RT Book, Section
A1 Iqbal, Sabah F.
A1 Pillai, Dinesh
A1 Brown, Kathleen M.
A1 Klein, Bruce L.
A2 Tenenbein, Milton
A2 Macias, Charles G.
A2 Sharieff, Ghazala Q.
A2 Yamamoto, Loren G.
A2 Schafermeyer, Robert
SR Print(0)
ID 1155296902
T1 Cystic Fibrosis
T2 Strange and Schafermeyer's Pediatric Emergency Medicine, 5e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259860751
LK accessemergencymedicine.mhmedical.com/content.aspx?aid=1155296902
RD 2025/01/14
AB Cystic  fibrosis  (CF)  is  the  most  common  life-limiting  autosomal  recessive  disease  among  Caucasians  in  the  United  States.Most  patients  with  CF  have  the  classic  triad  of  manifestations:  chronic  pulmonary  disease,  malabsorption  due  to  pancreatic  insufficiency,  and  elevated  concentrations  of  sweat  chloride  and  sodium.There  is  considerable  individual  variation  in  the  clinical  manifestations,  severity,  and  course  of  the  disease.In  a  patient  known  to  have  CF,  the  most  common  reason  for  presenting  to  the  emergency  department  (ED)  is  for  a  pulmonary  exacerbation.Gastrointestinal  (GI)  complications  include  malabsorption,  obstruction,  and  hematemesis.In  this  era  of  universal  newborn  screening,  it  is  rare  for  a  patient  to  present  with  undiagnosed  CF.