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Cystic Fibrosis (Obstructive) Cheat Sheet (DRAFT) by

ACNP CheatSheet

This is a draft cheat sheet. It is a work in progress and is not finished yet.

Cystic Fibrosis

Pathop­hys­iology
• Autosomal recessive predom­inantly in white patients. Defect in chloride channel protein causes impaired chloride and water transport, which leads to excess­ively thick, vicious secretions in the respir­atory tract, exocrine pancreas, sweat glands, GI, and GU tracts.
• Prognosis has improved with the median age of death now >30.
Treatment
• Pancreatic enzyme replac­ement, fat-so­luble vitamin supple­ments, chest PT, vaccin­ations (influenza and pneumo­coc­cal), antibi­otics for infect­ions, inhaled recomb­inant human deoxyr­ibo­nuc­lease (rhDNAse) breaks does the DNA in respir­atory mucus that clogs airways.
Symptoms
• Results in obstru­ctive lung disease with chronic infections (frequ­ently Pseudo­monas), pancreatic insuff­ici­ency, and other GI compli­cat­ions.
 

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