دورية أكاديمية

Circulating metabolic profile in idiopathic pulmonary fibrosis: data from the IPF-PRO Registry.

التفاصيل البيبلوغرافية
العنوان: Circulating metabolic profile in idiopathic pulmonary fibrosis: data from the IPF-PRO Registry.
المؤلفون: Summer R; Thomas Jefferson University, Philadelphia, PA, USA. ross.summer@jefferson.edu., Todd JL; Duke Clinical Research Institute, Durham, NC, USA.; Duke University Medical Center, Durham, NC, USA., Neely ML; Duke Clinical Research Institute, Durham, NC, USA.; Duke University Medical Center, Durham, NC, USA., Lobo LJ; University of North Carolina School of Medicine, Chapel Hill, NC, USA., Namen A; Wake Forest School of Medicine, Winston-Salem, NC, USA., Newby LK; Duke Clinical Research Institute, Durham, NC, USA.; Duke University Medical Center, Durham, NC, USA., Shafazand S; University of Miami, Miami, FL, USA., Suliman S; Banner University Medical Center, Phoenix, AZ, USA., Hesslinger C; Boehringer Ingelheim Pharma GmbH & Co. KG, Biberach, Germany., Keller S; Boehringer Ingelheim Pharma GmbH & Co. KG, Biberach, Germany., Leonard TB; Boehringer Ingelheim Pharmaceuticals, Inc, Ridgefield, CT, USA., Palmer SM; Duke Clinical Research Institute, Durham, NC, USA.; Duke University Medical Center, Durham, NC, USA., Ilkayeva O; Duke Molecular Physiology Institute, Durham, NC, USA.; Department of Medicine, Division of Endocrinology, Metabolism, and Nutrition, Duke University School of Medicine, Durham, NC, USA., Muehlbauer MJ; Duke Molecular Physiology Institute, Durham, NC, USA., Newgard CB; Duke Molecular Physiology Institute, Durham, NC, USA., Roman J; Jane and Leonard Korman Institute, Thomas Jefferson University, Philadelphia, PA, USA.
المصدر: Respiratory research [Respir Res] 2024 Jan 25; Vol. 25 (1), pp. 58. Date of Electronic Publication: 2024 Jan 25.
نوع المنشور: Clinical Study; Journal Article
اللغة: English
بيانات الدورية: Publisher: BioMed Central Ltd Country of Publication: England NLM ID: 101090633 Publication Model: Electronic Cited Medium: Internet ISSN: 1465-993X (Electronic) Linking ISSN: 14659921 NLM ISO Abbreviation: Respir Res Subsets: MEDLINE
أسماء مطبوعة: Publication: 2001- : London : BioMed Central Ltd.
Original Publication: London : Current Science Ltd., c2000-
مواضيع طبية MeSH: Carnitine*/analogs & derivatives , Idiopathic Pulmonary Fibrosis*/metabolism, Humans ; Ceramides ; Disease Progression ; Fatty Acids ; Metabolome ; Registries
مستخلص: Background: The circulating metabolome, reflecting underlying cellular processes and disease biology, has not been fully characterized in patients with idiopathic pulmonary fibrosis (IPF). We evaluated whether circulating levels of metabolites correlate with the presence of IPF, with the severity of IPF, or with the risk of clinically relevant outcomes among patients with IPF.
Methods: We analyzed enrollment plasma samples from 300 patients with IPF in the IPF-PRO Registry and 100 individuals without known lung disease using a set of targeted metabolomics and clinical analyte modules. Linear regression was used to compare metabolite and clinical analyte levels between patients with IPF and controls and to determine associations between metabolite levels and measures of disease severity in patients with IPF. Unadjusted and adjusted univariable Cox regression models were used to evaluate associations between circulating metabolites and the risk of mortality or disease progression among patients with IPF.
Results: Levels of 64 metabolites and 5 clinical analytes were significantly different between patients with IPF and controls. Among analytes with greatest differences were non-esterified fatty acids, multiple long-chain acylcarnitines, and select ceramides, levels of which were higher among patients with IPF versus controls. Levels of the branched-chain amino acids valine and leucine/isoleucine were inversely correlated with measures of disease severity. After adjusting for clinical factors known to influence outcomes, higher levels of the acylcarnitine C:16-OH/C:14-DC were associated with all-cause mortality, lower levels of the acylcarnitine C16:1-OH/C14:1DC were associated with all-cause mortality, respiratory death, and respiratory death or lung transplant, and higher levels of the sphingomyelin d43:2 were associated with the risk of respiratory death or lung transplantation.
Conclusions: IPF has a distinct circulating metabolic profile characterized by increased levels of non-esterified fatty acids, long-chain acylcarnitines, and ceramides, which may suggest a more catabolic environment that enhances lipid mobilization and metabolism. We identified select metabolites that were highly correlated with measures of disease severity or the risk of disease progression and that may be developed further as biomarkers.
Trial Registration: ClinicalTrials.gov; No: NCT01915511; URL: www.
Clinicaltrials: gov .
(© 2024. The Author(s).)
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فهرسة مساهمة: Keywords: Biomarkers; Interstitial lung diseases; Metabolomics; Pulmonary fibrosis
سلسلة جزيئية: ClinicalTrials.gov NCT01915511
المشرفين على المادة: 0 (acylcarnitine)
S7UI8SM58A (Carnitine)
0 (Ceramides)
0 (Fatty Acids)
تواريخ الأحداث: Date Created: 20240125 Date Completed: 20240206 Latest Revision: 20240206
رمز التحديث: 20240206
مُعرف محوري في PubMed: PMC10809477
DOI: 10.1186/s12931-023-02644-7
PMID: 38273290
قاعدة البيانات: MEDLINE
الوصف
تدمد:1465-993X
DOI:10.1186/s12931-023-02644-7