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  1. Article ; Online: Triterapia con moduladores de la proteína de conductancia de transmembrana en fibrosis quística.

    Fielbaum Colodro, Oscar / Vidal Grell, Alberto / Méndez Yarur, Alejandra / Sobarzo Clerc, Camila

    Andes pediatrica : revista Chilena de pediatria

    2023  Volume 93, Issue 6, Page(s) 898–905

    Abstract: Recent studies have shown that therapy with Cystic fibrosis transmembrane conductance regulator (CFTR) modulators in Cystic Fibrosis (CF) patients with Elexacaftor-Tezacaftor-Ivacaftor (ETI) decreases exacerbations and improves lung function and quality ... ...

    Title translation Tritherapy with cystic fibrosis transmembrane conductance regulator protein modulators in cystic fibrosis.
    Abstract Recent studies have shown that therapy with Cystic fibrosis transmembrane conductance regulator (CFTR) modulators in Cystic Fibrosis (CF) patients with Elexacaftor-Tezacaftor-Ivacaftor (ETI) decreases exacerbations and improves lung function and quality of life.
    Objective: to report the clinical response in the first patient in our setting treated with ETI.
    Clinical case: 14-year-old female patient with severe cystic fibrosis phenotype, heterozygous for the Phe508del mutation (Phe508del/1078delT), with moderate pulmonary involvement and pancreatic insufficiency (PI). The patient started ETI therapy after the disease entered a phase of clinical and pulmonary function deterioration. From the second visit (45 days) until the end of the follow-up (365 days), the patient experienced a significant improvement in the quality-of-life domains measured by the CFQ-R 14+ questionnaire. In the pulmonary function study at 45 and 365 days, FVC increased by 21% and 22%, FEV1 by 20% and 27%, and FEF 25-75 by 23% and 37%, respectively. Nutritional assessment para meters in the first six months of follow-up showed an increase in wBMI from 1.6 to 5.6 k. No adverse effects were observed.
    Conclusion: treatment with ETI in our CF patient showed a remarkable im provement in quality of life, pulmonary function, and nutritional status.
    MeSH term(s) Female ; Humans ; Adolescent ; Cystic Fibrosis/drug therapy ; Cystic Fibrosis/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Quality of Life ; Forced Expiratory Volume ; Mutation
    Chemical Substances Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6)
    Language Spanish
    Publishing date 2023-10-25
    Publishing country Chile
    Document type Case Reports ; English Abstract ; Journal Article
    ISSN 2452-6053
    ISSN (online) 2452-6053
    DOI 10.32641/andespediatr.v93i6.4361
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Reactance inversion in moderate to severe persistent asthma: low birth weight, prematurity effect, and bronchodilator response.

    González Vera, Ramiro / Vidal Grell, Alberto / Castro-Rodríguez, José A / Palomino Montenegro, María A / Méndez Yarur, Alejandra

    The Journal of asthma : official journal of the Association for the Care of Asthma

    2024  , Page(s) 1–7

    Abstract: Introduction: Reactance inversion (RI) has been associated with impaired peripheral airway function in persistent asthma. However, there is little to no data about the difference between asthmatic children with and without RI. This study aimed to detect ...

    Abstract Introduction: Reactance inversion (RI) has been associated with impaired peripheral airway function in persistent asthma. However, there is little to no data about the difference between asthmatic children with and without RI. This study aimed to detect clinical and lung function differences in moderate-severe asthmatic children with and without RI.
    Methods: This study was conducted between 2021 and 2022 in asthmatic school-age children. Impulse oscillometry (IOS) and spirometry were performed according to ATS/ERS standards.
    Results: A total of 62 patients, with a mean age of 8.4 years, 54.8% were males and were divided into three groups: group 1 (32.3%) with no RI, group 2 (27.4%) with RI but disappearing after bronchodilator test and group 3 (40.3%) with persistent RI after bronchodilator test. Children in groups 2 and 3 had significantly lower birth weights than in group 1. Group 2 had lower gestational age compared to group 1. FEV
    Conclusion: RI is frequently found in children with moderate-severe persistent asthma, particularly in those with a history of prematurity or low birth weight. In some patients, RI disappears after the bronchodilator test; however, it, persists in those with the worst pulmonary function. RI could be a small airway dysfunction marker.
    Language English
    Publishing date 2024-03-07
    Publishing country England
    Document type Journal Article
    ZDB-ID 603816-5
    ISSN 1532-4303 ; 0277-0903
    ISSN (online) 1532-4303
    ISSN 0277-0903
    DOI 10.1080/02770903.2024.2324865
    Database MEDical Literature Analysis and Retrieval System OnLINE

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