TY - JOUR
T1 - Clinical outcomes of splenectomy in children
T2 - Report of the splenectomy in congenital hemolytic anemia registry
AU - Splenectomy in Congenital Hemolytic Anemia (SICHA) Consortium
AU - Rice, Henry E.
AU - Englum, Brian R.
AU - Rothman, Jennifer
AU - Leonard, Sarah
AU - Reiter, Audra
AU - Thornburg, Courtney
AU - Brindle, Mary
AU - Wright, Nicola
AU - Heeney, Matthew M.
AU - Smithers, Charles
AU - Brown, Rebeccah L.
AU - Kalfa, Theodosia
AU - Langer, Jacob C.
AU - Cada, Michaela
AU - Oldham, Keith T.
AU - Scott, J. Paul
AU - St Peter, Shawn
AU - Sharma, Mukta
AU - Davidoff, Andrew M.
AU - Nottage, Kerri
AU - Bernabe, Kathryn
AU - Wilson, David B.
AU - Dutta, Sanjeev
AU - Glader, Bertil
AU - Crary, Shelley E.
AU - Dassinger, Melvin S.
AU - Dunbar, Levette
AU - Islam, Saleem
AU - Kumar, Manjusha
AU - Rescorla, Fred
AU - Bruch, Steve
AU - Campbell, Andrew
AU - Austin, Mary
AU - Sidonio, Robert
AU - Blakely, Martin L.
AU - Herzberg, Brittany
AU - Ferranti, Jeffrey M.
AU - Mouttalib, Sofia
AU - Nahm, Meredith
AU - Richesson, Rachel
AU - Snyder, Denise C.
AU - Ainsworth, Terri
AU - Bettger, Mark
AU - Chamorro, Ceci
AU - Kennel, Phyllis
AU - Levens, Justin
AU - Wilson, Joan
N1 - Publisher Copyright:
© 2014 Wiley Periodicals, Inc.
PY - 2015/3/1
Y1 - 2015/3/1
N2 - The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short-term adverse events (AEs) (≤30 days after surgery), and long-term AEs (31-365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1±1.8 g/dl, 52 week 12.8±1.6 g/dl; mean±SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short-term AEs and 11% rate of long-term AEs. As we accumulate more subjects and longer follow-up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision-making process.
AB - The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short-term adverse events (AEs) (≤30 days after surgery), and long-term AEs (31-365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1±1.8 g/dl, 52 week 12.8±1.6 g/dl; mean±SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short-term AEs and 11% rate of long-term AEs. As we accumulate more subjects and longer follow-up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision-making process.
UR - http://www.scopus.com/inward/record.url?scp=84923003687&partnerID=8YFLogxK
U2 - 10.1002/ajh.23888
DO - 10.1002/ajh.23888
M3 - Article
C2 - 25382665
AN - SCOPUS:84923003687
SN - 0361-8609
VL - 90
SP - 187
EP - 192
JO - American journal of hematology
JF - American journal of hematology
IS - 3
ER -