Hi, this is Tom, and in this episode I'm going to be going through cystic fibrosis,

and you can find notes at zero to finals.com and in the zero to finals pediatrics book,

and you can find flashcards and questions to train your knowledge at members.0to.0.com. And at the end of this episode,

we'll go through some questions to test you on what you've just heard. So let's jump straight in.

Cystic fibrosis, or CF, is an autosomal recessive genetic condition that affects the mucous glands.

The key consequences of cystic fibrosis are thick bioduct and pancreatic secretions,

thick airway secretions, and congenital bilateral absence of the vast deference in males.

Thick and sticky secretions can block the pancreatic and bile ducts,

preventing digestive enzymes, for example pancreatic lipase from entering the digestive tract.

Pancreatic lipase plays an important role in breaking down fats

so that they can be absorbed into the body.

Chronic obstruction of the billory system can lead to liver disease,

pancreatitis and diabetes.

Thick and sticky airway secretions reduce the airway clearance, resulting in a chronic cough,

bacterial colonization, meaning bacteria living in the airways, and recurrent infections.

Congenital absence of the vast deference causes male infertility.

Patients generally have healthy sperm, but the sperm have no way of getting from the testes

to the ejaculate.

Surgical sperm retrieval, followed by in vitro fertilization, can be used to treat male

infertility in cystic fibrosis. There is variation on how the individual is affected.

85% of patients with cystic fibrosis develop pancreatic insufficiency, 30 to 50% of adults with cystic fibrosis

develop cystic fibrosis diabetes, 30% of adults develop liver disease, and 95% of males are infertile

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