title: Steroid therapy may be effective in augmenting hemoglobin levels during hemolytic crises in children with hereditary spherocytosis.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, Edith Wolfson Medical Center, Holon, Israel. ballin@wolfson.health.gov.il
authors.forename: Ami
authors.initials: A
authors.lastname: Ballin
authors.forename: Orit
authors.initials: O
authors.lastname: Waisbourd-Zinman
authors.forename: Hitam
authors.initials: H
authors.lastname: Saab
authors.forename: Joanne
authors.initials: J
authors.lastname: Yacobovich
authors.forename: Meirah
authors.initials: M
authors.lastname: Zoldan
authors.forename: Shlomit
authors.initials: S
authors.lastname: Barzilai-Birenbaum
authors.forename: Isaac
authors.initials: I
authors.lastname: Yaniv
authors.forename: Hannah
authors.initials: H
authors.lastname: Tamary
mesh_terms: D002648:Child; D002675:Child, Preschool; D017707:Erythrocyte Transfusion; D004921:Erythropoietin / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006454:Hemoglobins / Q000187:drug effects* / Q000378:metabolism; D006801:Humans; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D009996:Osmotic Fragility / Q000187:drug effects; D011241:Prednisone / Q000627:therapeutic use*; D012189:Retrospective Studies; D013103:Spherocytosis, Hereditary / Q000188:drug therapy*
chemical_list: D005938:Glucocorticoids; D006454:Hemoglobins; D004921:Erythropoietin; D011241:Prednisone