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Related Experiment Videos

Partial growth hormone insensitivity in childhood

L M Carlsson1

  • 1Department of Research, Metabolic Unit, Kantonsspital, Basel, Switzerland.

Bailliere'S Clinical Endocrinology and Metabolism
|July 1, 1996
PubMed
Summary

Short children may have growth failure due to partial GH insensitivity (GHI), not just GH deficiency (GHD). Research identifies a subgroup with GH receptor defects causing partial GHI, impacting growth despite normal GH levels.

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Area of Science:

  • Pediatric Endocrinology
  • Growth Hormone Axis Research

Background:

  • Growth hormone (GH) responsiveness exists on a spectrum, from complete resistance (Laron syndrome) to normal sensitivity.
  • Partial GH insensitivity (GHI) is observed in some individuals with Laron-type dwarfism and short children with normal GH levels.
  • Current diagnosis of GH deficiency (GHD) relies on arbitrary GH concentration thresholds, assuming uniform GH sensitivity.

Purpose of the Study:

  • To investigate the role of partial GH insensitivity (GHI) in idiopathic short stature (ISS).
  • To identify subgroups of short children with reduced GH responsiveness.
  • To propose a more comprehensive approach to diagnosing short stature, considering both GH secretion and GH sensitivity.

Main Methods:

  • Identification of a subgroup of children with ISS exhibiting partial GHI.
  • Analysis of GH receptor defects as a cause for partial GHI syndrome.
  • Review of existing diagnostic criteria for GHD and auxological evaluation.

Main Results:

  • A subgroup of children with idiopathic short stature (ISS) has been identified with defects at the GH receptor level, leading to a partial-GHI syndrome.
  • Partial GHI can explain growth failure in some short children who do not meet conventional GHD criteria.
  • This suggests that reduced GH sensitivity, not just GH deficiency, contributes to short stature.

Conclusions:

  • Partial GH insensitivity is a significant factor in the growth failure of some short children.
  • Diagnostic evaluation for short stature should include assessment of GH secretion and GH responsiveness.
  • Considering GH sensitivity alongside GH levels offers a more complete understanding of growth disorders.

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