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Growth and factors affecting it in thalassemia major

Ashraf T Soliman1, Hany Khalafallah, Rasha Ashour

  • 1Department of Pediatrics, Hamad Medical Center, Doha, Qatar. atsoliman@yahoo.com

Hemoglobin
|December 17, 2009
PubMed

Insights

Children with thalassemia often experience growth issues, including short stature and reduced growth velocity. Growth hormone therapy shows benefits but suggests partial GH insensitivity in this population.

Area of Science:

  • Pediatric Endocrinology
  • Hematology
  • Growth Disorders

Background:

  • Thalassemia (T) is associated with significant growth impairment.
  • Many children with T exhibit short stature (HtSDS < -2) and reduced growth velocity (GVSDS < -1).
  • Biometric measurements like mid-arm circumference and triceps skin-fold thickness are decreased in thalassemic children compared to controls.

Purpose of the Study:

  • To investigate the growth characteristics and hormonal profiles in children with thalassemia.
  • To evaluate the response to growth hormone (GH) therapy and pubertal induction in this cohort.

Main Methods:

  • Assessment of height standard deviation score (HtSDS) and growth velocity standard deviation score (GVSDS).
  • Measurement of circulating insulin-like growth factor-I (IGF-I) concentrations.
  • Evaluation of growth hormone (GH) response to provocative tests and spontaneous nocturnal secretion.
  • Assessment of IGF-I generation after GH injection.
  • Analysis of GH therapy and human chorionic gonadotropin (hCG) induced pubertal changes.

Main Results:

  • Thalassemic children showed significantly lower IGF-I concentrations compared to normal children.
  • Half of short thalassemic children had defective GH response to provocation; others had defective nocturnal GH secretion.
  • IGF-I generation after GH injection was reduced in T children versus those with GH deficiency (GHD) or constitutional delay of growth and puberty (CDGP).
  • One year of GH therapy improved IGF-I, GV, and HtSDS in T children, but less than in GHD or CDGP groups, indicating partial GH insensitivity.
  • Pubertal induction with hCG increased IGF-I, GV, and HtSDS in thalassemic adolescents.

Conclusions:

  • Children with thalassemia exhibit multifaceted growth problems, including impaired GH secretion and action.
  • GH therapy and pubertal induction can improve growth in thalassemic children, though partial GH insensitivity is suggested.
  • Further research into optimizing growth management strategies for thalassemic patients is warranted.

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