormalities such as pulmonary fibrosis. However, the clinical features of pulmo...

    Authors: Toshikazu Watanabe, Tomoyuki Minezawa, Midori Hasegawa, Yasuhiro Goto, Takuya Okamura, Yosuke Sakakibara, Yoshikazu Niwa, Atsushi Kato, Masamichi Hayashi, Sumito Isogai, Masashi Kondo, Naoki Yamamoto, Naozumi Hashimoto and Kazuyoshi Imaizumi

    Citation: BMC Pulmonary Medicine 2019 19:194

    Content type: Research article

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  35. Connective tissue disease-associated interstitial lung disease (CTD-ILD) is associated with reduced quality of life and poor prognosis. Prior studies have not identified a consistent combination of variables t...

    Authors: Chrystal Chan, Christopher J. Ryerson, James V. Dunne and Pearce G. Wilcox

    Citation: BMC Pulmonary Medicine 2019 19:192

    Content type: Research article

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  36. In 2015, South Korea experienced an outbreak of Middle East respiratory syndrome (MERS), and our hospital experienced a nosocomial MERS infection. We performed a comprehensive analysis to identify the MERS tra...

    Authors: Hyun Kyun Ki, Sang Kuk Han, Jun Seong Son and Sang O Park

    Citation: BMC Pulmonary Medicine 2019 19:190

    Content type: Research article

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  37. Diffuse alveolar haemorrhage (DAH) is characterized by the diffuse accumulation of red blood cells within the alveoli, presence of ground glass opacities and/or consolidation on computed tomography (CT). Aside...

    Authors: Anna Stainer, Alex Rice, Anand Devaraj, Joseph Luke Barnett, Jacqueline Donovan, Maria Kokosi, Andrew Gordon Nicholson, Tom Cairns, Athol Umfrey Wells and Elisabetta Augusta Renzoni

    Citation: BMC Pulmonary Medicine 2019 19:185

    Content type: Case report

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  38. Chronic obstructive pulmonary disease (COPD) and pulmonary tuberculosis (PTB) share a number of common risk factors, including innate immunity-related genetic factors. In the present study, we compared the rol...

    Authors: Zhongqi Li, Xuhua Mao, Qiao Liu, Huan Song, Biyu He, Peiyi Shi, Qun Zhang, Xiaona Li and Jianming Wang

    Citation: BMC Pulmonary Medicine 2019 19:184

    Content type: Research article

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  39. Fibrotic interstitial lung disease (FILD) patients are typically dyspneic and exercise-intolerant with consequent impairment of health-related quality of life (HRQoL). Respiratory muscle dysfunction is among t...

    Authors: Pauliane Vieira Santana, Leticia Zumpano Cardenas, André Luis Pereira de Albuquerque, Carlos Roberto Ribeiro de Carvalho and Pedro Caruso

    Citation: BMC Pulmonary Medicine 2019 19:183

    Content type: Research article

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  40. Most international guidelines recommend empirical therapy for community-acquired pneumonia (CAP) to be based on site of care. Some patients with severe CAP are managed in general wards because of limited inten...

    Authors: Phunsup Wongsurakiat and Napat Chitwarakorn

    Citation: BMC Pulmonary Medicine 2019 19:179

    Content type: Research article

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  41. Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder that is associated with oculocutaneous albinism, bleeding diathesis, granulomatous colitis, and highly penetrant pulmonary fibrosis in some su...

    Authors: Martina Doubková, Jakub Trizuljak, Zuzana Vrzalová, Anna Hrazdirová, Ivona Blaháková, Lenka Radová, Šárka Pospíšilová and Michael Doubek

    Citation: BMC Pulmonary Medicine 2019 19:178

    Content type: Case report

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  42. 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

    Content type: Research article

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  43. 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

    Content type: Research article

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  44. 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

    Content type: Case report

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  45. 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

    Content type: Research article

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  46. 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

    Content type: Case report

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  47. 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

    Content type: Research article

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  48. 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

    Content type: Correspondence

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