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Growth hormone and dysmorphic syndromes

C Galasso1, G Scirè, B Boscherini

  • 1Department of Pediatrics, University Tor Vergata, Rome, Italy.

Hormone Research
|January 1, 1995
PubMed
Summary

This study reviews growth hormone secretion and treatment in children with dysmorphic syndromes causing short stature. It analyzes data for common conditions like Noonan syndrome and rare cases, informing growth hormone deficiency management.

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Area of Science:

  • Pediatric Endocrinology
  • Genetics
  • Growth Disorders

Background:

  • Short stature is a common feature in several dysmorphic syndromes.
  • Understanding growth hormone (GH) secretion is crucial for managing these conditions.

Purpose of the Study:

  • To investigate the pathogenesis of short stature in frequent dysmorphic syndromes.
  • To review somatrotropin (human growth hormone) secretion and response to GH replacement therapy in pediatric patients.

Main Methods:

  • Literature review of significant studies on GH secretion and treatment.
  • Analysis of case data for specific syndromes including Noonan, Silver-Russell, Prader-Willi, achondroplasia, hypochondroplasia, and Down syndrome.
  • Review of less frequent syndromes with diagnosed GH deficiency.

Main Results:

  • Summarizes findings on GH secretion patterns in various dysmorphic syndromes.
  • Evaluates the efficacy of human growth hormone replacement therapy in pediatric patients with short stature associated with these syndromes.
  • Highlights GH deficiency as a treatable factor in some rare dysmorphic conditions.

Conclusions:

  • Growth hormone deficiency and impaired response to treatment are key factors in short stature for many dysmorphic syndromes.
  • GH replacement therapy can be beneficial for select pediatric patients.
  • Further research into specific syndromes is warranted to optimize treatment strategies.

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