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. Research article

    Efficacy of 1, 5, and 20 mg oral sildenafil in the treatment of adults with pulmonary arterial hypertension: a randomized, double-blind study with open-label extension

    In a previous study, 6-minute walk distance (6MWD) improvement with sildenafil was not dose dependent at the 3 doses tested (20, 40, and 80 mg 3 times daily [TID]). This study assessed whether lower doses were...

    Carmine Dario Vizza, B. K. S. Sastry, Zeenat Safdar, Lutz Harnisch, Xiang Gao, Min Zhang, Manisha Lamba and Zhi-Cheng Jing

    BMC Pulmonary Medicine 2017 17:44

    Published on: 23 February 2017

  2. Case report

    Birt–Hogg–Dubé syndrome: a literature review and case study of a Chinese woman presenting a novel FLCN mutation

    The Birt–Hogg–Dubé (BHD) syndrome is a very rare autosomal dominant form of genodermatosis caused by germline mutations in the folliculin (FLCN) gene, which is mapped to the p11.2 region in chromosome 17. BHD com...

    Shengyu Hao, Fei Long, Fenglan Sun, Teng Liu, Daowei Li and Shujuan Jiang

    BMC Pulmonary Medicine 2017 17:43

    Published on: 21 February 2017

  3. Case report

    Elderly-onset hereditary pulmonary alveolar proteinosis and its cytokine profile

    Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by surfactant accumulation, and is caused by disruption of granulocyte/macrophage colony-stimulating factor (GM-CSF) signaling. Abnorma...

    Masayuki Ito, Kazuyuki Nakagome, Hiromitsu Ohta, Keiichi Akasaka, Yoshitaka Uchida, Atsushi Hashimoto, Ayako Shiono, Toshinori Takada, Makoto Nagata, Jun Tohyama, Koichi Hagiwara, Minoru Kanazawa, Koh Nakata and Ryushi Tazawa

    BMC Pulmonary Medicine 2017 17:40

    Published on: 17 February 2017

  4. Research article

    Upregulation of interleukin-33 and thymic stromal lymphopoietin levels in the lungs of idiopathic pulmonary fibrosis

    Innate T helper type 2 (Th2) immune responses mediated by interleukin (IL)-33, thymic stromal lymphopoietin (TSLP), and IL-25 have been shown to play an important role in pulmonary fibrosis of animal models; h...

    Jong-Uk Lee, Hun Soo Chang, Hyeon Ju Lee, Chang An Jung, Da Jeong Bae, Hyun Ji Song, Jong Sook Park, Soo-Taek Uh, Young Hoon Kim, Ki-Hyun Seo and Choon-Sik Park

    BMC Pulmonary Medicine 2017 17:39

    Published on: 15 February 2017

  5. Case report

    First time identification of Pandoraea sputorum from a patient with cystic fibrosis in Argentina: a case report

    Pandoraea species are considered emerging pathogens in the context of cystic fibrosis (CF) and are difficult to identify by conventional biochemical methods. These multidrug resistant ...

    Pablo F. Martina, Mónica Martínez, Guillermo Frada, Florencia Alvarez, Lorena Leguizamón, Claudia Prieto, Carolina Barrias, Marisa Bettiol, Antonio Lagares, Alejandra Bosch, Julián Ferreras and Martha Von Specht

    BMC Pulmonary Medicine 2017 17:33

    Published on: 7 February 2017

  6. Research article

    Hemosiderin-laden macrophages are an independent factor correlated with pulmonary vascular resistance in idiopathic pulmonary fibrosis: a case control study

    Increases in hemosiderin-laden macrophages (HLM) are reported to be observed in idiopathic pulmonary fibrosis (IPF). According to a recent study, significant correlation between hemosiderin deposition in the l...

    Jun Fukihara, Hiroyuki Taniguchi, Masahiko Ando, Yasuhiro Kondoh, Tomoki Kimura, Kensuke Kataoka, Taiki Furukawa, Takeshi Johkoh, Junya Fukuoka, Koji Sakamoto and Yoshinori Hasegawa

    BMC Pulmonary Medicine 2017 17:30

    Published on: 6 February 2017

  7. Research article

    Pulmonary oxygen uptake and muscle deoxygenation kinetics during heavy intensity cycling exercise in patients with emphysema and idiopathic pulmonary fibrosis

    Little is known about the mechanistic basis for the exercise intolerance characteristic of patients with respiratory disease; a lack of clearly defined, distinct patient groups limits interpretation of many st...

    Melitta A. McNarry, Nicholas K. Harrison, Tom Withers, Narendra Chinnappa and Michael J. Lewis

    BMC Pulmonary Medicine 2017 17:26

    Published on: 31 January 2017

  8. Study protocol

    A prospective pilot study of home monitoring in adults with cystic fibrosis (HOME-CF): protocol for a randomised controlled trial

    Home monitoring has the potential to detect early pulmonary exacerbations in people with cystic fibrosis (CF), with consequent improvements in health outcomes and healthcare associated costs. This study aims t...

    Jocelyn Choyce, Karen L. Shaw, Alice J. Sitch, Hema Mistry, Joanna L. Whitehouse and Edward F. Nash

    BMC Pulmonary Medicine 2017 17:22

    Published on: 23 January 2017

  9. Research article

    Cystic fibrosis physicians’ perspectives on the timing of referral for lung transplant evaluation: a survey of physicians in the United States

    Prior studies reveal that a significant proportion of patients with cystic fibrosis (CF) and advanced lung disease are not referred for lung transplant (LTx) evaluation. We sought to assess expert CF physician...

    Kathleen J. Ramos, Ranjani Somayaji, Erika D. Lease, Christopher H. Goss and Moira L. Aitken

    BMC Pulmonary Medicine 2017 17:21

    Published on: 19 January 2017

  10. Research article

    A novel reflex cough testing device

    The reflex cough test is useful for detecting silent aspiration, a risk factor for aspiration pneumonia. However, assessing the risk of aspiration pneumonia requires measuring not only the cough reflex but als...

    Kazunori Fujiwara, Katsuyuki Kawamoto, Yoko Shimizu, Takahiro Fukuhara, Satoshi Koyama, Hideyuki Kataoka, Hiroya Kitano and Hiromi Takeuchi

    BMC Pulmonary Medicine 2017 17:19

    Published on: 18 January 2017

  11. Case report

    Successful outcome following pneumonectomy in a teenage boy with cystic fibrosis: a case report

    Cystic fibrosis lung disease is generally a diffuse process however rarely one lung may become particularly damaged through chronic collapse and consolidation resulting in end-stage bronchiectasis with relativ...

    Zheyi Liew, Santosh Mallikarjuna, Asif Hasan, F. Kate Gould, Su Bunn, Matthew F. Thomas, Jim L. Lordan, Christopher O’Brien and Malcolm Brodlie

    BMC Pulmonary Medicine 2017 17:17

    Published on: 13 January 2017

  12. Research article

    Are risk predicting models useful for estimating survival of patients with rheumatoid arthritis-associated interstitial lung disease?

    Risk predicting models have been applied in idiopathic pulmonary fibrosis (IPF), but still not validated in patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). The purpose of this...

    Hanna M. Nurmi, Minna K. Purokivi, Miia S. Kärkkäinen, Hannu-Pekka Kettunen, Tuomas A. Selander and Riitta L. Kaarteenaho

    BMC Pulmonary Medicine 2017 17:16

    Published on: 13 January 2017

  13. Research article

    Identification and validation of differentially expressed transcripts by RNA-sequencing of formalin-fixed, paraffin-embedded (FFPE) lung tissue from patients with Idiopathic Pulmonary Fibrosis

    Idiopathic Pulmonary Fibrosis (IPF) is a lethal lung disease of unknown etiology. A major limitation in transcriptomic profiling of lung tissue in IPF has been a dependence on snap-frozen fresh tissues (FF). I...

    Milica Vukmirovic, Jose D. Herazo-Maya, John Blackmon, Vesna Skodric-Trifunovic, Dragana Jovanovic, Sonja Pavlovic, Jelena Stojsic, Vesna Zeljkovic, Xiting Yan, Robert Homer, Branko Stefanovic and Naftali Kaminski

    BMC Pulmonary Medicine 2017 17:15

    Published on: 12 January 2017

  14. Research article

    Effects of treadmill exercise versus Flutter® on respiratory flow and sputum properties in adults with cystic fibrosis: a randomised, controlled, cross-over trial

    Treadmill exercise and airway clearance with the Flutter® device have previously been shown to improve mucus clearance mechanisms in people with cystic fibrosis (CF) but have not been compared. It is therefore...

    Tiffany J. Dwyer, Rahizan Zainuldin, Evangelia Daviskas, Peter T. P. Bye and Jennifer A. Alison

    BMC Pulmonary Medicine 2017 17:14

    Published on: 11 January 2017

  15. Research article

    Characteristics associated with progression in patients with of nontuberculous mycobacterial lung disease : a prospective cohort study

    Patients with distinctive morphotype were more susceptible to nontuberculous mycobacterial lung disease (NTM-LD). However, little is known about the association between body morphotype and progression of NTM-L...

    Soo Jung Kim, Soon Ho Yoon, Sun Mi Choi, Jinwoo Lee, Chang-Hoon Lee, Sung Koo Han and Jae-Joon Yim

    BMC Pulmonary Medicine 2017 17:5

    Published on: 5 January 2017

  16. Case report

    Atrial septostomy and disease targeting therapy in pulmonary hypertension secondary to neurofibromatosis

    Neurofibromatosis type 1 (NF1) is a rare multisystem genetic disorder. During the course of the disease it can be rarely complicated with pulmonary hypertension (PH) which confers a dismal prognosis.

    George Giannakoulas, Panagiotis Savvoulidis, Vasilios Grosomanidis, Sophia-Anastasia Mouratoglou, Haralambos Karvounis and Stavros Hadjimiltiades

    BMC Pulmonary Medicine 2016 16:175

    Published on: 7 December 2016

  17. Debate

    Cystic fibrosis lung environment and Pseudomonas aeruginosa infection

    The airways of patients with cystic fibrosis (CF) are highly complex, subject to various environmental conditions as well as a distinct microbiota. Pseudomonas aeruginosa is recognized as one of the most importan...

    Anjali Y. Bhagirath, Yanqi Li, Deepti Somayajula, Maryam Dadashi, Sara Badr and Kangmin Duan

    BMC Pulmonary Medicine 2016 16:174

    Published on: 5 December 2016

  18. Research article

    Sera from severe trauma patients with pneumonia and without infectious complications have differential effects on neutrophil biology

    Major trauma patients (TP) developing imbalanced immune response are at high risk for infectious post-injury complications including pneumonia. Neutrophils play a central role in the host defense against bacte...

    B. Relja, R. Taraki, M. P. J. Teuben, K. Mörs, N. Wagner, S. Wutzler, F. Hildebrand, M. Perl and I. Marzi

    BMC Pulmonary Medicine 2016 16:171

    Published on: 1 December 2016

  19. Research article

    The balance between the serum levels of IL-6 and IL-10 cytokines discriminates mild and severe acute pneumonia

    To identify markers for earlier diagnosis of severe pneumonia, we assess the correlation between serum cytokine profile of children with different pneumonia severity.

    Rita de Cássia Coelho Moraes de Brito, Norma Lucena-Silva, Leuridan Cavalcante Torres, Carlos Feitosa Luna, Jaílson de Barros Correia and Giselia Alves Pontes da Silva

    BMC Pulmonary Medicine 2016 16:170

    Published on: 1 December 2016

  20. Research article

    Long-term clinical course of anti-glycyl tRNA synthetase (anti-EJ) antibody-related interstitial lung disease pathologically proven by surgical lung biopsy

    Anti-glycyl-tRNA synthetase (anti-EJ) antibody is occasionally positive in patients with interstitial lung disease (ILD). We aimed to define the clinical, radiological and pathological features of patients wit...

    Hajime Sasano, Eri Hagiwara, Hideya Kitamura, Yasunori Enomoto, Norikazu Matsuo, Tomohisa Baba, Shinichiro Iso, Koji Okudela, Tae Iwasawa, Shinji Sato, Yasuo Suzuki, Tamiko Takemura and Takashi Ogura

    BMC Pulmonary Medicine 2016 16:168

    Published on: 1 December 2016

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