BIOLOGY 1A03 Lecture Notes - Lecture 6: Cystic Fibrosis, Bronchoscopy, Endoscopy

• School: McMaster University
• Department: Biology
• Course: BIOLOGY 1A03
• Professor: Kim Dej

Document Summary

Bio 1a03 lecture 6 understanding cystic fibrosis. What is cystic fibrosis: cystic fibrosis is the most common fatal genetic disease, it affects 1/3600 newborns, the disease is autosomal recessive (both alleles must be present, cystic fibrosis affects the lungs and the digestive system. Symptoms: persistent cough, thick mucous production, wheezing, shortness of breath, chest infections and/or pneumonia, bowel disturbances (i. e. obstruction, salty tasting sweat. How cystic fibrosis works: the lumen of the bronchi get narrowed. Endoscopy of bronchi (bronchoscopy) to monitor mucus levels, measure how narrow/wide the lumen is getting to test the mucus levels. Respiratory health: goblet cells secrete mucus which trap any dust particles and bacteria. Cilia work together with goblet cells: airway surface liquid should have adequate volume to allow rhythmic ciliary beating and allow for mucociliary transport. Cystic fibrosis milestone and achievements: cftr gene was discovered in 1989 at the sick kids hospital by francis collins, lap-