Pulmonary radiographic findings in young children with cystic fibrosis

Pedrosa, Jesiana Ferreira; Ibiapina, Cássio da Cunha; Alvim, Cristina Gonçalves; Camargos, Paulo Augusto Moreira; Martins, Fabiana Paiva; Guimarães, Elizabet Vilar; Bedran, Renata Marcos

doi:10.1007/s00247-014-3112-z

Cited by 5 publications

(4 citation statements)

References 18 publications

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“…Initial radiographic findings of CF include air trapping and linear markings, and subsequent findings include nodular-cystic lesions representing bronchiectasis and mucus plugging (11). On CT, CF manifests as bronchiectasis, cellular bronchiolitis, mucus plugging, and mosaic attenuation (121314).…”

Section: Introductionmentioning

confidence: 99%

Chest CT Features of Cystic Fibrosis in Korea: Comparison with Non-Cystic Fibrosis Diseases

et al. 2017

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ObjectiveCystic fibrosis (CF) is a rare congenital disease in Korea, and its clinical and imaging findings are unclear. The objective of our study was to describe the clinical and CT features of CF in Korea and compare its features with those of other diseases mimicking CF.Materials and MethodsFrom November 1994 to December 2014, a presumptive diagnosis of CF was made in 23 patients based on clinical or radiological examination. After the exclusion of 10 patients without diagnostic confirmation, 13 patients were included in the study. A diagnosis of CF was made with the CF gene study. CT findings were evaluated for the presence and distribution of parenchymal abnormalities including bronchiectasis, tree-in-bud (TIB) pattern, mucus plugging, consolidation, and mosaic attenuation.ResultsOf the 13 patients, 7 (median age, 15 years) were confirmed as CF, 4 (median age, 19 years) had primary ciliary dyskinesia, 1 had bronchiectasis of unknown cause, and 1 had chronic asthma. CT of patients with CF showed bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging in all patients, with upper lung predominance (57%). In CT of the non-CF patients, bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging were also predominant features, with lower lung predominance (50%).ConclusionKorean patients with CF showed bilateral bronchiectasis, cellular bronchiolitis, mucus plugging, and mosaic attenuation, which overlapped with those of non-CF patients. CF gene study is recommended for the definitive diagnosis of CF in patients with these clinical and imaging features.

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Section: Introductionmentioning

confidence: 99%

Chest CT Features of Cystic Fibrosis in Korea: Comparison with Non-Cystic Fibrosis Diseases

et al. 2017

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“…The mean Brasfield score could vary in different study settings. For example, a study measuring pulmonary function in patients with CF showed that the mean Brasfield score ranged from 20.2 to 22.3 in children under 1 to 5 years old (19,20). This point highlights the imperative to delve into possible explanations behind the differences in the severity of pulmonary involvement in CF patients, such as nutritional status.…”

Section: Discussionmentioning

confidence: 99%

The Association of Serum Elements’ Levels with the Severity of Pulmonary Involvement in Cystic Fibrosis Patients

et al. 2023

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Background: Cystic fibrosis (CF) is a multisystem disease presenting different manifestations, including the involvement of the respiratory and gastrointestinal tract. Patients may be at risk for micronutrient depletion, particularly during illness and infection. However, the relationship between micronutrient depletion and pulmonary involvement is not established well. Objectives: This study aimed to determine the relationship between serum levels of calcium, phosphorus, magnesium, zinc, copper, selenium, and vitamin D with the severity of pulmonary involvement in patients with CF. Methods: This cross-sectional study was performed on 84 patients with CF referred to Mofid Children’s Hospital, Tehran, Iran, in 2017. The collected data included demographic and anthropometric information. Laboratory evaluations included magnesium, copper, zinc, selenium, complete blood cell count (CBC), and vitamin D levels. The severity of pulmonary involvement was determined based on the Brasfield scoring system (BSS). Pearson correlation coefficient, t test, χ2 test, or Fisher exact test was used for analyses. Results: The mean age of the children was 6.8 ± 3.5 years, and 36 patients (43%) were female. Based on the z score, 53 patients (63%) were malnourished. The severity of pulmonary involvement was mild in 25 patients (30%), moderate in 39 patients (46%), and severe in 20 patients (24%). The percentages of deficiency related to vitamin D, zinc, copper, and selenium were 56%, 38%, 32.1%, and 21.4%, respectively. Among trace elements, there was a statistically significant relationship between the severity of pulmonary involvement according to BSS and serum copper (P = 0.007 and r = -0.229) and selenium deficiency (P < 0.001 and r = -0.418). There was a significant relationship between malnutrition and vitamin D deficiency in patients (P = 0.034, r = 0.231). Conclusions: Serum copper concentration might affect respiratory function in CF patients. Selenium supplements should be added to patients’ diets. However, further interventional studies are required to investigate the possible causal relationships.

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“…Chest x-ray continues to be the most common method of monitoring structural damage in patients with CF [74] as it offers the advantages of low cost, high accessibility, and low radiation levels [75,76]. However, the low sensitivity of chest x-ray for detecting structural changes and the high interobserver variability [77,78] limit its role in early lung disease.…”

Section: Chest X-raymentioning

confidence: 99%

A multimodal approach to detect and monitor early lung disease in cystic fibrosis

Mondéjar-López

Horsley

Ratjen

et al. 2021

Expert Review of Respiratory Medicine

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Introduction: In the early stages, lung involvement in cystic fibrosis (CF) can be silent, with disease progression occurring in the absence of clinical symptoms. Irreversible airway damage is present in the early stages of disease; however, reliable biomarkers of early damage due to inflammation and infection that are universally applicable in day-to-day patient management have yet to be identified. Areas covered: At present, the main methods of detecting and monitoring early lung disease in CF are the lung clearance index (LCI), computed tomography (CT), and magnetic resonance imaging (MRI). LCI can be used to detect patients who may require more intense monitoring, identify exacerbations, and monitor responses to new interventions. High-resolution CT detects structural alterations in the lungs of CF patients with the best resolution of current imaging techniques. MRI is a radiation-free imaging alternative that provides both morphological and functional information. The role of MRI for short-term follow-up and pulmonary exacerbations is currently being investigated. Expert opinion: The roles of LCI and MRI are expected to expand considerably over the next few years. Meanwhile, closer collaboration between pulmonology and radiology specialties is an important goal toward improving care and optimizing outcomes in young patients with CF.

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Pulmonary radiographic findings in young children with cystic fibrosis

Abstract: The evaluation of radiographs using the Brasfield score demonstrated the most important pulmonary findings in cystic fibrosis and identified the age group when these alterations began to appear more pronounced.

Cited by 5 publications

References 18 publications

Chest CT Features of Cystic Fibrosis in Korea: Comparison with Non-Cystic Fibrosis Diseases

Chest CT Features of Cystic Fibrosis in Korea: Comparison with Non-Cystic Fibrosis Diseases

The Association of Serum Elements’ Levels with the Severity of Pulmonary Involvement in Cystic Fibrosis Patients

A multimodal approach to detect and monitor early lung disease in cystic fibrosis

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