TY  - CHAP
M1  - Book, Section
TI  - Sickle Cell Disease
A1  - Yamamoto, Loren G.
A2  - Tenenbein, Milton
A2  - Macias, Charles G.
A2  - Sharieff, Ghazala Q.
A2  - Yamamoto, Loren G.
A2  - Schafermeyer, Robert
PY  - 2019
T2  - Strange and Schafermeyer's Pediatric Emergency Medicine, 5e
AB  - Sickle  cell  disease  (SCD)  is  a  chronic  hemolytic  anemia  that  is  most  common  among  African  Americans,  but  may  occur  in  children  of  any  ethnic  background.  Patients  with  a  single  abnormal  gene  for  HbS  have  sickle  cell  trait  and  remain  essentially  asymptomatic.Acute  vasoocclusive  crisis  (VOC)  events  (painful  crisis  events)  are  the  most  common  complication  of  SCD  and  are  the  most  frequent  cause  of  emergency  department  visits.A  complete  blood  count  and  reticulocyte  count  should  be  obtained  every  time  a  patient  with  SCD  presents  to  the  emergency  department  (ED).Patients  with  SCD  presenting  with  a  new  infiltrate  on  chest  radiograph,  and  chest  pain,  fever,  and/or  respiratory  symptoms  have  acute  chest  syndrome  (AChS).  Therapy  for  AChS  consists  of  antibiotics,  pain  control,  respiratory  support,  and  possibly  transfusion.  All  children  with  AChS  should  be  admitted  to  the  hospital.A  blood  culture  should  be  obtained  and  parenteral  antibiotic  given  to  every  patient  with  SCD  and  fever  due  to  the  risk  of  sepsis  from  encapsulated  bacteria,  especially  Streptococcus  pneumoniae.Splenic  sequestration  crisis  occurs  when  red  blood  cells  (RBCs)  become  entrapped  in  the  spleen,  resulting  in  a  rapidly  enlarging  spleen  and  a  sudden  drop  in  hemoglobin.  The  mainstay  of  therapy  is  blood  transfusion.Stroke  occurs  in  11%  of  patients  with  SCD  under  20  years  of  age.  Patients  with  signs  and  symptoms  concerning  for  stroke  should  have  neuroimaging  performed  (preferably  magnetic  resonance  imaging  [MRI]  and  magnetic  resonance  angiography  [MRA])  and  consultation  with  a  hematologist  as  soon  as  possible.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/24
UR  - accessemergencymedicine.mhmedical.com/content.aspx?aid=1155748117
ER  -