RT Book, Section
A1 Lubsch, Lisa
A2 Sutton, S. Scott
SR Print(0)
ID 1158315734
T1 Cystic Fibrosis
T2 McGraw-Hill's NAPLEX® Review Guide, 3e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781260135923
LK accesspharmacy.mhmedical.com/content.aspx?aid=1158315734
RD 2022/05/16
AB Cystic  fibrosis  (CF)  is  a  life-threatening  genetic  disease  of  the  epithelial  cells  in  the  body,  especially  those  lining  the  intestinal  tract  and  airways  of  the  lungs.  Normally,  epithelial  cells  transport  chloride  through  the  cystic  fibrosis  transmembrane  regulator  (CFTR)  with  sodium  and  water  following  the  ion  flux.  CF  is  the  loss  of  the  function  of  the  CFTR  with  defective  movement  of  Cl  and  water  in  the  body.  Thus,  the  composition  of  fluid  secreted  by  the  pancreas,  hepatobiliary  tree,  reproductive  tract,  sweat  glands,  and  the  airways  is  thick  and  leads  to  obstruction  with  malfunction.  This  malfunction  eventually  leads  to  widespread  organ  system  disease  (Table  47-1).