RT Book, Section
A1 Voynow, Judith A.
A1 Mascarenhas, Maria
A1 Kelly, Andrea
A1 Scanlin, Thomas F.
A2 Grippi, Michael A.
A2 Elias, Jack A.
A2 Fishman, Jay A.
A2 Kotloff, Robert M.
A2 Pack, Allan I.
A2 Senior, Robert M.
A2 Siegel, Mark D.
SR Print(0)
ID 1122360051
T1 Cystic Fibrosis
T2 Fishman's Pulmonary Diseases and Disorders, 5e
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 978-0-07-179672-9
LK accessmedicine.mhmedical.com/content.aspx?aid=1122360051
RD 2024/04/19
AB Cystic  fibrosis  (CF)  is  a  common  inherited  disease  that  has  a  high  frequency  in  Caucasians.  The  disorder  affects  all  exocrine  glands,  with  symptoms  involving  the  lungs  and  pancreas  usually  dominating  the  clinical  picture.  Even  though  the  gene  responsible  for  CF  and  its  gene  product,  an  integral  membrane  glycoprotein,  have  been  identified,  two  aspects  of  the  disease  make  CF  particularly  difficult  to  both  diagnose  and  manage.  First,  there  is  tremendous  variability  in  the  degree  and  pattern  of  involvement  of  organs  in  different  persons.  In  addition,  we  lack  information  about  the  precise  details  of  the  molecular  and  cellular  pathogenesis  of  the  disease.  This  chapter  focuses  on  the  pathophysiology  and  management  of  CF.  Our  current  understanding  of  the  genetics  and  underlying  molecular  biology  is  highlighted.  Complications  of  the  disorder  are  addressed,  and  a  brief  discussion  of  relevant  psychosocial  and  reproductive  issues  is  provided.  Finally,  potential  future  directions  in  treatment  are  described.