What is the etiology of cystic fibrosis?

A five-month-old Caucasian female is brought into the clinic as the parent indicates that she has been having ongoing foul-smelling, greasy diarrhea. She seems to be small for her age and a bit sickly but, her parent’s state that she has a huge appetite. Upon examination you find that the patient is wheezing and you observe her coughing. After an extensive physical exam and work-up, the patient is diagnosed with cystic fibrosis.

What is the etiology of cystic fibrosis?
Describe in detail the pathophysiological process of cystic fibrosis.
Identify hallmark signs identified from the physical exam and symptoms.
Describe the pathophysiology of complications of cystic fibrosis.
What teaching related to her diagnosis would you provide the parents?

In addition to the textbook (McCance, K. L., Huether, S. E., Brashers, V. L., & Rote, N. S. (2013). Pathophysiology: The biologic basis for disease in adults and children (7th ed.). St. Louis, MO: Mosby.) utilize at least one peer-reviewed, evidence based resource to develop your post.

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