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Characterization of human alcohol dehydrogenase 4 and aldehyde dehydrogenase 2 as enzymes involved in the formation of 5-carboxylpirfenidone, a major metabolite of pirfenidone.
Drug Metab Dispos
January 2025
Drug Metabolism and Toxicology, Faculty of Pharmaceutical Sciences, Kanazawa University, Kakuma-machi, Kanazawa, Japan; WPI Nano Life Science Institute (WPI-NanoLSI), Kanazawa University, Kakuma-machi, Kanazawa, Japan.
Pirfenidone (PIR) is used in the treatment of idiopathic pulmonary fibrosis. After oral administration, it is metabolized by cytochrome P450 1A2 to 5-hydroxylpirfenidone (5-OH PIR) and further oxidized to 5-carboxylpirfenidone (5-COOH PIR), a major metabolite excreted in the urine (90% of the dose). This study aimed to identify enzymes that catalyze the formation of 5-COOH PIR from 5-OH PIR in the human liver.
View Article and Find Full Text PDFInitial therapy in patients with pulmonary arterial hypertension and cardiovascular comorbidities.
Eur Respir J
January 2025
INSERM UMR_S 999 « Pulmonary Hypertension: Pathophysiology and Novel Therapies », Hôpital Marie Lannelongue, Le Plessis-Robinson, France
Background: European guidelines recommend initial monotherapy in PAH patients with cardiovascular (CV) comorbidities based on the limited of evidence for combination therapy in this growing population.
Methods: A retrospective analysis was conducted on incident PAH patients enrolled in the French Pulmonary Hypertension Registry between 2009 and 2020. Propensity score matching was used to investigate initial dual oral combination therapy oral monotherapy in patients with at least one CV comorbidity (, hypertension, obesity, diabetes and coronary artery disease).
Patients with idiopathic pulmonary fibrosis have fatty lungs impacting respiratory physiology.
Pulmonology
December 2025
Respiratory Translational Research Group, Department of Laboratory Medicine, School of Health Sciences, College of Health and Medicine, University of Tasmania, Launceston, Australia.
The clinical meaning of the UIP pattern in fibrotic hypersensitivity pneumonitis on cryobiopsy: A multicentre retrospective study.
Pulmonology
December 2025
Department of Medical Specialities, Pulmonology Unit, GB Morgagni-L. Pierantoni Hospital, Forlì, Italy.
Fibrotic hypersensitivity pneumonitis (f-HP) is an interstitial lung disease in which various antigens in susceptible individuals may play a pathogenetic role. This study evaluates the role of transbronchial lung cryobiopsy (TBLC) and bronchoalveolar lavage (BAL) in identifying a UIP-like pattern and its association with fibrosis progression. We conducted a multicentre retrospective cohort study of patients diagnosed with f-HP who underwent BAL and TBLC between 2011 and 2023.
View Article and Find Full Text PDFTreatment patterns and clinical profile in progressive pulmonary fibrosis: a Japanese cross-sectional survey.
Front Med (Lausanne)
January 2025
Department of Respiratory Medicine, Faculty of Medicine, Kyorin University, Tokyo, Japan.
Background: There is a paucity of real-world data on patients with interstitial lung diseases (ILDs) that are progressive, other than idiopathic pulmonary fibrosis (IPF), including treatment patterns and attitudes toward treatment. This study aimed to investigate the diagnosis, clinical characteristics, treatment paradigm and current decision-making practices of IPF and progressive pulmonary fibrosis (PPF) in a Japanese real-world setting.
Methods: Data were drawn from the Adelphi Real World PPF-ILD Disease Specific Programme™, a cross-sectional survey with retrospective data collection of pulmonologists and rheumatologists in Japan from April to October 2022.
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