scholarly journals Acute Pulmonary Exacerbations in Cystic Fibrosis

2019 ◽  
Vol 40 (06) ◽  
pp. 792-803 ◽  
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.

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

2020 ◽  
pp. 1357633X2097200
Author(s):  
Beste Ozsezen ◽  
Nagehan Emiralioglu ◽  
Dilber A Tural ◽  
Birce Sunman ◽  
Halime N Buyuksahin ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Transmission Risk ◽  
Nutrition Status ◽  
Peak Time ◽  
Pulmonary Exacerbation ◽  
Pulmonary Exacerbations ◽  
The Times ◽  
Novel Coronavirus

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.

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

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.

scholarly journals Risk factors for lung disease progression in children with cystic fibrosis

2018 ◽  
Vol 51 (6) ◽  
pp. 1702509 ◽  
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.

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

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 575 ◽  
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.

scholarly journals Changes in airway inflammation during pulmonary exacerbations in patients with cystic fibrosis and primary ciliary dyskinesia

2015 ◽  
Vol 47 (3) ◽  
pp. 829-836 ◽  
Author(s):  
Felix Ratjen ◽  
Valerie Waters ◽  
Michelle Klingel ◽  
Nancy McDonald ◽  
Sharon Dell ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Inflammatory Response ◽  
Neutrophil Elastase ◽  
Primary Ciliary Dyskinesia ◽  
Bacterial Pathogens ◽  
Pulmonary Exacerbation ◽  
Elastase Activity ◽  
Cell Counts ◽  
Pulmonary Exacerbations ◽  
Ciliary Dyskinesia

Lung disease in patients with both primary ciliary dyskinesia (PCD) or cystic fibrosis (CF) is associated with impaired mucociliary clearance; however, clinical outcomes are typically worse in CF patients. We assessed whether CF and PCD patients differ in inflammatory response in the airways during pulmonary exacerbation.We first studied clinically stable PCD patients with a spectrum of bacterial pathogens to assess inflammatory response to different pathogens. Subsequently, PCD and CF patients with similar bacterial pathogens were studied at the time of a pulmonary exacerbation and after 21 days of antibiotics treatment. Qualitative and quantitative microbiology, cell counts, interleukin-8 concentrations, and neutrophil elastase activity were assessed in sputum samples obtained before and after treatment.In stable PCD patients, no significant differences were found in sputum inflammatory markers between individuals colonised with different bacterial pathogens. Pulmonary exacerbation severity assessed by a pulmonary exacerbation score and lung function decline from their previous baseline did not differ between CF and PCD patients. Bacterial density for Staphylococcus aureus and Haemophilus influenzae was higher in CF versus PCD (p<0.05), but absolute neutrophil counts were higher in PCD patients (p=0.02). While sputum elastase activity was similar in PCD and CF at the time of exacerbation, it decreased with antibiotic therapy in PCD (p<0.05) but not CF patients.PCD patients differ from those with CF in their responses to treatment of pulmonary exacerbations, with higher neutrophil elastase activity persisting in the CF airways at the end of treatment.

scholarly journals Molecular analysis of CFTR gene mutations among Iraqi cystic fibrosis patients

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Asal Gailan Abdul-Qadir ◽  
Bassam Musa Al-Musawi ◽  
Rabab Farhan Thejeal ◽  
Saad Abdul-Baqi Al-Omar
Keyword(s):  
Cystic Fibrosis ◽  
Molecular Analysis ◽  
Autosomal Recessive ◽  
Gene Mutations ◽  
Regional Studies ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Multisystem Disease ◽  
Polymorphic Variants ◽  
Cystic Fibrosis Transmembrane

Abstract Background Cystic fibrosis (CF) is an autosomal recessive multisystem disease that results from mutation(s) of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. More than 2100 mutations and polymorphisms have been reported in this gene so far. Incidence and genotyping of CF are under-identified in Iraq. This study aims to determine the types and frequencies of certain CFTR mutations among a sample of Iraqi CF patients. Two groups of patients were included: 31 clinically confirmed CF patients in addition to 47 clinically suspected patients of CF. All confirmed patients had typical, moderate-severe clinical presentation and course of the disease. Molecular analysis was performed on the majority of enrolled patients using the CF-stripAssay® kit supplied by ViennaLab diagnostics, GmbH, Austria. Results The mutation-detection rate from the tested 34 mutations in this study was 19.5% and the 8 detected mutations were as follows: 3120+1G>A and W1282X were found in 3 (4.17%) patients each; F508del and R1162X were found in 2 (2.78%) patients each; 3272-26A>G, R347P, I507del, and 2183AA>G were found in 1 (1.38%) patient each. Polymorphic variants of IVS8, namely 5T, 7T, and 9T, were detected in ~ 70%. These results were nearly similar to what was reported in regional countries. Conclusion Cystic fibrosis seems to be not rare as previously thought. 3120+1G>A and W1282X are the two most commonly detected mutations. F508del needs to be included in all future tests, while the I507del mutation was uniquely reported in this study but not in regional studies.

scholarly journals Lung clearance index in cystic fibrosis subjects treated for pulmonary exacerbations

2015 ◽  
Vol 46 (4) ◽  
pp. 1055-1064 ◽  
Author(s):  
Nicole Sonneveld ◽  
Sanja Stanojevic ◽  
Reshma Amin ◽  
Paul Aurora ◽  
Jane Davies ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Observational Studies ◽  
Forced Expiratory Volume ◽  
Response To Therapy ◽  
Lung Clearance ◽  
Clinical Events ◽  
Intravenous Antibiotics ◽  
Lung Clearance Index ◽  
Pulmonary Exacerbations ◽  
Relative Change

Pulmonary exacerbations are important clinical events for cystic fibrosis (CF) patients. Studies assessing the ability of the lung clearance index (LCI) to detect treatment response for pulmonary exacerbations have yielded heterogeneous results. Here, we conduct a retrospective analysis of pooled LCI data to assess treatment with intravenous antibiotics for pulmonary exacerbations and to understand factors explaining the heterogeneous response.A systematic literature search was performed to identify prospective observational studies. Factors predicting the relative change in LCI and spirometry were evaluated while adjusting for within-study clustering.Six previously reported studies and one unpublished study, which included 176 pulmonary exacerbations in both paediatric and adult patients, were included. Overall, LCI significantly decreased by 0.40 units (95% CI −0.60– −0.19, p=0.004) or 2.5% following treatment. The relative change in LCI was significantly correlated with the relative change in forced expiratory volume in 1 s (FEV1), but results were discordant in 42.5% of subjects (80 out of 188). Higher (worse) baseline LCI was associated with a greater improvement in LCI (slope: −0.9%, 95% CI −1.0– −0.4%).LCI response to therapy for pulmonary exacerbations is heterogeneous in CF patients; the overall effect size is small and results are often discordant with FEV1.

scholarly journals Three Novel CFTR Polymorphic Repeats Improve Segregation Analysis for Cystic Fibrosis

2009 ◽  
Vol 55 (7) ◽  
pp. 1372-1379 ◽  
Author(s):  
Ausilia Elce ◽  
Angelo Boccia ◽  
Giuseppe Cardillo ◽  
Sonia Giordano ◽  
Rossella Tomaiuolo ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Prenatal Diagnosis ◽  
Large Scale ◽  
Segregation Analysis ◽  
Preimplantation Diagnosis ◽  
Cftr Gene ◽  
Transmembrane Conductance Regulator ◽  
Large Scale Analysis ◽  
Allelic Distribution ◽  
Cftr Mutations

Abstract Background: Molecular diagnosis for cystic fibrosis (CF) is based on the direct identification of mutations in the CFTR gene [cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7)] (detection rate about 90% with scanning procedures) and on segregation analysis of intragenic polymorphisms for carrier and prenatal diagnosis in about 20% of CF families in which 1 or both causal mutations are unknown. Methods: We identified 3 novel intragenic polymorphic repeats (IVS3polyA, IVS4polyA, and IVS10CA repeats) in the CFTR gene and developed and validated a procedure based on the PCR followed by capillary electrophoresis for large-scale analysis of these polymorphisms and the 4 previously identified microsatellites (IVS1CA, IVS8CA, IVS17bTA, and IVS17bCA repeats) in a single run. We validated the procedure for both single- and 2-cell samples (for a possible use in preimplantation diagnosis), and on a large number of CF patients bearing different genotypes and non-CF controls. Results: The allelic distribution and heterozygosity results suggest that the 3 novel polymorphisms strongly contribute to carrier and prenatal diagnosis of CF in families in which 1 or both causal mutations have not been identified. At least 1 of the 4 previously identified microsatellites was informative in 78 of 100 unrelated CF families; at least 1 of all 7 polymorphisms was informative in 98 of the families. Finally, the analysis of haplotypes for the 7 polymorphisms revealed that most CF mutations are associated with different haplotypes, suggesting multiple slippage events but a single origin for most CFTR mutations. Conclusions: The analysis of the 7 polymorphisms is a rapid and efficient tool for routine carrier, prenatal, and preimplantation diagnosis of CF.

Sign in / Sign up

Export Citation Format

Share Document