Abstract | BACKGROUND: 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.
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Authors | Ami Ballin, Orit Waisbourd-Zinman, Hitam Saab, Joanne Yacobovich, Meirah Zoldan, Shlomit Barzilai-Birenbaum, Isaac Yaniv, Hannah Tamary |
Journal | Pediatric blood & cancer
(Pediatr Blood Cancer)
Vol. 57
Issue 2
Pg. 303-5
(Aug 2011)
ISSN: 1545-5017 [Electronic] United States |
PMID | 21480468
(Publication Type: Comparative Study, Journal Article)
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Copyright | Copyright © 2011 Wiley-Liss, Inc. |
Chemical References |
- Glucocorticoids
- Hemoglobins
- Erythropoietin
- Prednisone
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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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