Browsing by Subject "Cystic fibrosis"

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    Characterization of inflammatory reaction in upper airways of cystic fibrosis patients
    (Murcia : F. Hernández, 2000) Lesprit, E.; Escudier, E.; Roger, G.; Pruliere, V.; Lenoir, G.; Reinert, Ph.; Coste, A.
    Inflammatory cell populations have not been yet precisely evaluated in cystic fibrosis (CF) airways. We intended to characterize morphological modifications, inflammatory cell infiltration and cell proliferation in nasal tissues obtained from 15 CF patients and from 6 non-CF patients with nasal polyposis. Morphological analysis showed an intense inflammatory infiltration in CF and non-CF tissues with only few modifications in the epithelium from CF tissues. Inflammatory cell populations characterized by specific immunolabeling were quantified, showing a predominance of macrophages and T- and B-lymphocytes and only moderate numbers of neutrophils in CF tissues; in non-CF polyps, lymphocytes and eosinophils were abundant. Proliferating cell percentages quantified after proliferating cell nuclear antigen immunolabeling were 5.3+4.1% (mean t SD) in CF polyps and 3.1?1.2% in non-CF polyps in epithelium but were very low in lamina propria. Intense inflammation in nasal tissues from CF patients is therefore dominated by macrophages and lymphocytes rather than by neutrophils. While morphology is preserved, proliferation is high in epithelium from CF polyps. These findings should be regarded in the future for a better understanding of inflammation in CF airway disease.
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    Diabetes mellitus en pacientes pediátricos con fibrosis quística
    (Murcia: Servicio de Publicaciones de la Universidad de Murcia, 2019) Álvarez Ortega, Sofía; Serrano Gómez, Diego
    Introducción:La Fibrosis Quística (FQ) es una enfermedad hereditaria recesiva que afecta a varios órganos, fundamentalmente de los aparatos respiratorio y digestivo. La Diabetes Relacionada con la Fibrosis Quística (DRFQ) es una de sus principales complicaciones.Objetivos:Estudiar las complicaciones específicas de los pacientes pediátricos que padecen DRFQ y cómo afectan a su calidad de vida. Comparar las tasas de mortalidad de los pacientes pediátricos con FQ y con DRFQ. Poner de manifiesto la importancia del personal de enfermería en el manejo de los pacientes pediátricos con DRFQ.Metodología:Se llevó a cabo una revisión bibliográfica sistemática. Se incluyeron estudios publicados, en inglés y castellano, durante los últimos 10 años, y que analizaban una muestra de población de entre 0 y 9 años con FQ y/o DRFQ.Resultados:Se utilizaron 4 bases de datos para la búsqueda sistemática. Se analizaron 10 artículos para la resolución de los objetivos.Conclusiones:Las complicaciones que provocan la DRFQ, y los cuidados que requieren afectan a la calidad de vida de los pacientes. La progresión del diagnóstico y los tratamientos ha hecho que mejore su calidad de vida y que la diferencia de mortalidad entre los pacientes con FQ y con DRFQ se reduzca. El personal enfermero que atiende a los niños con DRFQ es un recurso de información esencial para los pacientes y sus familias. Las enfermeras deben transmitir la importancia de la adhesión a los tratamientos para conseguir una mejor calidad de vida.
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    Emerging relationship between CFTR, actin and tight junction organization in cystic fibrosis airway epithelium
    (Universidad de Murcia. Departamento de Biología Celular e Histología, 2017) Castellani, Stefano; Favia, Maria; Guerra, Lorenzo; Carbone, Anna Lucia; Abbattiscianni, Anna Claudia; Di Gioia, Sante; Casavola, Valeria; Conese, Massimo
    Cystic fibrosis (CF), one of the most common genetic disorders affecting primarily Caucasians, is due to mutations in the CF Transmembrane Conductance Regulator (CFTR) gene, encoding for a chloride channel also acting as regulator of other transmembrane proteins. In healthy subjects, CFTR is maintained in its correct apical plasma membrane location via the formation of a multiprotein complex in which scaffold proteins (such as NHERF1) and signaling molecules (such as cAMP and protein kinases) guarantee its correct functioning. In CF, a disorganized and dysfunctional airway epithelium brings an altered flux of ions and water into the lumen of bronchioles, consequent bacterial infections and an enormous influx of inflammatory cells (mainly polymorphonuclear neutrophils) into the airways. Recent evidence in healthy airway cells supports the notion that CFTR protein/function is strictly correlated with the actin cytoskeleton and tight junctions status. In CF cells, the most frequent CFTR gene mutation, F508del, has been shown to be associated with a disorganized actin cytoskeleton and altered tight junction permeability. Thus, the correct localization of CFTR on the apical plasma membrane domain through the formation of the scaffolding and signaling complex is likely fundamental to determine a physiological airway epithelium. The correction of CFTR mutations by either gene or drug therapies, as well as by stem cell-based interventions, can determine the resumption of a physiological organization of actin stress fibers and TJ structure and barrier function, further indicating the close interrelationship among these processes.
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    Impact of SARS-CoV-2 infection in patients with cystic fibrosis in Spain: Incidence and results of the national CF-COVID19-Spain survey
    (2020-06-08) Mondéjar López, Pedro Enrique; Quintana-Gallego, Esther; Girón-Moreno, Rosa M.; Cortell-Aznar, Isidoro; Balbuena-Maiz, Marta Ruiz de; Diab-Cáceres, Layla; Prados-Sánchez, Concepción; Álvarez-Fernández, Antonio; García-Marcos, Patricia W.; Peñalver Mellado, Carlos; Pastor-Vivero, M. Dolores; Olveira, Casilda; López-Neyra, Alejandro; Castillo-Corullón, Silvia; Palma-Milla, Samara; Pérez-Ruiz, Estela; Sole-Jover, Amparo; Barrio, M. Isabel; Sánchez-Solís de Querol, Manuel; Asensio de la Cruz, Óscar; CF-COVID19-Spain Registry Group; Cirugía, Pediatría y Obstetricia y Ginecología; Facultad de Medicina
    Background: Given the high incidence of confirmed infection by SARS-CoV-2 and mortality by COVID-19 in the Spanish population, its impact was analysed among persons with Cystic Fibrosis (CF) as a group at risk of a worse evolution. The possible causes of the incidence observed in them are explained and how CF Units have faced this health challenge is detailed. Methods: Retrospective descriptive observational study, for which a Spanish CF Patients with Confirmed COVID- 19 Registry is created, requesting information on number of people affected between 8 March–16 May 2020 and their clinical-demographic characteristics from the CF Units participating in the European Cystic Fibrosis Society Patient Registry (ECFSPR). The accumulated incidence is calculated, compared with that of the general population. Additionally, a survey (CF-COVID19-Spain) is carried out on prevention of SARS-CoV-2 infection, workings of CF Units and possible reasons for the incidence observed. Results: COVID-19 was diagnosed in eight CF patients, one of whom had received a lung transplant. The accu mulated incidence was 32/10000 in CF patients and 49/10000 in the general population. General death rate was 5.85/10000 while no CF patients included in the ECFSPR died. The characteristics of those affected and the results of the survey are described. Conclusions: Despite being considered a disease at high risk of severe COVID-19, the low incidence and mortality in CF patients in Spain contrasts with the figures for the general population. The possible factors that would explain such findings are discussed, with the help of the results of the CF-COVID19-Spain survey.
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    Secondary amyloidosis and cystic fibrosis. A morphological and histochemical study of five cases
    (Murcia : F. Hernández, 1987) Bontempini, L.; Ghimenton, C.; Colombari, R.; Malena, M.; Luzzolino, P.; Canciani, M.; Doglioni, C.; Sbabo, L.
    The pathological findings of five cases of amyloidosis associated with Cystic Fibrosis are reported. Rectal biopsy led to the diagnosis of amyloidosis in four patients. In three cases the diagnosis was confirmed at autopsy , with involvement of spleen, liver, kidneys, adrenal glands, thyroid and other organs. It seems that Secondary Amyloidosis provokes a significant, although rare, complication of Cystic Fibrosis as greater numbers of these patients survive into adulthood.