title: Growth hormone and insulin-like growth factor I axis and growth of children with different sickle cell anemia haplotypes.
abstract: BACKGROUND
  The purpose of this study was to examine the relationships between growth in children with sickle cell anemia and the different beta-globin haplotypes, as well as components of the insulin-like growth factor (IGF)/insulin-like growth factor binding protein (IGFBP) axis.
  
  
  METHODS
  Growth parameters and plasma concentrations of growth hormone (GH), IGF-I, and IGFBP-3 were studied in 41 children with sickle cell anemia whose haplotypes were defined.
  
  
  RESULTS
  Plasma concentrations of IGF-I (total, free, and free/total fraction) and IGFBP-3 were significantly reduced in all patients with sickle cell anemia compared with the healthy children. Patients with the CAR/CAR haplotype had significantly lower mean growth velocity compared with those with Ben/Ben. When the GH/IGF axis elements were compared in relation with the different haplotypes, total IGF-I levels in CAR/CAR patients were significantly lower compared with levels in patients with Ben/Ben. A positive correlation was found between hematocrit and total IGF-I and between fetal hemoglobin percentages and the z-scores for total IGF-I and IGFBP-3. There was a positive correlation between age, weight, height, bone age, and the various elements of the GH/IGF-I axis when all groups were considered, although the correlation was lost when the auxologic data were expressed as standard deviation score for age. Growth velocity and the z-score for growth velocity were not correlated with any element of the axis.
  
  
  CONCLUSIONS
  The positive relationship between hematocrit and fetal hemoglobin percentages with total IGF-I, free/total IGF-I, and IGFBP-3 in patients with sickle cell anemia could show that the delayed growth of these patients may be linked to intrinsic factors of the disease, which also determine the low circulating concentrations of the various elements of the GH/IGF-I axis. It is reasonable to assume that decrease of total IGF-I concentrations in patients with CAR/CAR haplotype is secondary to the severity of the disease.
authors.affiliation: Laboratory for Tumoral Biology, Fundação Pró-Sangue Hemocentro de São Paulo, Brazil.
authors.forename: S M
authors.initials: SM
authors.lastname: Luporini
authors.forename: I
authors.initials: I
authors.lastname: Bendit
authors.forename: R
authors.initials: R
authors.lastname: Manhani
authors.forename: O L
authors.initials: OL
authors.lastname: Bracco
authors.forename: L
authors.initials: L
authors.lastname: Manzella
authors.forename: D
authors.initials: D
authors.lastname: Giannella-Neto
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood / Q000235:genetics / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D017353:Gene Deletion; D005914:Globins / Q000235:genetics*; D006130:Growth Disorders / Q000097:blood / Q000209:etiology / Q000235:genetics; D006239:Haplotypes; D019382:Human Growth Hormone / Q000097:blood*; D006801:Humans; D018972:Insulin-Like Growth Factor Binding Protein 3 / Q000097:blood*; D007334:Insulin-Like Growth Factor I / Q000378:metabolism*; D008297:Male; D005810:Multigene Family
chemical_list: D018972:Insulin-Like Growth Factor Binding Protein 3; D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I; D005914:Globins