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Evaluation of growth hormone secretion and insulin-like growth factor-1 in prepubertal children with thalassemia

L Sağlamer1, L Ulukutlu, O Ercan

  • 1Department of Pediatrics, Istanbul University Cerrahpaşa Faculty of Medicine.

The Turkish Journal of Pediatrics
|April 1, 1992
PubMed

Insights

Children with thalassemia major often experience growth retardation despite normal growth hormone (GH) secretion. This study found GH deficiency in most prepubertal patients, suggesting regulatory issues affecting GH release.

Area of Science:

  • Pediatric Endocrinology
  • Hematology
  • Metabolic Disorders

Background:

  • Growth retardation is a common clinical manifestation in patients with thalassemia major.
  • Previous endocrine studies often indicated normal growth hormone (GH) secretion in these patients.

Purpose of the Study:

  • To evaluate GH response to arginine and L-dopa stimulation in prepubertal children with thalassemia major.
  • To assess insulin-like growth factor-1 (IGF-1) levels in these patients.
  • To investigate potential defects in GH secretion regulation.

Main Methods:

  • Study included 14 prepubertal children (9 males, 5 females) aged 2 to 10 years with thalassemia major.
  • GH response was assessed using intravenous arginine and oral L-dopa stimulation tests.
  • Serum IGF-1 levels were measured and compared to control levels.

Main Results:

  • Nine out of 14 patients exhibited GH deficiency (peak serum GH < 7 ng/ml) with arginine stimulation.
  • Two patients showed partial GH deficiency with arginine.
  • Similar results were observed with L-dopa stimulation, with 10 patients showing GH deficiency and one partial deficiency.
  • Serum IGF-1 levels were not statistically different from controls, though three patients had low IGF-1 values.
  • Three patients presented with elevated basal GH values.

Conclusions:

  • The findings suggest a defect in the regulatory mechanisms of GH secretion in children with thalassemia major.
  • Despite normal basal GH secretion in some cases, impaired GH response to stimulation tests indicates GH deficiency.
  • Further research into GH secretion regulation is warranted for effective management of growth in these patients.

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