TY  - CHAP
M1  - Book, Section
TI  - Cystic Fibrosis
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
Y1  - 2019
N1  - 
T2  - Strange and Schafermeyer's Pediatric Emergency Medicine, 5e
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.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/19
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1155296902
ER  -