Monitoring early lung disease in cystic fibrosis: where are we now?

Duncan, Julie; Aurora, Paul

doi:10.1183/20734735.010813

Cited by 6 publications

(5 citation statements)

References 94 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The identification of paediatric patients at risk of death through FEV 1 < 40% and use of oxygen might be too tardive in order to perform useful treatment actions to change the prognosis. Therefore, CF centres should implement diagnostic tools to identify patients at risk earlier, using lung CT scan and bronchoalveolar lavage; lung clearance index [ 37 ] to identify those patients who need more intensive nutritional and respiratory care. As a consequence, guidelines for the diagnosis and treatment of patients with early onset of severe respiratory disease, aggressive nutritional support for CF patients and improvement in early diagnosis of CFRD are desirable in the paediatric settings.…”

Section: Discussionmentioning

confidence: 99%

Cystic Fibrosis Mortality in Childhood. Data from European Cystic Fibrosis Society Patient Registry

Zolin

Bossi

Cirilli³

et al. 2018

IJERPH

View full text Add to dashboard Cite

Data collected in the European Cystic Fibrosis Society Patient Registry (ECFSPR) database were used to investigate whether risk factors for death in childhood and adolescents CF patients have different impact in countries of different income. In this way, it is possible to recognize where interventions could improve the quality of care and survival in these patients. We matched deceased and alive patients by age, country, year of follow-up. Multivariable logistic models were developed. In the years of this study, the ECFSPR collected information on 24,416 patients younger than 18 years: 7830 patients were from countries with low/middle income and 16,586 from countries with high income; among these the dead are 102 and 107 (p < 0.001), respectively. The use of oxygen, forced expiratory volume in one second (FEV1) below 40% and BMI standard deviation score (SDS) below −2 represent risk factors for death. However, some patients from countries with high income remain alive even if their values of FEV1% and BMI-SDS were low, and some deceased patients from countries with high income had high values of FEV1% (>60%). Evaluation of mortality in pediatric age may reflect the availability of resources for CF diagnosis and treatment in some countries.

show abstract

Section: Discussionmentioning

confidence: 99%

Cystic Fibrosis Mortality in Childhood. Data from European Cystic Fibrosis Society Patient Registry

Zolin

Bossi

Cirilli³

et al. 2018

IJERPH

View full text Add to dashboard Cite

show abstract

“…Several procedures have been employed to detect respiratory infections and to analyze inflammatory biomarkers in patients with CF, e.g., bronchoalveolar lavage fluid (BALF), nasal swab, cough swab, and induced sputum [9]. Children are not able to expectorate the sputum; thus, clinical monitoring of lung disease in children with CF requires the invasive collection of BALF [10,11].…”

Section: Introductionmentioning

confidence: 99%

Salivary Cytokines and Airways Disease Severity in Patients with Cystic Fibrosis

et al. 2020

View full text Add to dashboard Cite

About 50% of patients with cystic fibrosis (CF) have sinonasal complications, which include inferior turbinate hypertrophy (NTH) and/or nasal polyposis (NP), and different degrees of lung disease, which represents the main cause of mortality. Monitoring of sinonasal disease requires complex instrumental procedures, while monitoring of lung inflammation requires invasive collection of bronchoalveolar lavage fluid. The aim of this study was to investigate the associations between salivary cytokines levels and CF-related airway diseases. Salivary biochemical parameters and cytokines, i.e., interleukin-6 (IL-6), IL-8, and tumor necrosis factor alpha (TNF-α), were analyzed in resting saliva from healthy subjects and patients with CF. Patients with CF showed significantly higher levels of salivary chloride, IL-6, IL-8, and TNF-α and lower calcium levels than healthy subjects. Among patients with CF, IL-6 and IL-8 were significantly higher in patients with NTH, while TNF-α was significantly lower in patients with NP. A decreasing trend of TNF-α in patients with severe lung disease was also observed. On the other hand, we did not find significant correlation between cytokine levels and Pseudomonas aeruginosa or Stenotrophomonas maltophilia colonization. These preliminary results suggest that salivary IL-6 and IL-8 levels increase during the acute phase of sinonasal disease (i.e., NTH), while the end stages of pulmonary disease and sinonasal disease (i.e., NP) show decreased TNF-α levels.

show abstract

“…С учетом полученных данных была установлена нелинейная зависимость, которая позволяла оценить степень тяжести бронхиальной обструкции, выраженную в величине ОФВ 1…”

Section: результатыunclassified

“…В настоящее время муковисцидоз остается актуальной проблемой педиатрии. Тяжесть заболевания определяется преимущественно степенью поражения органов дыхания [1], поэтому у данной категории больных первоочередное значение приобретает мониторинг респираторной функции. Компьютерная бронхофонография способна выявлять признаки бронхиальной обструкции у детей любого возраста, в том числе у новорожденных, что является одним из основных преимуществ метода [2][3][4].…”

unclassified

An Innovative Approach to the Diagnosis of Bronchial Obstruction Syndrome in Children with Cystic Fibrosis

Павлинова¹,

Геппе²,

Malyshev³

et al. 2019

View full text Add to dashboard Cite

Monitoring early lung disease in cystic fibrosis: where are we now?

Cited by 6 publications

References 94 publications

Cystic Fibrosis Mortality in Childhood. Data from European Cystic Fibrosis Society Patient Registry

Cystic Fibrosis Mortality in Childhood. Data from European Cystic Fibrosis Society Patient Registry

Salivary Cytokines and Airways Disease Severity in Patients with Cystic Fibrosis

An Innovative Approach to the Diagnosis of Bronchial Obstruction Syndrome in Children with Cystic Fibrosis

Contact Info

Product

Resources

About