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Short stature with normal growth hormone and elevated IGF-I
T Momoi1, C Yamanaka, M Kobayashi
1Department of Paediatrics, Wakayama Red Cross Hospital, Japan.
European Journal of Pediatrics
|May 1, 1992
Summary
This study investigates a Japanese girl with severe short stature and high insulin-like growth factor I (IGF-I) levels. Findings suggest tissue-specific IGF-I receptor defects may cause elevated IGF-I, impacting growth.
Area of Science:
- Pediatrics
- Endocrinology
- Genetics
Background:
- Growth disorders in children can stem from complex hormonal imbalances.
- Insulin-like growth factor I (IGF-I) plays a crucial role in childhood growth and development.
Observation:
- A Japanese girl presented with intrauterine growth retardation, short stature, malar hypoplasia, up-slanting palpebral fissures, blue sclerae, and thin, stiff hair.
- Despite normal psychomotor development and menarche at 13 years 8 months, her height at 14 years 5 months was 132 cm (-4.6 SD).
- Growth hormone (GH) levels and responses to various stimuli were normal, but plasma IGF-I levels were significantly elevated.
Findings:
- The patient exhibited normal IGF-I bioactivity and binding to her skin fibroblasts.
- Plasma IGF-I fractions and binding patterns were similar to controls.
- Elevated IGF-I levels did not correlate with GH deficiency or impaired IGF-I action.
Implications:
- The study suggests that elevated IGF-I levels in this patient are not due to GH deficiency or impaired IGF-I bioactivity.
- Tissue-specific defects in IGF-I receptors are hypothesized as the underlying cause of the observed high IGF-I levels.
- This case highlights the complex regulation of IGF-I and its receptors in human growth.