PedAM
Pediatric Disease Annotations & Medicines
| Disease | respiratory failure |
| Phenotype | C0004623|bacterial infections |
| Sentences | 3 |
| PubMedID- 24104599 | This mirrors the described localization of wild-type cftr in the airway epithelium.31,32 ciliated cells are involved in transepithelial ion transport and mucociliary clearance in the lung and some reports suggest that cftr is expressed in ciliated cells within the surface epithelium, consistent with a role for cftr in regulating airway surface liquid volume.32,33 in the non-cf adult human airway, cftr exhibits a dual function as mediator of cl− secretion and regulator of epithelial sodium channels mediated na+ transport that maintains airway surface liquid volume homeostasis.9,34 indeed, in cf patients with homozygous δf508 mutation, there is no significant level of mature cftr protein or camp-dependent cl− secretion present in airway epithelial cells.32,35 defects in cftr in these cells are believed to directly influence the ion transport in the airway surface liquid that cause obstructive lung disease and chronic bacterial infections leading to eventual respiratory failure. |
| PubMedID- 22664493 | Poor mucociliary clearance with excessive mucus production causes obstructive lung disease like asthma and chronic bacterial infections leading to respiratory failure which is major cause of mortality51–54 (fig. |
| PubMedID- 23056764 | Excess mucus in the respiratory system of cf patients facilitates chronic bacterial infections, leading to respiratory failure, which is the major cause of mortality. |
Page: 1