Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index

Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index

Blog Article

Abstract Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting around 50,000 patients in Europe and 30,000 in United States.A mutation in CF trans-membrane conductance regulator (CFTR) gene changes a protein (a regulated chloride channel), which is expressed in many tissues.Defective CFTR results in reduced chloride secretion and an overage absorption of sodium across the epithelia, leading to thickened secretions in organs such as pancreas and lung.Gradually, there have been considerable improvements in the survival of people with CF, thanks to substantial changes in specialized CF care and the discovery of "Google News" y el impacto de la Ley de Propiedad Intelectual en la prensa: un nuevo amanecer para la información new CFTR modulators drugs.Nevertheless, lung disease remains the most common cause of death.

For these reasons improvement of sputum clearance is a major therapeutic aim in CF.So far, symptomatic mucolytic therapy is mainly based on inhalation of dornase alfa, hypertonic saline or mannitol, in combination with physiotherapy.The major component of mucus in CF is pus including viscous material such as polymerized DNA derived from degraded neutrophils.Dornase Pptc7 is an essential phosphatase for promoting mammalian mitochondrial metabolism and biogenesis alfa cleaves the DNA released from the neutrophils and reduces mucous viscosity, and further prevent airway infections and damage to the lung parenchyma.In this review we will summarize the current knowledge on dornase alfa in the treatment of CF lung disease, especially highlighting the positive effect on lung clearance index, a sensitive measure of ventilation inhomogeneity.