Results 41 to 50 of about 1,106,073 (318)

Novel folliculin gene mutations in Polish patients with Birt–Hogg–Dubé syndrome

Orphanet Journal of Rare Diseases, 2021
Background Birt–Hogg–Dubé syndrome (BHDS) is a rare, autosomal dominant, inherited disease caused by mutations in the folliculin gene (FLCN). The disease is characterised by skin lesions (fibrofolliculomas, trichodiscomas, acrochordons), pulmonary cysts ...
Elżbieta Radzikowska, Urszula Lechowicz, Jolanta Winek, Lucyna Opoka   +3 more
doaj   +1 more source

Local lung responses following endobronchial elastase and lipopolysaccharide instillation in sheep [PDF]

, 2006
Chronic lipopolysaccharide (LPS) exposure may contribute to the pathogenesis of a number of lung diseases including COPD and emphysema. We sought to develop a large-animal model of emphysema using repeated LPS administration into sheep lung segments.
Blundell, Richard, Rhind, Susan; id_orcid, McLean, Nicola, Rhind, Susan, Milne, Elspeth, Woodall, Chris, Collie, D David S, Baker, Alison, Sallenave, Jean-Michel   +8 more
core   +1 more source

Clinical characteristics and lung function in older children vertically infected with Human Immunodeficiency Virus in Malawi [PDF]

, 2015
T. M. was funded by the Commonwealth scholarship, with research costs from a grant fom Helse Nord Northern Norway Regional Health Authority. E. L. C., R. A. F., and J. R. are supported by Wellcome Trust Fellowships (Senior Fellowship in Clinical Sciences
Webb, Emily L, Rylance, Sarah J, van Oosterhout, Joep J, Emily L. Webb, Mwalukomo, Thandie, Anderson, Suzanne, O'Hare, Bernadette, Elizabeth L. Corbett, Corbett, Elizabeth L, Webb, Emily, Jamie Rylance, Ferrand, Rashida A., van Oosterhout, Joep J., Corbett, Elizabeth L., Sarah J. Rylance, Ferrand, Rashida A, Rashida A. Ferr, Rylance, Jamie, Joep J. Van Oosterhout, Rylance, Sarah, O'Hare, Bernadette Ann-Marie, Suzanne Anderson, Ie Mwalukomo   +22 more
core   +1 more source

A multicentre retrospective observational study on Polish experience of pirfenidone therapy in patients with idiopathic pulmonary fibrosis: the PolExPIR study

BMC Pulmonary Medicine, 2020
Background 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 real-world data (RWD) on the Polish
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, Alicja Siemińska, Krzysztof Sładek, Małgorzata Sobiecka, Karolina Szewczyk, Małgorzata Tomczak, Witold Tomkowski, Elżbieta Wiatr, Dariusz Ziora, Beata Żołnowska, Wojciech J. Piotrowski   +25 more
doaj   +1 more source

Interstitial lung disease [PDF]

European Respiratory Review, 2014
Interstitial lung diseases are a group of diffuse parenchymal lung disorders associated with substantial morbidity and mortality. Knowledge achieved in recent years has resulted in the publication of the new classification of idiopathic interstitial pneumonias, according to which there are three groups: major, rare and unclassified.
Antoniou, Katerina M., Margaritopoulos, George A., Tomassetti, Sara, Bonella, Francesco, Costabel, Ulrich, Poletti, Venerino   +5 more
openaire   +4 more sources

Pulmonary hypertension in diffuse parenchymal lung diseases — is there any benefit of PAH-specific therapy? [PDF]

, 2017
  Pulmonary hypertension (PH) is diagnosed in 40–50% of the patients with end-stage diffuse parenchymal lung diseases (DPLD), and it is associated with significant worsening of life expectancy. Latest ERS/ESC guidelines recommend best available treatment
Kacprzak, Aneta; I Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland, Szturmowicz, Monika; I Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland, Kuś, Jan; I Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland   +2 more
core   +2 more sources

Addressing geographical variation in the progression of non-communicable diseases in Peru: the CRONICAS cohort study protocol. [PDF]

, 2012
Background The rise in non-communicable diseases in developing countries has gained increased attention. Given that around 80% of deaths related to non-communicable diseases occur in low- and middle-income countries, there is a need for local knowledge ...
Smeeth, Liam, Miranda, J Jaime, Bernabe-Ortiz, Antonio, CRONICAS Cohort Study Group, Checkley, William, Gilman, Robert H   +5 more
core   +1 more source

Lung Ultrasonography in the Evaluation of Late Sequelae of COVID-19 Pneumonia—A Comparison with Chest Computed Tomography: A Prospective Study

Viruses
The onset of the COVID-19 pandemic allowed physicians to gain experience in lung ultrasound (LUS) during the acute phase of the disease. However, limited data are available on LUS findings during the recovery phase.
Katarzyna Zimna, Małgorzata Sobiecka, Jacek Wakuliński, Dorota Wyrostkiewicz, Ewa Jankowska, Monika Szturmowicz, Witold Z. Tomkowski   +6 more
doaj   +1 more source

Sustained Therapeutic Efficacy of Intravenous Plasminogen Concentrate in Pediatric Patients With Type 1 Plasminogen Deficiency: An Analysis of Dosing Parameters and Clinical Outcomes

Pediatric Blood &Cancer, EarlyView.
ABSTRACT Background Type 1 plasminogen deficiency (PLGD‐1) is an ultra‐rare autosomal recessive disorder caused by variants in the PLG gene and affects approximately 1.6 individuals per million. The condition is characterized by decreased plasminogen levels and impaired function, resulting in fibrin‐rich lesions on mucous membranes throughout the body.
Charles Nakar, Karen Thibaudeau, Mary Mathias, Heather McDaniel, Madlen Reschke, Klaus Tenbrock, Joseph M. Parker, Amy Shapiro   +7 more
wiley   +1 more source

Diagnostic and prognostic biomarkers for chronic fibrosing interstitial lung diseases with a progressive phenotype [PDF]

, 2020
Biomarkers have the potential to become central to the clinical evaluation and monitoring of patients with chronic fibrosing interstitial lung diseases with a progressive phenotype.
Inoue, Y., Kaner, R. J., Maher, Toby M, Moiseev, S., Maher, T. M., Brown, K. K., Kaner, Robert J, Inoue, Yoshikazu, Tomassetti, Sara, Moiseev, Sergey, Kuwana, Masataka, Kuwana, M., Guiot, Julien, Brown, Kevin K, Tomassetti, S.   +14 more
core   +1 more source