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Steroid therapy may be effective in augmenting hemoglobin levels during hemolytic crises in children with hereditary spherocytosis.

AbstractBACKGROUND:
The course of hereditary spherocytosis (HS) may be subject to hemolytic episodes, sometimes requiring blood transfusion. The aim of this study was to evaluate the efficacy of a short course of steroid therapy in elevating hemoglobin levels during hemolytic crisis.
PROCEDURE:
The files of all patients followed for HS from 1968 to 2009 at our hospital were reviewed. Outcome of hemolytic crises was compared between steroid-treated and untreated patients; patients given packed red blood cell transfusion(s) or erythropoietin treatment were excluded. A good outcome was defined as an increase of at least 20% in hemoglobin level from the nadir within 1 week.
RESULTS:
Of the 118 patients with HS who attended our hospital during the study period, 20 were treated with steroids and 9 received no treatment. Mean nadir hemoglobin level in both groups was 6.9 g/dl. The study group had a total of 50 steroid-treated hemolytic crises of which 37 (74%) responded favorably to treatment. Treatment failure was significantly associated with a low dose (<1 mg/kg/day) or short duration (<1 week) of treatment. The nine untreated patients had 16 hemolytic crises, of which 25% had a good outcome. Steroid therapy was significantly more effective than no therapy in increasing hemoglobin level (P = 0.015) in these hemolytic crises.
CONCLUSION:
Steroid therapy may be effective in augmenting hemoglobin levels during hemolytic crises in patients with moderate HS and eventually will result in a reduced need for RBC transfusion.
AuthorsAmi Ballin, Orit Waisbourd-Zinman, Hitam Saab, Joanne Yacobovich, Meirah Zoldan, Shlomit Barzilai-Birenbaum, Isaac Yaniv, Hannah Tamary
JournalPediatric blood & cancer (Pediatr Blood Cancer) Vol. 57 Issue 2 Pg. 303-5 (Aug 2011) ISSN: 1545-5017 [Electronic] United States
PMID21480468 (Publication Type: Comparative Study, Journal Article)
CopyrightCopyright © 2011 Wiley-Liss, Inc.
Chemical References
  • Glucocorticoids
  • Hemoglobins
  • Erythropoietin
  • Prednisone
Topics
  • Child
  • Child, Preschool
  • Erythrocyte Transfusion
  • Erythropoietin (therapeutic use)
  • Female
  • Glucocorticoids (therapeutic use)
  • Hemoglobins (drug effects, metabolism)
  • Humans
  • Logistic Models
  • Male
  • Multivariate Analysis
  • Osmotic Fragility (drug effects)
  • Prednisone (therapeutic use)
  • Retrospective Studies
  • Spherocytosis, Hereditary (drug therapy)

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