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Short stature in children with sickle cell anemia correlates with alterations in the IGF-I axis

Paulo F Collett-Solberg1, Don Fleenor, William H Schultz

  • 1Rio de Janeiro, Brazil, Department ofPediatrics, Duke University Medical Center, Durham, NC, USA. paulosolberg@netscape.net

Journal of Pediatric Endocrinology & Metabolism : JPEM
|April 3, 2007
PubMed

Insights

Children with sickle cell anemia (SCA) often experience short stature due to growth failure. This study reveals abnormalities in the Insulin-like Growth Factor-I (IGF-I) axis that worsen with age in these children.

Area of Science:

  • Pediatric Endocrinology
  • Hematology
  • Growth Disorders

Background:

  • Children with sickle cell anemia (SCA) frequently exhibit short stature.
  • Alterations in the Insulin-like Growth Factor-I (IGF-I) axis are hypothesized to contribute to growth failure in SCA.

Purpose of the Study:

  • To investigate the IGF-I axis in children with SCA and short stature compared to those with normal stature.
  • To identify specific abnormalities within the IGF-I axis contributing to growth failure.

Main Methods:

  • Assessed Insulin-like Growth Factor-I (IGF-I) and IGF Binding Protein-3 (IGFBP-3) levels using Radioimmunoassay (RIA).
  • Evaluated IGFBP-3 proteolysis via protease activity assays and Western immunoblots.
  • Compared IGF-I axis parameters between SCA children with short stature (below 25th percentile) and normal stature (above 50th percentile).

Main Results:

  • Both short and normally growing SCA children showed low IGF-I and IGFBP-3 standard deviation scores (SDS).
  • In short statured patients, IGF-I SDS correlated with height velocity SDS (p = 0.018).
  • IGFBP-3 SDS decreased with age (p = 0.0054) and IGFBP-3 was proteolyzed in both groups, with lower intact IGFBP-3 in short statured children (p = 0.028).

Conclusions:

  • Children with SCA exhibit significant abnormalities in the IGF-I axis.
  • These IGF-I axis abnormalities, including reduced IGF-I and IGFBP-3 and increased IGFBP-3 proteolysis, are associated with short stature and worsen with age in SCA patients.

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