TY  - CHAP
M1  - Book, Section
TI  - Cystic Fibrosis
A1  - Egan, Marie E.
A2  - Zaoutis, Lisa B.
A2  - Chiang, Vincent W.
PY  - 2017
T2  - Comprehensive Pediatric Hospital Medicine, 2e
AB  - Cystic  fibrosis  (CF)  is  a  multisystem  disease  affecting  the  gastrointestinal,  respiratory,  and  reproductive  tracts  and  the  sweat  glands.  It  is  the  most  common  life-shortening  autosomal  recessive  disorder  in  the  Caucasian  population,  affecting  approximately  1  in  2500  to  3300  live  births.  However,  CF  occurs  among  persons  of  all  races  and  ethnicities.  Although  the  classic  CF  triad  is  chronic  obstructive  lung  disease,  exocrine  pancreatic  insufficiency,  and  sweat  gland  abnormalities,  CF  can  mimic  many  other  common  pediatric  conditions.  Its  presentation  can  vary  from  classic  CF  with  severe  manifestations  early  in  life  to  later  presentations  with  mild  or  even  atypical  symptoms.1-4  In  2009,  newborn  screening  for  CF  became  mandatory  in  all  50  states.5-7  With  the  implementation  of  universal  newborn  screening  for  CF,  clinical  care  guidelines  were  developed  to  aggressively  identify  and  address  respiratory  and  nutritional  problems  prior  to  the  development  of  symptoms.8-11  Each  of  these  factors  has  relevance  to  the  pediatric  hospitalist,  who  may  evaluate  and  treat  patients  with  chronic  respiratory  or  gastrointestinal  symptoms  in  which  the  differential  diagnosis  includes  CF  or  manage  disease-related  complications  in  individuals  with  known  CF.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1146122155
ER  -