Telephone surveillance during 2019 novel coronavirus disease: Is it a helpful diagnostic tool for detecting acute pulmonary exacerbations in children with chronic lung disease?

Introduction The global burden of the 2019 novel coronavirus disease pandemic on the healthcare system, as well as the high transmission risk of coronavirus disease has led to the use of alternative strategies for evaluation of children with chronic conditions. In this context, telemedicine has become the effective and affordable choice. In this study we aimed to evaluate the efficiency of telephone visits to determine pulmonary exacerbations and hospitalization rates of children with cystic fibrosis and interstitial lung disease. Methods A total of 119 children with cystic fibrosis or interstitial lung disease were enrolled and provided cases in which telephone visits were applied during the peak time of the coronavirus disease pandemic in our country. The recordings of respiratory, gastrointestinal and other symptoms, nutrition status, rate of acute pulmonary exacerbation, treatments initiated by telephone visits, referral to hospital and hospitalization were established from the electronic health reports of the patients. Results Thirteen patients (10.9%) were symptomatic, 12 of them (10%) were diagnosed with acute pulmonary exacerbation. One patient was diagnosed with peripheral facial paralysis. Nine patients were recalled to the hospital and seven patients (5.8%) were hospitalised. Discussion Using telemedicine the health status of patients can be defined, and patients can be guided on proper healthcare that they need, especially during the times of pandemics which we are facing. Communication with patients while minimising the risk of exposure to coronavirus disease is an important advantage of telemedicine. Telemedicine will have to be implemented on our daily medical practice in the near future.

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Risk factors for lung disease progression in children with cystic fibrosis

European Respiratory Journal ◽

10.1183/13993003.02509-2017 ◽

2018 ◽

Vol 51 (6) ◽

pp. 1702509 ◽

Cited By ~ 5

Author(s):

Marieke van Horck ◽

Kim van de Kant ◽

Bjorn Winkens ◽

Geertjan Wesseling ◽

Vincent Gulmans ◽

...

Keyword(s):

Risk Factors ◽

Cystic Fibrosis ◽

Lung Disease ◽

Disease Progression ◽

Potential Risk ◽

Allergic Bronchopulmonary Aspergillosis ◽

Pulmonary Exacerbation ◽

Exacerbation Rate ◽

Potential Risk Factors ◽

Pulmonary Exacerbations

To identify potential risk factors for lung disease progression in children with cystic fibrosis (CF), we studied the longitudinal data of all children with CF (aged ≥5 years) registered in the Dutch CF Registry (2009–2014).Lung disease progression was expressed as a decline in lung function (forced expiratory volume in 1 s (FEV1) % pred) and pulmonary exacerbation rate. Potential risk factors at baseline included sex, age, best FEV1 % pred, best forced vital capacity % pred, genotype, body mass index z-score, pancreatic insufficiency, medication use (proton pump inhibitors (PPIs), prophylactic antibiotics and inhaled corticosteroids), CF-related diabetes, allergic bronchopulmonary aspergillosis and colonisation with Pseudomonas aeruginosa.The data of 545 children were analysed. PPI use was associated with both annual decline of FEV1 % pred (p=0.017) and future pulmonary exacerbation rate (p=0.006). Moreover, lower FEV1 % pred at baseline (p=0.007), prophylactic inhaled antibiotic use (p=0.006) and pulmonary exacerbations in the baseline year (p=0.002) were related to pulmonary exacerbations in subsequent years.In a cohort of Dutch children with CF followed for 5 years, we were able to identify several risk factors for future exacerbations. In particular, the association between PPI use and lung disease progression definitely requires further investigation.

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Hyperpolarised 129Xe magnetic resonance imaging to monitor treatment response in children with cystic fibrosis

European Respiratory Journal ◽

10.1183/13993003.02188-2018 ◽

2019 ◽

Vol 53 (5) ◽

pp. 1802188 ◽

Cited By ~ 18

Author(s):

Jonathan H. Rayment ◽

Marcus J. Couch ◽

Nancy McDonald ◽

Nikhil Kanhere ◽

David Manson ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Cystic Fibrosis ◽

Magnetic Resonance ◽

Lung Disease ◽

Treatment Response ◽

Forced Expiratory Volume ◽

Response To Therapy ◽

Breath Hold ◽

Pulmonary Exacerbation ◽

Resonance Imaging

Pulmonary magnetic resonance imaging using hyperpolarised 129Xe gas (XeMRI) can quantify ventilation inhomogeneity by measuring the percentage of unventilated lung volume (ventilation defect per cent (VDP)). While previous studies have demonstrated its sensitivity for detecting early cystic fibrosis (CF) lung disease, the utility of XeMRI to monitor response to therapy in CF is unknown. The aim of this study was to assess the ability of XeMRI to capture treatment response in paediatric CF patients undergoing inpatient antibiotic treatment for a pulmonary exacerbation.15 CF patients aged 8–18 years underwent XeMRI, spirometry, plethysmography and multiple-breath nitrogen washout at the beginning and end of inpatient treatment of a pulmonary exacerbation. VDP was calculated from XeMRI images obtained during a static breath hold using semi-automated k-means clustering and linear binning approaches.XeMRI was well tolerated. VDP, lung clearance index and the forced expiratory volume in 1 s all improved with treatment; however, response was not uniform in individual patients. Of all outcome measures, VDP showed the largest relative improvement (−42.1%, 95% CI −52.1–−31.9%, p<0.0001).These data support further investigation of XeMRI as a tool to capture treatment response in CF lung disease.

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Year in review 2014: Interstitial lung disease, physiology, sleep and ventilation, acute respiratory distress syndrome, cystic fibrosis, bronchiectasis and rare lung disease

Respirology ◽

10.1111/resp.12532 ◽

2015 ◽

Vol 20 (5) ◽

pp. 834-845 ◽

Cited By ~ 2

Author(s):

Toby M. Maher ◽

Amanda Piper ◽

Yuanlin Song ◽

Marcos I. Restrepo ◽

Neil D. Eves

Keyword(s):

Cystic Fibrosis ◽

Acute Respiratory Distress Syndrome ◽

Interstitial Lung Disease ◽

Respiratory Distress Syndrome ◽

Respiratory Distress ◽

Lung Disease ◽

Distress Syndrome ◽

Review 2014

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Procalcitonin predicts the severity of cystic fibrosis pulmonary exacerbations and readmissions in adult patients: a prospective cohort study

Journal of Investigative Medicine ◽

10.1136/jim-2019-001183 ◽

2020 ◽

Vol 68 (4) ◽

pp. 856-863

Author(s):

Kristina L Bailey ◽

Peter J Murphy ◽

Olena K Lineberry ◽

Matthew R Haack ◽

John D Dickinson ◽

...

Keyword(s):

Cystic Fibrosis ◽

Antibiotic Treatment ◽

Hospital Admissions ◽

Clinical Care ◽

Forced Expiratory Volume ◽

Pulmonary Exacerbation ◽

Inclusion Criteria ◽

Exclusion Criteria ◽

Pulmonary Exacerbations ◽

Time Of Admission

Patients with cystic fibrosis (CF) experience multiple pulmonary exacerbations throughout their lifetime, resulting in repeated antibiotic exposure and hospital admissions. Reliable diagnostic markers to guide antibiotic treatment in patients with CF, however, are lacking. Given that the CF airway is characterized by persistent and frequent bacterial infection, our goal was to determine if procalcitonin (PCT) could be used as a severity and prognostic marker of CF exacerbation. We enrolled 40 participants at the time of diagnosis of CF pulmonary exacerbation. Inclusion criteria: age ≥19 years with exacerbation requiring antibiotics as determined by the treating physician. Exclusion criteria: antibiotics initiated more than 48 hours prior to enrollment, and pregnancy. Blood samples were collected on enrollment day and after 7–10 days of treatment. Of the 40 patients enrolled, 23 (57.5%) had detectable levels of PCT (≥0.05 ng/mL). PCT levels were significantly associated with pulmonary exacerbation scores (p=0.01) and per cent decrease in forced expiratory volume in 1 second (FEV1) (p=0.01) compared with the best in the last 12 months. Those who had worsening PCT during treatment had less improvement in FEV1 (p=0.001) and were more likely to be readmitted to the hospital sooner (p<0.0001). Likewise, those who had a detectable PCT at the time of admission were more likely to be readmitted sooner (p=0.03). PCT elevation during antibiotic treatment is associated with less improvement in FEV1 and earlier readmission. A detectable PCT level occurs only in more severe CF exacerbations. Multicenter trials are needed to confirm whether PCT may play a role in the clinical care of patients with CF.

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Acute Pulmonary Exacerbations in Cystic Fibrosis

Seminars in Respiratory and Critical Care Medicine ◽

10.1055/s-0039-1697975 ◽

2019 ◽

Vol 40 (06) ◽

pp. 792-803 ◽

Cited By ~ 2

Author(s):

Christopher H. Goss

Keyword(s):

Cystic Fibrosis ◽

Large Scale ◽

Clinical Epidemiology ◽

Transmembrane Conductance Regulator ◽

Pulmonary Exacerbation ◽

Multisystem Disease ◽

Clinical Events ◽

Treatment Paradigm ◽

Pulmonary Exacerbations ◽

Epidemiology Studies

AbstractWith the improving survival of cystic fibrosis (CF) patients and the advent of highly effective cystic fibrosis transmembrane conductance regulator therapy, the clinical spectrum of this complex multisystem disease continues to evolve. One of the most important clinical events for patients with CF in the course of this disease is an acute pulmonary exacerbation. Clinical and microbial epidemiology studies of CF pulmonary exacerbations continue to provide important insight into the disease course, prognosis, and complications. This work has now led to a number of large scale clinical trials with the goal of improving the treatment paradigm for CF pulmonary exacerbation. The primary goal of this review is to provide a summary of the pathophysiology, the clinical epidemiology, microbial epidemiology, outcome and the treatment of CF pulmonary exacerbation.

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Adult cystic fibrosis: association of acute pulmonary exacerbations and increasing severity of lung disease with auxotrophic mutants of Pseudomonas aeruginosa.

Thorax ◽

10.1136/thx.48.10.1002 ◽

1993 ◽

Vol 48 (10) ◽

pp. 1002-1005 ◽

Cited By ~ 13

Author(s):

R F Taylor ◽

M E Hodson ◽

T L Pitt

Keyword(s):

Cystic Fibrosis ◽

Pseudomonas Aeruginosa ◽

Lung Disease ◽

Auxotrophic Mutants ◽

Pulmonary Exacerbations

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Gas Exchange in Disease: Asthma, Chronic Obstructive Pulmonary Disease, Cystic Fibrosis, and Interstitial Lung Disease

Comprehensive Physiology ◽

10.1002/cphy.c090012 ◽

2011 ◽

pp. 663-697 ◽

Cited By ~ 23

Author(s):

Iven H. Young ◽

Peter T.P. Bye

Keyword(s):

Chronic Obstructive Pulmonary Disease ◽

Cystic Fibrosis ◽

Interstitial Lung Disease ◽

Gas Exchange ◽

Lung Disease ◽

Pulmonary Disease ◽

Chronic Obstructive ◽

Obstructive Pulmonary Disease

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Recent advances in the understanding and management of cystic fibrosis pulmonary exacerbations

F1000Research ◽

10.12688/f1000research.13926.1 ◽

2018 ◽

Vol 7 ◽

pp. 575 ◽

Cited By ~ 7

Author(s):

Kate Skolnik ◽

Bradley S. Quon

Keyword(s):

Quality Of Life ◽

Cystic Fibrosis ◽

Significant Proportion ◽

Pulmonary Exacerbation ◽

Aggressive Treatment ◽

Profound Impact ◽

Recent Advances ◽

Intravenous Antibiotics ◽

Pulmonary Exacerbations

Pulmonary exacerbations are common events in cystic fibrosis and have a profound impact on quality of life, morbidity, and mortality. Pulmonary exacerbation outcomes remain poor and a significant proportion of patients fail to recover their baseline lung function despite receiving aggressive treatment with intravenous antibiotics. This focused review provides an update on some of the recent advances that have taken place in our understanding of the epidemiology, pathophysiology, diagnosis, and management of pulmonary exacerbations in cystic fibrosis as well as direction for future study.

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