Bronchial tree‐shaped mucous plug in cystic fibrosis: imaging‐guided management

Salamone, Ignazio; Mondello, B; Lucanto, Maria Cristina; Cristadoro, Simona; Lombardo, Mariangela; Barone, Mario

doi:10.1002/rcr2.214

Cited by 13 publications

(9 citation statements)

References 5 publications

(6 reference statements)

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“…1a. This is likely to be caused by the presence of mucus obstruction which is a feature of this animal model 27,28 . Our previous synchrotron-based study 24 used Figure 1.…”

Section: Results and Analysismentioning

confidence: 99%

Quantification of muco-obstructive lung disease variability in mice via laboratory X-ray velocimetry

Werdiger

Donnelley

Dubsky

et al. 2020

Sci Rep

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To effectively diagnose, monitor and treat respiratory disease clinicians should be able to accurately assess the spatial distribution of airflow across the fine structure of lung. This capability would enable any decline or improvement in health to be located and measured, allowing improved treatment options to be designed. Current lung function assessment methods have many limitations, including the inability to accurately localise the origin of global changes within the lung. However, X-ray velocimetry (XV) has recently been demonstrated to be a sophisticated and non-invasive lung function measurement tool that is able to display the full dynamics of airflow throughout the lung over the natural breathing cycle. In this study we present two developments in XV analysis. Firstly, we show the ability of laboratory-based XV to detect the patchy nature of cystic fibrosis (CF)-like disease in β-ENaC mice. Secondly, we present a technique for numerical quantification of CF-like disease in mice that can delineate between two major modes of disease symptoms. We propose this analytical model as a simple, easy-to-interpret approach, and one capable of being readily applied to large quantities of data generated in XV imaging. Together these advances show the power of XV for assessing local airflow changes. We propose that XV should be considered as a novel lung function measurement tool for lung therapeutics development in small animal models, for CF and for other muco-obstructive diseases. Cystic fibrosis (CF) is a progressive, chronic and debilitating genetic disease caused by mutations in the CF Transmembrane-conductance Regulator (CFTR) gene. Unrelenting CF airway disease begins early in infancy and produces a steady deterioration in quality of life, ultimately leading to premature death. Effective lung health assessment tools must capture the patchy nature of muco-obstructive lung diseases such as cystic fibrosis, and this is particularly important during the early stages of the disease when local treatments could be applied to prevent disease progression. Assessments of overall lung health in humans and animals are typically made using lung function tests that screen for abnormalities by measuring the flow of gas in the airways. Spirometry is the most common tool for assessing lung function, however it measures global airflow at the mouth. This means that parameters such as FEV 1 are a single, global measure of the health of the entire lung, are effort-dependent, and provide no information about the location of muco-obstructive disease. Multiple breath wash-in or wash-out techniques that measure the lung-clearance index (LCI) are sensitive to some changes in CF disease as they reflect the health of both the small and large airways 1, 2 , but can only provide limited regional airflow information. LCI is rarely reported in laboratory animal studies. The forced oscillation technique (FOT) measures the resistive properties of the respiratory system and has been used in obstructive lung disease assessment, although the re...

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“…1a. This is likely to be caused by the presence of mucus obstruction which is a feature of this animal model 27,28 . Our previous synchrotron-based study 24 used Figure 1.…”

Section: Results and Analysismentioning

confidence: 99%

Quantification of muco-obstructive lung disease variability in mice via laboratory X-ray velocimetry

Werdiger

Donnelley

Dubsky

et al. 2020

Sci Rep

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“…Salamone et al . (2017) published a case report where they mechanically removed bronchial tree-shaped mucous plug in a cystic fibrosis patient 9 . Park et al mentioned that mucus impaction and plastic bronchitis are usually self-limited or responsive to medical therapy, with a good prognosis, although their patient did not respond to oral corticosteroid or intrabronchial instillation of acetylcysteine and continued to deteriorate despite medical therapy 8 .…”

Section: Discussionmentioning

confidence: 99%

Case Report: Mucus plug in bronchus mimicking a bronchial solid foreign body obstruction

Devkota¹,

He²,

Zhang³

2017

F1000Res

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Bronchial foreign body obstruction is common in all clinical settings. Obstruction of the airway due to foreign bodies and foreign body aspiration are major causes of childhood mortality and morbidity, which are a big challenge to manage. Occasionally, bronchial obstruction may be due to mucus plugs or other endogenous factors. Here we describe a case of bronchial obstruction caused by mucus plug formation that was managed conservatively in a one-year old boy. The patient was suffering from a cough and noisy breathing for 2 days prior to coming to our hospital, when he experienced sudden onset of difficulty in breathing and a severe cough. At the time of presentation his vital sign readings were:- HR 186 bpm, RR 46/min, BP 78/40 MmHg, temp 36.9°C and SPO2 68%. He was given oxygen immediately and nebulization was started. Chest CT scan was performed that suggested the presence of a right bronchial foreign body with right sided obstructive emphysema. The patient was stable with oxygenation and nebulization with ipratropium bromide, albuterol, normal saline and budesonide before the CT scan. Therefore, we conclude that symptoms resembling foreign body obstruction are not always aspirated or inhaled, and sometimes secreted sputum forms a plug, which mimics the symptoms of foreign body obstruction.

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“…In emergency situation it is the first choice. Bronchoscopy is required to achieve lung expansion [ 4 , 5 ].…”

Section: Treatmentmentioning

confidence: 99%

Pleural Effusion or Main Left Bronchus Mucus Obstruction: To Drain or Not to Drain? Decision-Making for Young Surgeon on Call

Coco¹,

Leanza²

2018

Case Reports in Radiology

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Mucous plugs occur in a number of pulmonary conditions. Central right or left bronchus mucus plug causes complete pulmonary collapse making it an emergency life-threatening case. We describe the case of an 80-year-old man that, in postoperative period after a urological intervention, has had a progressive tachypnea and dyspnea during hospitalization for urological problems. Young surgeon on call was called.

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Bronchial tree‐shaped mucous plug in cystic fibrosis: imaging‐guided management

Cited by 13 publications

References 5 publications

Quantification of muco-obstructive lung disease variability in mice via laboratory X-ray velocimetry

Quantification of muco-obstructive lung disease variability in mice via laboratory X-ray velocimetry

Case Report: Mucus plug in bronchus mimicking a bronchial solid foreign body obstruction

Pleural Effusion or Main Left Bronchus Mucus Obstruction: To Drain or Not to Drain? Decision-Making for Young Surgeon on Call

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