RT Book, Section
A1 Saddi, Vishal
A1 Ooi, Chee Y.
A1 Jaffe, Adam
A2 Kline, Mark W.
SR Print(0)
ID 1182917191
T1 Cystic Fibrosis
T2 Rudolph's Pediatrics, 23e
YR 2018
FD 2018
PB McGraw-Hill Education
PP New York, NY
SN 9781259588594
LK accesspediatrics.mhmedical.com/content.aspx?aid=1182917191
RD 2024/04/24
AB Cystic  fibrosis  (CF)  is  the  most  common  lethal  genetic  condition  in  the  Caucasian  population.  Significant  progress  has  been  made  in  diagnosis  and  treatment,  which  has  led  to  dramatically  increased  survival  and  reduced  morbidity  rates.  The  localization  of  the  gene  encoding  the  CF  transmembrane  conductance  regulator  (CFTR)  in  1989  enabled  a  deeper  understanding  of  the  disease  pathophysiology  and  led  to  the  development  of  novel  therapeutic  avenues  correcting  the  basic  defect  instead  of  fire-fighting  the  downstream  consequences  of  the  disease.