Monitoring disease progression in childhood bronchiectasis

Ramsey, Kathryn; Schultz, André

doi:10.3389/fped.2022.1010016

Cited by 3 publications

(4 citation statements)

References 72 publications

(46 reference statements)

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“…In addition, there is a need and recent interest for noninvasive biomarkers to investigate bronchiectasis. 15,24,32 To date, the existing literature related to this finding appears inconsistent. In this study, we found that bronchiectasis reversibility was not rare, occurring in a substantial proportion of the study population.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, there is a need and recent interest for non‐invasive biomarkers to investigate bronchiectasis 15,24,32 . To date, the existing literature related to this finding appears inconsistent.…”

Section: Discussionmentioning

confidence: 99%

“…22,23 Thus, the question of bronchiectasis reversibility remains unresolved. 24 Therefore, the goal of this study was to assess the longitudinal clinical validity of UTE-MRI to assess changes in disease severity over time among CF patients undergoing ETI treatment, by using the standard Bhalla score, a clinical scoring system that enables assessing lung structural damages. The secondary objectives were to detect reversibility in structural damage by UTE-MRI such as bronchiectasis, to investigate its reproducibility, to describe the characteristics of patients with and without bronchiectasis reversibility and to assess the correlation between UTE-MRI scorings to pulmonary function tests (PFTs) and CT.…”

mentioning

confidence: 99%

“…Conventional MR methods showcased a reduction in lung structure but the studies combined elements such as wall thickening, bronchiectasis, and mucus plugging 21 ; thereby neglecting a dedicated study on bronchiectasis 22,23 . Thus, the question of bronchiectasis reversibility remains unresolved 24 …”

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confidence: 99%

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Longitudinal Evaluation of Bronchial Changes in Cystic Fibrosis Patients Undergoing Elexacaftor/Tezacaftor/Ivacaftor Therapy Using Lung MRI With Ultrashort Echo‐Times

David,

Benlala,

Bui

et al. 2023

Magnetic Resonance Imaging

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BackgroundLung magnetic resonance imaging (MRI) with ultrashort echo‐times (UTE‐MRI) allows high‐resolution and radiation‐free imaging of the lung structure in cystic fibrosis (CF). In addition, the combination of elexacaftor/tezacaftor/ivacaftor (ETI) has improved CF clinical outcomes such as need for hospitalization. However, the effect on structural disease still needs longitudinal evaluation at high resolution.PurposeTo analyze the effects of ETI on lung structural alterations using UTE‐MRI, with a focus on bronchiectasis reversibility.Study TypeRetrospective.PopulationFifty CF patients (mean age 24.3 ± 9.2; 23 males).Field Strength/Sequence1.5 T, UTE‐MRI.AssessmentAll subjects completed both UTE‐MRI and pulmonary function tests (PFTs) during two annual visits (M0 and M12), and 30 of them completed a CT scan. They initiated ETI treatment after M0 within a maximum of 3 months from the annual examinations. Three observers scored a clinical MRI Bhalla score on UTE‐MRI. Bronchiectasis reversibility was defined as a reduction in both outer and inner bronchial dimensions. Correlations were searched between the Bhalla score and PFT such as the forced expiratory volume in 1 second percentage predicted (FEV1%p).Statistical TestsComparison was assessed using the paired t‐test, correlation using the Spearman correlation test with a significance level of 0.05. Concordance and reproducibility were assessed using intraclass correlation coefficient (ICC).ResultsThere was a significant improvement in MRI Bhalla score after ETI treatment. UTE‐MRI demonstrated bronchiectasis reversibility in a subgroup of 18 out of 50 CF patients (36%). These patients with bronchiectasis reversibility were significantly younger, with lower severity of wall thickening but no difference in mucus plugging extent (P = 0.39) was found. The reproducibility of UTE‐MRI evaluations was excellent (ICC ≥ 0.95), was concordant with CT scan (N = 30; ICC ≥ 0.90) and significantly correlated to FEV1% at PFT at M0 (N = 50; r = 0.71) and M12 (N = 50; r = 0.72).Data ConclusionUTE‐MRI is a reproducible tool for the longitudinal follow‐up of CF patients, allowing to quantify the response to ETI and demonstrating the reversibility of some structural alterations such as bronchiectasis in a substantial fraction of this study population.Level of Evidence4Technical EfficacyStage 2

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Longitudinal Evaluation of Bronchial Changes in Cystic Fibrosis Patients Undergoing Elexacaftor/Tezacaftor/Ivacaftor Therapy Using Lung MRI With Ultrashort Echo‐Times

David,

Benlala,

Bui

et al. 2023

Magnetic Resonance Imaging

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Efficacy and safety of macrolides in the treatment of children with bronchiectasis: a meta-analysis

Song

Zhang

et al. 2023

Pediatr Res

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Therapeutic Management of Bronchiectasis in Children and Adolescents: A Concise Narrative Review

Faverio,

Franco,

Landoni

et al. 2024

JCM

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Introduction: Bronchiectasis, characterized by airway dilation, mucus hypersecretion, and recurrent exacerbations, is increasingly recognized in children and adolescents. Recent guidelines from the European Respiratory Society (ERS) and Thoracic Society of Australia and New Zealand (TSANZ) emphasize early diagnosis and optimized management. This review explores therapeutic strategies for pediatric bronchiectasis. Materials and methods: Our review involved a comprehensive search of English-language literature in the PubMed and EMBASE databases until December 2023, focusing on observational studies, interventions, reviews, and guidelines in pediatric bronchiectasis. Results: Management strategies encompass airway clearance techniques, mucoactive agents, pulmonary rehabilitation, bronchodilators and inhaled corticosteroids tailored to individual needs and age-appropriate techniques. Antibiotics play key roles in preventing exacerbations, eradicating pathogens, and managing acute exacerbations, which are guided by culture sensitivities and symptoms. Long-term antibiotic prophylaxis, particularly macrolides, aims to reduce exacerbations, although concerns about antibiotic resistance persist. Vaccinations, including pneumococcal and influenza vaccines, are crucial for preventing infections and complications. Surgery and lung transplantation are reserved to severe, refractory cases after failure of medical therapies. Conclusions: The optimal management of pediatric bronchiectasis requires a multidisciplinary approach, including physiotherapy, pharmacotherapy, and vaccinations, tailored to individual needs and guided by evidence-based guidelines. Further research is needed to refine diagnostic and therapeutic strategies and improve outcomes for affected children and adolescents.

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Monitoring disease progression in childhood bronchiectasis

Cited by 3 publications

References 72 publications

Longitudinal Evaluation of Bronchial Changes in Cystic Fibrosis Patients Undergoing Elexacaftor/Tezacaftor/Ivacaftor Therapy Using Lung MRI With Ultrashort Echo‐Times

Longitudinal Evaluation of Bronchial Changes in Cystic Fibrosis Patients Undergoing Elexacaftor/Tezacaftor/Ivacaftor Therapy Using Lung MRI With Ultrashort Echo‐Times

Efficacy and safety of macrolides in the treatment of children with bronchiectasis: a meta-analysis

Therapeutic Management of Bronchiectasis in Children and Adolescents: A Concise Narrative Review

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