INTRODUCTION: Pirfenidone, an antifibrotic drug, slows-down the disease progression in idiopathic pulmonary fibrosis (IPF) over 12 months, however limited data on the decline of lung function and overall survival (OS) in real-world cohorts on longer follow-up exists. PATIENTS/METHODS: Of the enrolled Czech IPF patients (n = 841) from an EMPIRE registry, 383 (45.5%) received pirfenidone, 218 (25.9%) no-antifibrotic treatment and 240 (28.5%) were excluded (missing data, nintedanib treatment). The 2- and 5-yrs OS and forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO) were investigated at treatment initiation and 6, 12, 18 and 24 months' follow-up. RESULTS: During a 2-yr follow-up, less than a quarter of the patients progressed on pirfenidone as assessed by the decline of ≥10% FVC (17.0%) and ≥ 15% DLCO (14.3%). On pirfenidone, the DLCO (≥10%) declines at 6, 12, 18 and 24 months' and DLCO (≥15%) declines at 6, 18 and 24 months' follow-up were associated with increased mortality. The DLCO decline showed higher predictive value for mortality than FVC decline. In patients with no-antifibrotics, FVC and DLCO declines were not predictive for mortality. Pirfenidone increased 5-yrs OS over no-antifibrotic treatment (55.9% vs 31.5% alive, P = 0.002). CONCLUSION: Our study observed the 2-yrs sustained effect of pirfenidone on the decline of lung function and survival in the real-world patient's IPF cohort. DLCO decline of ≥10% shows a potential as a mortality predictor in IPF patients on pirfenidone, and should be routinely evaluated during follow-up examinations.

• MeSH
• antiflogistika nesteroidní farmakologie   terapeutické užití   MeSH
• idiopatická plicní fibróza diagnóza   farmakoterapie   epidemiologie   MeSH
• kohortové studie MeSH
• lidé středního věku MeSH
• lidé MeSH
• míra přežití trendy   MeSH
• následné studie MeSH
• progrese nemoci * MeSH
• pyridony farmakologie   terapeutické užití   MeSH
• registrace * MeSH
• respirační funkční testy trendy   MeSH
• senioři MeSH
• vitální kapacita účinky léků   fyziologie   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika MeSH

Determination of biomarkers in body fluids provides information about the overall state of the organism 1. Among commonly determined markers in clinical laboratories belong AAs. The AAs (Fig. 1), basic structural units of peptides, as well as their derivatives occupy many metabolic and biochemical roles in human body 2,3. The AAs belong to zwitterions containing acidic and basic functional groups (carboxyl groups and amino, respectively). Zwitterions have neutral character at a certain pH known as the isoelectric point due the dissociation of acidic and basic groups. Therefore, the charge of AAs depend on the number of functional groups and pH environment 4. Determination of AAs levels in the body fluids such as blood and/or plasma 5,6, cerebrospinal fluid 6,7, urine 6,8, saliva 6,7 or amniotic fluid 9 represents the significant clinical indicator not only for control of the nutritional state of the organism, but for a number of metabolic disorders 10. Examples can be inherited metabolic disorders such as phenylketonuria (PKU) (an enzyme deficiency in phenylalanine hydroxylase (PAH) (Fig. 2)) 11 or maple syrup urine disease (an enzyme deficiency in branched-chain α-ketoacid dehydrogenase (BCKD) (Fig. 3)) 12. There are many methods for AAs determination employing various techniques such as high pressure liquid chromatography (HPLC) 13 or gas chromatography (GC) 14. During the 80's of the 20th century CE became a routine laboratory technique 15. Recently, this method has ranked among very useful tools for separation and determination of AAs 2,9,16,17, biogenic amines 18 or peptides 19 and is often used as an alternative to HPLC or GC due to its electrophoretic separation mechanism 16,20. In addition, a main advantage is very low sample and solvent consumption, speed of analysis and excellent separation efficiency 2,16,21-23. Besides, due to the high resolving power is CE a promising method for the analysis of biological fluids (complex mixtures of many metabolites) 2,22. The essential part of the CE instrumentation is a detector. In photometric and fluorescence detection is problem with a limited presence of chromophores and fluorofores. Because of that, the derivatization procedures prior CE are usually used, which is the one of the limiting factors 24. Therefore, there are attempts to analyze AAs without derivatization employing various strategies such as indirect detection 25, electrochemical detection (conductometric detection 6,8, amperometric detection 26) or CE in combination with mass spectrometry (MS) 5,11.

• MeSH
• aminokyseliny * krev   metabolismus   moč   MeSH
• biologické markery MeSH
• elektroforéza kapilární * MeSH
• lidé MeSH
• tělesné tekutiny * chemie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• práce podpořená grantem MeSH
• přehledy MeSH