RT Book, Section
A1 Doherty, Gerard M.
SR Print(0)
ID 58108522
T1 Cystic Fibrosis
T2 Quick Answers Surgery
YR 2010
FD 2010
PB The McGraw-Hill Companies
PP New York, NY
SN 
LK accesssurgery.mhmedical.com/content.aspx?aid=58108522
RD 2024/04/19
AB •  Serious  congenital  disorder,  autosomal  recessive  disorder•  Most  common  mutation  is  deletion  of  amino  acid  in  position  508  (Phe)•  Defect  in  chloride  transport,  results  in  more  NaCl  absorption  in  the  airway•  Defect  occurs  in  apocrine  sweat  glands  tracheobronchial  tree,  pancreas,  GI  tract•  Airway  secretions  are  low  in  volume  and  high  in  viscosity•  Mucoid  plugs  form  and  are  rubbery,  semisolid,  gray  to  greenish  yellow  in  color  resulting  in  impaction•  Often  history  of  recurrent  upper  respiratory  tract  infection,  fever,  and  chest  pain