Evidence for partial growth hormone insensitivity among patients with idiopathic short stature. The National Cooperative Growth Study.

Abstract

OBJECTIVE:

To determine whether some patients with idiopathic short stature have partial resistance to growth hormone (GH). Patients with idiopathic short stature have decreased serum levels of the GH receptor-related GH-binding protein (GHBP), and low GHBP levels are associated with complete GH insensitivity (Laron) syndrome. We hypothesized that patients with idiopathic short stature and low GHBP levels may also have a degree of GH insensitivity.

DESIGN:

Retrospective analysis of patients in a multicenter study.

SETTING:

Ninety-six National Cooperative Growth Study centers in the United States and Canada.

SUBJECTS:

Five hundred eleven patients with idiopathic short stature who were treated with GH. All patients had a baseline height standard deviation score of less than -2 and a maximum stimulated GH level greater than 10 micrograms/L. Of these, 101 (20%) had a baseline GHBP standard deviation score of -2 or less.

RESULTS:

The patients with low GHBP levels, in comparison with those with normal GHBP levels, had a lower mean extracted standard deviation score for insulin-like growth factor I (-3.3 +/- 1.1 vs -2.5 +/- 1.4; p < 0.0001) but mean 12-hour GH values (2.8 +/- 1.1 vs 2.3 +/- 1.1 micrograms/L; p <0.0001). The differences between groups were statistically significant after control for age and weight-for-height standard deviation score. Among prepubertal patients, there was no significant difference between the low and normal GHBP groups in mean pretreatment or first-year growth rate (p = 0.74, 0.61 respectively) with comparable doses of GH.

CONCLUSIONS:

Patients with idiopathic short stature and low GHBP levels, compared with those with normal GHBP levels, had significantly lower standardized levels of insulin-like growth factor I, and higher mean 12-hour GH levels, which suggest partial GH insensitivity. There was no significant correlation of GHBP levels with the growth response to exogenous GH.

• Authors: K M Attie, L M Carlsson, A C Rundle, B M Sherman
• Journal: The Journal of pediatrics (J Pediatr) Vol. 127 Issue 2 Pg. 244-50 (Aug 1995) ISSN: 0022-3476 [Print] United States
• PMID: 7636649 (Publication Type: Comparative Study, Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)

Chemical References

• Carrier Proteins
• Insulin-Like Growth Factor I
• Growth Hormone
• somatotropin-binding protein

Topics

• Body Height (drug effects)
• Carrier Proteins (blood)
• Case-Control Studies
• Child
• Female
• Growth Disorders (blood, drug therapy)
• Growth Hormone (blood, deficiency, therapeutic use)
• Humans
• Insulin-Like Growth Factor I (analysis)
• Linear Models
• Male
• Reference Values
• Retrospective Studies