FIBROSIS QUÍSTICA UNA ENFERMEDAD PULMONAR: PATOGENIA Y OBJETIVOS TERAPÉUTICOS ACTUALIZADOS

Abstract

Chemical fibrosis represents a condition of congenital origin with autosomal recessive transmission that affects the gene that produces the CFRT protein, which constitutes a channel for chloride transport. The deficiency of this transport produces the appearance of thick secretions in different organs that cannot be eliminated, with the consequent cellular destruction and appearance of fibrosis, at the lung level, recurrent infections occur that worsen the inflammatory condition and advance towards the fibrotic healing process, at the gastrointestinal level it generates impairment in intestinal absorption as well as a deficit in the production of pancreatic secretions which will lead to the appearance of malnutrition, the diagnosis is based on family history, neonatal screening for immunoreactive trypsin, measurements of chloride in sweat and identification of genetic mutations, treatment is directed towards preventing the accumulation of secretions at the lung level through secretion elimination techniques using bronchodilators, antiinflammatory therapy, hypertonic saline solutions and antibiotic treatment when infection appears. In patients with lung damage incompatible with life, lung transplantation is the last step in treatment.