RT Book, Section
A1 Carden, Marcus
A1 Glassberg, Jeffrey
A1 Morris, Claudia R.
A2 Gladwin, Mark T.
A2 Kato, Gregory J.
A2 Novelli, Enrico M.
SR Print(0)
ID 1179345373
T1 Emergent Clinical Complications of Sickle Cell Disease
T2 Sickle Cell Disease
YR 2021
FD 2021
PB McGraw-Hill Education
PP New York, NY
SN 9781260458596
LK hemonc.mhmedical.com/content.aspx?aid=1179345373
RD 2024/04/24
AB Sickle  cell  disease  (SCD)  comprises  a  group  of  recessively  inherited  hemoglobinopathies  that  impact  approximately  100,000  adults  and  children  of  various  ethnicities  and  backgrounds  in  the  United  States  and  millions  more  worldwide.1,2  Although  the  clinical  features  of  SCD  vary  from  patient  to  patient,  common  clinical  complaints  in  the  emergency  department  (ED)  result  from  a  persistent  hemolytic  anemia  and  vasculopathy  within  the  microcirculation,  which  leads  to  repeated  episodes  of  vaso-occlusion  and  ischemia-reperfusion  that  can  result  in  acute  and  chronic  end-organ  damage  of  every  organ  system.3,4  Patients  with  SCD  have  a  shortened  life  expectancy  compared  to  the  general  population  due  to  these  complications;  however,  most  children  with  SCD  in  the  United  States  can  now  expect  to  live  to  adulthood  thanks  to  modern  medical  advances,  including  mandatory  neonatal  hemoglobinopathy  screening,  disease-specific  immunization  practices,  early  initiation  of  penicillin  prophylaxis,  hydroxyurea  therapy,  transfusion  protocols,  and  parent  education.5