Question

What does the nurse recognize as the primary factor responsible for multiple clinical manifestations of cystic fibrosis?

1 Hyperactivity of sweat glands
2 Atrophic changes in mucosal wall of intestines
3 Hypoactivity of the autonomic nervous system
4 Increased viscosity of mucous gland secretions

Answer 1

The primary factor responsible for the clinical symptoms of cystic fibrosis is the increased viscosity of mucus gland secretions due to a mutation in the CFTR gene, which causes defective Cl- ion transport. Therefore, the correct option is 4.

Step-by-step explanation:

The primary factor responsible for the multiple clinical manifestations of cystic fibrosis is increased viscosity of mucus gland secretions. Cystic fibrosis is an autosomal recessive genetic disease caused by a mutation in the CFTR gene. This mutation leads to a defective ion channel protein, which results in the inability to transport Cl- ions effectively out of the cell. Without proper ion transport, the water concentration gradient necessary for normal mucus consistency is not established, leading to the production of thick and sticky mucus. This mucus obstructs the airways, pancreatic ducts, and other mucus-secreting organs, leading to frequent lung infections and malabsorption of nutrients.