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1.
J Asthma ; : 1-7, 2024 Mar 07.
Artigo em Inglês | MEDLINE | ID: mdl-38426671

RESUMO

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. FEV1 and FEF25-75 of forced vital capacity were significantly lower in groups 2 and 3. In group 3, R5, AX, R5-20, and R5-R20/R5 ratios were significantly higher. Bronchodilator responses (BDR) in X5c, AX, and R5-R20 were significantly different between groups and lower in group 3. 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.

2.
Andes Pediatr ; 93(6): 898-905, 2022 Dec.
Artigo em Espanhol | MEDLINE | ID: mdl-37906807

RESUMO

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.


Assuntos
Fibrose Cística , Feminino , Humanos , Adolescente , Fibrose Cística/tratamento farmacológico , Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Qualidade de Vida , Volume Expiratório Forçado , Mutação
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