The Relation of CFTR-Genotype and Associated Comorbidities to Development of Pulmonary Atelectasis in Cystic Fibrosis Patients

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Características de las atelectasias como complicación pulmonar en la fibrosis quística / No disponible

Objetivos: Determinar la prevalencia de las atelectasias en la fibrosis quística (FQ) en la Comunidad de Madrid, con el fin de encontrar factores de riesgo predisponentes. Material y métodos. Se ha llevado a cabo un estudio retrospectivo en las unidades de FQ de la Comunidad de Madrid, de aquellos que habían padecido atelectasias. Se estudiaron: infecciones, complicaciones pulmonares y comorbilidades, tratamiento previo y posterior, el empleo de la fibrobroncoscopia y su respuesta, el diagnóstico mediante tomografía computarizada (TC) y la resolución o no de la atelectasia. Resultados: 16 (2,66%) pacientes habían padecido atelectasias, siete hombres (43,8%), con una edad media a la primera atelectasia de 23,81 ± 11,2 años. 14 (87,5%) presentaron infección bronquial crónica (IBC), especialmente por S. aureus meticilin-sensible y P. aeruginosa. Tres (18,8%) habían padecido aspergilosis broncopulmonar alérgica (ABPA) y uno hemoptisis (6,3%) con embolización. No hubo diferencias significativas en los tratamientos usados antes y después. En 10 (62,5%) se realizó fibrobroncoscopia, mejorando ocho pacientes. La TC demostró mejoría radiológica en un 62,5%, completa en siete pacientes. Hubo diferencias significativas entre FEV1/FVC, FEV1 y FVC en la evolución de los pacientes; entre el número total de exacerbaciones del año previo y del posterior (p= 0,012) y entre las exacerbaciones graves del año previo y las últimas registradas (p= 0,013). Cinco (31,25%) tuvieron una evolución tórpida tras el evento. Conclusiones: Una función pulmonar con obstrucción moderada-grave, y haber padecido otras complicaciones respiratorias (ABPA y IBC) podrían predisponer a padecer atelectasias en FQ. Además, esta complicación empeora las exacerbaciones y la función pulmonar

Aim: To determine the prevalence of atelectasis in cystic fibrosis (CF) in Madrid Community, in order to find predisposing risk factors. Material and methods. A retrospective study in the CF Units of Madrid Community was carried out, of the patients who suffered atelectasis We studied: infections, pulmonary complications and comorbilities, prior and subsequent treatment, the use of broncoschopy and its results; the diagnosis using computed tomography (CT), and the resolution or not the atelectasis. Results: 16 (2,66%) patients has suffered atelectasis (2,66%), 7 males (43,8%) with a mean aged with the first atelectasis was 23,81 ± 11,2 year-old 14 (87,5%) presented chronic bronchial infection (CBI), specially by meticillinsensitive S and P. aeruginosa. Three (18,8%) had suffered allergic bronchopulmonary aspergillosis (ABPA) and one (6,3%) haemoptysis with embolization. There were no significant differences with prior and subsequent treatment. A bronchoscopy was performed in 10 patients (62,5%): improving in eight cases. A radiological improve was observed using a CT in 10 (62,5%) patients, complete in seven patients. We have found significant differences in FEV1/FVC, FEV1 and FVC, in the evolution of the patients; between the total number of exacerbations of the previous year and the following one. Five (31.25%) had a torpid evolution after the event. Conclusions: Pulmonary function with moderate to severe obstruction and other respiratory complications (ABPA and CBI) may predispose to atelectasis in CF. In addition, this complication worsens exacerbations and pulmonary function

Predicting high risk of exacerbations in bronchiectasis: the E-FACED score.

BACKGROUND: Although the FACED score has demonstrated a great prognostic capacity in bronchiectasis, it does not include the number or severity of exacerbations as a separate variable, which is important in the natural history of these patients. OBJECTIVE: Construction and external validation of a new index, the E-FACED, to evaluate the predictive capacity of exacerbations and mortality. METHODS: The new score was constructed on the basis of the complete cohort for the construction of the original FACED score, while the external validation was undertaken with six cohorts from three countries (Brazil, Argentina, and Chile). The main outcome was the number of annual exacerbations/hospitalizations, with all-cause and respiratory-related deaths as the secondary outcomes. A statistical evaluation comprised the relative weight and ideal cut-off point for the number or severity of the exacerbations and was incorporated into the FACED score (E-FACED). The results obtained after the application of FACED and E-FACED were compared in both the cohorts. RESULTS: A total of 1,470 patients with bronchiectasis (819 from the construction cohorts and 651 from the external validation cohorts) were followed up for 5 years after diagnosis. The best cut-off point was at least two exacerbations in the previous year (two additional points), meaning that the E-FACED has nine points of growing severity. E-FACED presented an excellent prognostic capacity for exacerbations (areas under the receiver operating characteristic curve: 0.82 for at least two exacerbations in 1 year and 0.87 for at least one hospitalization in 1 year) that was statistically better than that of the FACED score (0.72 and 0.78, P<0.05, respectively). The predictive capacities for all-cause and respiratory mortality were 0.87 and 0.86, respectively, with both being similar to those of the FACED. CONCLUSION: E-FACED score significantly increases the FACED capacity to predict future yearly exacerbations while maintaining the score's simplicity and prognostic capacity for death.

Unidad de Fibrosis Quística del Hospital Universitario Ramón y Cajal / Cystic fibrosis unit of the Hospital Universitario Ramón y Cajal

La Unidad de Fibrosis Quistica del Hospital Universitario Ramón y Cajal, centro de referencia de la Comunidad de Madrid, fue pionera en los años noventa, en la atención a los pacientes con esta patología grave, con una visión futurista, al centrarse en la atención multidisciplinar de un equipo (tanto del área pediátrica como de adultos), que acompaña al paciente desde la infancia a la edad adulta, lo que ha contribuido, de forma clave, a la mejora en la calidad y esperanza de vida de estos pacientes. A lo largo de los años, la Unidad ha ido creciendo en todos los aspectos y es, hoy, ejemplo para muchas otras patologías (AU)

The Cystic Fibrosis Unit of the University Hospital Ramón y Cajal, reference center of the Community of Madrid, was a pioneer in the 19903, in the care given to patients with this serious condition. It has had a futuristic view, on focusing on multidisciplinary care of a team (both in the pediatric and adult area), accompanying the patient from Childhood to the adult age. This has played a critical role to the improvement in the quality of life and life expectancy of these patients. Over these years, the Unit has been growing in all the aspects and is currently an example for many other conditions (AU)

Antibioterapia inhalada. Nuevas perspectivas / No disponible

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Antibioterapia inhalada en pacientes con fibrosis quística / Inhaled antibiotic treatment in patients with cystic fibrosis

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Tratamiento con azitromicina a largo plazo en un paciente con bronquiectasias idiopáticas / Long-term treatment with azithromycin in a patient with idiopathic bronchiectasis

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Actualización de criterios para trasplante pulmonar en la fibrosis pulmonar idiopática / Current criteria for lung transplantation in idiopathic pulmonary fibrosis

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[Gastroesophageal reflux, pulmonary and gastric function in patients with cystic fibrosis. Results of a randomized trial]. / Reflujo gastroesofágico, función pulmonar y función gástrica en pacientes con fibrosis quística. Resultados de un estudio randomizado.

We studied ten patients with Cystic fibrosis. The purposes of this study were to investigate the presence of gastroesophageal reflux and establish the probable association between gastroesophageal reflux and pulmonary and gastric involvement. All 10 patients underwent 24-hour esophageal pH recording, spirometry and gastric function. Abnormal reflux index was found in all these patients. Lung function was pathologic in the 3 older children. There were no relationship between the severity of the gastroesophageal reflux and the degree of pulmonary damage. No patient has gastric acid hypersecretion. Eight of 10 patients had steatorrhea. Our findings confirm the high frequence of gastroesophageal reflux in cystic fibrosis.