Cystic fibrosis in adult age

Lerín, M.; Prados, Concepción; Martínez, María Teresa; Máiz, Luís; Girón, Rosa; Solé, Amparó; Cabanillas, J.J.; Álvarez-Sala, R.

doi:10.1016/j.rceng.2014.05.001

Cited by 4 publications

(7 citation statements)

References 28 publications

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“…In the current study, we found that 23.9% presented a poor evolution, which led us to believe that atelectasis could adversely affect the survival of patients with CF, by favoring respiratory infections and a worsening of lung function. The mean age of these patients was lower than those published (more than 40 years) [1]. It is well known that pulmonary exacerbations are associated with a aggravation of the disease [13,14].…”

Section: Regarding the Location Of Atelectasis Five Patientsmentioning

confidence: 57%

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Atelectasis with Torpid Evolution in Patients with Cystic Fibrosis

Prados‐Sánchez¹,

Martínez-Redondo²,

Máiz-Carro³

et al. 2018

Ann Pulmonol

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Section: Regarding the Location Of Atelectasis Five Patientsmentioning

confidence: 57%

“…Cystic fibrosis (CF) is a chronic and progressive disease. Respiratory complications, usually, afect the quality of life, morbidity and prognosis of the disease [1][2][3][4]. Within respiratory complications, atelectasis is which the least medical literature has generated, without consensus on its etiology, prognosis and treatment [5].…”

Section: Introductionmentioning

confidence: 99%

Atelectasis with Torpid Evolution in Patients with Cystic Fibrosis

Prados‐Sánchez¹,

Martínez-Redondo²,

Máiz-Carro³

et al. 2018

Ann Pulmonol

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“…the end of the study, versus 2 [1][2][3] in the control group (P ¼ 0.008). Likewise, 47 (87.7%) patients were diagnosed with chronic bronchial infection at the end of the study.…”

Section: Pulmonary Atelectasis As a Poor Prognostic Factor In Cfmentioning

confidence: 96%

“…Exacerbations and chronic bronchial infection. The average number of exacerbations was 3 [1][2][3][4][5] in the case group in the year before Comparisons between the groups were made using the chi-square test. Comparisons between the groups were made using the chi-square test.…”

Section: Pulmonary Atelectasis As a Poor Prognostic Factor In Cfmentioning

confidence: 99%

“…Respiratory impairment has an incidence of 95% in adulthood, although the severity of its presentation varies, and it constitutes the primary cause of morbidity and mortality in patients with CF. 1 The prognosis for CF has evolved favorably in recent years because of neonatal screening, the emergence of new therapeutic tools, early treatment of respiratory infections, the maintenance of adequate nutrition, and multidisciplinary care, achieving a median survival exceeding 40 years. 2 Among the respiratory complications of CF, atelectasis (lung collapse) is associated with the fewest medical publications, with no consensus on its etiology, prognosis, or treatment.…”

Section: Introductionmentioning

confidence: 99%

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Should atelectasis be considered a pulmonary complication and indicator of poor prognosis in cystic fibrosis?

Martínez Redondo,

Carpio Segura,

Zamarrón De Lucas

et al. 2024

J Int Med Res

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Objective This study examined whether bronchoscopy leads to clinicoradiological improvement in cystic fibrosis (CF) and the predictive factors. The study also investigated whether pulmonary atelectasis is a poor prognostic factor in CF. Methods This multicenter, case–control, observational, retrospective study included two groups of patients with CF: a case group (patients with persistent atelectasis who were followed-up at least for 2 years) and a control group (patients without atelectasis matched 1:1 by sex and age [±3 years]). We recorded demographic data, lung function test results, pulmonary complications, comorbidities, treatments (including bronchoscopies, surgery and transplantation), and deaths. Results Each group included 55 patients (case group: 20 men, mean age 25.4 ± 10.4 years; control group: 20 men, mean age 26.1 ± 11.4 years). Bronchoscopy did not lead to clinicoradiological improvement. Allergic bronchopulmonary aspergillosis (ABPA) was more frequent in the case group. Patients in the case group more frequently used inhaled steroids, their pre-atelectasis lung function was statistically worse, and they had more exacerbations during follow-up. Conclusion Moderate-to-severe pulmonary disease and ABPA can favor atelectasis. Pulmonary atelectasis can be a poor prognostic factor in CF because it increases exacerbations. Despite our results, we recommend enhancing treatment, including bronchoscopy, to prevent persistent atelectasis.

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How are the ancient cystic fibrosis patients? Cystic fibrosis diagnosed over 60 years-old

Prados

Lerín

Cabanillas

et al. 2017

Respiratory Medicine Case Reports

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Background and aimsTo specify the prevalence of patients diagnosed with CF at age of ≥60 year-old and to analyze their characteristics.Patients and methodsObservational study of CF patients which were diagnosed at age ≥60 year-old. The analyzed variables were: age, sex, nationality, lung function parameters, conditions present at diagnosis, microbiological characteristics and genetic findings.Resultseight patients were included. 7 patients were female (87.5%) with a mean age of 70.6 years (median 71.5 years, range 60–78 years). The most important findings were: sweat test >60 mEq/l; heterozygotes F508del; bronchiectasis in CT; methicillin-sensitive Staphylococcus aureus (50%) in sputum. The most patients presented a normal or mild obstructive lung function.ConclusionsCF must also be considered a disease diagnosed in adulthood, incorporating the sweat test within the usual techniques of differential diagnosis in patients with different diseases associated with CF, because genetic counselling is esencial.

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Cystic fibrosis in adult age

Cited by 4 publications

References 28 publications

Atelectasis with Torpid Evolution in Patients with Cystic Fibrosis

Atelectasis with Torpid Evolution in Patients with Cystic Fibrosis

Should atelectasis be considered a pulmonary complication and indicator of poor prognosis in cystic fibrosis?

How are the ancient cystic fibrosis patients? Cystic fibrosis diagnosed over 60 years-old

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