The Multiple Faces of Non–Cystic Fibrosis Bronchiectasis. A Cluster Analysis Approach

Martínez‐García, Miguel Ángel; Vendrell, Montserrat; Girón, Rosa; Máiz-Carro, Luis; Rosa, David de la; Gracia, Javier de; Olveira, Casilda

doi:10.1513/annalsats.201510-678oc

Cited by 68 publications

(33 citation statements)

References 32 publications

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“…Clinical features included demographics, comorbidities, and clinical, radiological, functional, and microbiological variables. Patients were grouped in four clusters denoted as (i) “ Pseudomonas ,” (ii) “other chronic infection,” (iii) “daily sputum,” and (iv) “dry bronchiectasis.” Using similar approach, Martinez Garcia and colleagues identified four clinical phenotypes according to patient's gender, severity of the disease, and exacerbation rate . It would be interesting to apply a similar cluster analysis approach in children with bronchiectasis, thus identifying children who would benefit from long‐term macrolide therapy.…”

Section: The Role Of Macrolides In Non‐cf Bronchiectasismentioning

confidence: 99%

A review of non‐cystic fibrosis bronchiectasis in children with a focus on the role of long‐term treatment with macrolides

Boustany¹,

Gachelin²,

Colomban³

et al. 2019

Pediatric Pulmonology

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Bronchiectasis is a rare chronic airway disease arising from several respiratory and systemic diseases. The grade of evidence for specific treatment of childhood bronchiectasis unrelated to cystic fibrosis (CF) is low with very few randomized controlled trials. Treatment has been based mainly on evidence from studies in adults with non-cystic fibrosis bronchiectasis and patients with cystic fibrosis. Recently, longterm treatment with macrolides has been proposed. These molecules offer the advantage of anti-inflammatory and immunomodulatory properties in addition to their antibacterial properties. A total of three randomized double-blind placebo-controlled trials conducted in adults showed that macrolides taken for 6-12 months led to a significant reduction in exacerbation rates. Only one long-term, randomized doubleblind placebo-controlled trial was conducted in the pediatric population. It showed that azithromycin administered weekly for up to 24 months reduced pulmonary exacerbations. Further randomized controlled studies are needed to determine the optimal dose and duration of treatment with macrolides. The clinical profile of children who would benefit from this treatment also needs to be determined. K E Y W O R D S macrolides, non-cystic-fibrosis bronchiectasis, pulmonary exacerbations

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Section: The Role Of Macrolides In Non‐cf Bronchiectasismentioning

confidence: 99%

A review of non‐cystic fibrosis bronchiectasis in children with a focus on the role of long‐term treatment with macrolides

Boustany¹,

Gachelin²,

Colomban³

et al. 2019

Pediatric Pulmonology

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“…12,13 Since the widespread use of computed tomography (CT) in the identification and diagnosis of bronchiectasis, there has been increasing global interest in phenotyping patients with bronchiectasis. [14][15][16][17][18] The aim of this study was to describe the clinical, physiological and sputum characteristics in an Australian group of patients hospitalized to a metropolitan healthcare provider with an infective exacerbation of bronchiectasis. Western Health (WH) serves over 800 000 people in the Western suburbs of Melbourne.…”

Section: Introductionmentioning

confidence: 99%

Patients hospitalized with an infective exacerbation of bronchiectasis unrelated to cystic fibrosis: Clinical, physiological and sputum characteristics

2017

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“…Based on our analysis, approximately 50% of non‐PCD gene carriers had a history of cigarette smoking, which could have caused the acquired ciliary defects. Indeed, earlier studies have recognized that bronchiectasis patients with a significant smoking history have more severe clinical disease, a higher incidence of exacerbations, and increased risk of death from respiratory causes . It is also of interest to note that a significant number of carriers of PCD gene mutations have ultrastructural defects, and even gene carriers without CRS demonstrate ultrastructural ciliary abnormalities.…”

Section: Discussionmentioning

confidence: 99%

Prevalence of chronic rhinosinusitis in bronchiectasis patients suspected of ciliary dyskinesia

McCormick

Weeks

Rivers

et al. 2019

Int Forum Allergy Rhinol

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Background: Mucociliary clearance is a main defense mechanism of the airway and is impaired in ciliary dyskinesia. The objective of this study was to evaluate the prevalence of chronic rhinosinusitis (CRS) and its characteristics in bronchiectasis patients suspected of harboring ciliary dyskinesia. Methods:Bronchiectasis patients referred to a rhinology clinic for nasal brush biopsy (NBB) were included in this study. NBB was performed using a cure age technique whereby ciliated epithelial cells were obtained from the surface of the inferior nasal turbinate. Results of transmission electron microscopy findings, primary ciliary dyskinesia (PCD) gene (35 genes) analyses (Invitae), and sinus computed tomography (CT) scans were reviewed.Results: Twenty-three patients (age, 54 ± 2.9 years) were referred for NBB between 2015 and 2018. Thirteen patients (56.5%) met the criteria for diagnosis of CRS. Nineteen patients had ciliary ultrastructural defects. The most common finding was compound cilia (n = 11, 47.8%). Five patients (21.7%) had central microtubule defects (CMD) with higher forced expiratory volume in 1 second (FEV 1 ) at the time of referral than those without CMD (CMD + , 91 ± 3.7%; CMD − , 73.5 ± 5.7%; p = 0.023). Of 15 subjects with a PCD gene panel, 67% (9 of 15) carried at least 1 gene associated with PCD. Only 1 patient reached diagnosis of PCD. Approximately 50% of non-PCD carriers had a smoking history (p < 0.05). Lund-Mackay scores did not significantly differ between PCD and non-PCD carriers (p = 0.72). Conclusion:Nearly half of bronchiectasis patients referred for NBB had concurrent CRS. The presence of ciliary abnormalities was not amplified in bronchiectasis patients with CRS compared to those without CRS. Extrinsic factors may be related to ciliary structural abnormalities in non-PCD gene carriers. C 2019 ARS-AAOA, LLC.

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The Multiple Faces of Non–Cystic Fibrosis Bronchiectasis. A Cluster Analysis Approach

Abstract: Using cluster analysis, it is possible to separate patients with bronchiectasis into distinct clinical phenotypes with different prognoses.

Cited by 68 publications

References 32 publications

A review of non‐cystic fibrosis bronchiectasis in children with a focus on the role of long‐term treatment with macrolides

A review of non‐cystic fibrosis bronchiectasis in children with a focus on the role of long‐term treatment with macrolides

Patients hospitalized with an infective exacerbation of bronchiectasis unrelated to cystic fibrosis: Clinical, physiological and sputum characteristics

Prevalence of chronic rhinosinusitis in bronchiectasis patients suspected of ciliary dyskinesia

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