Is bronchoscopy an obsolete tool in cystic fibrosis? The role of bronchoscopy in cystic fibrosis and its clinical use

Abstract

Cystic fibrosis (CF) is a progressive life threatening multisystem genetic disease which affects the CF transmembrane conductance regulator channel. Respiratory causes remain the most common mortality in CF. With the onset of newborn screening, initiating treatments both for prophylaxis and disease management, optimizing nutritional support, and developing therapies targeting CF transmembrane conductance regulator protein, this has significantly changed the face of managing this devastating disease. Bronchoscopy and related procedures such as bronchoalveolar lavage (BAL), transbronchial biopsies, and protected brush sampling have been looked at in the management of CF as patients with CF continue to live longer with the help of newer therapies, the microbiome in the lung becomes less diverse along with increased occurrences for noninfectious causes of airway diseases. Though bronchoscopy has been used in conjunction with other modalities such as computed tomography and sputum induction providing a better understanding of the progression of the disease, it still remains valuable in the diagnosis and management of CF.

Consenso Chileno Para La Atención Integral De Niños Y Adultos Con Fibrosis Quística

“…• En pacientes trasplantados para evaluar: Infecciones, revisión de los sitios de anastomosis por posibles complicaciones quirúrgicas (estenosis bronquial), realizar biopsia transbronquial, vigilancia de recuentos celulares y marcadores de rechazo (103,104) . • En hemoptisis (no masiva) para intentar localizar sitio de sangramiento.…”

The clinical yield of bronchoscopy in the management of cystic fibrosis: A retrospective multicenter study

“…Lung disease in CF is caused by defective mucociliary clearance, due to the cystic fibrosis transmembrane conductance regulator protein dysfunction in the airway epithelium leading to the accumulation of thick mucus, obstructing the airways 3 . CF lung disease is complicated by chronic infection, airway destruction, and eventually, respiratory failure 4,5 …”

“…3 CF lung disease is complicated by chronic infection, airway destruction, and eventually, respiratory failure. 4,5 Pulmonary infections are the main driver of chronic pulmonary morbidity in CF, which is also the leading cause of mortality. 5,6 The most common pathogens in CF are Staphylococcus aureus and Haemophilus influenza in young children and Pseudomonas aeruginosa in older children and adults.…”

“… 3 CF lung disease is complicated by chronic infection, airway destruction, and eventually, respiratory failure. 4 , 5 …”

Nutritional characterization of children and adolescents with cystic fibrosis

“…Se produce como consecuencia de mutaciones que afectan a un gen, el cual codifica para una proteína de membrana, cuya función es actuar como canal de cloro y se denomina Regulador de Conductancia Transmembrana de FQ o CFTR por sus siglas en inglés: Cystic Fibrosis (2)(3)(4)(5) Transmembrane Conductance Regulator . El CFTR es esencial para la regulación de los movimientos de (3) sal y agua a través de las membranas celulares .…”