title: Effects of red blood cell transfusion on resting energy expenditure in adolescents with sickle cell anemia.
abstract: BACKGROUND
  Previous studies indicate that resting energy expenditure is elevated in children with sickle cell anemia, possibly caused in part by hemolysis and increased erythropoietic activity. The purpose of the present investigation was to determine whether erythrocyte transfusion normalizes resting energy expenditure in sickle cell anemia.
  
  
  METHODS
  Five adolescents with sickle cell anemia (12-16 years old; 4 boys, 1 girl) were studied before and 1 week after erythrocyte transfusion before elective surgery or at the initial transfusion for growth failure. Resting energy expenditure was measured by indirect calorimetry, and laboratory measures were determined by routine, validated methods. Data comparisons were by nonparametric analysis.
  
  
  RESULTS
  After erythrocyte transfusion, total hemoglobin levels increased (difference (D) = 15 g/l; p < 0.05), whereas hemoglobin S (D = -0.36; p < 0.05) and reticulocyte count (D = -0.12; p < 0.05) decreased. Mean pretransfusion resting energy expenditure was elevated to 124% above predicted levels (p < 0.05) and increased further to 134% above prediction (p < 0.05 vs. pretransfusion levels). Plasma triiodothyronine (T3) levels increased (D = 0.17 nmol/l; p < 0.05), reverse T3 (rT3) levels tended to decline (D = -0.04 nmol/l; p = 0.14), and rT3/T3 decreased (D = -0.03; p < 0.05). Plasma insulin-like growth factor-I (IGF-I) levels were low-normal before transfusion and did not change, despite the change in resting energy expenditure.
  
  
  CONCLUSIONS
  The results confirm that resting energy expenditure is elevated in patients with sickle cell anemia. However, resting energy expenditure further increased after transfusion, despite decreased erythropoietic activity. A posttransfusion decrease in rT3/T3 may contribute to the increased resting energy expenditure. That there was no change in IGF-I implies that the growth hormone-IGF system is not involved in posttransfusion regulation of resting energy expenditure. Therefore, our data are not consistent with the hypothesis that increased resting energy expenditure in sickle cell anemia is directly related to erythropoietic activity. The mechanisms by which resting energy expenditure increases after transfusion in sickle cell anemia require additional investigation.
authors.affiliation: Department of Gastroenterology and Nutrition, Children's Hospital Oakland, California 94609, USA.
authors.forename: P
authors.initials: P
authors.lastname: Harmatz
authors.forename: M B
authors.initials: MB
authors.lastname: Heyman
authors.forename: J
authors.initials: J
authors.lastname: Cunningham
authors.forename: P D
authors.initials: PD
authors.lastname: Lee
authors.forename: L
authors.initials: L
authors.lastname: Styles
authors.forename: K
authors.initials: K
authors.lastname: Quirolo
authors.forename: L
authors.initials: L
authors.lastname: Kopp-Hoolihan
authors.forename: J
authors.initials: J
authors.lastname: Ghiron
authors.forename: R L
authors.initials: RL
authors.lastname: Hintz
authors.forename: E
authors.initials: E
authors.lastname: Vichinsky
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000378:metabolism* / Q000628:therapy*; D001481:Basal Metabolism; D002153:Calorimetry, Indirect; D002648:Child; D017707:Erythrocyte Transfusion; D005260:Female; D006454:Hemoglobins; D006801:Humans; D008297:Male
chemical_list: D006454:Hemoglobins