RESUMEN
Previous studies have identified abnormalities in the oxidative responses of the neutrophil in cystic fibrosis (CF), but it is unclear whether such changes relate to loss of membrane cystic fibrosis transmembrane conductance regulator (CFTR) or to the inflammatory environment present in this disease. The aim of the present study was to determine whether neutrophils from CF patients demonstrate an intrinsic abnormality of the respiratory burst. The respiratory burst activity of neutrophils isolated from stable DeltaF508 homozygote CF patients and matched healthy controls was quantified by both chemiluminscence and cytochrome C reduction. Expression of NADPH oxidase components and CFTR was determined by Western blotting and RT-PCR. The oxidative output from neutrophils from CF in response to receptor-linked and particulate stimuli did not differ from that of controls. Expression of NADPH oxidase components was identical in CF and non-CF neutrophils. While low levels of CFTR mRNA could be identified in the normal human neutrophil, we were unable to detect CFTR protein in human neutrophil lysates or immunoprecipitates. CFTR has no role in controlling neutrophil oxidative activity; previously reported differences in neutrophil function between CF and non-CF subjects most likely relate to the inflammatory milieu from which the cells were isolated.
Asunto(s)
Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/metabolismo , Fibrosis Quística/inmunología , Neutrófilos/metabolismo , Especies Reactivas de Oxígeno/metabolismo , Adulto , Western Blotting , Fibrosis Quística/metabolismo , Femenino , Expresión Génica/inmunología , Humanos , Masculino , NADPH Oxidasas/metabolismo , Neutrófilos/inmunología , Fosfoproteínas/metabolismo , Neumonía/inmunología , Neumonía/metabolismo , ARN Mensajero/metabolismo , Especies Reactivas de Oxígeno/inmunología , Estallido Respiratorio/inmunología , Reacción en Cadena de la Polimerasa de Transcriptasa InversaRESUMEN
Mucus plugging plays a vital role in the pathophysiology of fatal and near fatal asthma as demonstrated in various postmortem studies. There is a paucity of published literature on how to manage mucus plugging in adult patients with refractory asthma exacerbation not responding to conventional therapies as compared with its paediatric cohort. We describe a dramatic improvement with the use of rhDNase, following bronchoalveolar lavage in an intubated adult female patient, with status asthmaticus refractory to conventional treatment.